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Sample records for partly meromictic hypertrophic

  1. Ecology of Meromictic Lakes

    NARCIS (Netherlands)

    Gulati, R.D.; Zadereev, E.S.; Degermendzhy, A.G.

    2017-01-01

    This volume presents recent advances in the research on meromictic lakes and a state-of-the art overview of this area. After an introduction to the terminology and geographic distribution of meromictic lakes, three concise chapters describe their physical, chemical and biological features. The

  2. Techniques for Optimizing Surgical Scars, Part 2: Hypertrophic Scars and Keloids.

    Science.gov (United States)

    Potter, Kathryn; Konda, Sailesh; Ren, Vicky Zhen; Wang, Apphia Lihan; Srinivasan, Aditya; Chilukuri, Suneel

    2017-01-01

    Surgical management of benign or malignant cutaneous tumors may result in noticeable scars that are of great concern to patients, regardless of sex, age, or ethnicity. Techniques to optimize surgical scars are discussed in this three-part review. Part 2 focuses on scar revision for hypertrophic and keloids scars. Scar revision options for hypertrophic and keloid scars include corticosteroids, bleomycin, fluorouracil, verapamil, avotermin, hydrogel scaffold, nonablative fractional lasers, ablative and fractional ablative lasers, pulsed dye laser (PDL), flurandrenolide tape, imiquimod, onion extract, silicone, and scar massage.

  3. HYPERTROPHIC CARDIOMYOPATHY AS A PART OF INHERITED MALFORMATION SYNDROMES IN INFANTS

    Directory of Open Access Journals (Sweden)

    M.V. Tural'chuk

    2011-01-01

    Full Text Available The data of clinical and instrumental examination of two infantile patients with obstructive hypertrophic cardiomyopathy in association with marked multisystem involvement as a picture of inherited malformation syndromes are given.Key words: infants, hypertrophic cardiomyopathy, LEOPARD syndrome, Noonan syndrome.(Voprosy sovremennoi pediatrii — Current Pediatrics. 2011; 10 (3: 166–169

  4. Modelling assessment of End Pit Lakes meromictic potential

    International Nuclear Information System (INIS)

    2006-11-01

    The use of End Pit Lakes have been proposed as a remediation solution for oil sands reclamation and operational waters. This report modelled the main factors controlling the occurrence of stratification in Pit Lakes in order to establish design and management guidelines for the Cumulative Environmental Management Association's End Pit Lake Sub-group. The study focused on End Pit Lake size, depth, starting lake salinity concentrations, inflow rates and inflow salinity flux, and investigated their influence on density gradients. One-dimensional modelling and limited 2-D modelling simulations were conducted to examine meromictic potential for a large range of End Pit Lake configurations and conditions. Modelling results showed that fall is the governing season for determining meromixis. The expelling of salt from saline water upon ice formation and its effect on stratification potential and the effect of fresh water loading on stratification potential during spring melt events were not observed to be dominant factors governing meromictic potential for the scenarios examined in the study. Results suggested that shallow End Pit Lakes showed a high turn-over rate with seasonal heating and cooling cycles. Moderately deep End Pit Lakes demonstrated a meromictic potential that was inversely proportional to lake size and require higher starting salinities. With a 2 or 10 million m 3 /yr inflow rate and a 5 parts per thousand starting salinity, a 50 m deep End Pit Lake achieved meromixis at all 3 size ranges considered in the study. Results also showed that the rate of influent salinity decrease was the least important of the parameters influencing meromixis. It was observed that meromixis was a temporary condition in all of the End Pit Lake scenarios envisioned due to the lack of a constant, positive salt replenishment over the long term. It was concluded that further 3-D modelling is required to represent littoral areas as well as to account for extreme winter conditions. A

  5. Assessment of hypertrophic cardiomyopathy by ECG gated cardiac computed tomography

    International Nuclear Information System (INIS)

    Takeuchi, Kazuhide; Tanaka, Chujiro; Oku, Hisao

    1981-01-01

    The applicability of ECG gated cardiac computed tomography (CT) in 12 patients with hypertrophic cardiomyopathy was examined. Six of the 12 patients had hypertrophic obstructive cardiomyopathy, including one patient with mid-ventricular obstruction. Three of the 12 patients had hypertrophic non-obstructive cardiomyopathy, and three had apical hypertrophic cardiomyopathy. The diagnosis of hypertrophic cardiomyopathy was confirmed by the angiocardiogram in all patients. Cardiac CT was performed after intravenous administration of contrast media usually given as a bolus injection. The gantry was set with positive 20 0 tilt angle. In all patients with hypertrophic obstructive cardiomyopathy except for mid-ventricular obstruction, the hypertrophied interventricular septum in the basal and mid portions was observed, and the left ventricular cavity was narrowed in systole. In a patient with mid-ventricular obstruction, the marked hypertrophied interventricular septum and antero-lateral papillary muscle were observed. In diastole, the left ventricular cavity was narrow and divided into two parts. The apical cavity was completely disappeared in systole. In all patients with hypertrophic non-obstructive cardiomyopathy, the diffuse hypertrophied interventricular septum was observed in diastole. In systole, the apical portion of the left ventricular cavity was markedly narrow and antero-lateral papillary muscle was hypertrophic. In all patients with apical hypertrophic cardiomyopathy, the marked apical hypertrophy of the left ventricular wall was observed in diastole. It is concluded that ECG gated cardiac CT could estimate myocardial wall motion and thickness and differentiate the types of hypertrophic cardiomyopathy each other. (author)

  6. An unusual ST-segment elevation: apical hypertrophic cardiomyopathy shows the ace up its sleeve.

    Science.gov (United States)

    de Santis, Francesco; Pergolini, Amedeo; Zampi, Giordano; Pero, Gaetano; Pino, Paolo Giuseppe; Minardi, Giovanni

    2013-01-01

    Apical hypertrophic cardiomyopathy is part of the broad clinical and morphologic spectrum of hypertrophic cardiomyopathy. We report a patient with electrocardiographic abnormalities in whom acute coronary syndrome was excluded and apical hypertrophic cardiomyopathy was demonstrated by careful differential diagnosis. Copyright © 2012 Sociedade Portuguesa de Cardiologia. Published by Elsevier España. All rights reserved.

  7. Sources of carbon and sulfur nutrition for consumers in three meromictic lakes of New York State

    International Nuclear Information System (INIS)

    Fry, B.

    1986-01-01

    The trophic importance of bacterioplankton as a source of carbon and sulfur nutrition for consumers in meromictic lakes was tested using stable carbon (delta 13 C) and sulfur (delta 34 S) isotopic measurements. Studies in three lakes near Syracuse, New York, showed that most consumers ultimately derive their C and S nutrition from a mixture of terrestrial detritus, phytoplankton, and littoral vegetation, rather than from bacterioplankton. Food webs in these meromictic lakes are thus similar to those in other lakes that lack dense populations of bacterioplankton

  8. Hypertrophic Cardiomyopathy Association

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    ... be donated to Hypertrophic Cardiomyopathy Association. iGive.com - Online Shopping Joing iGive.com to earn money for the ... it works, check out the iGive website . AmazonSmile - Online Shopping Amazon donates 0.5% of the purchase price ...

  9. Infantile hypertrophic pyloric stenosis

    DEFF Research Database (Denmark)

    Pedersen, Rikke Neess; Garne, Ester; Loane, Maria

    2008-01-01

    OBJECTIVE: The objective of this study was to present epidemiologic data on infantile hypertrophic pyloric stenosis (IHPS) from seven well-defined European regions, and to compare incidence and changes in incidence over time between these regions. METHODS: This was a population-based study using ...

  10. Infantile hypertrophic pyloric stenosis

    International Nuclear Information System (INIS)

    Breivik, K.; Soereide, J.A.; Bland, J.

    1990-01-01

    During an eight-year period, 40 patients were operated consecutively for pyloric stenosis. The most common symptom was projectile vomiting, which occurred in 92.5% of the cases. On examination only three patients had a palpable hypertrophic pylorus. In 39 patients a preoperative X-ray examination with contrast was necessary to confirm the diagnosis. A pyleromyotomy was performed in all patients. The diagnosis routines and the results of the treatment are discussed. 16 refs., 2 figs., 3 tabs

  11. Hypertrophic Cardiomyopathy: Clinical Update.

    Science.gov (United States)

    Geske, Jeffrey B; Ommen, Steve R; Gersh, Bernard J

    2018-05-01

    Hypertrophic cardiomyopathy (HCM) is the most common heritable cardiomyopathy, manifesting as left ventricular hypertrophy in the absence of a secondary cause. The genetic underpinnings of HCM arise largely from mutations of sarcomeric proteins; however, the specific underlying mutation often remains undetermined. Patient presentation is phenotypically diverse, ranging from asymptomatic to heart failure or sudden cardiac death. Left ventricular hypertrophy and abnormal ventricular configuration result in dynamic left ventricular outflow obstruction in most patients. The goal of therapeutic interventions is largely to reduce dynamic obstruction, with treatment modalities spanning lifestyle modifications, pharmacotherapies, and septal reduction therapies. A small subset of patients with HCM will experience sudden cardiac death, and risk stratification remains a clinical challenge. This paper presents a clinical update for diagnosis, family screening, clinical imaging, risk stratification, and management of symptoms in patients with HCM. Copyright © 2018 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

  12. Anaerobic Oxidation of Methane in a French meromictic lake (Lake Pavin): Who is responsible?

    Science.gov (United States)

    Grossi, V.; Attard, E.; Birgel, D.; Schaeffer, P.; Jézéquel, D.; Lehours, A.

    2012-12-01

    Methane is an important greenhouse gas and its biogeochemical cycle is of primary significance to the global carbon cycle. The Anaerobic Oxidation of Methane (AOM) has been estimated to be responsible for >90% of methane consumption. This biogeochemical process has been increasingly documented during the last two decades but the underlying microbial processes and their key agents remain incompletely understood. Freshwater lakes account for 2-10% of the total emissions of methane and are therefore an important part of the global methane cycle. Lake Pavin is a French meromictic crater lake with unusual hydrological characteristics: its morphology (depth >92m, mean diameter 750m) induce that waters below 60m are never mixed with overlying waters and remain permanently anoxic. The deep anoxic waters of Lake Pavin contain high concentrations (i.e. 4 mM) of methane but, contrary to other aquatic systems, almost no methane escapes from the lake. Previous biogeochemical and modeling studies suggest that methane is preferentially consumed within the oxic-anoxic transition zone (ca. 55-60 m depth) but that ca. 30% of methane oxidation occurs in the anoxic part of the lake. Phylogenetic (16S rRNA) analyses showed that ANME generally involved in AOM (ANME-1, -2 and -3) are not present in Lake Pavin. Other archaeal groups that do not have any cultured representatives so far appear well represented in the anoxic parts of the lake but their implication in AOM is not demonstrated. The analysis of lipid biomarkers using GC-MS and LC-MS revealed the presence of a low diversity of archaeal-specific biomarkers in the superficial sediments and in the anoxic waters of the lake. Archaeol and caldarcheaol (GDGT-0) are the two main archaeal core lipids detected; other biomarkers generally present in ANME such as pentamethylicosane or hydroxyarchaeol are not present. However, the stable carbon isotopic composition of archaeol (δ13C = -18‰) and of the biphytane chain of GDGT-0 (δ13C

  13. Formation of hypertrophic scars: Evolution and susceptibility

    NARCIS (Netherlands)

    Mahdavian Delavary, B.; van der Veer, W.M.; Ferreira, J.A.; Niessen, F.B.

    2012-01-01

    Formation of hypertrophic scars is a common complication of wound healing, and at present little is known about the incidence and risk factors. Our aim was to analyse the incidence, progression, and regression of postoperative hypertrophic scars over time and to identify risk factors of hypertrophic

  14. Idiopathic hypertrophic subaortic stenosis. I

    International Nuclear Information System (INIS)

    Kvam, G.

    1980-01-01

    Biplane left ventricular cineangiographies in 4 patients with typical obstructive idiopathic hypertrophic subaortic stenosis (IHSS) and in control patients with normal left ventricles were analysed. In the protruding hypertrophic muscular interventricular septum of IHSS a markedly reduced shortening occurs in either direction during the systolic contraction. It does not bend towards the right ventricle. It is suggested that the septum of IHSS acts as a suspender during the systolic contraction, thereby accounting for the fast stroke volume ejection and the high ejection fraction of IHSS. (Auth.)

  15. Living with hypertrophic cardiomyopathy.

    Science.gov (United States)

    Subasic, Kim

    2013-12-01

    The purpose of this study is to provide an insider's account of what it is like to live with hypertrophic cardiomyopathy (HCM), a genetic cardiovascular illness that carries the risk for sudden cardiac death. This study aims to reveal how HCM impacts the family and guides the decision whether or not to pursue genetic testing, how the physical limitations associated with HCM alter being-in-the-world, and how HCM alters social relationships. Fifteen adults with HCM were recruited for a longitudinal, phenomenological, qualitative study through purposive sampling and word of mouth. A total of 45 interviews were conducted by the researcher at a time and place designated by the participant between August 2011 and January 2012. The first interview with each participant was conducted in person. While efforts were made to conduct all interviews in person, a total of three interviews were conducted by telephone as requested by three participants due to scheduling conflicts. Through methods of interpretive phenomenology, three audio-recorded, semistructured interviews occurred over the course of 3 months. Detailed narratives were solicited and transcribed verbatim. Methodological and analytical documentation was supported with the identification of key phrases, similar experiences, themes, and documentation of the rationale for decisions throughout the research process. Participation in genetic testing carries a multitude of personal, familial, financial, and emotional implications. The results of a genetic test elicited an emotional response regardless of whether the results were negative, positive, or inconclusive. Living with a potentially life-threatening illness altered identity, disrupted social relationships, and generated chronic fear and uncertainty. A new normal was re-ordered or transformed by the demands and limitations posed by HCM, and by the person's concerns, priorities, and the meaning of the illness. Results from this study underscore the need for healthcare

  16. Myocardial ischemia in hypertrophic cardiomyopathy

    International Nuclear Information System (INIS)

    Lima Filho, Moyses de Oliveira; Figueiredo, Geraldo L.; Simoes, Marcus V.; Pyntia, Antonio O.; Marin Neto, Jose Antonio

    2000-01-01

    Myocardial ischemia in hypertrophic cardiomyopathy is multifactorial and explains the occurrence of angina, in about 50% of patients. The pathophysiology of myocardial ischemia may be explained by the increase of the ventricular mass and relative paucity of the coronary microcirculation; the elevated ventricular filling pressures and myocardial stiffness causing a compression of the coronary microvessels; the impaired coronary vasodilator flow reserve caused by anatomic and functional abnormalities; and the systolic compression of epicardial vessel (myocardial bridges). Myocardial ischemia must be investigated by perfusion scintigraphic methods since its presence influences the prognosis and has relevant clinical implications for management of patients. Patients with hypertrophic cardiomyopathy and documented myocardial ischemia usually need to undergo invasive coronary angiography to exclude the presence of concomitant atherosclerotic coronary disease. (author)

  17. Hypertrophic osteopathy and megaoesophagus in a cat.

    Science.gov (United States)

    Mills, J

    2010-01-01

    A case of hypertrophic osteopathy in a stray cat is reported. It was not known how long periosteal proliferation had been present prior to the time of first presentation. A few months later, megaoesophagus became apparent. Computer tomography was performed at least 18 months after periosteal proliferation had first become evident, but an underlying neoplasia was not identifed in association with the hypertrophic osteopathy. The hypertrophic osteopathy and the megaoesophagus were both successfully medically managed, and the cat continued to do well 33 months after the hypertrophic osteopathy was first evident.

  18. Methanolobus psychrotolerans sp. nov., a psychrotolerant methanoarchaeon isolated from a saline meromictic lake in Siberia.

    Science.gov (United States)

    Chen, Sheng-Chung; Huang, Hsing-Hua; Lai, Mei-Chin; Weng, Chieh-Yin; Chiu, Hsiu-Hui; Tang, Sen-Lin; Rogozin, Denis Yu; Degermendzhy, Andrey G

    2018-04-01

    A psychrotolerant, methylotrophic methanogen, strain YSF-03 T , was isolated from the saline meromictic Lake Shira in Siberia. Cells of strain YSF-03 T were non-motile, irregular cocci and 0.8-1.2 µm in diameter. The methanogenic substrates utilized by strain YSF-03 T were methanol and trimethylamine. The temperature range of growth for strain YSF-03 T was from 0 to 37 °C. The optimum growth conditions were 30-37 °C, pH 7.0-7.4 and 0.17 M NaCl. The G+C content of the genome of strain YSF-03 T was 41.3 mol%. Phylogenetic analysis revealed that strain YSF-03 T was most closely related to Methanolobus profundi MobM T (98.15 % similarity in 16S rRNA gene sequence). Genome relatedness between strain YSF-03 T and MobM T was computed using the Genome-to-Genome Distance Calculator and average nucleotide identity, which gave values of 23.5 and 79.3 %, respectively. Based on the morphological, phenotypic, phylogenetic and genomic relatedness data presented here, it is evident that strain YSF-03 T represents a novel species of the genus Methanolobus, for which the name Methanolobus psychrotolerans sp. nov. is proposed. The type strain is YSF-03 T (=BCRC AR10049 T =DSM 104044 T =NBRC 112514 T ).

  19. Phototrophic Fe(II-oxidation in the chemocline of a ferruginous meromictic lake

    Directory of Open Access Journals (Sweden)

    Xavier Alexis eWalter

    2014-12-01

    Full Text Available Precambrian Banded Iron Formation (BIF deposition was conventionally attributed to the precipitation of iron-oxides resulting from the abiotic reaction of ferrous iron (Fe(II with photosynthetically-produced oxygen. Earliest traces of oxygen date from 2.7 Ga, thus raising questions as to what may have caused BIF precipitation before oxygenic photosynthesis evolved. The discovery of anoxygenic phototrophic bacteria thriving through the oxidation of Fe(II has provided support for a biological origin for some BIFs, but despite reports suggesting that anoxygenic phototrophs may oxidize Fe(II in the environment, a model ecosystem of an ancient ocean where they are demonstrably active was lacking. Here we show that anoxygenic phototrophic bacteria contribute to Fe(II oxidation in the water column of the ferruginous sulfate-poor, meromictic lake La Cruz (Spain. We observed in-situ photoferrotrophic activity through stimulation of phototrophic carbon uptake in the presence of Fe(II, and determined light-dependent Fe(II-oxidation by the natural chemocline microbiota. Moreover, a photoferrotrophic bacterium most closely related to Chlorobium ferrooxidans was enriched from the ferruginous water column. Our study for the first time demonstrates a direct link between anoxygenic photoferrotrophy and the anoxic precipitation of Fe(III-oxides in a ferruginous water column, providing a plausible mechanism for the bacterial origin of BIF’s before the advent of free oxygen.

  20. Hypertrophic Nonunion Humerus Mimicking an Enchondroma

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    N. K. Magu

    2014-01-01

    Full Text Available Introduction. Although fractures of humeral shaft show excellent results with conservative management, nonunion does occur. Case Report. We bring forth the case of a young male with a 1.5-year-old hypertrophic nonunion of the humerus mimicking an enchondroma. The initial X-ray images of the patient appeared to be an enchondroma, which only on further evaluation and histopathological analysis was diagnosed conclusively to be a hypertrophic nonunion. Discussion. Enchondromas are often incidentally diagnosed benign tumours. It is however not common to misdiagnose a hypertrophic nonunion to be an enchondroma. We present this case to highlight the unique diagnostic dilemma the treating team had to face.

  1. Laser application for hypertrophic rhinitis

    Science.gov (United States)

    Inouye, Tetsuzo; Tanabe, Tetsuya; Nakanoboh, Manabu; Ogura, Masami

    1995-05-01

    The CO2 and KTP/532 lasers have been used in the treatment of an allergic and hypertrophic rhinitis for the past several years. As we know, the laser enables a surgeon to perform the operation with minimum hemorrhage and minimized pain, during and after the procedure. Additionally many of these operations can be performed under local anesthesia instead of general anesthesia, on an outpatient basis. The laser is used to irradiate the mucous membranes of the inferior turbinates. Vaporization and cutting is easily done. Post operative management of the local operated area is easy. The advantages of laser surgery over regular surgical techniques are supreme for intranasal operations when performed under local anesthesia.

  2. Quantitative analysis of biogeochemically controlled density stratification in an iron-meromictic lake

    Science.gov (United States)

    Nixdorf, E.; Boehrer, B.

    2015-11-01

    Lake stratification controls the cycling of dissolved matter within the water body. This is of particular interest in the case of meromictic lakes, where permanent density stratification of the deep water limits vertical transport, and a chemically different (reducing) milieu can be established. As a consequence, the geochemical setting and the mixing regime of a lake can stabilize each other mutually. We attempt a quantitative approach to the contribution of chemical reactions sustaining the density stratification. As an example, we chose the prominent case of iron meromixis in Waldsee near Doebern, a small lake that originated from near-surface underground mining of lignite. From a data set covering 4 years of monthly measured electrical conductivity profiles, we calculated summed conductivity as a quantitative variable reflecting the amount of electro-active substances in the entire lake. Seasonal variations followed the changing of the chemocline height. Coinciding changes of electrical conductivities in the monimolimnion indicated that a considerable share of substances, precipitated by the advancing oxygenated epilimnion, re-dissolved in the remaining anoxic deep waters and contributed considerably to the density stratification. In addition, we designed a lab experiment, in which we removed iron compounds and organic material from monimolimnetic waters by introducing air bubbles. Precipitates could be identified by visual inspection. Eventually, the remaining solutes in the aerated water layer looked similar to mixolimnetic Waldsee water. Due to its reduced concentration of solutes, this water became less dense and remained floating on nearly unchanged monimolimnetic water. In conclusion, iron meromixis as seen in Waldsee did not require two different sources of incoming waters, but the inflow of iron-rich deep groundwater and the aeration through the lake surface were fully sufficient for the formation of iron meromixis.

  3. Pyrosequencing analysis of the protist communities in a High Arctic meromictic lake: DNA preservation and change

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    Sophie eCharvet

    2012-12-01

    Full Text Available High Arctic meromictic lakes are extreme environments characterized by cold temperatures, low nutrient inputs from their polar desert catchments and prolonged periods of low irradiance and darkness. These lakes are permanently stratified with an oxygenated freshwater layer (mixolimnion overlying a saline, anoxic water column (monimolimnion. The physical and chemical properties of the deepest known lake of this type in the circumpolar Arctic, Lake A, on the far northern coast of Ellesmere Island, Canada, have been studied over the last 15 years, but little is known about the lake’s biological communities. We applied high-throughput sequencing of the V4 region of the 18S ribosomal RNA gene to investigate the protist communities down the water column at three sampling times: under the ice at the end of winter in 2008, during an unusual period of warming and ice-out the same year, and again under the ice in mid-summer 2009. Sequences of many protist taxa occurred throughout the water column at all sampling times, including in the deep anoxic layer where growth is highly unlikely. Furthermore, there were sequences for taxonomic groups including diatoms and marine taxa, which have never been observed in Lake A by microscopic analysis. However the sequences of other taxa such as ciliates, chrysophytes, Cercozoa and Telonema varied with depth, between years and during the transition to ice-free conditions. These results imply that there are seasonally active taxa in the surface waters of the lake that are sensitive to depth and change with time. DNA from these taxa is superimposed upon background DNA from multiple internal and external sources that is preserved in the deep, cold, largely anoxic water column.

  4. Subaortic membrane mimicking hypertrophic cardiomyopathy.

    Science.gov (United States)

    Anderson, Mark Joseph; Arruda-Olson, Adelaide; Gersh, Bernard; Geske, Jeffrey

    2015-11-04

    A 34-year-old man was referred for progressive angina and exertional dyspnoea refractory to medical therapy, with a presumptive diagnosis of hypertrophic cardiomyopathy (HCM). Transthoracic echocardiography (TTE) revealed asymmetric septal hypertrophy without systolic anterior motion of the mitral valve leaflet and with no dynamic left ventricular outflow tract (LVOT) obstruction. However, the LVOT velocity was elevated at rest as well as with provocation, without the characteristic late peaking obstruction seen in HCM. Focused TTE to evaluate for suspected fixed obstruction demonstrated a subaortic membrane 2.2 cm below the aortic valve. Coronary CT angiography confirmed the presence of the subaortic membrane and was negative for concomitant coronary artery disease. Surgical resection of the subaortic membrane and septal myectomy resulted in significant symptomatic relief and lower LVOT velocities on postoperative TTE. This case reminds the clinician to carefully evaluate for alternative causes of LVOT obstruction, especially subaortic membrane, as a cause of symptoms mimicking HCM. 2015 BMJ Publishing Group Ltd.

  5. Prevention and curative management of hypertrophic scar formation

    NARCIS (Netherlands)

    Bloemen, M.C.; Veer, van der W.M.; Ulrich, M.; Zuijlen, van P.P.; Niessen, F.B.; Middelkoop, E.

    2009-01-01

    Although hypertrophic scarring commonly occurs following burns, many aspects such as incidence of and optimal treatment for scar hypertrophy remain unclear. This review will focus on hypertrophic scar formation after burn in particular, exploring multiple treatment options and describing their

  6. Hypertrophic osteoarthropathy associated with alcoholic liver disease without cirrhosis.

    Science.gov (United States)

    Varju, T; Lesch, M; Adorján, A

    1986-01-01

    Two cases of secondary hypertrophic osteoarthropathy associated with alcoholic liver disease without cirrhosis are reported. Conditions which can be associated with hypertrophic osteoarthropathy and theoretical factors which can play a role in its pathomechanism are briefly discussed.

  7. Clinical-radiological experiences in patients with hypertrophic cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Hofmann, A; Bonse, G; Beck, B; Sauter, E; Sundermeyer, R; Gunkel, L V

    1986-09-01

    The hypertrophic cardiomyopathy shows a series of interesting clinical and radiological problems, discussed in case of selected patients. A special difficult problem arises in the differential diagnosis of hypertrophic cardiomyopathy and cardiac disease secondary to systemic hypertension.

  8. The KCNE genes in hypertrophic cardiomyopathy: a candidate gene study

    DEFF Research Database (Denmark)

    Hedley, Paula L; Haundrup, Ole; Andersen, Paal S

    2011-01-01

    The gene family KCNE1-5, which encode modulating β-subunits of several repolarising K+-ion channels, has been associated with genetic cardiac diseases such as long QT syndrome, atrial fibrillation and Brugada syndrome. The minK peptide, encoded by KCNE1, is attached to the Z-disc of the sarcomere...... as well as the T-tubules of the sarcolemma. It has been suggested that minK forms part of an "electro-mechanical feed-back" which links cardiomyocyte stretching to changes in ion channel function. We examined whether mutations in KCNE genes were associated with hypertrophic cardiomyopathy (HCM), a genetic...

  9. Apical Hypertrophic Cardiomyopathy in Association with PulmonaryArtery Hypertension

    Directory of Open Access Journals (Sweden)

    Mehdi Peighambari

    2012-09-01

    Full Text Available Apical Hypertrophic Cardiomyopathy is an uncommon condition constituting 1% -2% of the cases with Hypertrophic Cardiomyopathy (HCM diagnosis. We interestingly report two patients with apical hypertrophic cardiomyopathy in association with significant pulmonary artery hypertension without any other underlying reason for pulmonary hypertension. The patients were assessed by echocardiography, cardiac catheterization and pulmonary function parameters study.

  10. Pregnancy in women with hypertrophic cardiomyopathy

    NARCIS (Netherlands)

    Pieper, P. G.; Walker, F.

    Hypertrophic cardiomyopathy (HCM) is increasingly being diagnosed in pregnant women. Women with HCM generally tolerate pregnancy well. The risk is however higher in women who are symptomatic before pregnancy or in those with severe left ventricular outflow tract obstruction. The incidence of

  11. Extensive hypertrophic lupus erythematosus: Atypical presentation

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    Tarun Narang

    2012-01-01

    Full Text Available Lupus erythematosus (LE is a disease with a wide spectrum of cutaneous and systemic manifestations. Clinical features of patients with LE show a great variation, and for this reason it is difficult to develop a unifying concept of this disease. Our objective is to present a case of hypertrophic LE with atypical morphology and extensive involvement, who responded favorably to isotretinoin. Diagnosis of hypertrophic lupus erythematosus (HLE was confirmed by characteristic histopathological findings. Combination therapy with isotretinoin and hydroxychloroquine resulted in flattening and repression of previously refractory skin lesions. Sometimes, HLE lesions may present a diagnostic and therapeutic dilemma. In long standing lesions, squamous cell carcinoma may arise. Therefore, HLE requires adequate therapy with clinical and histopathological follow up.

  12. Magnetic resonance imaging in hypertrophic cardiomyopathy

    International Nuclear Information System (INIS)

    Ichida, Fukiko; Hamamichi, Yuuji; Hashimoto, Ikuo; Tsubata, Shinichi; Miyazaki, Ayumi; Okada, Toshio; Futatsuya, Ryuusuke; Okada, Eikichi

    1994-01-01

    To evaluate the capability of magnetic resonance imaging (MRI) in the anatomical diagnosis and tissue characterization, 8 children with hypertrophic cardiomyopathy were studied comparing with echocardiography and 201 Tl myocardial imaging. The severity and distribution of hypertrophy were comparable on echocardiography and MRI. MRI was superior to echocardiography to demonstrate the apical hypertrophy. In 4 patients with severe hypertrophy, heterogenous high signal intensity was demonstrated in the site of hypertrophy, which was enhanced by T 2 weighted imaging. In the patient with decreased cardiac performance and progressed cardiac failure, the heterogeneity and high signal intensity progressed in one year interval. Simultaneously performed 201 Tl myocardial imaging showed patchy perfusion defect. Histological findings of the left ventricle demonstrated hypertrophy, degeneration and marked dysarray of the myocytes and fibrosis. MRI has the potential ability for the evaluation and sequential monitoring of myocardial tissue characterization as well as cardiac anatomy in childhood hypertrophic cardiomyopathy. (author)

  13. Magnetic resonance imaging in hypertrophic cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Ichida, Fukiko; Hamamichi, Yuuji; Hashimoto, Ikuo; Tsubata, Shinichi; Miyazaki, Ayumi; Okada, Toshio; Futatsuya, Ryuusuke; Okada, Eikichi [Toyama Medical and Pharmaceutical Univ. (Japan)

    1994-02-01

    To evaluate the capability of magnetic resonance imaging (MRI) in the anatomical diagnosis and tissue characterization, 8 children with hypertrophic cardiomyopathy were studied comparing with echocardiography and [sup 201]Tl myocardial imaging. The severity and distribution of hypertrophy were comparable on echocardiography and MRI. MRI was superior to echocardiography to demonstrate the apical hypertrophy. In 4 patients with severe hypertrophy, heterogenous high signal intensity was demonstrated in the site of hypertrophy, which was enhanced by T[sub 2] weighted imaging. In the patient with decreased cardiac performance and progressed cardiac failure, the heterogeneity and high signal intensity progressed in one year interval. Simultaneously performed [sup 201]Tl myocardial imaging showed patchy perfusion defect. Histological findings of the left ventricle demonstrated hypertrophy, degeneration and marked dysarray of the myocytes and fibrosis. MRI has the potential ability for the evaluation and sequential monitoring of myocardial tissue characterization as well as cardiac anatomy in childhood hypertrophic cardiomyopathy. (author).

  14. Left ventricular function in hypertrophic cardiomyopathy

    International Nuclear Information System (INIS)

    Takahashi, Hiromi; Yamaguchi, Ryutaro; Ifuku, Masayasu

    1985-01-01

    The present study was to investigate of left ventricular (LV) function during exercise in 26 patients with hypertrophic cardiomyopathy(HCM) usingTc-99m equilibrium angiocardiography, and to elucidate the mechanism of impaired functional reserve during exercise. In patients with HCM, LV ejection fraction decreased from 65 ± 8 (mean ± SD) % at rest to 59 ± 18 % at peak exercise, in contrast to an increase among controls (from 56 ± 9 % to 64 ± 9 %). As compared with resting values, cardiac output increased to 168 ± 24 % at peak exercise in HCM, but the increase was significantly less than that in controls (215 ± 47 %). Stroke volume decreased gradually to 83 ± 16 % during exercise in HCM, while it increased to 114 ± 10 % at an exercise level of half intensity, and it decreased slightly to 106 ± 16 % at peak exercise. LV end-systolic volume decreased among controls to 78 ± 27 % at peak exercise, but remained unchanged in HCM (118 ± 58 %). An increase in peak ejection rate at peak exercise was less in HCM than in controls (143 ± 26 % vs 170 ± 42 %). No significant differences were observed between the two groups concerning changes in indices of LV diastolic function including LV end-diastolic volume, peak filling rate or 1/3 filling rate during exercise. In the analysis of LV function curves, pulmonary arterial diastolic pressure increased to a greater extent in HCM than in controls (19 ± 6 mmHg vs 11 ± 6 mmHg); whereas, an increase in the stroke work index was less in HCM (80 ± 26 g.m/m 2 /beat vs 121 ± 21 g.m/m 2 /beat) at peak exercise. Thus, the LV function curve shifted downward and to the right in patients with HCM. The above findings indicate that LV functional reserve during exercise is impaired, especially as to systolic function in patients with HCM, while deterioration of diastolic function may be partly compromised by elevated filling pressure. (J.P.N.)

  15. Infantile hypertrophic pyloric stenosis. Infantil hypertrofisk pylorusstenose

    Energy Technology Data Exchange (ETDEWEB)

    Breivik, K.; Soereide, J.A.; Bland, J. (Rogaland Central Hospital, Stavanger (Norway))

    1990-09-01

    During an eight-year period, 40 patients were operated consecutively for pyloric stenosis. The most common symptom was projectile vomiting, which occurred in 92.5% of the cases. On examination only three patients had a palpable hypertrophic pylorus. In 39 patients a preoperative X-ray examination with contrast was necessary to confirm the diagnosis. A pyleromyotomy was performed in all patients. The diagnosis routines and the results of the treatment are discussed. 16 refs., 2 figs., 3 tabs.

  16. Impact of late glacial climate variations on stratification and trophic state of the meromictic lake Längsee (Austria: validation of a conceptual model by multi proxy studies

    Directory of Open Access Journals (Sweden)

    Jens MÜLLER

    2002-02-01

    Full Text Available Selected pigments, diatoms and diatom-inferred phosphorus (Di-TP concentrations of a late glacial sediment core section of the meromictic Längsee, Austria, were compared with tephra- and varve-dated pollen stratigraphic and geochemical results. A conceptual model was adopted for Längsee and evaluated using multi proxy data. During the unforested late Pleniglacial, a holomictic lake stage with low primary productivity prevailed. Subsequent to the Lateglacial Betula expansion, at about 14,300 cal. y BP, okenone and isorenieratene, pigments from purple and green sulphur bacteria, indicate the onset of anoxic conditions in the hypolimnion. The formation of laminae coincides with this anoxic, meromictic period with high, though fluctuating, amounts of okenone that persisted throughout the Lateglacial interstadial. The occurrence of unlaminated sediment sections of allochthonous origin, and concurrent low concentrations of okenone, were related to cool and wet climate fluctuations during this period, probably coupled with a complete mixing of the water column. Two of these oscillations of the Lateglacial interstadial have been correlated tentatively with the Aegelsee and Gerzensee oscillations in the Alps. The latter climate fluctuation divides a period of enhanced anoxia and primary productivity, correlated with the Alleröd chronozone. Continental climate conditions were assumed to be the main driving forces for meromictic stability during Alleröd times. In addition, calcite dissolution due to severe hypolimnetic anoxia, appear to have supported meromictic stability. Increased pigment concentrations, which are in contrast to low diatom-inferred total phosphorus (Di- TP, indicate the formation of a productive metalimnion during this period, probably due to a clear-water phase (low catchment erosion, increased temperatures, and a steep gradient between the phosphorus enriched hypolimnion and the oligotrophic epilimnion. Meltwater impacts from an

  17. Hypertrophic pachymeningitis accompanying neuromyelitis optica spectrum disorder: A case report.

    Science.gov (United States)

    Kon, Tomoya; Nishijima, Haruo; Haga, Rie; Funamizu, Yukihisa; Ueno, Tatsuya; Arai, Akira; Suzuki, Chieko; Nunomura, Jin-ichi; Baba, Masayuki; Takahashi, Toshiyuki; Tomiyama, Masahiko

    2015-10-15

    We report a case of idiopathic cerebral hypertrophic pachymeningitis accompanying neuromyelitis optica spectrum disorder. No other identifiable cause of pachymeningitis was detected. Corticosteroid therapy was effective for both diseases. Hypertrophic pachymeningitis is closely related to autoimmune inflammatory disease of the central nervous system. This case supports the hypothesis that hypertrophic pachymeningitis can be a rare comorbidity of neuromyelitis optica spectrum disorder. Copyright © 2015. Published by Elsevier B.V.

  18. Cushing's syndrome in pregnancy and neonatal hypertrophic obstructive cardiomyopathy.

    Science.gov (United States)

    Fayol, L; Masson, P; Millet, V; Simeoni, U

    2004-10-01

    Cushing's syndrome is rare in pregnancy but can cause spontaneous abortion, stillbirth or premature birth. We report a case of transient hypertrophic obstructive cardiomyopathy in a newborn whose mother had hypercortisolism due to a primary adrenal lesion. There was no family history of hypertrophic obstructive cardiomyopathy. Follow-up revealed complete resolution of the cardiac abnormalities in the infant. Cushing's syndrome in the mother resolved after delivery. Although maternal hypercortisolism seldom results in symptomatic hypercortisolism in the newborn, hypertrophic obstructive cardiomyopathy can occur.

  19. Powering up the biogeochemical engine: The impact of exceptional ventilation of a deep meromictic lake on the lacustrine redox, nutrient, and methane balances

    Directory of Open Access Journals (Sweden)

    Moritz Felix Lehmann

    2015-08-01

    Full Text Available The Lake Lugano North Basin has been meromictic for several decades, with anoxic waters below 100m depth. Two consecutive cold winters in 2005 and 2006 induced exceptional deep mixing, leading to a transient oxygenation of the whole water column. With the ventilation of deep waters and the oxidation of large quantities of reduced solutes, the lake's total redox-balance turned positive, and the overall hypolimnetic oxygen demand of the lake strongly decreased. The disappearance of 150 t dissolved phosphorous (P during the first ventilation in March 2005 is attributed to the scavenging of water-column-borne P by newly formed metal oxyhydroxides and the temporary transfer to the sediments. The fixed nitrogen (N inventory was reduced by ~30% (~1000 t. The water-column turnover induced the nitratation of the previously NO3--free deep hypolimnion by oxidation of large amounts of legacy NH4+ and by mixing with NO3--rich subsurface water masses. Sediments with a strong denitrifying potential, but NO3--starved for decades, were brought in contact with NO3--replete waters, invigorating benthic denitrification and rapid fixed N loss from the lake in spite of the overall more oxygenated conditions. Similarly, a large microbial aerobic CH4 oxidation (MOx potential in the hypolimnion was capitalized with the ventilation of the deep basin. Almost all CH4, which had been built up over more than 40 years (~2800 t, was removed from the water column within 30 days. However, boosted MOx could only partly explain the disappearance of the CH4. The dominant fraction (75% of the CH4 evaded to the atmosphere, through storage flux upon exposure of anoxic CH4-rich water to the atmosphere. As of today, the North Basin seems far from homeostasis regarding its fixed N and CH4 budgets, and the deep basin's CH4 pool is recharging at a net production rate of ~66 t y-1. The size of impending CH4 outbursts will depend on the frequency and intensity of exceptional mixing events in

  20. Hypoxia impedes hypertrophic chondrogenesis of human multipotent stromal cells.

    Science.gov (United States)

    Gawlitta, Debby; van Rijen, Mattie H P; Schrijver, Edmée J M; Alblas, Jacqueline; Dhert, Wouter J A

    2012-10-01

    Within the field of bone tissue engineering, the endochondral approach to forming bone substitutes represents a novel concept, where cartilage will undergo hypertrophic differentiation before its conversion into bone. For this purpose, clinically relevant multipotent stromal cells (MSCs), MSCs, can be differentiated into the chondrogenic lineage before stimulating hypertrophy. Controversy exists in literature on the oxygen tensions naturally present during this transition in, for example, the growth plate. Therefore, the present study focused on the effects of different oxygen tensions on the progression of the hypertrophic differentiation of MSCs. Bone marrow-derived MSCs of four human donors were expanded, and differentiation was induced in aggregate cultures. Normoxic (20% oxygen) and hypoxic (5%) conditions were imposed on the cultures in chondrogenic or hypertrophic differentiation media. After 4 weeks, the cultures were histologically examined and by real-time polymerase chain reaction. Morphological assessment showed the chondrogenic differentiation of cultures from all donors under normoxic chondrogenic conditions. In addition, hypertrophic differentiation was observed in cultures derived from all but one donor. The deposition of collagen type X was evidenced in both chondrogenically and hypertrophically stimulated cultures. However, mineralization was exclusively observed in hypertrophically stimulated, normoxic cultures. Overall, the progression of hypertrophy was delayed in hypoxic compared with normoxic groups. The observed delay was supported by the gene expression patterns, especially showing the up-regulation of the late hypertrophic markers osteopontin and osteocalcin under normoxic hypertrophic conditions. Concluding, normoxic conditions are more beneficial for hypertrophic differentiation of MSCs than are hypoxic conditions, as long as the MSCs possess hypertrophic potential. This finding has implications for cartilage tissue engineering as well

  1. Estimation of lake water - groundwater interactions in meromictic mining lakes by modelling isotope signatures of lake water.

    Science.gov (United States)

    Seebach, Anne; Dietz, Severine; Lessmann, Dieter; Knoeller, Kay

    2008-03-01

    A method is presented to assess lake water-groundwater interactions by modelling isotope signatures of lake water using meteorological parameters and field data. The modelling of delta(18)O and deltaD variations offers information about the groundwater influx into a meromictic Lusatian mining lake. Therefore, a water balance model is combined with an isotope water balance model to estimate analogies between simulated and measured isotope signatures within the lake water body. The model is operated with different evaporation rates to predict delta(18)O and deltaD values in a lake that is only controlled by weather conditions with neither groundwater inflow nor outflow. Comparisons between modelled and measured isotope values show whether the lake is fed by the groundwater or not. Furthermore, our investigations show that an adaptation of the Craig and Gordon model [H. Craig, L.I. Gordon. Deuterium and oxygen-18 variations in the ocean and the marine atmosphere. In Stable Isotopes in Oceanographic Studies and Paleotemperature, Spoleto, E. Tongiorgi (Ed.), pp. 9-130, Consiglio Nazionale delle Ricerche, Laboratorio di Geologia Nucleare, Pisa (1965).] to specific conditions in temperate regions seems necessary.

  2. Idiopathic hypertrophic cranial pachymeningitis associated with Sweet's Syndrome

    International Nuclear Information System (INIS)

    Cano, Antonio; Ribes, Ramon; Riva, Andres de la; Rubio, Fernando Lopez; Sanchez, Carmen; Sancho, Jose L.

    2002-01-01

    A case of hypertrophic cranial pachymeningitis associated with Sweet's Syndrome is presented. Both entities have been described in association with several other chronic systemic inflammatory diseases and autoimmune conditions. To our knowledge the coexistence between Sweet's Syndrome and hypertrophic cranial pachymeningitis has not been reported up to date. We suggest a possible autoimmune or dysimmune mechanism in the pathogenesis of these two entities

  3. Fabry Disease in Families With Hypertrophic Cardiomyopathy

    DEFF Research Database (Denmark)

    Adalsteinsdottir, Berglind; Palsson, Runolfur; Desnick, Robert J

    2017-01-01

    BACKGROUND: The screening of Icelandic patients clinically diagnosed with hypertrophic cardiomyopathy resulted in identification of 8 individuals from 2 families with X-linked Fabry disease (FD) caused by GLA(α-galactosidase A gene) mutations encoding p.D322E (family A) or p.I232T (family B...... asymmetrical, and had similar late gadolinium enhancement patterns. Ischemic stroke and severe white matter lesions were more frequent among family A men, but neither family A nor family B men had overt renal disease. Family A and family B heterozygotes had less severe or no clinical manifestations...

  4. Hypertrophic Cardiomyopathy—Past, Present and Future

    Directory of Open Access Journals (Sweden)

    Alphonsus C. Liew

    2017-12-01

    Full Text Available Hypertrophic cardiomyopathy (HCM is the most common genetic cardiomyopathy with a prevalence of 1 in 500 in the general population. Since the first pathological case series at post mortem in 1957, we have come a long way in its understanding, diagnosis and management. Here, we will describe the history of our understanding of HCM including the initial disease findings, diagnostic methods and treatment options. We will review the current guidelines for the diagnosis and management of HCM, current gaps in the evidence base and discuss the new and promising developments in this field.

  5. Pseudotumoural hypertrophic neuritis of the facial nerve

    OpenAIRE

    Zanoletti, E; Mazzoni, A; Barbò, R

    2008-01-01

    In a retrospective study of our cases of recurrent paralysis of the facial nerve of tumoural and non-tumoural origin, a tumour-like lesion of the intra-temporal course of the facial nerve, mimicking facial nerve schwannoma, was found and investigated in 4 cases. This was defined as, pseudotumoral hypertrophic neuritis of the facial nerve. The picture was one of recurrent acute facial palsy with incomplete recovery and imaging of a benign tumour. It was different from the well-known recurrent ...

  6. Hypoxia inhibits hypertrophic differentiation and endochondral ossification in explanted tibiae.

    Directory of Open Access Journals (Sweden)

    Jeroen C H Leijten

    Full Text Available Hypertrophic differentiation of growth plate chondrocytes induces angiogenesis which alleviates hypoxia normally present in cartilage. In the current study, we aim to determine whether alleviation of hypoxia is merely a downstream effect of hypertrophic differentiation as previously described or whether alleviation of hypoxia and consequent changes in oxygen tension mediated signaling events also plays an active role in regulating the hypertrophic differentiation process itself.Fetal mouse tibiae (E17.5 explants were cultured up to 21 days under normoxic or hypoxic conditions (21% and 2.5% oxygen respectively. Tibiae were analyzed on growth kinetics, histology, gene expression and protein secretion.The oxygen level had a strong influence on the development of explanted fetal tibiae. Compared to hypoxia, normoxia increased the length of the tibiae, length of the hypertrophic zone, calcification of the cartilage and mRNA levels of hypertrophic differentiation-related genes e.g. MMP9, MMP13, RUNX2, COL10A1 and ALPL. Compared to normoxia, hypoxia increased the size of the cartilaginous epiphysis, length of the resting zone, calcification of the bone and mRNA levels of hyaline cartilage-related genes e.g. ACAN, COL2A1 and SOX9. Additionally, hypoxia enhanced the mRNA and protein expression of the secreted articular cartilage markers GREM1, FRZB and DKK1, which are able to inhibit hypertrophic differentiation.Collectively our data suggests that oxygen levels play an active role in the regulation of hypertrophic differentiation of hyaline chondrocytes. Normoxia stimulates hypertrophic differentiation evidenced by the expression of hypertrophic differentiation related genes. In contrast, hypoxia suppresses hypertrophic differentiation of chondrocytes, which might be at least partially explained by the induction of GREM1, FRZB and DKK1 expression.

  7. Diagnostic imaging of hypertrophic pyloric stenosis (HPS)

    International Nuclear Information System (INIS)

    Frkovic, M.; Seronja Kuhar, M.; Perhoc, Z.; Barbaric-Babic, V.; Molnar, M.; Vukovic, J.

    2001-01-01

    Background. Imaging of the abdomen in children with suspected hypertrophic pyloric stenosis has been traditionally performed by plain film radiography and upper gastrointestinal contrast studies. In many clinical situations, this approach has been modified or replaced by ultrasound examination. The authors aimed to analyse the value of diagnostic algorithm in children with hypertrophic pyloric stenosis confirmed at surgery in our hospital. Patients and methods. The authors made a five year retrospective review of hospital records of all children operated on for HPS in Clinical Hospital Centre Zagreb - Rebro and found out that 14 boys, between 2 (17 days) and 10 weeks of life (75 days) underwent surgery due to HPS. Results. Specific radiographic signs were: string sign, double track sign, elongation and narrowing of pyloric canal, mushroom sign, gastric distension with fluid and beak sign. Ultrasound was performed in 9 patients, one of them was false negative (sonographer admitted that he had no experience), the rest were positive. Conclusions. If the physical examination is negative or equivocal, sonography by an experienced sonographer must be performed. If the ultrasound finding is negative, than the infant should undergo to barium upper gastrointestinal studies (UGI). If HPS isn't a primary diagnostic question, it's better to perform UGI first in order to make a correct diagnosis. (author)

  8. Clinical Presentation and Natural History of Hypertrophic Cardiomyopathy in RASopathies.

    Science.gov (United States)

    Calcagni, Giulio; Adorisio, Rachele; Martinelli, Simone; Grutter, Giorgia; Baban, Anwar; Versacci, Paolo; Digilio, Maria Cristina; Drago, Fabrizio; Gelb, Bruce D; Tartaglia, Marco; Marino, Bruno

    2018-04-01

    RASopathies are a heterogeneous group of genetic syndromes characterized by mutations in genes that regulate cellular processes, including proliferation, differentiation, survival, migration, and metabolism. Excluding congenital heart defects, hypertrophic cardiomyopathy is the most frequent cardiovascular defect in patients affected by RASopathies. A worse outcome (in terms of surgical risk and/or mortality) has been described in a specific subset of Rasopathy patients with early onset, severe hypertrophic cardiomyopathy presenting with heart failure. New short-term therapy with a mammalian target of rapamycin inhibitor has recently been used to prevent heart failure in these patients with a severe form of hypertrophic cardiomyopathy. Copyright © 2017 Elsevier Inc. All rights reserved.

  9. Cardiovascular magnetic resonance in hypertrophic cardiomyopathy

    International Nuclear Information System (INIS)

    Shiozaki, Afonso Akio; Parga, Jose Rodrigues; Arteaga, Edmundo; Rochitte, Carlos Eduardo; Tassi, Eduardo Marinho

    2007-01-01

    Hypertrophic cardiomyopathy (HCM) is the most frequent genetic cardiac disease that causes sudden death in young people, with an incidence of 1:500 adults. The routinely used criteria for worst prognosis have limited sensitivity and specificity. Thus, the estimated risk of evolving to dilated cardiomyopathy or sudden death is somewhat inaccurate, leading to management uncertainty of HCM patients. Therefore, an accurate noninvasive method for the diagnosis of HCM with prognostic value is of great importance. In the last years, Cardiovascular Magnetic Resonance (CMR) emerged not only as a diagnostic tool, but also as a study with prognostic values, by characterizing myocardial fibrosis with great accuracy in HCM patients. Additionally, CMR identifies the types of hypertrophy, analyses the ventricular function, estimates the intraventricular gradient and allows the determination of differential diagnosis. Moreover, CMR can uniquely access myocardial fibrosis in HCM. (author)

  10. MT-CYB mutations in hypertrophic cardiomyopathy

    DEFF Research Database (Denmark)

    Hagen, Christian M; Aidt, Frederik H; Havndrup, Ole

    2013-01-01

    Mitochondrial dysfunction is a characteristic of heart failure. Mutations in mitochondrial DNA, particularly in MT-CYB coding for cytochrome B in complex III (CIII), have been associated with isolated hypertrophic cardiomyopathy (HCM). We hypothesized that MT-CYB mutations might play an important...... and m.15482T>C; p.S246P were identified. Modeling showed that the p.C93Y mutation leads to disruption of the tertiary structure of Cytb by helix displacement, interfering with protein-heme interaction. The p.S246P mutation induces a diproline structure, which alters local secondary structure and induces...... of HCM patients. We propose that further patients with HCM should be examined for mutations in MT-CYB in order to clarify the role of these variants....

  11. Hypertrophic Synovitis of the Facet Joint Causing Root Pain

    Directory of Open Access Journals (Sweden)

    Koichi Iwatsuki M.D.

    2008-01-01

    Full Text Available Osteoarthritic changes in the facet joints are common in the presence of degenerative disc disease. Changes in the joint capsule accompany changes in the articular surfaces. Intraspinal synovial cysts that cause radicular pain, cauda equina syndrome, and myelopathy have been reported; however, there have been few reports in orthopedic or neurosurgical literature regarding hypertrophic synovitis of the facet joint presenting as an incidental para-articular mass. Here, we report a case of hypertrophic synovitis causing root pain. We describe the case of a 65-year-old man suffering from right sciatica and right leg pain in the L5 nerve-root dermatome for 1 year; magnetic resonance imaging (MRI revealed an enhanced mass around the L4–5 facet joint. We investigated this mass pathologically. After right medial facetectomy, the symptoms resolved. Pathological investigation revealed this mass was hypertrophic synovitis. Hypertrophic synovitis of the facet joint might cause root pain.

  12. Before and After Photos: Treatment of Hypertrophic Scars

    Science.gov (United States)

    ... for Every Season How to Choose the Best Skin Care Products In This Section Dermatologic Surgery What is dermatologic ... for Every Season How to Choose the Best Skin Care Products Before and after photos: Treatment of hypertrophic scars ...

  13. MRI and MR tractography in bilateral hypertrophic olivary degeneration

    Directory of Open Access Journals (Sweden)

    Debraj Sen

    2014-01-01

    Full Text Available Hypertrophic olivary degeneration is a trans-synaptic neuronal degeneration associated with hypertrophy of the inferior olivary nucleus due to a lesion in the triangle of Guillain-Mollaret. Familiarity with this entity on magnetic resonance imaging (MRI is essential to avoid other erroneous ominous diagnoses. We present a case of bilateral hypertrophic olivary degeneration and discuss the etiopathogenesis and MRI findings in this entity. The contributory role of MR tractography in the diagnosis is also highlighted.

  14. MRI and MR tractography in bilateral hypertrophic olivary degeneration.

    Science.gov (United States)

    Sen, Debraj; Gulati, Yoginder S; Malik, Virender; Mohimen, Aneesh; Sibi, Eranki; Reddy, Deepak Chandra

    2014-10-01

    Hypertrophic olivary degeneration is a trans-synaptic neuronal degeneration associated with hypertrophy of the inferior olivary nucleus due to a lesion in the triangle of Guillain-Mollaret. Familiarity with this entity on magnetic resonance imaging (MRI) is essential to avoid other erroneous ominous diagnoses. We present a case of bilateral hypertrophic olivary degeneration and discuss the etiopathogenesis and MRI findings in this entity. The contributory role of MR tractography in the diagnosis is also highlighted.

  15. Hypertrophic pyloric stenosis: tips and tricks for ultrasound diagnosis

    OpenAIRE

    Costa Dias, Sílvia; Swinson, Sophie; Torrão, Helena; Gonçalves, Lígia; Kurochka, Svitlana; Vaz, Carlos Pina; Mendes, Vasco

    2012-01-01

    We describe a systematic approach to the ultrasound (US) examination of the antropyloric region in children. US is the modality of choice for the diagnosis of hypertrophic pyloric stenosis (HPS). The imaging features of the normal pylorus and the diagnostic findings in HPS are reviewed and illustrated in this pictorial essay. Common difficulties in performing the examination and tips to help overcome them will also be discussed. Main Messages • Hypertrophic Pyloric Stenosis is defined by thic...

  16. Hypertrophic cardiomyopathy with mid-ventricular obstruction and apical aneurysm

    Directory of Open Access Journals (Sweden)

    N.D. Oryshchyn

    2016-11-01

    Full Text Available A case report of apical left ventricular aneurysm in patient with hypertrophic cardiomyopathy with mid-ventricular obstruction (diagnosis and surgical treatment is presented. We revealed apical aneurysm and mid-ventricular obstruction during echocardiography and specified anatomical characteristics of aneurysm during computer tomography. There was no evidence of obstructive coronary artery disease during coronary angiography. Taking into consideration multiple cerebral infarcts, aneurysm resection and left ventricular plastics was performed. Electronic microscopy of myocardium confirmed the diagnosis of hypertrophic cardiomyopathy.

  17. MRI and MR tractography in bilateral hypertrophic olivary degeneration

    International Nuclear Information System (INIS)

    Sen, Debraj; Gulati, Yoginder S.; Malik, Virender; Mohimen, Aneesh; Sibi, Eranki; Reddy, Deepak Chandra

    2014-01-01

    Hypertrophic olivary degeneration is a trans-synaptic neuronal degeneration associated with hypertrophy of the inferior olivary nucleus due to a lesion in the triangle of Guillain-Mollaret. Familiarity with this entity on magnetic resonance imaging (MRI) is essential to avoid other erroneous ominous diagnoses. We present a case of bilateral hypertrophic olivary degeneration and discuss the etiopathogenesis and MRI findings in this entity. The contributory role of MR tractography in the diagnosis is also highlighted

  18. Microbial processes in the Kanda Bay, a meromictic water body artifically separated from the White Sea.

    Science.gov (United States)

    Savvichev, A S; Demidenko, N A; Krasnova, E D; Kalmatskaya, O V; Kharcheva, A N; Ivanov, M V

    2017-05-01

    Sings of meromixis are found by means of microbiological and biogeochemical investigations in the southernn part of the Kanda Bay, an artificial water body separated front the White Sea with a railway dam. The concentration of oxygen in the bottom layer attained 1.9 mmol/L, intensity of the process of microbial sulfate reduction, 3.0 μmol of sulfur/(L day). The concentration of dissolved methane, 3.7 μmol/L. Isotopic composition of carbon in methane (δ 13 C (CH 4 ) =-79.2‰) indicates to its microbial genesis. At present, Kanda Bay is a sole in Russia man-made marine water body for which there are data on the rate of microbial processes responsible for formation of bottom water layer containing hydrogen sulfide and methane.

  19. Comparison among patients with hypertrophic cardiomyopathy, hypertrophic cardiomyopathy with hypertension and hypertensive heart disease by 123I-BMIPP myocardial scintigraphy

    International Nuclear Information System (INIS)

    Yoneyama, Satoshi; Sugihara, Hiroki; Ito, Kazuki

    1997-01-01

    The usefulness of 123 I-BMIPP myocardial SPECT in discriminating hypertrophic cardiomyopathy (46 patients), hypertrophic cardiomyopathy with hypertension (23 patients), and hypertensive hypertrophic heart (20 patients) was studied. SPECT image was divided into 17 domains, and dimension of decreased accumulation was decided visually at each domain as four classes called defect score (DS). Summation of DS (TDS) of each group was used to compare frequency and dimension of decreased accumulation, and characteristic of each site. Frequency of decreased accumulation and TDS in hypertrophic cardiomyopathy were similar in dimension with those in hypertrophic cardiomyopathy with hypertension, and those data in hypertensive hypertrophic heart were lower than those in above-mentioned 2 groups. In the cases of hypertrophic cardiomyopathy and hypertrophic cardiomyopathy with hypertension, decreased accumulation site was similar and was anterior wall-septum junction, septum-posterior wall junction and apex of heart. In the case of hypertensive hypertrophic heart, decreased accumulation site was only the posterior wall. Frequency, dimension and site of decreased accumulation in hypertrophic cardiomyopathy were different from those in hypertensive hypertrophic heart, and BMIPP was thought to be useful in discriminating these diseases. (K.H.)

  20. Cardiac norepinephrine kinetics in hypertrophic cardiomyopathy

    International Nuclear Information System (INIS)

    Brush, J.E. Jr.; Eisenhofer, G.; Garty, M.; Stull, R.; Maron, B.J.; Cannon, R.O. III; Panza, J.A.; Epstein, S.E.; Goldstein, D.S.

    1989-01-01

    We examined the uptake and release of norepinephrine in the cardiac circulation and other regional vascular beds in 11 patients with hypertrophic cardiomyopathy (HCM) and in 10 control subjects during simultaneous infusion of tracer-labeled norepinephrine and isoproterenol. Cardiac neuronal uptake of norepinephrine was assessed by comparing regional removal of tracer-labeled norepinephrine with that of tracer-labeled isoproterenol (which is not a substrate for neuronal uptake) and by the relation between production of dihydroxyphenylglycol (DHPG), an exclusively intraneuronal metabolite of norepinephrine, and regional spillover of norepinephrine. Cardiac extraction of norepinephrine averaged 59 +/- 17% in the patients with HCM, significantly less than in the control subjects (79 +/- 13%, p less than 0.05), whereas cardiac extraction of isoproterenol was similar in the two groups (13 +/- 23% versus 13 +/- 14%), indicating that neuronal uptake of norepinephrine was decreased in the patients with HCM. The cardiac arteriovenous difference in norepinephrine was significantly larger in the patients with HCM than in the control subjects (73 +/- 77 versus 13 +/- 50 pg/ml, p less than 0.05), as was the product of the arteriovenous difference in norepinephrine and coronary blood flow (7.3 +/- 7.3 versus 0.8 +/- 3.0 ng/min, p less than 0.05)

  1. Syncope in children with hypertrophic cardiomyopathy

    Directory of Open Access Journals (Sweden)

    I. V. Leontyeva

    2014-01-01

    Full Text Available Seventy children aged 7 to 17 years with hypertrophic cardiomyopathy (HCM were examined; among them there were 11 syncope patients and 5 presyncope patients. The screening program included standard electrocardiography (ECG, Doppler echocardiogra-phy, 24-hour Holter ECG monitoring, and an incremental exercise testing (Bruce treadmill test. The markers of myocardial electrical instability were determined. In the children with HCM, syncope was established to be heterogeneous; it had an arrhythmogenic origin and, in most cases, occurred in the presence of tachyarrhythmia (44% or bradyarrythmia (25%; its vasovagal genesis was probable in one third of the examinees. The children with syncope were typified by the asymmetric, obstructive form of HCM, at the same tone there was most commonly left ventricular hypertrophy concurrent with left atrial enlargement. 24-hour Holter monitoring showed that bradycardia was prevalent in the patients, 3 patients were found to have more than 2-second cardiac rhythm pauses caused by second-degree atrioventricular block in 1 case or by sick sinus syndrome in 2. Nonsustained ventricular tachycardia was noted in two patients. The children with syncope were typified by the signs of myocardial electrical instability as a reduction in the early phase of heart rate turbulence and by impaired QT/RR interval adaptation as hyperadaptation. The paper presents the developed management tactics for children with syncope and indications for the implantation of a cardioverter defibrillator, a pacemaker, or an ECG loop recorder.

  2. MR imaging findings of hypertrophic olivary degeneration

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Do Joong; Jeon, Pyung; Kim, Dong Ik [Yonsei Univ. College of Medicine, Seoul (Korea, Republic of)

    1997-06-01

    To describe the magnetic resonance (MR) imaging findings of hypertrophic olivary degeneration (HOD) MR images of seven patients with HOD were retrospectively reviewed. Two were women and five were men, and they were aged between 48 and 65 (mean 58) years. Imaging examinations were performed with a 1.5-T unit, and the findings were used to evaluate the size and signal intensity of olivary lesions. The time interval from hemorrhagic ictus to MR imaging was between two and 30 months. Follow-up examinations were performed in two patients. All four patients with hemorrhages involving the central tegmental tract in the pons or midbrain showed ipsilateral HOD. Among these four, bilateral HOD was seen in one patient with hemorrhage involving the bilateral central tegmental tract, and in another with tegmental hemorrhage extending to the ipsilateral superior cerebellar peduncle. One patient with cerebellar hemorrhage involving the dentate nucleus had contralateral HOD. Two patients with multiple hemorrhages involving both the pons and cerebellum showed bilateral HOD. Axial MR images showed mild enlargement of the involved olivary mucleus, with high signal intensity on both proton density and T2 weighted images. There was no apparent enhancement on postcontrast T1-weighted images. MR imaging can clearly distinguish secondary olivary degeneration from underlying pathology involving the central tegmental tract in the pons or midbrain and cerebellum. These olivary abnormalities should not, however, be mistaken for primary medullary lesions.

  3. MR imaging findings of hypertrophic olivary degeneration

    International Nuclear Information System (INIS)

    Kim, Do Joong; Jeon, Pyung; Kim, Dong Ik

    1997-01-01

    To describe the magnetic resonance (MR) imaging findings of hypertrophic olivary degeneration (HOD) MR images of seven patients with HOD were retrospectively reviewed. Two were women and five were men, and they were aged between 48 and 65 (mean 58) years. Imaging examinations were performed with a 1.5-T unit, and the findings were used to evaluate the size and signal intensity of olivary lesions. The time interval from hemorrhagic ictus to MR imaging was between two and 30 months. Follow-up examinations were performed in two patients. All four patients with hemorrhages involving the central tegmental tract in the pons or midbrain showed ipsilateral HOD. Among these four, bilateral HOD was seen in one patient with hemorrhage involving the bilateral central tegmental tract, and in another with tegmental hemorrhage extending to the ipsilateral superior cerebellar peduncle. One patient with cerebellar hemorrhage involving the dentate nucleus had contralateral HOD. Two patients with multiple hemorrhages involving both the pons and cerebellum showed bilateral HOD. Axial MR images showed mild enlargement of the involved olivary mucleus, with high signal intensity on both proton density and T2 weighted images. There was no apparent enhancement on postcontrast T1-weighted images. MR imaging can clearly distinguish secondary olivary degeneration from underlying pathology involving the central tegmental tract in the pons or midbrain and cerebellum. These olivary abnormalities should not, however, be mistaken for primary medullary lesions

  4. Medicinal Plants for the Treatment of Hypertrophic Scars

    Directory of Open Access Journals (Sweden)

    Qi Ye

    2015-01-01

    Full Text Available Hypertrophic scar is a complication of wound healing and has a high recurrence rate which can lead to significant abnormity in aesthetics and functions. To date, no ideal treatment method has been established. Meanwhile, the underlying mechanism of hypertrophic scarring has not been clearly defined. Although a large amount of scientific research has been reported on the use of medicinal plants as a natural source of treatment for hypertrophic scarring, it is currently scattered across a wide range of publications. Therefore, a systematic summary and knowledge for future prospects are necessary to facilitate further medicinal plant research for their potential use as antihypertrophic scar agents. A bibliographic investigation was accomplished by focusing on medicinal plants which have been scientifically tested in vitro and/or in vivo and proved as potential agents for the treatment of hypertrophic scars. Although the chemical components and mechanisms of action of medicinal plants with antihypertrophic scarring potential have been investigated, many others remain unknown. More investigations and clinical trials are necessary to make use of these medical plants reasonably and phytotherapy is a promising therapeutic approach against hypertrophic scars.

  5. Characteristics of hypertrophic cardiomyopathy on delayed contrast-enhanced MRI

    International Nuclear Information System (INIS)

    Yan Chaowu; Zhao Shihua; Li Hua; Jiang Shiliang; Lu Minjie; Zhang Yan; Wei Yunqing; Ling Jian; Fang Wei

    2010-01-01

    Objective: To analyze the characteristics of hypertrophic cardiomyopathy (HCM) on delayed contrast-enhanced cardiac magnetic resonance imaging (CMRI). Methods: All patients underwent delayed contrast-enhanced CMRI. The left ventricle was divided into 9 segments to assess the location, extent and function of the hypertrophic segments. The t test was applied for the statistics. Results: Of 154 patients, delayed enhancement of' hypertrophic segment was found in 95 cases and non-delayed enhancement in 59 cases. The thickness and number of hypertrophic segment in patients with delayed enhancement were larger than those with non-delayed enhancement [(24.8±5.5) mm vs (20.4± 3.8) mm, t=3.82, P<0.05; (3.3±1.9) vs (2.4±1.7), t=2.26, P<0.05], and the age was younger [(46.0±15.2) years vs (55.0±11.9) years, t=-3.67, P<0.05]. The diffuse enhancement was found in 62 patients, and confluent enhancement in 33 patients. Confluent enhancement was found in all 14 patients after the alcohol ablation procedure. Conclusion: The age, thickness and number of hypertrophic segments in patients with delayed enhancement are different from those with non-delayed enhancement. (authors)

  6. Keratinocyte-derived growth factors play a role in the formation of hypertrophic scars

    NARCIS (Netherlands)

    Niessen, FB; Andriessen, MP; Schalkwijk, J; Visser, L; Timens, W

    In predisposed individuals, wound healing can lead to hypertrophic scar or keloid formation, characterized by an overabundant extracellular matrix. It has recently been shown that hypertrophic scars are accompanied by abnormal keratinocyte differentiation and proliferation, and significantly

  7. Metastases of Hepatocellular Carcinoma Misdiagnosed as Isolated Hypertrophic Cardiomyopathy.

    Science.gov (United States)

    Greco, Assunta; De Masi, Roberto; Orlando, Stefania; Metrangolo, Antonio; Zecca, Vittorio; Morciano, Giancarlo; De Donno, Antonella; Bagordo, Francesco; Piccinni, Giancarlo

    At present, cardiac metastasis of hepatocellular carcinoma is rarely mentioned in the literature. We report a hepatocellular carcinoma patient with cardiac metastasis misdiagnosed as hypertrophic cardiomyopathy in 2011. Two years later, on presentation of syncope, an abnormal ventricular septal size was recorded by ultrasound scan, and was subsequently shown by magnetic resonance imaging to be a tumour lesion. A myocardial biopsy confirmed infiltration of hepatocellular carcinoma. This observation underlines the risk of hepatocellular carcinoma cardiac metastasis, manifested in its infiltrative form as hypertrophic cardiomyopathy. In conclusion, we suggest that the ultrasound appearance of hypertrophic cardiomyopathy in hepatocellular carcinoma patients should be seen as a "red flag" and recommend the introduction of magnetic resonance imaging assessment of transplant candidates.

  8. Spontaneous coronary artery dissection associated with apical hypertrophic cardiomyopathy

    International Nuclear Information System (INIS)

    Tuncer, M.; Gumrukcuoglu, H.A.; Ekim, H.; Gunes, Y.; Simsek, H.

    2010-01-01

    Apical hypertrophic cardiomyopathy (HCM) is a relatively uncommon inherited disease. Spontaneous coronary artery dissection (SCAD) is also uncommonly observed, which often occurs in pregnant or post partum women but is rare in men. This report describes a 38 years old man with apical hypertrophic cardiomyopathy who developed SCAD leading to acute inferior myocardial infarction. After emergent appendectomy operation at another hospital, he was immediately transferred to the Cardiology Department of our hospital due to acute myocardial infarction. He emergently underwent coronary angiography which showed a long dissection involving the right coronary. He underwent an emergent CABG with cardiopulmonary bypass. Postoperative recovery was uneventful and he was discharged. According to our knowledge, no case of spontaneous coronary artery dissection associated with apical hypertrophic cardiomyopathy unrelated to postpartum period or oral contraceptive use has been reported so far. (author)

  9. Use of cisplatin for control of metastatic malignant mesenchymoma and hypertrophic osteopathy in a dog

    International Nuclear Information System (INIS)

    Hahn, K.A.; Richardson, R.C.

    1989-01-01

    Cisplatin (cis-diammine-dichloroplatinum) treatment induced partial remission of pulmonary metastatic malignant mesenchymoma and nearly complete radiographic remission of hypertrophic osteopathy in a 14-year-old Beagle. Cisplatin was given once every 3 weeks. Clinical signs of hypertrophic osteopathy resolved one week after initiation of treatment. Partial remission of pulmonary metastases and partial radiographic remission of hypertrophic osteopathy was seen 6 weeks after initiation of treatment. Previous treatment of neoplasia-related hypertrophic osteopathy has consisted of removal of the initiating mass or vagotomy. In this case, appropriate chemotherapy was used to control clinical signs and progression of hypertrophic osteopathy

  10. Magnetic resonance imaging in familial hypertrophic cardiomyopathy associated with abnormal thallium perfusion and cardiac enzymes

    Energy Technology Data Exchange (ETDEWEB)

    Nishimura, Tsunehiko; Nagata, Seiki; Sakakibara, Hiroshi

    1988-05-01

    Gated magnetic resonance imaging (MRI) was performed in 6 patients with familial hypertrophic cardiomyopathy associated with abnormal thallium perfusion, and 12 patients with ordinary hypertrophic cardiomyopathy. The patients with ordinary hypertrophic cardiomyopathy and abnormal thickening of the septal wall and normal left ventricular dimensions, while the patients with familial hypertrophic cardiomyopathy had focal wall thinning (usually involving the apical-septal wall) and dilated left ventricle in addition to hypertrophied heart. The quantitative measurement for cardiac dimensions using MRI was similar to that found on echocardiography in all cases. In addition, inhomogeneous signal intensities at left ventricular wall were observed in 3 cases of familial hypertrophic cardiomyopathy, which may suggest the existence of myocardial fibrosis. Gated MRI should be performed for early detection and follow-up of hypertrophic cardiomyopathy, since some patients will progress from hypertrophic cardiomyopathy to dilated cardiomyopathy.

  11. Orchidectomy Ameliorates the Vascular Hypertrophic Effect of A ...

    African Journals Online (AJOL)

    Dr Olaleye Samuel

    testosterone replacement (TR) (10mg/kg sustanon 250® i.m) once in 3 weeks. They were either placed ... hypertrophic effect of a HSD by reducing vascular smooth muscle proliferation and ..... NADPH oxidase plays a role in beta-tissue growth.

  12. Danon’s disease as a cause of hypertrophic cardiomyopathy

    Directory of Open Access Journals (Sweden)

    I. V. Leontyeva

    2015-01-01

    Full Text Available Hypertrophic cardiomyopathy is the most common inherited disease of the myocardium. The causes of the disease are heterogeneous; its primary form results from mutations in the genes encoding cardiac sarcomeric proteins; its secondary (metabolic and syndromic forms develop due to mutations in the genes encoding non-sarcomeric proteins. Glycogenosis is the most common cause of the metabolic ones of hypertrophic cardiomyopathy. Danon’s disease (lysosome-associated membrane protein 2 (LAMP2-cardiomyopathy is a form of glycogenosis and it is characterized by a typical triad: hypertrophic cardiomyopathy, mental retardation, and skeletal myopathy. The disease occurs with mutations in the LAMP2 gene; X-linked dominant inheritance. LAMP2-cardiomyopathy does not virtually differ in its clinical manifestations from the severe form of hypertrophic cardiomyopathy, which results from mutations in the sarcomeric protein genes. The disease is characterized by a poor progressive course with the high probability of causing sudden death or with the progression of severe heart failure. Implantation of a cardioverter defibrillator is a main method to prevent sudden cardiac death. 

  13. Penetrance of Hypertrophic Cardiomyopathy in Children Who Are Mutation Positive

    NARCIS (Netherlands)

    Vermeer, Alexa M. C.; Clur, Sally-Ann B.; Blom, Nico A.; Wilde, Arthur A. M.; Christiaans, Imke

    2017-01-01

    Objectives To investigate the presence of hypertrophic cardiomyopathy (HCM) at first cardiac evaluation and during follow-up and cardiac events in predictively tested children who are mutation positive. Study design The study included 119 predictively tested children who were mutation positive, with

  14. Hypertrophic Pyloric stenosis: Pre- and post-operative sonographic findings

    International Nuclear Information System (INIS)

    Park, Joung Suk; Han, Douk Sub; Oh, Jong Sub; Kim, Min Jung; Gi, Joo Yun; Park, Byung Ran; Kim, Se Jong; Koh, Kang Suk; Kim, Byung Kun

    1993-01-01

    The authors retrospectively analysed the ultrasonographic findings of 43 cases of surgically confirmed hypertrophic pyloric stenosis and their postoperative findings of sonograms taken at 1 month(n=40) or 3 month(n=5) after pyloromyotomy. In preoperative study, the thickened pyloric muscle was isoechoic or slight hypoechoic relative to liver on the midline longitudinal view and appeared as a 'nonuniform acoustic ring' on the transverse view. The results of measurement in the all cases with hypertrophic pyloric stenosis were the pyloric muscle thickness ≥ 3.8 mm, the pyloric diameter ≥ 14 mm, the pyloric channel length ≥ 16 mm, the pyloric muscle volume ≥ 2.21 cm 3 and the pyloric muscle index ≥ 0.57, respectively. It usually required 1 month after operation for the hypertrophied muscle to resolve in 36 of 41 patients. The normalized pyloric muscle appeared more hypoechoic than that of adjacent parenchyma, and the nonuniform echogenicity of the pyloric muscle disappeared. Five patients whose hypertrophied muscle did not return to normal range on 1 month's follow-up sonogram were follow-up 3 months later again, and we have ascertained their resolution in all of them. In conclusion, the pre-operative sonographic findings of infantile hypertrophic pyloric stenosis were different quantitatively and qualitatively from of those of post-operative follow-up ultrasound exam. High-resolution real time sonography is a safe and accurate method for the diagnosis of hypertrophic pyloric stenosis and useful in postpyloromyotomy follow-up

  15. Hypertrophic osteopathy in a pony with a pituitary adenoma

    International Nuclear Information System (INIS)

    Sweeney, C.R.; Stebbins, K.E.; Schelling, C.G.; Beech, J.; Schilling, D.A.

    1989-01-01

    Hypertrophic osteopathy was diagnosed in a pony that had no antemortem or postmortem evidence of an intrathoracic lesion. With a history of hirsutism in an aged pony, a pituitary adenoma was suspected, and evaluation of plasma cortisol and insulin values and their response to thyrotropin-releasing hormone supported the diagnosis

  16. A case of hypertrophic osteoarthropathy in a Belgian blue cow

    OpenAIRE

    Guyot, Hugues; Sandersen, Charlotte; Rollin, Frédéric

    2011-01-01

    A 12-year-old cow was presented with chronic respiratory disease and lameness. Chronic pleuritis, pneumonia, and bronchial carcinoma were found as well as periosteal proliferation on limb bones. Ancillary tests and necropsy confirmed a combined pathology of pulmonary inflammation and neoplasm, and hypertrophic pulmonary osteopathy.

  17. Hypertrophic cardiomyopathy in infants: clinical features and natural history

    International Nuclear Information System (INIS)

    Maron, B.J.; Tajik, A.J.; Ruttenberg, H.D.; Graham, T.P.; Atwood, G.F.; Victorica, B.E.; Lie, J.T.; Roberts, W.C.

    1982-01-01

    The clinical and morphologic features of hypertrophic cardiomyopathy in 20 patients recognized as having cardiac disease in the first year of life are described. Fourteen of these 20 infants were initially suspected of having heart disease solely because a heart murmur was identified. However, the infants showed a variety of clinical findings, including signs of marked congestive heart failure (in the presence of nondilated ventricular cavities and normal or increased left ventricular contractility) and substantial cardiac enlargement on chest radiograph. Other findings were markedly different from those usually present in older children and adults with hypertrophic cardiomyopathy (e.g., right ventricular hypertrophy on the ECG and cyanosis). Consequently, in 14 infants, the initial clinical diagnosis was congenital cardiac malformation other than hypertrophic cardiomyopathy. The clinical course was variable in these patients, but the onset of marked congestive heart failure in the first year of life appeared to be an unfavorable prognostic sign; nine of the 11 infants with congestive heart failure died within the first year of life. In infants with hypertrophic cardiomyopathy, unlike older children and adults with this condition, sudden death was less common (two patients) than death due to progressive congestive heart failure

  18. The emerging role of antineoplastic agents in the treatment of keloids and hypertrophic scars: a review.

    Science.gov (United States)

    Shridharani, Sachin M; Magarakis, Michael; Manson, Paul N; Singh, Navin K; Basdag, Basak; Rosson, Gedge D

    2010-03-01

    The management of keloids and hypertrophic scars continues to challenge health-care providers. Though both forms of pathologic scarring are distinct entities at the macro and microscopic level, their etiologies and treatment are often similar. Potential treatment approaches are progressing, and combinations of treatment options have been proposed in the literature with promising outcomes. The treatment evolution has reached a level where molecular therapeutic modalities are being investigated. Currently, no gold standard treatment exists. Overall success rates and patient satisfaction seem to be slowly climbing, but additional investigational studies must continue to be performed. Several studies have investigated antineoplastic agents, and there seems to be a marked improvement in rates of recurrence, patient satisfaction, and overall quality of scar when these agents are used. Intralesional injection and/or wound irrigation with interferon-a2b, interferon-g, mitomycin-C, bleomycin, or 5-fluorouracil seems to have a positive effect on the reduction of pathologic scars. There is mounting evidence that these drugs used alone or in combination therapy, have the potential to be an integral part of the treatment paradigm for hypertrophic scars and keloids.

  19. Echocardiography Differences Between Athlete's Heart Hearth and Hypertrophic Cardiomyopathy.

    Science.gov (United States)

    Kreso, Amir; Barakovic, Fahir; Medjedovic, Senad; Halilbasic, Amila; Klepic, Muhamed

    2015-10-01

    Among long term athletes there is always present hypertrophy of the left ventricle walls as well as increased cardiac mass. These changes are the result of the heart muscle adaptation to load during the years of training, which should not be considered as pathology. In people suffering from hypertrophic cardiomyopathy (HCM), there is also present hypertrophy of the left ventricle walls and increased mass of the heart, but these changes are the result of pathological changes in the heart caused by a genetic predisposition for the development HCM of. Differences between myocardial hypertrophy in athletes and HCM are not clearly differentiated and there are always dilemmas between pathological and physiological hypertrophy. The goal of the study is to determine and compare the echocardiographic cardiac parameters of longtime athletes to patients with hypertrophic cardiomyopathy. The study included 60 subjects divided into two groups: active athletes and people with hypertrophic cardiomyopathy. Mean values of IVSd recorded in GB is IVSd=17.5 mm (n=20, 95% CI, 16.00-19.00 mm), while a significantly smaller mean value is recorded in GA, IVSd=10.0 mm (n=40, 95% CI, 9.00-11.00 mm). The mean value of the left ventricle in diastole (LVDd) recorded in the GA is LVDd=51 mm (n=40; 95% CI, 48.00 to 52.00 mm), while in the group with hypertrophic cardiomyopathy (GB) mean LVDd value is 42 mm (n=20; 95% CI, 40.00 to 48.00 mm). The mean value of the rear wall of the left ventricle (LVPWd) recorded in the GA is LVDd=10 mm (n=40; 95% CI, 9.00-10.00 mm) while in the group with hypertrophic cardiomyopathy (GB) mean LVDd is 14 mm (n=20; 95% CI, 12.00 to 16.00 mm). The mean of the left ventricle during systole (LVSD) observed in GA is LVSD=34 mm (n=40; 95% CI, 32.00 to 36.00 mm), while in the group with hypertrophic cardiomyopathy (GB) mean LVSD is 28 mm (n=20; 95% CI, 24.00 to 28.83 mm). The mean ejection fraction (EF%) observed in GA is EF=60% (n=40; 95% CI, 56.41 to 63.00%), while in

  20. Abnormalities in the basement membrane structure promote basal keratinocytes in the epidermis of hypertrophic scars to adopt a proliferative phenotype.

    Science.gov (United States)

    Yang, Shaowei; Sun, Yexiao; Geng, Zhijun; Ma, Kui; Sun, Xiaoyan; Fu, Xiaobing

    2016-05-01

    The majority of studies on scar formation have mainly focused on the dermis and little is known of the involvement of the epidermis. Previous research has demonstrated that the scar tissue-derived keratinocytes are different from normal cells at both the genetic and cell biological levels; however, the mechanisms responsible for the fundamental abnormalities in keratinocytes during scar development remain elusive. For this purpose, in this study, we used normal, wound edge and hypertrophic scar tissue to examine the morphological changes which occur during epidermal regeneration as part of the wound healing process and found that the histological structure of hypertrophic scar tissues differed from that of normal skin, with a significant increase in epidermal thickness. Notably, staining of the basement membrane (BM) appeared to be absent in the scar tissues. Moreover, immunofluorescence staining for cytokeratin (CK)10, CK14, CK5, CK19 and integrin-β1 indicated the differential expression of cell markers in the epidermal keratinocytes among the normal, wound edge and hypertrophic scar tissues, which corresponded with the altered BM structures. By using a panel of proteins associated with BM components, we validated our hypothesis that the BM plays a significant role in regulating the cell fate decision of epidermal keratinocytes during skin wound healing. Alterations in the structure of the BM promote basal keratinocytes to adopt a proliferative phenotype both in vivo and in vitro.

  1. Hypertrophic Obstructive Cardiomyopathy Masked by Tako-Tsubo Syndrome: A Case Report

    Directory of Open Access Journals (Sweden)

    Y. Daralammori

    2012-01-01

    Full Text Available Introduction. Left ventricular outflow obstruction might be part of the pathophysiological mechanism of Tako-tsubo cardiomyopathy. This obstruction can be masked by Tako-tsubo cardiomyopathy and diagnosed only by followup. Case Presentation. A 70-year-old female presented with Tako-tsubo cardiomyopathy and masked obstructive hypertrophic cardiomyopathy at presentation. Conclusion. Tako-tsubo cardiomyopathy typically presents like an acute MI and is characterized by severe, but transient, regional left ventricular systolic dysfunction. Prompt evaluation of the coronary status is, therefore, mandatory. The prognosis under medical treatment of heart failure symptoms and watchful waiting is favourable. Previous studies showed that LVOT obstruction might be part of the pathophysiological mechanism of TCM. This paper supports this theory. However, TCM may also mask any preexisting LVOT obstruction.

  2. Plant Wax n-Alkane and n-Alkanoic Acid Signatures Overprinted by Microbial Contributions and Old Carbon in Meromictic Lake Sediments

    Science.gov (United States)

    Makou, Matthew; Eglinton, Timothy; McIntyre, Cameron; Montluçon, Daniel; Antheaume, Ingrid; Grossi, Vincent

    2018-01-01

    Specific n-alkanes and n-alkanoic acids are commonly used as biomarkers in paleoenvironmental reconstruction, yet any individual homologue may originate from multiple biological sources. Here we improve source and age controls for these compounds in meromictic systems by measuring the radiocarbon (14C) ages of specific homologues preserved in twentieth century Lake Pavin (France) sediments. In contrast to many studies, 14C ages generally decreased with increasing carbon chain length, from 7.3 to 2.6 ka for the C14-C30 n-alkanoic acids and from 9.2 to 0.3 ka for the C21-C33 n-alkanes. Given a known hard water effect, these values suggest that aquatic microbial sources predominate and contributed to most of the homologues measured. Only the longest chain n-alkanes exclusively represent inputs of higher plant waxes, which were previously sequestered in soils over centennial to millennial timescales prior to transport and deposition. These findings suggest that biomarker source and age should be carefully established for lacustrine settings.

  3. Prosthodontist contribution in treating post-burn hypertrophic facial scars

    Directory of Open Access Journals (Sweden)

    Padmanabhan T

    2010-01-01

    Full Text Available The formation of hypertrophic scars is common following healing of the burn wound, particularly in children. The face is one of the areas of the body most frequently affected by burns. Scar formation as a result of burn wounds leads to contraction of the formed granulation tissue, which causes both aesthetic and functional impairment for the patient. Scarring has major psychological and physical repercussions. Scarring on the face and visible regions of the body can be very distressing for the patient. Prevention of scars involves early and continuous use of a compressive orthesis. However, their efficacy is often limited to the facial region because of the contours of this area of body. This paper describes a clinical case of post-burn hypertrophic scars treated with silicone gel sheeting applied with pressure under custom made auto-polymerizing resin stent.

  4. Myocardial ischemia in hypertrophic cardiomyopathy; Isquemia miocardica na cardiomiopatia hipertrofica

    Energy Technology Data Exchange (ETDEWEB)

    Lima Filho, Moyses de Oliveira; Figueiredo, Geraldo L.; Simoes, Marcus V.; Pyntia, Antonio O.; Marin Neto, Jose Antonio [Sao Paulo Univ., Ribeirao Preto, SP (Brazil). Faculdade de Medicina. Div. de Cardiologia

    2000-08-01

    Myocardial ischemia in hypertrophic cardiomyopathy is multifactorial and explains the occurrence of angina, in about 50% of patients. The pathophysiology of myocardial ischemia may be explained by the increase of the ventricular mass and relative paucity of the coronary microcirculation; the elevated ventricular filling pressures and myocardial stiffness causing a compression of the coronary microvessels; the impaired coronary vasodilator flow reserve caused by anatomic and functional abnormalities; and the systolic compression of epicardial vessel (myocardial bridges). Myocardial ischemia must be investigated by perfusion scintigraphic methods since its presence influences the prognosis and has relevant clinical implications for management of patients. Patients with hypertrophic cardiomyopathy and documented myocardial ischemia usually need to undergo invasive coronary angiography to exclude the presence of concomitant atherosclerotic coronary disease. (author)

  5. Evaluation of left cardiac function by exercise in hypertrophic cardiomyopathy

    International Nuclear Information System (INIS)

    Konishi, Tokuji; Horayama, Norihisa; Hamada, Masayuki; Nakano, Takeshi; Takezawa, Hideo

    1981-01-01

    Left ventricular systolic and diastolic features at rest and exercise in hypertrophic cardiomyopathy were evaluated by Fourier analysis of blood pool scintigraphy (intracorporeal labelling with sup(99m)Tc-RBC). In the normal group (17 subjects), the left ventricular ejection fraction showed a linear increase, but no abnormality of regional ventricular wall motion, by multistage exercises. The hypertrophic cardiomyopathy group showed higher left ventricular ejection fractions at rest than those of the normal group, and in the HCM group (non-obstructive, from morphological features; 7 cases) the left ventricular ejection fraction did not increase any more when it reached a certain plateau in accordance with increased stress. In the HOCM (obstructive; 5 cases), the left ventricular ejection fraction showed a decreasing tendency as the stress was increased and also showed contractile abnormalities from the left ventricular center to the apex. Fourier analysis was effective for the evaluation of these changes. (Chiba, N.)

  6. Clinical Features and Echocardiographic Findings in Children with Hypertrophic Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Cristina Blesneac

    2013-12-01

    Full Text Available Background: Hypertrophic cardiomyopathy, one of the most common inherited cardiomyopathies, is a heterogeneous disease resulting from sarcomeric protein mutations, with an incidence in the adult population of 1:500. Current information on the epidemiology and outcomes of this disease in children is limited. Methods: Thirty-four children diagnosed with hypertrophic cardiomyopathy in the Pediatric Cardiology Department from Tîrgu Mureș were evaluated concerning familial and personal history, clinical, paraclinical and therapeutic aspects. Hypertrophic cardiomyopathy was defined by the presence of a hypertrophied, non-dilated ventricle, in the absence of a cardiac or systemic disease that could produce ventricular hypertrophy. Results: The youngest diagnosed child was a neonate, a total of 10 patients being diagnosed until 1 year of age. In 6 cases a positive familial history was found. Noonan syndrome was found in 2 cases. Only 21 patients were symptomatic, the predominant symptoms being shortness of breath on exertion with exercise limitations. Left ventricular outflow tract obstruction was present in 21 cases (61.7%. Twenty-four patients were on β-blocking therapy, while 4 patients underwent septal myectomy. Conclusions: Hypertrophic cardiomyopathy is a heterogeneous disorder in terms of evolution, age of onset, type and extent of hypertrophy, and the risk of sudden death. It can affect children of any age. There is a need for a complex evaluation, including familial and personal anamnesis, clinical examination, electrocardiogram and echocardiography of all patients. It is highly important to develop screening strategies, including genetic testing, for an early diagnosis, especially in asymptomatic patients with a positive familial background

  7. Cardiovascular magnetic resonance in hypertrophic cardiomyopathy and infiltrative cardiomyopathy

    OpenAIRE

    Schofield, Rebecca; Manacho, Katia; Castelletti, Silvia; Moon, James C.

    2016-01-01

    Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiac disease. Cardiac imaging plays a key role in the diagnosis and management, with cardiovascular magnetic resonance (CMR) an important modality. CMR provides a number of different techniques in one examination: structure and function, flow imaging and tissue characterisation particularly with the late gadolinium enhancement (LGE) technique. Other techniques include vasodilator perfusion, mapping (especially T1 mapping and ex...

  8. Hypertrophic neuropathy in Noonan syndrome with multiple lentigines.

    Science.gov (United States)

    Maridet, Claire; Sole, Guilhem; Morice-Picard, Fanny; Taieb, Alain

    2016-06-01

    RASopathies comprise several genetic syndromes with mainly cardio-facial-cutaneous manifestations. We report a patient with Noonan syndrome with multiple lentigines (NSML) due to a PTPN11 (p.Thr468Met) mutation associated with hypertrophic neuropathy of lumbar plexus in an adult woman, initially referred for neuropathic pain. Differential diagnosis of neurofibromatosis type 1 (NF1) and other RASopathies is difficult without molecular testing. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

  9. Hypertrophic anterior cervical osteophytes causing dysphagia and airway obstruction.

    Science.gov (United States)

    Lin, Harrison W; Quesnel, Alicia M; Holman, Allison S; Curry, William T; Rho, Michael B

    2009-10-01

    Hyperostosis of anterior cervical vertebral osteophytes can produce otolaryngological symptoms ranging from mild dysphagia, dysphonia, and foreign body sensation to severe food impaction and stridulous dyspnea. Airway compromise necessitating a tracheostomy is very rare. We discuss the case of an elderly man who presented with progressive dysphagia and a large hypopharyngeal mass as his initial manifestations of hypertrophic anterior cervical osteophytes. After a biopsy of the mass, the patient went into airway distress due to bilateral vocal fold fixation by the enlarging mass and consequently required a surgical airway. A combined team approach to the removal of the osteophytes successfully resolved his symptoms. The clinical, diagnostic, radiologic, and therapeutic principles involved in this case are presented and discussed. The recognition of hypertrophic osteophytes as a potential cause of common otolaryngological symptoms in the elderly population is paramount, as these symptoms can rapidly progress and lead to life-threatening airway obstruction. Medical and surgical interventions can be employed for the treatment of hypertrophic anterior cervical osteophytes, and they often result in favorable outcomes.

  10. Clinical studies on myocardial damage in hypertrophic cardiomyopathy using exercise testing with thallium-201 myocardial scintigraphy

    International Nuclear Information System (INIS)

    Igarashi, Yutaka

    1986-01-01

    Twenty-eight patients with hypertrophic cardiomyopathy (HCM) associated with normal coronary angiogram were categorized into four groups according to the distribution of disproportional hypertrophy in the left ventriculography and biventriculography. The circumferential profiles based on the scintigram were analyzed with reference to the relationship between washout rate and disproportional hypertrophy. As compared with the control group, the mean values of initial uptake in the four HCM-groups decreased in the apical segments and the lower part of anteroseptal segments which were reflected disproportional hypertrophy. The mean values increased significantly in the upper part of anteroseptal segments which projected the largest amount of myocardium three-dimensionally. The mean values of washout rate in the four HCM-groups decreased significantly in all segments, especially in the segments which were reflected in disproportional hypertrophy, and also decreased equally in the segments with the increased initial uptake. In comparison between the segmental values in each HCM-patient and those in the control group, the initial uptake and/or washout rate decreased below the mean value-2SD of the control group in the profiles of 17 out of 28 HCM-patients. The segments with decreased washout rate and normal limit of initial uptake were observed in 12 out of 17 patients. In 25 segments out of the total segments in HCM-patients, an initial uptake and/or washout rate fell down below the normal limit. The washout rate decreased, the initial uptake remained normal in 21 out of 25 segments, and 16 out of 21 segments were reflected in disproportionally hypertrophic wall. In the posterolateral segment which is the thinnest wall in the left ventricular wall of HCM, the mean value of washout rate significantly decreased in 20 patients with normal limit of initial uptake, as compared with the control group. (J.P.N.)

  11. Tindallia Californiensis sp. nov.: A New Halo-Alkaliphilic Primary Anaerobe, Isolated from Meromictic soda Mono Lake in California and the Correction of Diagnosis for Genus Tindallia

    Science.gov (United States)

    Pikuta, Elena; Marsic, Damien; Hoover, Richard B.; Kevbrin, Vadim; Whitman, William B.; Krader, Paul; Cleland, Dave; Six, N. Frank (Technical Monitor)

    2002-01-01

    A novel extremely halo-alkaliphilic, bacterium strain APO (sup T) was isolated from sediments of the athalassic, meromictic, soda Mono Lake in California. Gram positive, spore-forming, slightly curved rods with sizes 0.6-0.7x 2.5-4.0 micrometers which occur singly, in pairs or short curved chains. Cells, are motile by singular subcentral flagellum. Strain APO (sup T) is mesophilic: growth was observed over the temperature range of +10 C to +48 C (optimum +37 C), NaCl concentration range 1-20 %, wt/vol (optimum 3-5%, wt/vol) and pH range 8.0-11.0 (optimum pH 9.5). The novel isolate is strictly halo-alkaliphilic, requires sodium chloride in medium, obligately anaerobic and catalase-negative. Strain APO (sup T) is organo-heterotroph with fermentative type of metabolism, and uses as substrates: peptone, badotryptone, casamino acids, yeast extract, L-serine, L-lysine, L-histidine, L-arginine, and pyruvate. The main end products of growth on peptone medium were: lactate, acetate, propionate, and ethanol. Strain APO (sup T) is resistant to kanamycin, but sensitive to chloramphenicol, tetracycline, and gentamycin. The sum of G+C in DNA is 44.4 mol% (by HPLC method). On the bait of physiological and molecular properties, the isolate was considered as novel species of genus Tindallia; and the name Tindallia californiensis sp. nov., is proposed for new isolate (type strain APO (sup T) - ATCC BAA_393(sup T) = DSMZ 14871 (sup T)).

  12. A prospective study of time to healing and hypertrophic scarring in paediatric burns: every day counts.

    Science.gov (United States)

    Chipp, Elizabeth; Charles, Lisa; Thomas, Clare; Whiting, Kate; Moiemen, Naiem; Wilson, Yvonne

    2017-01-01

    It is commonly accepted that burns taking longer than 3 weeks to heal have a much higher rate of hypertrophic scarring than those which heal more quickly. However, some of our patients develop hypertrophic scars despite healing within this 3-week period. We performed a prospective study of 383 paediatric burns treated non-operatively at a regional burns centre over a 2-year period from May 2011 to April 2013. Scar assessment was performed by a senior burns therapist using the Vancouver Scar Scale. Overall rates of hypertrophic scarring were 17.2%. Time to healing was the strongest predictor of developing hypertrophic scarring, and the earliest hypertrophic scar developed in a patient who was healed after 8 days. The risk of hypertrophic scarring was multiplied by 1.138 for every additional day taken for the burn wound to heal. There was a trend towards higher rates of hypertrophic scarring in non-white skin types but this did not reach statistical significance. The risk of hypertrophic scarring increases with every day and, therefore, every effort should be made to get the wound healed as quickly as possible, even within the traditional 3-week period usually allowed for healing. We believe that the traditional dogma of aiming for healing within 3 weeks is overly simplistic and should be abandoned: in paediatric burns, every day counts. Not applicable.

  13. Data on exercise and cardiac imaging in a patient cohort with hypertrophic cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Lars A. Dejgaard

    2017-12-01

    Full Text Available Data presented in this paper are supplementary material to our study “Vigorous exercise in patients with hypertrophic cardiomyopathy” [1]. The current article presents supplementary data on collection and analyses of exercise parameters and genetic data in the original research article. Keywords: Hypertrophic cardiomyopathy, Exercise, Genetics, Arrhythmia

  14. Survival and sudden cardiac death after septal ablation for hypertrophic obstructive cardiomyopathy

    DEFF Research Database (Denmark)

    Jensen, Morten Kvistholm; Havndrup, Ole; Hassager, Christian

    2011-01-01

    Reports of long-term survival and the risk of sudden cardiac death (SCD) after percutaneous transluminal septal myocardial ablation (PTSMA) in patients with hypertrophic obstructive cardiomyopathy (HOCM) are sparse.......Reports of long-term survival and the risk of sudden cardiac death (SCD) after percutaneous transluminal septal myocardial ablation (PTSMA) in patients with hypertrophic obstructive cardiomyopathy (HOCM) are sparse....

  15. Role of beta adrenoceptors in the hypertrophic response to thyroxine

    International Nuclear Information System (INIS)

    Eliades, D.; Weiss, H.R.

    1989-01-01

    The ability of beta-adrenoceptor blockade to reduce the hypertrophic response to thyroxine (T4, 0.5 mg/kg per day, s.c.) was tested in New Zealand white rabbits. Two beta-adrenergic blocking agents, one a full antagonist (propranolol, 9.6 mg/kg per day) and the other a partial agonist (pindolol, 0.96 mg/kg per day) were administered in combination with T4 in an effort to reduce myocardial hypertrophy. A 3 and 16 day group were generated to test the time course of the hypertrophic and receptor responses. Coronary blood flow was measured using radioactive microspheres, and beta-adrenoceptor number and affinity were measured using 125I(-) pindolol as the radioligand. T4 increased coronary blood flow to 1.95 times control values in the 3 day group and 2.2 times control levels in the 16 day group; beta-adrenoceptor number was increased similarly in 3 and 16 day groups to 1.9 times control Bmax levels. Heart weight (HW) to body weight (BW) ratios were significantly increased in only the 16 day group to 1.22 and 1.61 times control, respectively. Treatment with propranolol + T4 blunted the coronary blood flow increase, but receptor upregulation occurred to the same extent as with either substance alone. The HW/BW was increased to 1.49 times control. Pindolol + T4 did not decrease coronary blood flow but blocked beta-adrenoceptor upregulation. The HW was reduced to control levels and the HW/BW ratio was 1.40 times control and significantly decreased from T4 alone. Thus, pindolol was effective in reducing the hypertrophic response to T4, whereas propranolol was only moderately effective in doing so

  16. Lumican alleviates hypertrophic scarring by suppressing integrin-FAK signaling

    International Nuclear Information System (INIS)

    Zhao, Yuqian; Li, Xueyong; Xu, Xiaoli; He, Zhi; Cui, Lei; Lv, Xiaoxing

    2016-01-01

    Hypertrophic scarring (HS) is an overcompensation of wound healing that increases the risk of cosmetic disfigurement and functional impairment. No gold standard has been established for the treatment or prevention of HS. Our study aims to elucidate the expression and function of lumican in the pathogenesis of HS as well as the underlying mechanism involved in this procedure. An animal model of HS (rabbit ear) was established, and the Ad-lumican vectors were locally injected. Primary fibroblasts isolated from patients with hypertrophic burn scars were used in vitro. Histological and molecular changes in HS pathogenesis were evaluated. The results showed that lumican is significantly reduced in HS tissues and fibroblasts from HS patients as compared to normal skin or cells. Lumican levels were further suppressed in response to TGF-β stimulation. However, lumican upregulation effectively thinned the scar area and inhibited fibroblast proliferation and the cell cycle. Meanwhile, Ad-lumican administration suppressed the deposition of extracellular matrix, such as collagen and CTGF. Ad-lumican injected animals or fibroblasts presented comparable integrin α 2 β 1 expression while greatly reduced phosphorylation of FAK compared to the negative control. Moreover, Ad-lumican administration largely enhanced the binding of lumican to integrin α 2 β 1 and may thus inhibit the signaling propagation of collagen-integrin α 2 β 1 . Overall, the restoration of lumican levels contributed to suppressing the HS progression by inhibiting collagen-integrin α 2 β 1 -FAK signaling. - Highlights: • Lumican is downregulated during hypertrophic scar formation. • Lumican inhibits fibroblast proliferation. • Lumican inhibits extracellular matrix deposition. • Lumican suppresses collagen-integrin-FAK signaling.

  17. Evaluation of the diagnosis for hypertrophic cardiomyopathy (HCM) with SPECT

    International Nuclear Information System (INIS)

    Li Jiaxiu

    1992-01-01

    A heart phantom-7070 was used to measure the wall thickness of cardiac chambers. Two methods were employed: (1) profile curve measurement, (2) calculation of the thickness of cardiac walls. 9 normal cases and 13 patients with hypertrophic cardiomyopathy were studied using 99m Tc-CDI SPECT. 4 patterns were obtained: (1) Local hypertrophy of ventricular septum; (2) The predominant hypertrophy localized in left ventricular lateral wall; (3) Markedly hypertrophied septum and also involving left ventricular walls, especially the apical region; (4) Markedly hypertrophied papillary muscles with perfusion defects in the left wall and septum. These results suggest that myocardial SPECT is a promising and noninvasive method for the diagnosis of HCM

  18. Private mitochondrial DNA variants in danish patients with hypertrophic cardiomyopathy

    DEFF Research Database (Denmark)

    Hagen, Christian M; Aidt, Frederik H; Havndrup, Ole

    2015-01-01

    Hypertrophic cardiomyopathy (HCM) is a genetic cardiac disease primarily caused by mutations in genes coding for sarcomeric proteins. A molecular-genetic etiology can be established in ~60% of cases. Evolutionarily conserved mitochondrial DNA (mtDNA) haplogroups are susceptibility factors for HCM......>G, and MT-CYB: m.15024G>A, p.C93Y remained. A detailed analysis of these variants indicated that none of them are likely to cause HCM. In conclusion, private mtDNA mutations are frequent, but they are rarely, if ever, associated with HCM....

  19. Hypertrophic osteoarthropathy in association with pulmonary tuberculosis: a case report

    International Nuclear Information System (INIS)

    Moreira, Luiza Beatriz Melo; Marchiori, Edson; Melo, Alessandro Severo Alves de; Pinto, Ana Lucia de Araujo; Monteiro, Nicolau Pedro

    2002-01-01

    In this article the authors report a case of a patient with hypertrophic osteoarthropathy associated with pulmonary tuberculosis. This disorder is a rare complication of tuberculosis and may be associated with severe and fulminant disease. The imaging methods used in the diagnosis of this disease included lower limbs radiographs that showed bilateral periosteal reaction, and chest radiographs and computed tomography that revealed a mass in the upper lobe of the right lung. Although a fine needle aspiration was suggestive of tuberculosis, the definitive diagnosis was made after thoracotomy and histopathological examination of the mass. The patient presented symptomatic and radiological improvement after treatment of tuberculosis. (author)

  20. Myoadenylate deaminase deficiency, hypertrophic cardiomyopathy and gigantism syndrome.

    Science.gov (United States)

    Skyllouriotis, M L; Marx, M; Bittner, R E; Skyllouriotis, P; Gross, M; Wimmer, M

    1997-07-01

    We report a 20-year-old man with gigantism syndrome, hypertrophic cardiomyopathy, muscle weakness, exercise intolerance, and severe psychomotor retardation since childhood. Histochemical and biochemical analysis of skeletal muscle biopsy revealed myoadenylate deaminase deficiency; molecular genetic analysis confirmed the diagnosis of primary (inherited) myoadenylate deaminase deficiency. Plasma, urine, and muscle carnitine concentrations were reduced. L-Carnitine treatment led to gradual improvement in exercise tolerance and cognitive performance; plasma and tissue carnitine levels returned to normal, and echocardiographic evidence of left ventricular hypertrophy disappeared. The combination of inherited myoadenylate deaminase deficiency, gigantism syndrome and carnitine deficiency has not previously been described.

  1. Lung carcinoma with hypertrophic osteoarthropathy in a teenager

    Directory of Open Access Journals (Sweden)

    Jeremy Whelan

    2011-03-01

    Full Text Available Hypertrophic osteoarthropathy (HOA characterised by arthralgia, clubbing and periosteal proliferation of long bones, is rarely encountered in children and adolescents. Whereas in adults over 80% of cases are associated with malignancy, in children the majority of cases are due to non-neoplastic causes such as cystic fibrosis, bilary atresia and congenital heart disease. Up to 5% of adults with lung cancer demonstrate signs of HOA. However, lung cancer is extremely uncommon in children and young people. Here we report a case of lung adenocarcinoma in an 18 year old male associated with HOA present both at diagnosis and at subsequent disease progression.

  2. Is there a minimum intensity threshold for resistance training-induced hypertrophic adaptations?

    Science.gov (United States)

    Schoenfeld, Brad J

    2013-12-01

    In humans, regimented resistance training has been shown to promote substantial increases in skeletal muscle mass. With respect to traditional resistance training methods, the prevailing opinion is that an intensity of greater than ~60 % of 1 repetition maximum (RM) is necessary to elicit significant increases in muscular size. It has been surmised that this is the minimum threshold required to activate the complete spectrum of fiber types, particularly those associated with the largest motor units. There is emerging evidence, however, that low-intensity resistance training performed with blood flow restriction (BFR) can promote marked increases in muscle hypertrophy, in many cases equal to that of traditional high-intensity exercise. The anabolic effects of such occlusion-based training have been attributed to increased levels of metabolic stress that mediate hypertrophy at least in part by enhancing recruitment of high-threshold motor units. Recently, several researchers have put forth the theory that low-intensity exercise (≤50 % 1RM) performed without BFR can promote increases in muscle size equal, or perhaps even superior, to that at higher intensities, provided training is carried out to volitional muscular failure. Proponents of the theory postulate that fatiguing contractions at light loads is simply a milder form of BFR and thus ultimately results in maximal muscle fiber recruitment. Current research indicates that low-load exercise can indeed promote increases in muscle growth in untrained subjects, and that these gains may be functionally, metabolically, and/or aesthetically meaningful. However, whether hypertrophic adaptations can equal that achieved with higher intensity resistance exercise (≤60 % 1RM) remains to be determined. Furthermore, it is not clear as to what, if any, hypertrophic effects are seen with low-intensity exercise in well-trained subjects as experimental studies on the topic in this population are lacking. Practical

  3. FACTORS INFLUENCING THE OCCURRENCE OF HYPERTROPHIC SCARS AMONG POSTOPERATIVE PATIENTS IN GARUT, INDONESIA

    Directory of Open Access Journals (Sweden)

    Andri Nugraha

    2017-02-01

    Full Text Available Background: Hypertrophic scar causes physical and psychological problems. Thus understanding the factors related to the occurrence of hypertrophic scar tissue is needed. Little is known about its influencing factors in Indonesia, especially in Garut. Objective: This study aims to examine the relationships between hypertrophic scar and its influencing factors, and identify the most dominant factor of the occurrence of hypertrophic scars. Methods: This was an observational case control study using retrospective approach in Polyclinic of Surgery of Regional Public Hospital of dr. Slamet of Garut Regency. There were 40 samples recruited in this study by purposive sampling, which was divided to be case group (20 patients and control group (20 patients. Data were collected using Stony Brook Scar Evaluation Scale by observation and documentation of the medical records of patients. Data were analyzed using logistic regression analysis Results: Findings indicated that there were significant relationships between the surgical wound infection (p = 0.02, family history (p = 0.026, and type of suture (p = 0.043 with the occurrence of hypertrophic scars. The most dominant factor on the occurrence of hypertrophic scars was type of suture, acid polyglactin 910. The variables that had no significant relationships with the occurrence of hypertrophic scar tissue were age (p = 0.34, area of surgical wound (p = 0.177, and smoking habit (p = 0.479. Conclusion: There were significant relationships between infection of surgical wound, genetic history, the type of suture, and the occurrence of hypertrophic scar tissue. The most dominant factor that influenced the occurrence of hypertrophic scar tissue was the type of suture. Therefore, it is suggested to health professionals to modify the using of acid polyglactin 910 sutures, and nurses particularly need to provide the information regarding the family history and genetic-related hypertrophic scar, and prevent the

  4. Symmetric Dimethylarginine in Cats with Hypertrophic Cardiomyopathy and Diabetes Mellitus

    DEFF Research Database (Denmark)

    Langhorn, R.; Kieler, I. N.; Koch, J.

    2018-01-01

    Background: Symmetric dimethylarginine (SDMA) has been increasingly used as a marker of early chronic kidney disease (CKD) in cats, but little is known about the influence of comorbidities on SDMA in this species. Hypothesis: Hypertrophic cardiomyopathy (HCM) and diabetes mellitus (DM), independe......Background: Symmetric dimethylarginine (SDMA) has been increasingly used as a marker of early chronic kidney disease (CKD) in cats, but little is known about the influence of comorbidities on SDMA in this species. Hypothesis: Hypertrophic cardiomyopathy (HCM) and diabetes mellitus (DM......), independently of CKD, are associated with changes in serum SDMA. Animals: Ninety-four cats (17 with CKD, 40 with HCM, 17 with DM, and 20 healthy controls). Methods: Case-control study. Clinical examination, echocardiography, ECG, blood pressure, CBC, biochemistry, thyroxine, and SDMA measurement were performed....... Urinalysis was performed in controls and cats with CKD and DM. Analysis of variance was used to compare overall differences in the log-transformed SDMA data among groups. A random forest algorithm was applied to explore which clinical and other factors influenced serum SDMA. Results: Median (range) serum...

  5. Ablation of hypertrophic septum using radiofrequency energy: an alternative for gradient reduction in patient with hypertrophic obstructive cardiomyopathy?

    Science.gov (United States)

    Riedlbauchová, Lucie; Janoušek, Jan; Veselka, Josef

    2013-06-01

    Alcohol septal ablation and surgical myectomy represent accepted therapeutic options for treatment of symptomatic patients with hypertrophic obstructive cardiomyopathy. Long-term experience with radiofrequency ablation of arrhythmogenic substrates raised a question if this technique might be effective for left ventricular outflow tract (LVOT) gradient reduction. We report on a 63-year-old patient with recurrence of symptoms 1 year after alcohol septal ablation (ASA) leading originally to a significant reduction of both symptoms and gradient. Due to a new increase of gradient in the LVOT up to 200 mm Hg with corresponding worsening of symptoms and due to refusal of surgical myectomy by the patient, endocardial radiofrequency ablation of the septal hypertrophy (ERASH) was indicated. Radiofrequency ablation was performed in the LVOT using irrigated-tip ablation catheter; the target site was identified using intracardiac echocardiography and electroanatomical CARTO mapping. ERASH caused an immediate gradient reduction due to hypokinesis of the ablated septum. At 2-month follow-up exam, significant clinical improvement was observed, together with persistent gradient reduction assessed with Doppler echocardiography. Echocardiography and magnetic resonance revealed persistent septal hypokinesis and slight thinning of the ablated region. Septal ablation using radiofrequency energy may be a promising alternative or adjunct to the treatment of hypertrophic obstructive cardiomyopathy. Intracardiac echocardiography and electroanatomical CARTO mapping enable exact lesion placement and preservation of atrioventricular conduction.

  6. Squamous Cell Carcinoma Arising in Hypertrophic Lichen Planus: A Review and Analysis of 38 Cases.

    Science.gov (United States)

    Knackstedt, Thomas J; Collins, Lindsey K; Li, Zhongze; Yan, Shaofeng; Samie, Faramarz H

    2015-12-01

    Hypertrophic lichen planus is a chronic variant of lichen planus with controversial malignant association. To describe and analyze the relationship of squamous cell carcinoma (SCC) and hypertrophic lichen planus. A retrospective chart review of patients with hypertrophic lichen planus and SCC was performed at the authors' institution. Thereafter, scientific databases were searched for articles reporting cases of SCC arising in hypertrophic lichen planus. Patient demographics, immune status, lichen planus features, and SCC data points were extracted for each patient and evaluated. Thirty-eight cases of SCC in hypertrophic lichen planus occurred in 16 women, average age: 61.4, and 22 men, average age: 51.3, after a lag time of 88 days to 40 years. Squamous cell carcinoma was uniformly located on the lower extremity. Men had larger SCC than women (p = .027) and a significantly longer lag time to SCC development (p = .002). Long lag time was associated with a smaller SCC size (p = .032). In the past, hypertrophic lichen planus and SCC have been considered isolated diseases. Based on an increasing number of cases, the association between hypertrophic lichen planus and keratinocyte malignancies warrants surveillance.

  7. Conversion of 3H-testosterone to dihydrotestosterone in human hypertrophic prostatic tissue

    International Nuclear Information System (INIS)

    Baranowska, B.; Zgliczynski

    1979-01-01

    The aim of the study was to develop a simple method for the determination of the conversion of testosterone to 5α-dihydrotestosterone (5α-DHT) after incubation of human hypertrophic prostatic tissue with 3 H-testosterone. The mean conversion rate of 3 H-testosterone to 5α-DHT in hypertrophic prostatic tissue was found to be higher than in normal and carcinomatous tissue. The results indicate that androgen metabolism in the hypertrophic prostatic gland is enhanced. (orig.) [de

  8. Conversion of /sup 3/H-testosterone to dihydrotestosterone in human hypertrophic prostatic tissue

    Energy Technology Data Exchange (ETDEWEB)

    Baranowska, B; Zgliczynski, [Centre of Postgraduate Medical Education, Warsaw (Poland). Clinic of Endocrinology

    1979-12-01

    The aim of the study was to develop a simple method for the determination of the conversion of testosterone to 5..cap alpha..-dihydrotestosterone (5..cap alpha..-DHT) after incubation of human hypertrophic prostatic tissue with /sup 3/H-testosterone. The mean conversion rate of /sup 3/H-testosterone to 5..cap alpha..-DHT in hypertrophic prostatic tissue was found to be higher than in normal and carcinomatous tissue. The results indicate that androgen metabolism in the hypertrophic prostatic gland is enhanced.

  9. Hypertrophic cardiomyopathy secondary to hepatitis C virus-related vasculitis.

    Science.gov (United States)

    Cavalli, Giulio; Berti, Alvise; Fragasso, Gabriele; De Cobelli, Francesco

    2016-12-01

    : Almost invariably associated with chronic HCV infection, cryoglobulinemic vasculitis is a small-vessel vasculitis commonly affecting the skin, kidneys, and peripheral nervous system. Cardiac involvement, possibly due to cardiac microcirculation involvement, is an utterly rare and severe complication. We describe a case of hypertrophic cardiomyopathy secondary to cryoglobulinemic vasculitis. Evaluation with transthoracic cardiac ultrasound and cardiac MRI evidenced severe left ventricular hypertrophy and diffuse hypokinesia, a marked decrease in left ventricular ejection fraction, and a subtle late enhancement of inferior and lateral left ventricular walls. Upon clinical stabilization, the patient received treatment with anti-CD20 monoclonal antibody rituximab. Clinical and radiological follow-up with cardiac ultrasound and cardiac MRI documented a dramatic and sustained clinical improvement, with marked reduction of left ventricular hypertrophy, resolution of late enhancement, recovery of left ventricular contractility and function.

  10. Various manifestations of hypertrophic cardiomyopathy on ultrafast computed tomography

    International Nuclear Information System (INIS)

    Sekiya, Tohru; Karikomi, Masahito; Ohshiro, Masaya; Iwakami, Masayoshi; Takamoto, Toshihiko; Sakamoto, Tsuguya

    1992-01-01

    Ultrafast computed tomography was performed in 30 patients with hypertrophic cardiomyopathy and images were assessed on variability of left ventricular hypertrophy, the pattern of left ventricular contraction, right ventricular hypertrophy, dilatation of the left atrium, and thickening of the mitral valve. Fifteen (50.0%) of 30 patients had asymmetric septal hypertrophy, six (20.0%) had diffuse hypertrophy, and nine (30.0%) had apical hypertrophy. In eleven patients with asymmetric septal hypertrophy and two with apical hypertrophy, non-hypertrophied segments in end-diastole showed vigorous contraction. Sixteen patients showed homogeneous left ventricular contraction and one showed partial apical contraction. Right ventricular hypertrophy was noted in 12 patients (40.0%), dilatation of the left atrium in 13 patients (43.3%), and mitral valve thickening in three (10.0%). Ultrafast computed tomography was useful in the evaluation of apical hypertrophy and right ventricular hypertrophy, which could be difficult to obtain by echocardiography. (author)

  11. Serious arrhythmias in patients with apical hypertrophic cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Okishige, Kaoru; Sasano, Tetsuo; Yano, Kei; Azegami, Kouji; Suzuki, Kou; Itoh, Kuniyasu [Yokohama Red Cross Hospital (Japan)

    2001-05-01

    We report cases of serious arrhythmias associated with apical hypertrophic cardiomyopathy (AHCM). Thirty-one patients were referred to our institute to undergo further assessment of their AHCM from 1988 to 1999. Three patients with nonsustained ventricular tachycardia demonstrated an {sup 123}I-MIBG regional reduction in the tracer uptake. In two patients with ventricular fibrillation (VF), the findings from {sup 123}I-MIBG imaging revealed regional sympathetic denervation in the inferior and lateral regions. Electrophysiologic study demonstrated reproducible induction of VF in aborted sudden death and presyncopal patients, resulting in the need for an implantable defibrillator device and amiodarone in each patient. Patients with refractory atrial fibrillation with a rapid ventricular response suffered from serious congestive heart failure. A prudent assessment and strategy in patients with this disease would be indispensable in avoiding a disastrous outcome. (author)

  12. Hypertrophic cardiomyopathy: a heart in need of an energy bar?

    Directory of Open Access Journals (Sweden)

    Styliani eVakrou

    2014-08-01

    Full Text Available Hypertrophic cardiomyopathy (HCM has been recently recognized as the most common inherited cardiovascular disorder, affecting 1 in 500 adults worldwide. HCM is characterized by myocyte hypertrophy resulting in thickening of the ventricular wall, myocyte disarray, interstitial and/or replacement fibrosis, decreased ventricular cavity volume and diastolic dysfunction. HCM is also the most common cause of sudden death in the young. A large proportion of patients diagnosed with HCM have mutations in sarcomeric proteins. However, it is unclear how these mutations lead to the cardiac phenotype, which is variable even in patients carrying the same causal mutation. Abnormalities in calcium cycling, oxidative stress, mitochondrial dysfunction and energetic deficiency have been described, constituting the basis of therapies in experimental models of HCM and HCM patients. This review focuses on evidence supporting the role of cellular metabolism and mitochondria in HCM.

  13. Cardiac symptoms before sudden cardiac death caused by hypertrophic cardiomyopathy

    DEFF Research Database (Denmark)

    Lynge, Thomas Hadberg; Risgaard, Bjarke; Jabbari, Reza

    2016-01-01

    AIMS: Hypertrophic cardiomyopathy (HCM) is a frequent cause of sudden cardiac death (SCD) among the young (SCDY). The aim of this study was to characterize symptoms before SCDY due to HCM. METHODS AND RESULTS: Through review of all death certificates, we identified all SCDs in Danes aged 1-35 years...... in 2000-2009. Nationwide we included all deaths (n = 8756) and identified 431 autopsied SCDYs. All available records from hospitals and general practitioners were retrieved. To compare symptoms, we included a control groups consisting of traffic accident victims (n = 74). In the 10-year study period, 431...... autopsied SCDY cases were reviewed and 38 cases (9%) were included, of which 22 (58%) had morphologic findings diagnostic of HCM and 16 (42%) had findings suggestive, but not diagnostic, of HCM ('possible HCM'). Cardiac symptoms >1 h prior to death were reported in 21 (55%) of cases, and 16 (42%) sought...

  14. Ultrastructural myocardial changes in seven cats with spontaneous hypertrophic cardiomyopathy

    DEFF Research Database (Denmark)

    Christiansen, Liselotte Bruun; Prats Gavalda, Clara; Hyttel, Poul

    2015-01-01

    OBJECTIVES: Hypertrophic cardiomyopathy (HCM) is the most common heart disease in cats and shares clinical and pathological characteristics with human HCM. Little is known about the pathogenic mechanisms underlying development of spontaneous feline HCM. ANIMALS: The study population consisted...... of seven cats diagnosed with HCM and eight age-matched cats with no evidence of cardiac disease. METHODS: Fresh myocardial biopsies taken from the middle of the left ventricular posterior free wall were obtained and examined with transmission electron microscopy. RESULTS: Electron microscopic examination...... showed ultrastructural aberrations of the myocardial cytoarchitecture and of the interstitium in the seven cats with HCM. In the most severely affected cats the myofibrils were disorganized and subsarcolemmal mitochondria were depleted. In control cats, contraction band artifacts were commonly seen...

  15. The Portuguese Registry of Hypertrophic Cardiomyopathy: Overall results.

    Science.gov (United States)

    Cardim, Nuno; Brito, Dulce; Rocha Lopes, Luís; Freitas, António; Araújo, Carla; Belo, Adriana; Gonçalves, Lino; Mimoso, Jorge; Olivotto, Iacopo; Elliott, Perry; Madeira, Hugo

    2018-01-01

    We report the results of the Portuguese Registry of Hypertrophic Cardiomyopathy, an initiative that reflects the current spectrum of cardiology centers throughout the territory of Portugal. A direct invitation to participate was sent to cardiology departments. Baseline and outcome data were collected. A total of 29 centers participated and 1042 patients were recruited. Four centers recruited 49% of the patients, of whom 59% were male, and mean age at diagnosis was 53±16 years. Hypertrophic cardiomyopathy (HCM) was identified as familial in 33%. The major reason for diagnosis was symptoms (53%). HCM was obstructive in 35% of cases and genetic testing was performed in 51%. Invasive septal reduction therapy was offered to 8% (23% of obstructive patients). Most patients (84%) had an estimated five-year risk of sudden death of <6%. Thirteen percent received an implantable cardioverter-defibrillator. After a median follow-up of 3.3 years (interquartile range [P25-P75] 1.3-6.5 years), 31% were asymptomatic. All-cause mortality was 1.19%/year and cardiovascular mortality 0.65%/year. The incidence of heart failure-related death was 0.25%/year, of sudden cardiac death 0.22%/year and of stroke-related death 0.04%/year. Heart failure-related death plus heart transplantation occurred in 0.27%/year and sudden cardiac death plus equivalents occurred in 0.53%/year. Contemporary HCM in Portugal is characterized by relatively advanced age at diagnosis, and a high proportion of invasive treatment of obstructive forms. Long-term mortality is low; heart failure is the most common cause of death followed by sudden cardiac death. However, the burden of morbidity remains considerable, emphasizing the need for disease-specific treatments that impact the natural history of the disease. Copyright © 2017 Sociedade Portuguesa de Cardiologia. Publicado por Elsevier España, S.L.U. All rights reserved.

  16. Efficacy of Gd-DTPA-enhanced MRI in hypertrophic cardiomyopathy

    International Nuclear Information System (INIS)

    Okamoto, Shinya; Aoki, Toshikazu; Konishi, Tokuji; Nakano, Takeshi; Yamakado, Kyoichiro; Sakuma, Hajime; Takeda, Kann; Nakagawa, Takashi

    1991-01-01

    The cabability of magnetic resonance (MR) imaging to detect tissue characterization or myocardial degeneration process of the hypertrophied myocardium was evaluated in 15 patients with hypertrophic cardiomyopathy. T1-weighted MR images were obtained with a 1.5 T MR unit by using ECG-gated spin-echo techniques. MR images were visually reviewed before and after enhancement of Gd-DTPA. Four patients had an increase in signal intensity mainly in the endocardium of the left ventricular septum on non-enhanced MR images, 3 of whom had widespread high intensity in addition to two-thirds of the wall. Gd-DTPA enhanced-MR images showed high intensity over the whole septum in 5 patients and also in the antero-lateral endocardium in 4 patients. Decreased intensity on non-enhanced MR images, as shown in 4 patients, became clear on enhanced-MR images. According to findings on enhanced-MR images, signal intensity was defined as normal (N), septum (S), and diffuse (D). Patients in Group D tended to be younger and have more frequently family history. Regarding both interventricular septum thickness and left ventricular posterior wall thickness, there was no significant difference among the three groups. Both left ventricular diastolic diameter and left ventricular systolic diameter were significantly larger in Group D than the other two groups. Left ventricular ejection fraction was significantly lower in both Group S and Group D. Widespread abnormal intensity on Gd-DTPA enhanced MR images was associated with findings similar to dilated cardiomyopathy, such as dilated left ventricular lumen and decreased ejection fraction. Gd-DTPA enhanced MR imaging seemed to be useful for visualizing myocardial degeneration in hypertrophic cardiomyopathy.(N.K.)

  17. MTO1 mutations are associated with hypertrophic cardiomyopathy and lactic acidosis and cause respiratory chain deficiency in humans and yeast.

    Science.gov (United States)

    Baruffini, Enrico; Dallabona, Cristina; Invernizzi, Federica; Yarham, John W; Melchionda, Laura; Blakely, Emma L; Lamantea, Eleonora; Donnini, Claudia; Santra, Saikat; Vijayaraghavan, Suresh; Roper, Helen P; Burlina, Alberto; Kopajtich, Robert; Walther, Anett; Strom, Tim M; Haack, Tobias B; Prokisch, Holger; Taylor, Robert W; Ferrero, Ileana; Zeviani, Massimo; Ghezzi, Daniele

    2013-11-01

    We report three families presenting with hypertrophic cardiomyopathy, lactic acidosis, and multiple defects of mitochondrial respiratory chain (MRC) activities. By direct sequencing of the candidate gene MTO1, encoding the mitochondrial-tRNA modifier 1, or whole exome sequencing analysis, we identified novel missense mutations. All MTO1 mutations were predicted to be deleterious on MTO1 function. Their pathogenic role was experimentally validated in a recombinant yeast model, by assessing oxidative growth, respiratory activity, mitochondrial protein synthesis, and complex IV activity. In one case, we also demonstrated that expression of wt MTO1 could rescue the respiratory defect in mutant fibroblasts. The severity of the yeast respiratory phenotypes partly correlated with the different clinical presentations observed in MTO1 mutant patients, although the clinical outcome was highly variable in patients with the same mutation and seemed also to depend on timely start of pharmacological treatment, centered on the control of lactic acidosis by dichloroacetate. Our results indicate that MTO1 mutations are commonly associated with a presentation of hypertrophic cardiomyopathy, lactic acidosis, and MRC deficiency, and that ad hoc recombinant yeast models represent a useful system to test the pathogenic potential of uncommon variants, and provide insight into their effects on the expression of a biochemical phenotype. © 2013 The Authors. *Human Mutation published by Wiley Periodicals, Inc.

  18. Determination of multidirectional myocardial deformations in cats with hypertrophic cardiomyopathy by using two-dimensional speckle-tracking echocardiography.

    Science.gov (United States)

    Suzuki, Ryohei; Mochizuki, Yohei; Yoshimatsu, Hiroki; Teshima, Takahiro; Matsumoto, Hirotaka; Koyama, Hidekazu

    2017-12-01

    Objectives Hypertrophic cardiomyopathy, a primary disorder of the myocardium, is the most common cardiac disease in cats. However, determination of myocardial deformation with two-dimensional speckle-tracking echocardiography in cats with various stages of hypertrophic cardiomyopathy has not yet been reported. This study was designed to measure quantitatively multidirectional myocardial deformations of cats with hypertrophic cardiomyopathy. Methods Thirty-two client-owned cats with hypertrophic cardiomyopathy and 14 healthy cats serving as controls were enrolled and underwent assessment of myocardial deformation (peak systolic strain and strain rate) in the longitudinal, radial and circumferential directions. Results Longitudinal and radial deformations were reduced in cats with hypertrophic cardiomyopathy, despite normal systolic function determined by conventional echocardiography. Cats with severely symptomatic hypertrophic cardiomyopathy also had lower peak systolic circumferential strain, in addition to longitudinal and radial strain. Conclusions and relevance Longitudinal and radial deformation may be helpful in the diagnosis of hypertrophic cardiomyopathy. Additionally, the lower circumferential deformation in cats with severe hypertrophic cardiomyopathy may contribute to clinical findings of decompensation, and seems to be related to severe cardiac clinical signs. Indices of multidirectional myocardial deformations by two-dimensional speckle-tracking echocardiography may be useful markers and help to distinguish between cats with hypertrophic cardiomyopathy and healthy cats. Additionally, they may provide more detailed assessment of contractile function in cats with hypertrophic cardiomyopathy.

  19. Use of bilidase for the treatment of experimental hypertrophic postburn cicatrices.

    Science.gov (United States)

    Loladze, M; Alibegashvili, M; Turmanidze, Ts; Iashvili, B; Kutivadze, D; Chanishvili, T

    2005-01-01

    The efficiency of bilidase (hyaluronidase preparation) was studied in guinea pigs with experimental postburn cicatrices. Bilidase promoted normalization of the structure and histochemical picture of new cicatricial tissue. The drug can be used for the treatment of hypertrophic postburn cicatrices.

  20. Safety profile and utility of treadmill exercise in patients with high-gradient hypertrophic cardiomyopathy

    DEFF Research Database (Denmark)

    Sorensen, Lars Lindholm; Liang, Hsin-Yueh; Pinheiro, Aurelio

    2017-01-01

    BACKGROUND: Exercise echocardiography in the evaluation of hypertrophic cardiomyopathy (HCM) provides valuable information for risk stratification, selection of optimal treatment, and prognostication. However, HCM patients with left ventricular outflow tract gradients ≥30mm Hg are often excluded ...

  1. Angiotensin II type 2 receptors and cardiac hypertrophy in women with hypertrophic cardiomyopathy

    NARCIS (Netherlands)

    J. Deinum (Jacob); J.M. van Gool (Jeanette); M.J.M. Kofflard (Marcel); A.H.J. Danser (Jan); F.J. ten Cate (Folkert)

    2001-01-01

    textabstractThe development of left ventricular hypertrophy in subjects with hypertrophic cardiomyopathy (HCM) is variable, suggesting a role for modifying factors such as angiotensin II. Angiotensin II mediates both trophic and antitrophic effects, via angiotensin II type 1

  2. Comparison Between Clinical and Echocardiographic Findings in Infants and Children Diagnosed with Hypertrophic Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Cristina Blesneac

    2015-06-01

    Full Text Available Background: Hypertrophic cardiomyopathy is a rather common hereditary disease with an autozomal dominant character, caused by mutations of genes that code for proteins of the cardiac sarcomere. The observed prevalence of this disease is much lower in pediatric patients compared to adults, because it’s late gene expression. Hypertrophic cardiomyopathy presenting in infancy has been shown to have a very high mortality.

  3. Value of noninvasive diagnostic procedures in cardiology: typical findings in hypertrophic obstructive cardiomyopathy

    International Nuclear Information System (INIS)

    Riebeling, V.; Bubenheimer, P.

    1984-01-01

    Routine chest X-ray often yields poor information for diagnosis of heart disease. The diagnostic value of invasive procedures in cardiology is generally accepted. The patient's as well as the physician's risk of the examination, however, has to be considered. A high number of heart diseases, e.g. hypertrophic obstructive cardiomyopathy (HOCM) is mainly detected by noninvasive procedures such as auscultation, electrocardiography, phonomechanocardiography, echocardiography, physical manoeuvres, and pharmacological provocation tests. Typical findings in hypertrophic obstructive cardiomyopathy are demonstrated. (orig.) [de

  4. Nerve growth factor receptor immunostaining suggests an extrinsic origin for hypertrophic nerves in Hirschsprung's disease.

    OpenAIRE

    Kobayashi, H; O'Briain, D S; Puri, P

    1994-01-01

    The expression of nerve growth factor receptor in colon from 20 patients with Hirshsprung's disease and 10 controls was studied immunohistochemically. The myenteric and submucous plexuses in the ganglionic bowel and hypertrophic nerve trunks in the aganglionic bowel displayed strong expression of nerve growth factor receptor. The most important finding was the identical localisation of nerve growth factor receptor immunoreactivity on the perineurium of both hypertrophic nerve trunks in Hirshs...

  5. ACE I/D polymorphism in Indian patients with hypertrophic cardiomyopathy and dilated cardiomyopathy

    DEFF Research Database (Denmark)

    Rai, Taranjit Singh; Dhandapany, Perundurai Subramaniam; Ahluwalia, Tarun Veer Singh

    2008-01-01

    The study was carried to determine the association of angiotensin converting enzyme (ACE) insertion/deletion (I/D) polymorphism with the risk of hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), and restrictive cardiomyopathy (RCM).......The study was carried to determine the association of angiotensin converting enzyme (ACE) insertion/deletion (I/D) polymorphism with the risk of hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), and restrictive cardiomyopathy (RCM)....

  6. Idiopathic hypertrophic cranial pachymeningitis associated with Sweet's Syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Cano, Antonio E-mail: acano@hrs.sas.junta-andalucia.es; Ribes, Ramon; Riva, Andres de la; Rubio, Fernando Lopez; Sanchez, Carmen; Sancho, Jose L

    2002-11-01

    A case of hypertrophic cranial pachymeningitis associated with Sweet's Syndrome is presented. Both entities have been described in association with several other chronic systemic inflammatory diseases and autoimmune conditions. To our knowledge the coexistence between Sweet's Syndrome and hypertrophic cranial pachymeningitis has not been reported up to date. We suggest a possible autoimmune or dysimmune mechanism in the pathogenesis of these two entities.

  7. [Gene mutation and clinical phenotype analysis of patients with Noonan syndrome and hypertrophic cardiomyopathy].

    Science.gov (United States)

    Liu, X H; Ding, W W; Han, L; Liu, X R; Xiao, Y Y; Yang, J; Mo, Y

    2017-10-02

    Objective: To analyze the gene mutations and clinical features of patients with Noonan syndrome and hypertrophic cardiomyopathy. Method: Determined the mutation domain in five cases diagnosed with Noonan syndrome and hypertrophic cardiomyopathy and identified the relationship between the mutant domain and hypertrophic cardiomyopathy by searching relevant articles in pubmed database. Result: Three mutant genes (PTPN11 gene in chromosome 12, RIT1 gene in chromosome 1 and RAF1 gene in chromosome 3) in five cases all had been reported to be related to hypertrophic cardiomyopathy. The reported hypertrophic cardiomyopathy relevant genes MYPN, MYH6 and MYBP3 had also been found in case 1 and 2. Patients with same gene mutation had different clinical manifestations. Both case 4 and 5 had RAF1 mutation (c.770C>T). However, case 4 had special face, low IQ, mild pulmonary artery stenosis, and only mild ventricular hypertrophy. Conclusion: Noonan syndrome is a genetic heterogeneity disease. Our study identified specific gene mutations that could result in Noonan syndrome with hypertrophic cardiomyopathy through molecular biology methods. The results emphasize the importance of gene detection in the management of Noonan syndrome.

  8. Sudden cardiac arrest in a young patient with hypertrophic cardiomyopathy and zero canonical risk factors: the inherent limitations of risk stratification in hypertrophic cardiomyopathy.

    Science.gov (United States)

    Kohorst, John J; Bos, J Martijn; Hagler, Donald J; Ackerman, Michael J

    2014-01-01

    Hypertrophic cardiomyopathy is the most common heritable cardiovascular disease and a common cause of sudden cardiac death (SCD) in young adolescents and athletes. Clinical risk stratification for SCD is predicated on the presence of established risk factors; however, this assessment is far from perfect. Herein, we present a 16-year-old male who was resuscitated successfully from his sentinel event of out-of-hospital cardiac arrest. Prior to this event, he was asymptomatic and lacked all traditional SCD-predisposing risk factors for hypertrophic cardiomyopathy. © 2013 Wiley Periodicals, Inc.

  9. Echocardiography as a Screening Test for Myocardial Scarring in Children with Hypertrophic Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Gregory Compton

    2016-01-01

    Full Text Available Introduction. Hypertrophic cardiomyopathy (HCM is burdened with morbidity and mortality including tachyarrhythmias and sudden cardiac death. These complications are attributed in part to the formation of proarrhythmic scars in the myocardium. The presence of extensive LGE is a risk factor for adverse outcomes in HCM. Late gadolinium enhancement (LGE cardiac magnetic resonance imaging (cMRI is the standard for the noninvasive evaluation of myocardial scars. However, echocardiography represents an attractive screening tool for myocardial scarring. The aim of this study was to compare the suitability of echocardiography to detect myocardial scars to the standard of cMRI-LGE. Methods. The cMRI studies and echocardiograms from 56 consecutive children with HCM were independently evaluated for the presence of cMRI-LGE and echocardiographic evidence of scarring by expert readers. Results. Echocardiography had a high sensitivity (93% and negative predictive value (94% in comparison to LGE. The false positive rate was high, leading to a low specificity (37% and a low positive predictive value (35%. Conclusions. Given the poor specificity and positive predictive value, echocardiography is not a suitable screening test for the presence of myocardial scarring in children with HCM. However, children without echocardiographic evidence of myocardial scarring may not need to undergo cardiac magnetic resonance imaging to “rule in” LGE.

  10. Ultrasonographic features of normalization of the pylorus after pyloromyotomy for hypertrophic pyloric stenosis.

    Science.gov (United States)

    Yoshizawa, J; Eto, T; Higashimoto, Y; Saitou, T; Maie, M

    2001-04-01

    The purpose of this study was to describe the time course, early postoperative changes, and morphologic features of normalization of the pylorus after pyloromyotomy for hypertrophic pyloric stenosis. The subjects were 17 infants (9 boys, 8 girls) who underwent umbilical incision Ramstedt pyloromyotomy. The pyloric muscle mass was measured immediately before the operation and then at intervals from 3 days to 6 months after the operation using a 7.5-MHz ultrasound probe. In longitudinal section, the dorsal part of the pyloric muscle thickened transiently and then thinned to normal values by 5 months after the operation. It was 5.1 +/- 0.8 mm (mean +/- SD) preoperatively, increased to 6.0 +/- 0.3 mm by day 3 after the operation (P thick preoperatively, thickened to 4.6 +/- 0.4 mm by 3 days after the operation (P thickness within the first few postoperative days followed by a slow decrease that reaches normal thickness (thickness is accompanied by a gradual decrease in length to 75% of the preoperative value by 5 months. The morphologic features in this normalization are first a wedge (day 3), then a flat tire (days 7 and 14), and finally an elongated ring (5 months). J Pediatr Surg 36:582-586. Copyright 2001 by W.B. Saunders Company.

  11. The Signaling Pathways Involved in Chondrocyte Differentiation and Hypertrophic Differentiation

    Directory of Open Access Journals (Sweden)

    Jianmei Li

    2016-01-01

    Full Text Available Chondrocytes communicate with each other mainly via diffusible signals rather than direct cell-to-cell contact. The chondrogenic differentiation of mesenchymal stem cells (MSCs is well regulated by the interactions of varieties of growth factors, cytokines, and signaling molecules. A number of critical signaling molecules have been identified to regulate the differentiation of chondrocyte from mesenchymal progenitor cells to their terminal maturation of hypertrophic chondrocytes, including bone morphogenetic proteins (BMPs, SRY-related high-mobility group-box gene 9 (Sox9, parathyroid hormone-related peptide (PTHrP, Indian hedgehog (Ihh, fibroblast growth factor receptor 3 (FGFR3, and β-catenin. Except for these molecules, other factors such as adenosine, O2 tension, and reactive oxygen species (ROS also have a vital role in cartilage formation and chondrocyte maturation. Here, we outlined the complex transcriptional network and the function of key factors in this network that determine and regulate the genetic program of chondrogenesis and chondrocyte differentiation.

  12. Review of current therapies for secondary hypertrophic pulmonary osteoarthropathy.

    Science.gov (United States)

    Nguyen, Sheila; Hojjati, Mehrnaz

    2011-01-01

    Hypertrophic osteoarthropathy (HOA) is a disabling condition that may occur secondarily to primary lung cancer. It is characterized by digital clubbing, arthralgia/arthritis, and periostosis of the tubular bones. The pain associated with HOA can be disabling and often refractory to conventional analgesics. We performed a comprehensive review of the literature using the PubMed database on treatment modalities available for HOA. We found 52 relevant articles-40 case reports, six case series, two review papers, and four combined case series and review papers. There were no randomized controlled trials reported. We then classified treatments used for HOA into two categories: (1) treatment of primary cause (i.e., resection of tumor, chemotherapy, radiotherapy, treatment of infection, etc.) and (2) symptomatic treatments (i.e., bisphosphonates, octreotide, NSAIDs, vagotomy, etc.). Subsequently, we summarized the main findings for each treatment. Although the clinical diagnosis of HOA has existed for over 100 years, the pathogenesis mechanism has not yet been elucidated, and treatment options for this condition remain experimental. Primary treatment is the most widely reported modality to be efficacious. In cases which primary therapy is not possible, several symptomatic treatment modalities are suggested, with various degree of success. Further research is needed to clarify the pathophysiological mechanism of HOA as to appropriately direct therapy.

  13. Magnetic resonance imaging of hypertrophic cardiomyopathy. Evaluation of diastolic function

    International Nuclear Information System (INIS)

    Schwarz, F.; Reiser, M.F.; Theisen, D.; Schwab, F.; Beckmann, B.M.; Schuessler, F.; Kaeaeb, S.; Zinsser, D.; Goelz, T.

    2013-01-01

    Hypertrophic cardiomyopathy (HCM) has a prevalence of approximately 0.2% and is clinically asymptomatic in many patients or presents with unspecific symptoms. This explains the importance of imaging for the diagnosis of HCM as well as for the assessment of the clinical course. The definitive finding in HCM is myocardial hypertrophy with thickening of the ventricular wall ≥ 15 mm. While echocardiography is an excellent screening tool magnetic resonance imaging (MRI) allows a comprehensive analysis of the heart in HCM. This includes a detailed analysis of the distribution and extent of myocardial hypertrophy, a thorough evaluation of systolic and diastolic cardiac function, the assessment of the presence and extent of dynamic outflow tract obstruction as well as the description of the systolic anterior motion (SAM) phenomenon of the mitral valve with secondary mitral insufficiency. When contrast material is administered, additional information about myocardial perfusion as well as the presence and extent of myocardial fibrosis can be obtained. This study compared systolic functional parameters as well as end systolic and end diastolic wall thickness of patients with and without diastolic dysfunction. (orig.) [de

  14. Lysosome-associated hypertrophic cardiomyopathy (Danon's disease in two siblings

    Directory of Open Access Journals (Sweden)

    I. V. Leontyeva

    2015-01-01

    Full Text Available The paper presents a clinical observation of two siblings with Danon's disease (lysosome-associated cardiomyopathy verified by genetic examination. Heart lesion in Danon's disease bears a phenotypic similarity to the primary forms of hypertrophic cardiomyopathy; in this connection the correct etiology of the disease has remained long unestablished. The presence of laboratory markers as the significantly raised levels of transaminases, creatine phosphokinase, and lactate dehydrogenase was as a guide for suspecting the metabolic origin of the disease. Two siblings with a similar LAMP gene mutation were observed to have a different clinical course: a severer clinical course of cardiomyopathy with extreme myocardial hypertrophy, myocardial electric instability, and mental development retardation in one case and a more favorable course in the other; although a 2-year follow-up also revealed negative changes. For the prevention of sudden cardiac death, a cardioverter defibrulator was implanted and continuous therapy with p-adrenoblockers was performed. The specific feature of the cases was no symptoms of skeletal myopathy, moderate mental retardation only in the elder brother, no evidence of an accessory atrioventricular junction despite the fact that there were ECG manifestations of Wolff-Parkinson-White syndrome

  15. Standard guidelines of care: Keloids and hypertrophic scars

    Directory of Open Access Journals (Sweden)

    Somesh Gupta

    2011-01-01

    Full Text Available Keloids and hypertrophic scars (HTS are the result of overgrowth of fibrous tissue, following healing of a cutaneous injury, and cause morbidity. There are several treatment modalities which are useful for the management of keloids, though no single modality is completely effective. The most commonly used modalities are pressure, silicone gel sheet, intralesional steroids, 5-fluorouracil (5 FU, cryotherapy, surgical excision, and lasers. They may be used either singly or, as is done more commonly, in combinations. Any qualified dermatologist who has attained postgraduate qualification in dermatology can treat keloids and HTS. Some procedures, such as cryosurgery and surgical excision, may require additional training in dermatologic surgery. Most modalities for keloids, including intralesional injections and mechanical therapies such as pressure and silicone gel based products, can be given/prescribed on OPD basis. Surgical excision requires a minor operation theater with the facility to handle emergencies. It is important to counsel the patient about the nature of the problem. One should realize that keloid will only improve and not disappear completely. Patients should be informed about the high recurrence rates. Different modalities carry risk of adverse effects and complications and the treating physician needs to be aware of these and patients should be informed about them.

  16. Hypertrophic osteopathy characterized by nuclear scintigraphy in a horse

    International Nuclear Information System (INIS)

    Long, M.T.; Foreman, J.H.; Wallig, M.A.; Chambers, M.D.; Losonsky, J.M.; Muhlbauer, M.C.

    1994-01-01

    A five year old American Saddlebred gelding was admitted to the University of Illinois Veterinary Medical Teaching Hospital with a history of multiple leg lameness, depression and intermittent fever for a duration of six months. Physical examination revealed the horse to be underweight, depressed and a febrile. No abnormalities were detected during auscultation of the heart and lungs. All limbs possessed multiple hard swellings of the distal long bones and digits. The horse walked witha stiff gait and was reluctant to trot. Nuclear scintigraphy of the distal limbs revealed multiple areas of focally increased uptake in all limbs. Radiographs of several sites on the distal limbs showed evidence of periosteal new bone production corresponding to the areas of abnormal uptake. The horse was euthanized and at necropsy chronic, multifocal, fibrous pericarditis and epicarditis were observed grossly. The left atrial myocardium contained areas of osseous metaplasia. Histological evaluation of the distal long bones revealed proliferative periosteal new bone formation consistent with a diagnosis of hypertrophic osteopathy

  17. The changing epidemiology of infantile hypertrophic pyloric stenosis in Scotland.

    Science.gov (United States)

    Sommerfield, T; Chalmers, J; Youngson, G; Heeley, C; Fleming, M; Thomson, G

    2008-12-01

    The aetiology of infantile hypertrophic pyloric stenosis (IHPS) has not been fully elucidated. Since the 1990s, a sharp decline in IHPS has been reported in various countries. Recent research from Sweden reported a correlation between falling rates of IHPS and of sudden infant death syndrome (SIDS). This was attributed to a reduction in the number of infants sleeping in the prone position following the "Back to Sleep" campaign. To describe the changing epidemiology of IHPS in Scotland, to examine the relationship between IHPS and SIDS rates and to examine trends in other factors that may explain the observed reduction in IHPS incidence. Incidence rates of IHPS and SIDS were derived from routine data and their relationship analysed. Trends in mean maternal age, maternal smoking, mean birth weight and breastfeeding rates were also examined. The whole of Scotland between 1981 and 2004. IHPS incidence fell from 4.4 to 1.4 per 1000 live births in Scotland between 1981 and 2004. Rates were consistently higher in males, although the overall incidence patterns in males and females were similar. Rates showed a positive relationship with deprivation. The fall in the incidence of IHPS preceded the fall in SIDS by 2 years and the incidence of SIDS displayed less variability than that of IHPS. Significant temporal trends were also observed in other maternal and infant characteristics. There has been a marked reduction in Scotland's IHPS incidence, but this is unlikely to be a consequence of a change in infant sleeping position.

  18. Invasive alien species water hyacinth Eichhornia crassipes as abode for macroinvertebrates in hypertrophic Ramsar Site, Lake Xochimilco, Mexico.

    Science.gov (United States)

    Rocha-Ramirez, A; Robles-Valderrama, E; Ramirez-Flores, E

    2014-11-01

    This paper presents information on the density, diversity and functional feeding groups of macroinvertebrate assemblages associated with water hyacinth in Antiguo Canal Cuemanco, part of Lake Xochimilco in Mexico City. Rare (low frequency and density) and dominant (high frequency and density) taxa prevailed in the assemblages, with the most predominant being Hyalella azteca, Chironomus plumosus and Ischnura denticollis. Nonmetric Multidimensional Scaling confirmed two climatic seasons: warm-rainy and cold-dry; the former with the highest diversity and density of taxa. Canonical Correspondence Analysis showed that conductivity, nitrates and turbidity explained the density variations of taxa. Antiguo Canal Cuemanco waters are spatially homogeneous with the characteristics of hypertrophic shallow lakes, inhabited by scrapers and gathering-collectors. The species found were tolerant to organic pollution.

  19. Enhanced secretion of TIMP-1 by human hypertrophic scar keratinocytes could contribute to fibrosis.

    Science.gov (United States)

    Simon, Franck; Bergeron, Daniele; Larochelle, Sébastien; Lopez-Vallé, Carlos A; Genest, Hervé; Armour, Alexis; Moulin, Véronique J

    2012-05-01

    Hypertrophic scars are a pathological process characterized by an excessive deposition of extracellular matrix components. Using a tissue-engineered reconstructed human skin (RHS) method, we previously reported that pathological keratinocytes induce formation of a fibrotic dermal matrix. We further investigated keratinocyte action using conditioned media. Results showed that conditioned media induce a similar action on dermal thickness similar to when an epidermis is present. Using a two-dimensional electrophoresis technique, we then compared conditioned media from normal or hypertrophic scar keratinocytes and determined that TIMP-1 was increased in conditioned media from hypertrophic scar keratinocytes. This differential profile was confirmed using ELISA, assaying TIMP-1 presence on media from monolayer cultured keratinocytes and from RHS. The dermal matrix of these RHS was recreated using mesenchymal cells from three different origins (skin, wound and hypertrophic scar). The effect of increased TIMP-1 levels on dermal fibrosis was also validated independently from the mesenchymal cell origin. Immunodetection of TIMP-1 showed that this protein was increased in the epidermis of hypertrophic scar biopsies. The findings of this study represent an important advance in understanding the role of keratinocytes as a direct potent modulator for matrix degradation and scar tissue remodeling, possibly through inactivation of MMPs. Copyright © 2011 Elsevier Ltd and ISBI. All rights reserved.

  20. Osteoarthritis: Control of human cartilage hypertrophic differentiation. Research highlight van: Gremlin1, frizzled-related protein, and Dkk-1 are key regulators of human articular cartilage homeostasis

    NARCIS (Netherlands)

    Buckland, J.; Leijten, Jeroen Christianus Hermanus; van Blitterswijk, Clemens; Karperien, Hermanus Bernardus Johannes

    2012-01-01

    Disruption of articular cartilage homeostasis is important in osteoarthritis (OA) pathogenesis, key to which is activation of articular chondrocyte hypertrophic differentiation. Healthy articular cartilage is resistant to hypertrophic differentiation, whereas growth-plate cartilage is destined to

  1. QT prolongation and sudden cardiac death risk in hypertrophic cardiomyopathy.

    Science.gov (United States)

    Patel, Salma I; Ackerman, Michael J; Shamoun, Fadi E; Geske, Jeffrey B; Ommen, Steve R; Love, William T; Cha, Stephen S; Bos, Johan M; Lester, Steven J

    2018-03-07

    Risk assessment for sudden cardiac death (SCD) in hypertrophic cardiomyopathy (HCM) remains complex. The goal of this study was to assess electrocardiogram (ECG)-derived risk factors on SCD in a large HCM population Methods: Retrospective review of adults with HCM evaluated at Mayo Clinic, Rochester, MN from 1 December 2002 to 31 December 2012 was performed. Data inclusive of ECG and 24-hour ambulatory Holter monitor were assessed. SCD events were documented by ventricular fibrillation (VF) noted on implantable cardioverter defibrillator (ICD), or appropriate VT or VF-terminating ICD shock. Overall, 1615 patients (mean age 53.7 ± 15.2 years; 943 males, 58.4%) were assessed, with mean follow-up 2.46 years and 110 SCD events. Via logistic regression (n = 820), the odds of SCD increased with increasing number of conventional risk factors. With one risk factor the OR was 4.88 (p  450 to this logistic regression model had OR 1.722 (p = .04, CI 1.01-2.937) to predict SCD. QTc ≥ 450 was a significant predictor for death (HR 1.88, p = .021, CI 1.10-3.20). There was no correlation between sinus bradycardia, sinus tachycardia, first degree AV block, atrial fibrillation, left bundle branch block, right bundle branch block, premature atrial complexes, premature ventricular complexes, supraventricular tachycardia, PR interval, QRS interval and SCD. Prolonged QTc was a risk factor for SCD and death even when controlling for typical risk factors.

  2. Biventricular / Left Ventricular Pacing in Hypertrophic Obstructive Cardiomyopathy: An Overview

    Directory of Open Access Journals (Sweden)

    Radu Vatasescu, MD

    2012-05-01

    Full Text Available Hypertrophic cardiomyopathy (HCM is an autosomal dominant inherited genetic disease characterized by compensatory pathological left ventricle (LV hypertrophy due to sarcomere dysfunction. In an important proportion of patients with HCM, the site and extent of cardiac hypertrophy results in severe obstruction to LV outflow tract (LVOT, contributing to disabling symptoms and increasing the risk of sudden cardiac death (SCD. In patients with progressive and/or refractory symptoms despite optimal pharmacological treatment, invasive therapies that diminish or abolish LVOT obstruction relieve heart failure-related symptoms, improve quality of life and could be associated with long-term survival similar to that observed in the general population. The gold standard in this respect is surgical septal myectomy, which might be supplementary associated with a reduction in SCD. Percutaneous techniques, particularly alcohol septal ablation (ASA and more recently radiofrequency (RF septal ablation, can achieve LVOT gradient reduction and symptomatic benefit in a large proportion of HOCM patients at the cost of a supposedly limited septal myocardial necrosis and a 10-20% risk of chronic atrioventricular block. After an initial period of enthusiasm, standard DDD pacing failed to show in randomized trials significant LVOT gradient reductions and objective improvement in exercise capacity. However, case reports and recent small pilot studies suggested that atrial synchronous LV or biventricular (biV pacing significantly reduce LVOT obstruction and improve symptoms (acutely as well as long-term in a large proportion of severely symptomatic HOCM patients not suitable to other gradient reduction therapies. Moreover, biV/LV pacing in HOCM seems to be associated with significant LV reverse remodelling.

  3. Quantitative measurement of hypertrophic scar: interrater reliability and concurrent validity.

    Science.gov (United States)

    Nedelec, Bernadette; Correa, José A; Rachelska, Grazyna; Armour, Alexis; LaSalle, Léo

    2008-01-01

    Research into the pathophysiology and treatment of hypertrophic scar (HSc) remains limited by the heterogeneity of scar and the imprecision with which its severity is measured. The objective of this study was to test the interrater reliability and concurrent validity of the Cutometer measurement of elasticity, the Mexameter measurement of erythema and pigmentation, and total thickness measure of the DermaScan C relative to the modified Vancouver Scar Scale (mVSS) in patient-matched normal skin, normal scar, and HSc. Three independent investigators evaluated 128 sites (severe HSc, moderate or mild HSc, donor site, and normal skin) on 32 burn survivors using all of the above measurement tools. The intraclass correlation coefficient, which was used to measure interrater reliability, reflects the inherent amount of error in the measure and is considered acceptable when it is >0.75. Interrater reliability of the totals of the height, pliability, and vascularity subscales of the mVSS fell below the acceptable limit ( congruent with0.50). The individual subscales of the mVSS fell well below the acceptable level (0.89) for each study site with the exception of severe scar. Mexameter and DermaScan C reliability measurements were acceptable for all sites (>0.82). Concurrent validity correlations with the mVSS were significant except for the comparison of the mVSS pliability subscale and the Cutometer maximum deformation measure comparison in severe scar. In conclusion, the Mexameter and DermaScan C measurements of scar color and thickness of all sites, as well as the Cutometer measurement of elasticity in all but the most severe scars shows high interrater reliability. Their significant concurrent validity with the mVSS confirms that these tools are measuring the same traits as the mVSS, and in a more objective way.

  4. J Waves for Predicting Cardiac Events in Hypertrophic Cardiomyopathy.

    Science.gov (United States)

    Tsuda, Toyonobu; Hayashi, Kenshi; Konno, Tetsuo; Sakata, Kenji; Fujita, Takashi; Hodatsu, Akihiko; Nagata, Yoji; Teramoto, Ryota; Nomura, Akihiro; Tanaka, Yoshihiro; Furusho, Hiroshi; Takamura, Masayuki; Kawashiri, Masa-Aki; Fujino, Noboru; Yamagishi, Masakazu

    2017-10-01

    This study sought to investigate whether the presence of J waves was associated with cardiac events in patients with hypertrophic cardiomyopathy (HCM). It has been uncertain whether the presence of J waves predicts life-threatening cardiac events in patients with HCM. This study evaluated consecutive 338 patients with HCM (207 men; age 61 ± 17 years of age). A J-wave was defined as J-point elevation >0.1 mV in at least 2 contiguous inferior and/or lateral leads. Cardiac events were defined as sudden cardiac death, ventricular fibrillation or sustained ventricular tachycardia, or appropriate implantable cardiac defibrillator therapy. The study also investigated whether adding the J-wave in a conventional risk model improved a prediction of cardiac events. J waves were seen in 46 (13.6%) patients at registration. Cardiac events occurred in 31 patients (9.2%) during median follow-up of 4.9 years (interquartile range: 2.6 to 7.1 years). In a Cox proportional hazards model, the presence of J waves was significantly associated with cardiac events (adjusted hazard ratio: 4.01; 95% confidence interval [CI]: 1.78 to 9.05; p = 0.001). Compared with the conventional risk model, the model using J waves in addition to conventional risks better predicted cardiac events (net reclassification improvement, 0.55; 95% CI: 0.20 to 0.90; p = 0.002). The presence of J waves was significantly associated with cardiac events in HCM. Adding J waves to conventional cardiac risk factors improved prediction of cardiac events. Further confirmatory studies are needed before considering J-point elevation as a marker of risk for use in making management decisions regarding risk in patients with HCM. Copyright © 2017 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

  5. Decreased coronary reserve in patients with hypertrophic cardiomyopathy

    International Nuclear Information System (INIS)

    Yamaguchi, Ryutaro

    1987-01-01

    To assess coronary flow reserve in patients with hypertrophic cardiomyopathy (HCM), split dose thallium-201 dipyridamole (DP) myocardial scintigraphy was performed. Subjects included 30 HCM patients with asymmetric septal hypertrophy and normal coronary angiogram, 10 hypertensive patients with left ventricular hypertrophy (posterior wall thickness ≥ 15 mm) and 13 healthy controls. Coronary reserve index (CRI) was calculated as a ratio of myocardial thallium uptake after dipyridamole (0.5 mg/kg) to the baseline value. HCM patients showed a significantly lower CRI (177 %) as compared with controls (281 %) or hypertensive patients (214 %), and 17 of them had abnormally decreased CRI below the normal range (mean-2SD of controls). These HCM patients with abnormal CRI showed significantly more frequent family histrory of HCM (71 vs 31 %), and a greater degree of systolic narrowing of the septal perforator as compared with those normal CRI. Maximal work loads were significantly lower (82 vs 106 watts) in those with abnormal CRI, 31 % developed ST depression at 80 watts. However, patients with abnormal CRI did not differ from those with normal CRI in septal and posterior wall thickness, left ventricular end-diastolic pressure, and in the degree of systolic narrowing of the left anterior descending artery. In the segmental CRI analysis, 13 HCM patients showed abnormal CRI in the septal and/or apical segments, while 8 patients presented diffuse CRI decrease, including the non-hypertrophied posterior segment. These findings indicate that 57 % of HCM patients have impaired coronary vasodilatory reserve, which may not only be a consequence of left ventricular hypertrophy, elevated left ventricular end-diastolic pressure, and systolic narrowing of the coronary artery, but may be related to small vessel coronary disease. (J.P.N.)

  6. Interaction of Adverse Disease Related Pathways in Hypertrophic Cardiomyopathy.

    Science.gov (United States)

    Rowin, Ethan J; Maron, Martin S; Chan, Raymond H; Hausvater, Anais; Wang, Wendy; Rastegar, Hassan; Maron, Barry J

    2017-12-15

    Hypertrophic cardiomyopathy (HC) has been characterized as a generally progressive genetic heart disease, creating an ominous perspective for patients and managing cardiologists. We explored the HC disease burden and interaction of adverse clinical pathways to clarify patient expectations over long time periods in the contemporary therapeutic era. We studied 1,000 consecutive HC patients (52 ± 17 years) at Tufts Medical Center, followed 9.3 ± 8 years from diagnosis, employing a novel disease pathway model: 46% experienced a benign course free of adverse pathways, but 42% of patients progressed along 1 major pathway, most commonly refractory heart failure to New York Heart Association class III or IV requiring surgical myectomy (or alcohol ablation) or heart transplant; repetitive or permanent atrial fibrillation; and least commonly arrhythmic sudden death events. Eleven percent experienced 2 of these therapeutic end points at different times in their clinical course, most frequently the combination of advanced heart failure and atrial fibrillation, whereas only 1% incurred all 3 pathways. Freedom of progression from 1 to 2 disease pathways, or from 2 to 3 was 80% and 93% at 5 years, respectively. Annual HC-related mortality did not differ according to the number of pathways: 1 (0.8%), 2 (0.8%), or 3 (2.4%) (p = 0.56), and 93% of patients were in New York Heart Association classes I or II at follow-up. In conclusion, it is uncommon for HC patients to experience multiple adverse (but treatable) disease pathways, underscoring the principle that HC is not a uniformly progressive disease. These observations provide a measure of clarity and/or reassurance to patients regarding the true long-term disease burden of HC. Copyright © 2017 Elsevier Inc. All rights reserved.

  7. Cardiovascular magnetic resonance in hypertrophic cardiomyopathy and infiltrative cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Rebecca Schofield

    2016-11-01

    Full Text Available Hypertrophic cardiomyopathy (HCM is the most common inherited cardiac disease. Cardiac imaging plays a key role in the diagnosis and management, with cardiovascular magnetic resonance (CMR an important modality. CMR provides a number of different techniques in one examination: structure and function, flow imaging and tissue characterisation particularly with the late gadolinium enhancement (LGE technique. Other techniques include vasodilator perfusion, mapping (especially T1 mapping and extracellular volume quantification [ECV] and diffusion-weighted imaging with its potential to detect disarray. Clinically, the uses of CMR are diverse. The imaging must be considered within the context of work-up, particularly the personal and family history, Electrocardiogram (ECG and echocardiogram findings. Subtle markers of possible HCM can be identified in genotype positive left ventricular hypertrophy (LVH-negative subjects. CMR has particular advantages for assessment of the left ventricle (LV apex and is able to detect both missed LVH (apical and basal antero-septum, when the echocardiography is normal but the ECG abnormal. CMR is important in distinguishing HCM from both common phenocopies (hypertensive heart disease, athletic adaptation, ageing related changes and rarer pheno and/or genocopies such as Fabry disease and amyloidosis. For these, in particular the LGE technique and T1 mapping are very useful with a low T1 in Fabry’s, and high T1 and very high ECV in amyloidosis. Moreover, the tissue characterisation that is possible using CMR offers a potential role in patient risk stratification, as scar is a very strong predictor of future heart failure. Scar may also play a role in the prediction of sudden death. CMR is helpful in follow-up assessment, especially after septal alcohol ablation and myomectomy.

  8. Determinants of myocardial energetics and efficiency in symptomatic hypertrophic cardiomyopathy

    International Nuclear Information System (INIS)

    Timmer, Stefan A.J.; Germans, Tjeerd; Goette, Marco J.W.; Ruessel, Iris K.; Dijkmans, Pieter A.; Knaapen, Paul; Rossum, Albert C. van; Lubberink, Mark; Lammertsma, Adriaan A.; Berg, Jurrien M. ten; Cate, Folkert J. ten

    2010-01-01

    Next to hypertrophy, hypertrophic cardiomyopathy (HCM) is characterized by alterations in myocardial energetics. A small number of studies have shown that myocardial external efficiency (MEE), defined by external work (EW) in relation to myocardial oxidative metabolism (MVO 2 ), is reduced. The present study was conducted to identify determinants of MEE in patients with HCM by use of dynamic positron emission tomography (PET) and cardiovascular magnetic resonance imaging (CMR). Twenty patients with HCM (12 men, mean age: 55.2 ± 13.9 years) and 11 healthy controls (7 men, mean age: 48.1 ± 10 years) were studied with [ 11 C]acetate PET to assess MVO 2 . CMR was performed to determine left ventricular (LV) volumes and mass (LVM). Univariate and multivariate analyses were employed to determine independent predictors of myocardial efficiency. Between study groups, MVO 2 (controls: 0.12 ± 0.04 ml.min -1 .g -1 , HCM: 0.13 ± 0.05 ml.min -1 .g -1 , p = 0.64) and EW (controls: 9,139 ± 2,484 mmHg.ml, HCM: 9,368 ± 2,907 mmHg.ml, p = 0.83) were comparable, whereas LVM was significantly higher (controls: 99 ± 21 g, HCM: 200 ± 76 g, p 2 -terminal pro-brain natriuretic peptide (NT-proBNP) and serum free fatty acid levels (all p 2 , impaired EW generation per gram of myocardial tissue and subsequent deteriorated myocardial efficiency. Mechanical external efficiency could independently be predicted by SV and LVM. (orig.)

  9. The presence of lysylpyridinoline in the hypertrophic cartilage of newly hatched chicks

    Science.gov (United States)

    Orth, M. W.; Martinez, D. A.; Cook, M. E.; Vailas, A. C.

    1993-01-01

    The presence of lysylpyridinoline (LP) as a nonreducible cross-link in appreciable quantities has primarily been limited to the mineralized tissues, bone and dentin. However, the results reported here show that LP is not only present in the hypertrophic cartilage of the tibiotarsus isolated from newly hatched broiler chicks, but it is approx. 4-fold as concentrated as hydroxylysylpyridinoline (HP). Bone and articular cartilage surrounding the hypertrophic cartilage do not contain measurable quantities of LP. Purified LP has a fluorescent scan similar to purified HP and literature values, confirming that we indeed were measuring LP. Also, the cartilage lesion produced by immature chondrocytes from birds with tibial dyschondroplasia had LP but the HP:LP ratio was > 1. Thus, the low HP:LP ratio could be a marker for hypertrophic cartilage in avians.

  10. Myocardial glucose metabolism in patients with hypertrophic cardiomyopathy. Assessment by F-18-FDG PET study

    International Nuclear Information System (INIS)

    Uehara, Toshiisa; Ishida, Yoshio; Hayashida, Kohei

    1998-01-01

    In an investigation of myocardial metabolic abnormalities in hypertrophic myocardium, the myocardial glucose metabolism was evaluated with F-18-fluorodeoxyglucose (FDG) positron emission tomography (PET) in 32 patients with hypertrophic cardiomyopathy, and the results were compared with those in 9 patients with hypertensive heart disease. F-18-FDG PET study was performed in the fasting and glucose-loading states. The myocardial regional %dose uptake was calculated quantitatively. The average regional %dose uptake in the fasting state in the patients with asymmetric septal hypertrophy and dilated-phase hypertrophic cardiomyopathy was significantly higher than that in the patients with hypertensive heart disease (0.75±0.34%, 0.65±0.25%, and 0.43±0.22%/100 g myocardium, respectively). In contrast, the average %dose uptake in the glucose-loading state in the patients with asymmetric septal hypertrophy and dilated-phase hypertrophic cardiomyopathy was not significantly different from that in patients with hypertensive heart disease (1.17±0.49%, 0.80±0.44% and 0.99±0.45%, respectively). The patients with apical hypertrophy had also low %dose uptake in the fasting state (0.38±0.21%) as in the hypertensive heart disease patients, so that the characteristics of asymmetric septal hypertrophy and dilated-phase hypertrophic cardiomyopathy are considered to be high FDG uptake throughout the myocardium in the fasting state. Patients with apical hypertrophy are considered to belong to other disease categories metabolically. F-18-FDG PET study is useful in the evaluation of the pathophysiologic diagnosis of patients with hypertrophic cardiomyopathy. (author)

  11. Hypertrophic Pachymeningitis and the Syndrome of Inappropriate Antidiuretic Hormone Secretion: Coincidence or Cause?

    Science.gov (United States)

    Harsch, Igor Alexander; Schiffer, Anne; Konturek, Peter C

    2017-01-01

    To investigate a potential cause of the syndrome of inappropriate antidiuretic hormone secretion (SIADH). A 70-year-old female patient had nausea and collapsed. Although euvolemic, pathological laboratory findings showed hyponatremia and hypoosmolality, and cerebral magnetic resonance imaging showed hypertrophic pachymeningitis. Secondary hypertrophic pachymeningitis was excluded. Other nonneurological reasons for SIADH were also excluded. Moderate fluid restriction restored an almost normal serum osmolality and sodium. This case of SIADH was conservatively treated with moderate fluid restriction that almost restored normal serum osmolality and sodium levels. © 2017 S. Karger AG, Basel.

  12. Myectomy and LA-to-LV Conduit for Severe Calcific Mitral Stenosis and Hypertrophic Cardiomyopathy.

    Science.gov (United States)

    Meghji, Zahara; Nguyen, Anita; Geske, Jeffrey B; Schaff, Hartzell V

    2018-02-26

    Severe calcific mitral valve stenosis can rarely occur concomitantly with obstructive hypertrophic cardiomyopathy. In these patients, surgical decalcification of the stenotic mitral valve followed by mitral valve replacement carries significant operative risk and may result in paravalvular leakage, atrioventricular groove disruption, and excessive bleeding. We report the first 2 cases of obstructive hypertrophic cardiomyopathy with severe calcific mitral valve stenosis successfully treated with concomitant transaortic septal myectomy and bypass of the stenotic mitral valve using a valved left atrium to left ventricular conduit. Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  13. Management of an asymptomatic patient with the apical variant of hypertrophic cardiomyopathy.

    Science.gov (United States)

    Trojan, Meghan K Borden; Biederman, Robert W

    2017-07-01

    Healthcare professionals are faced with challenging decisions regarding patient evaluation and management on a daily basis. Once a diagnosis is made, additional challenges include how to proceed with the management. Here, we present an eighty-two-year-old female who was incidentally diagnosed with the apical variant of hypertrophic cardiomyopathy on a transthoracic echocardiogram. She was found to have newly diagnosed atrial fibrillation, but was otherwise asymptomatic from a cardiomyopathy standpoint. No specific guidelines exist for this patient population. Therefore, how does one proceed with the management of an asymptomatic patient with the apical variant of hypertrophic cardiomyopathy? © 2017, Wiley Periodicals, Inc.

  14. Biventricular Hypertrophic Cardiomyopathy in a Child with LEOPARD Syndrome: a Case Report

    Directory of Open Access Journals (Sweden)

    Blesneac Cristina

    2017-12-01

    Full Text Available Background: LEOPARD syndrome is a complex dysmorphogenetic disorder of inconstant penetrance and various morphologic expressions. The syndrome is an autosomal dominant disease that features multiple lentigines, electrocardiographic changes, eye hypertelorism, pulmonary valve stenosis or hypertrophic cardiomyopathy, genital malformations, and a delayed constitutional growth hearing loss, which can be associated with rapidly progressive severe biventricular obstructive hypertrophic cardiomyopathy. No epidemiologic data are available on the real incidence of LEOPARD syndrome; however, this seems to be a rare disease, being often underdiagnosed, as many of its features are mild.

  15. Hypertrophic scars and keloids in surgery: current concepts.

    Science.gov (United States)

    Song, Colin

    2014-09-01

    Hypertrophic scars and keloids remain a challenge in surgery. We appreciate that our understanding of the process at cellular and molecular level, profound as it is, when it comes to the clinical evidence much is left to be desired. Although the bench to bedside conundrum remains, the science of translational research calls for an even higher level of cooperation between the scientist and the clinician for the impetus to succeed.The clinicians alerted us to the possible theories in the pathogenesis of keloid formation, inter alia, the ischemia theory, mast cell theory, immune theory, transforming growth factor β interaction, mechanical theory, and the melanocyte stimulating hormone theory. All of the above presupposed a stimulus that would result in an uncontrolled upregulation of collagen and extracellular matrix expression in the pathogenesis of the keloid. This bedside to bench initiative, as in true science, realized more ponderables than possibilities.By the same token, research into the epidermal-mesenchymal signaling, molecular biology, genomics, and stem cell research holds much promise in the bench top arena. To assess efficacy, many scar assessment scores exist in the literature. The clinical measurement of scar maturity can aid in determining end points for therapeutics. Tissue oxygen tension and color assessment of scars by standardized photography proved to be useful.In surgery, the use of dermal substitutes holds some promise as we surmise that quality scars that arise from dermal elements, molecular and enzyme behavior, and balance. Although a systematic review shows some benefit for earlier closure and healing of wounds, no such review exists at this point in time for the use of dermal substitutes in scars.Adipose-derived stem cell, as it pertains to scars, will hopefully realize the potential of skin regeneration rather than by repair in which we are familiar with as well as the undesirable scarring as a result of healing through the inflammatory

  16. Quantitative measurement of hypertrophic scar: intrarater reliability, sensitivity, and specificity.

    Science.gov (United States)

    Nedelec, Bernadette; Correa, José A; Rachelska, Grazyna; Armour, Alexis; LaSalle, Léo

    2008-01-01

    The comparison of scar evaluation over time requires measurement tools with acceptable intrarater reliability and the ability to discriminate skin characteristics of interest. The objective of this study was to evaluate the intrarater reliability and sensitivity and specificity of the Cutometer, the Mexameter, and the DermaScan C relative to the modified Vancouver Scar Scale (mVSS) in patient-matched normal skin, normal scar (donor sites), and hypertrophic scar (HSc). A single investigator evaluated four tissue types (severe HSc, less severe HSc, donor site, and normal skin) in 30 burn survivors with all four measurement tools. The intraclass correlation coefficient (ICC) for the Cutometer was acceptable (> or =0.75) for the maximum deformation measure for the donor site and normal skin (>0.78) but was below the acceptable range for the HSc sites and all other parameters. The ICC for the Mexameter erythema (>0.75) and melanin index (>0.89) and the DermaScan C total thickness measurement (>0.82) were acceptable for all sites. The ICC for the total of the height, pliability, and vascularity subscales of the mVSS was acceptable (0.81) for normal scar but below the acceptable range for the scar sites. The DermaScan C was clearly able to discriminate HSc from normal scar and normal skin based on the total thickness measure. The Cutometer was less discriminating but was still able to discriminate HSc from normal scar and normal skin. The Mexameter erythema index was not a good discriminator of HSc and normal scar. Receiver operating characteristic curves were generated to establish the best cutoff point for the DermaScan C total thickness and the Cutometer maximum deformation, which were 2.034 and 0.387 mm, respectively. This study showed that although the Cutometer, the DermaScan C, and the Mexameter have measurement properties that make them attractive substitutes for the mVSS, caution must be used when interpreting results since the Cutometer has a ceiling effect when

  17. Right ventricular mechanics in hypertrophic cardiomyopathy using feature tracking

    Science.gov (United States)

    Badran, Hala Mahfouz; Soliman, Mahmood; Hassan, Hesham; Abdelfatah, Raed; Saadan, Haythem; Yacoub, Magdi

    2013-01-01

    Objectives: Right ventricular (RV) mechanics in hypertrophic cardiomyopathy (HCM) are poorly understood. We investigate global and regional deformation of the RV in HCM and its relationship to LV phenotype, using 2D strain vector velocity imaging (VVI). Methods: 100 HCM patients (42% females, 41 ± 19 years) and 30 control patients were studied using VVI. Longitudinal peak systolic strain (ϵsys), strain rate (SR), time to peak (ϵ) (TTP), displacement of RV free wall (RVFW) and septal wall were analyzed. Similar parameters were quantified in LV septal, lateral, anterior and inferior segments. Intra-V-delay was defined as SD of TTP. Inter-V-delay was estimated from TTP difference between the most delayed LV segment & RVFW. Results: ϵsys and SR of both RV & LV, showed loss of base to apex gradient and significant decline in HCM (p < 0.001). Deformation variables estimated from RVFW were strongly correlated with each other (r = 0.93, p < 0.0001). Both were directly related to LV ϵsys, SRsys, SRe, ejection fraction (EF)%, RVFW displacement (P < 0.001) and inversely related to age, positive family history (p < 0.004, 0.005), RV wall thickness, maximum wall thickness (MWT), intra-V-delay, LA volume (P < 0.0001), LVOT gradient (p < 0.02, 0.005) respectively. ROC curves were constructed to explore the cut-off point that discriminates RV dysfunction. Global and RVFW ϵsys: − 19.5% shows 77, 70% sensitivity & 97% specificity, SRsys: − 1.3s− 1 shows 82, 70% sensitivity & 30% specificity. Multivariate analyses revealed that RVFW displacement (β = − 0.9, p < 0.0001) and global LV SRsys (β = 5.9, p < 0.0001) are independent predictors of global RV deformation. Conclusions: Impairment of RV deformation is evident in HCM using feature tracking. It is independently influenced by LV mechanics and correlated to the severity of LV phenotype. RVFW deformation analysis and global RV assessment are comparable. PMID:24689019

  18. Contemporary Natural History and Management of Nonobstructive Hypertrophic Cardiomyopathy.

    Science.gov (United States)

    Maron, Martin S; Rowin, Ethan J; Olivotto, Iacopo; Casey, Susan A; Arretini, Anna; Tomberli, Benedetta; Garberich, Ross F; Link, Mark S; Chan, Raymond H M; Lesser, John R; Maron, Barry J

    2016-03-29

    Left ventricular outflow tract gradients are absent in an important proportion of patients with hypertrophic cardiomyopathy (HCM). However, the natural course of this important patient subgroup remains largely unresolved. The authors systematically employed exercise (stress) echocardiography to define those patients without obstruction to left ventricular outflow at rest and/or under physiological exercise and to examine their natural history and clinical course to create a more robust understanding of this complex disease. We prospectively studied 573 consecutive HCM patients in 3 centers (44 ± 17 years; 66% male) with New York Heart Association functional class I/II symptoms at study entry, including 249 in whom left ventricular outflow tract obstruction was absent both at rest and following physiological exercise (<30 mm Hg; nonobstructive HCM) and retrospectively assembled clinical follow-up data. Over a median follow-up of 6.5 years, 225 of 249 nonobstructive patients (90%) remained in classes I/II, whereas 24 (10%) developed progressive heart failure to New York Heart Association functional classes III/IV. Nonobstructive HCM patients were less likely to experience advanced limiting class III/IV symptoms than the 324 patients with outflow obstruction (1.6%/year vs. 7.4%/year rest obstruction vs. 3.2%/year provocable obstruction; p < 0.001). However, 7 nonobstructive patients (2.8%) did require heart transplantation for progression to end stage versus none of the obstructive patients. HCM-related mortality among nonobstructive patients was low (n = 8; 0.5%/year), with 5- and 10-year survival rates of 99% and 97%, respectively, which is not different from expected all-cause mortality in an age- and sex-matched U.S. population (p = 0.15). HCM patients with nonobstructive disease appear to experience a relatively benign clinical course, associated with a low risk for advanced heart failure symptoms, other disease complications, and HCM-related mortality, and

  19. Left Atrial Mechanical Function and Global Strain in Hypertrophic Cardiomyopathy.

    Directory of Open Access Journals (Sweden)

    Kyung-Jin Kim

    Full Text Available Atrial fibrillation is the most common arrhythmia and is associated with adverse outcomes in hypertrophic cardiomyopathy (HCM. Although left atrial (LA remodeling and dysfunction are known to associate with the development of atrial fibrillation in HCM, the changes of the LA in HCM patients remain unclear. This study aimed to evaluate the changes in LA size and mechanical function in HCM patients compared to control subjects and to determine the characteristics of HCM associated with LA remodeling and dysfunction.Seventy-nine HCM patients (mean age, 54 ± 11 years; 76% were men were compared to 79 age- and sex-matched controls (mean age, 54 ± 11 years; 76% were men and 20 young healthy controls (mean age, 33 ± 5 years; 45% were men. The LA diameter, volume, and mechanical function, including global strain (ε, were evaluated by 2D-speckle tracking echocardiography. The phenotype of HCM, maximal left ventricular (LV wall thickness, LV mass, and presence and extent of late gadolinium enhancement (LGE were evaluated with cardiac magnetic resonance imaging.HCM patients showed increased LA volume index, impaired reservoir function, and decreased LA ε compared to the control subjects. When we divided the HCM group according to a maximal LA volume index (LAVImax of 38.7 ml/m2 or LA ε of 21%, no significant differences in the HCM phenotype and maximal LV wall thickness were observed for patients with LAVImax >38.7 ml/m2 or LA ε ≤21%. Conversely, the LV mass index was significantly higher both in patients with maximal LA volume index >38.7 ml/m2 and with LA ε ≤21% and was independently associated with LAVImax and LA ε. Although the LGE extent was increased in patients with LA ε ≤21%, it was not independently associated with either LAVImax or LA ε.HCM patients showed progressed LA remodeling and dysfunction; the determinant of LA remodeling and dysfunction was LV mass index rather than LV myocardial fibrosis by LGE-magnetic resonance

  20. MicroRNA-140 Provides Robustness to the Regulation of Hypertrophic Chondrocyte Differentiation by the PTHrP-HDAC4 Pathway.

    Science.gov (United States)

    Papaioannou, Garyfallia; Mirzamohammadi, Fatemeh; Lisse, Thomas S; Nishimori, Shigeki; Wein, Marc N; Kobayashi, Tatsuya

    2015-06-01

    Growth plate chondrocytes go through multiple differentiation steps and eventually become hypertrophic chondrocytes. The parathyroid hormone (PTH)-related peptide (PTHrP) signaling pathway plays a central role in regulation of hypertrophic differentiation, at least in part, through enhancing activity of histone deacetylase 4 (HDAC4), a negative regulator of MEF2 transcription factors that drive hypertrophy. We have previously shown that loss of the chondrocyte-specific microRNA (miRNA), miR-140, alters chondrocyte differentiation including mild acceleration of hypertrophic differentiation. Here, we provide evidence that miR-140 interacts with the PTHrP-HDAC4 pathway to control chondrocyte differentiation. Heterozygosity of PTHrP or HDAC4 substantially impaired animal growth in miR-140 deficiency, whereas these mutations had no effect in the presence of miR-140. miR-140-deficient chondrocytes showed increased MEF2C expression with normal levels of total and phosphorylated HDAC4, indicating that the miR-140 pathway merges with the PTHrP-HDAC4 pathway at the level of MEF2C. miR-140 negatively regulated p38 mitogen-activated protein kinase (MAPK) signaling, and inhibition of p38 MAPK signaling reduced MEF2C expression. These results demonstrate that miR-140 ensures the robustness of the PTHrP/HDAC4 regulatory system by suppressing MEF2C-inducing stimuli. © 2014 American Society for Bone and Mineral Research © 2015 American Society for Bone and Mineral Research. © 2014 American Society for Bone and Mineral Research.

  1. Altering the architecture of tissue engineered hypertrophic cartilaginous grafts facilitates vascularisation and accelerates mineralisation.

    Directory of Open Access Journals (Sweden)

    Eamon J Sheehy

    Full Text Available Cartilaginous tissues engineered using mesenchymal stem cells (MSCs can be leveraged to generate bone in vivo by executing an endochondral program, leading to increased interest in the use of such hypertrophic grafts for the regeneration of osseous defects. During normal skeletogenesis, canals within the developing hypertrophic cartilage play a key role in facilitating endochondral ossification. Inspired by this developmental feature, the objective of this study was to promote endochondral ossification of an engineered cartilaginous construct through modification of scaffold architecture. Our hypothesis was that the introduction of channels into MSC-seeded hydrogels would firstly facilitate the in vitro development of scaled-up hypertrophic cartilaginous tissues, and secondly would accelerate vascularisation and mineralisation of the graft in vivo. MSCs were encapsulated into hydrogels containing either an array of micro-channels, or into non-channelled 'solid' controls, and maintained in culture conditions known to promote a hypertrophic cartilaginous phenotype. Solid constructs accumulated significantly more sGAG and collagen in vitro, while channelled constructs accumulated significantly more calcium. In vivo, the channels acted as conduits for vascularisation and accelerated mineralisation of the engineered graft. Cartilaginous tissue within the channels underwent endochondral ossification, producing lamellar bone surrounding a hematopoietic marrow component. This study highlights the potential of utilising engineering methodologies, inspired by developmental skeletal processes, in order to enhance endochondral bone regeneration strategies.

  2. Altering the architecture of tissue engineered hypertrophic cartilaginous grafts facilitates vascularisation and accelerates mineralisation.

    Science.gov (United States)

    Sheehy, Eamon J; Vinardell, Tatiana; Toner, Mary E; Buckley, Conor T; Kelly, Daniel J

    2014-01-01

    Cartilaginous tissues engineered using mesenchymal stem cells (MSCs) can be leveraged to generate bone in vivo by executing an endochondral program, leading to increased interest in the use of such hypertrophic grafts for the regeneration of osseous defects. During normal skeletogenesis, canals within the developing hypertrophic cartilage play a key role in facilitating endochondral ossification. Inspired by this developmental feature, the objective of this study was to promote endochondral ossification of an engineered cartilaginous construct through modification of scaffold architecture. Our hypothesis was that the introduction of channels into MSC-seeded hydrogels would firstly facilitate the in vitro development of scaled-up hypertrophic cartilaginous tissues, and secondly would accelerate vascularisation and mineralisation of the graft in vivo. MSCs were encapsulated into hydrogels containing either an array of micro-channels, or into non-channelled 'solid' controls, and maintained in culture conditions known to promote a hypertrophic cartilaginous phenotype. Solid constructs accumulated significantly more sGAG and collagen in vitro, while channelled constructs accumulated significantly more calcium. In vivo, the channels acted as conduits for vascularisation and accelerated mineralisation of the engineered graft. Cartilaginous tissue within the channels underwent endochondral ossification, producing lamellar bone surrounding a hematopoietic marrow component. This study highlights the potential of utilising engineering methodologies, inspired by developmental skeletal processes, in order to enhance endochondral bone regeneration strategies.

  3. Osteogenic Treatment Initiating a Tissue-Engineered Cartilage Template Hypertrophic Transition.

    Science.gov (United States)

    Fu, J Y; Lim, S Y; He, P F; Fan, C J; Wang, D A

    2016-10-01

    Hypertrophic chondrocytes play a critical role in endochondral bone formation as well as the progress of osteoarthritis (OA). An in vitro cartilage hypertrophy model can be used as a platform to study complex molecular mechanisms involved in these processes and screen new drugs for OA. To develop an in vitro cartilage hypertrophy model, we treated a tissue-engineered cartilage template, living hyaline cartilaginous graft (LhCG), with osteogenic medium for hypertrophic induction. In addition, endothelial progenitor cells (EPCs) were seeded onto LhCG constructs to mimic vascular invasion. The results showed that osteogenic treatment significantly inhibited the synthesis of endostatin in LhCG constructs and enhanced expression of hypertrophic marker-collagen type X (Col X) and osteogenic markers, as well as calcium deposition in vitro. Upon subcutaneous implantation, osteogenic medium-treated LhCG constructs all stained positive for Col X and showed significant calcium deposition and blood vessel invasion. Col X staining and calcium deposition were most obvious in osteogenic medium-treated only group, while there was no difference between EPC-seeded and non-seeded group. These results demonstrated that osteogenic treatment was of the primary factor to induce hypertrophic transition of LhCG constructs and this model may contribute to the establishment of an in vitro cartilage hypertrophy model.

  4. Hypertrophic Cardiomyopathy Associated with Mid-cavity Obstruction and High Left Intraventricular Pressure

    Science.gov (United States)

    A. Bejiqi, Ramush; J. Retkoceri, Ragip; Sh. Bejiqi, Hana

    2011-01-01

    We report a case of a child, with a rare form of the idiopathic hypertrophic cardiomyopathy, associated with mid-cavity obstruction and high intraventricular peak pressure. Cardiomyopathy, diagnosed antenataly, was followed postnataly and, despite of a lot echocardiographic findings - the growing, development and clinical signs are minimal. PMID:23407799

  5. Hypertrophic Cardiomyopathy Associated with Mid-cavity Obstruction and High Left Intraventricular Pressure

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    A. Bejiqi, Ramush; J. Retkoceri, Ragip; Sh. Bejiqi, Hana

    2011-01-01

    We report a case of a child, with a rare form of the idiopathic hypertrophic cardiomyopathy, associated with mid-cavity obstruction and high intraventricular peak pressure. Cardiomyopathy, diagnosed antenataly, was followed postnataly and, despite of a lot echocardiographic findings - the growing, development and clinical signs are minimal.

  6. Enhanced in Vivo Delivery of 5-Fluorouracil by Ethosomal Gels in Rabbit Ear Hypertrophic Scar Model

    Directory of Open Access Journals (Sweden)

    Yan Wo

    2014-12-01

    Full Text Available Applying Ethosomal Gels (EGs in transdermal drug delivery systems has evoked considerable interest because of their good water-solubility and biocompatibility. However, there has not been an explicit description of applying EGs as a vehicle for hypertrophic scars treatment. Here, a novel transdermal EGs loaded with 5-fluorouracil (5-FU EGs was successfully prepared and characterized. The stability assay in vitro revealed that 5-FU EGs stored for a period of 30 days at 4 ± 1 °C had a better size stability than that at 25 ± 1 °C. Furthermore, using confocal laser scanning microscopy, EGs labeled with Rhodamine 6 G penetrated into the deep dermis of the hypertrophic scar within 24 h in the rabbit ear hypertrophic model suggested that the EGs were an optional delivery carrier through scar tissues. In addition, the value of the Scar Elevation Index (SEI of 5-FU EGs group in the rabbit ear scar model was lower than that of 5-FU Phosphate Buffered Saline gel and Control groups. To conclude, these results suggest that EGs delivery system loaded 5-fluorouracil is a perfect candidate drug for hypertrophic scars therapy in future.

  7. Evidences of autologous fat grafting for the treatment of keloids and hypertrophic scars

    Directory of Open Access Journals (Sweden)

    VINÍCIUS ZOLEZI DA SILVA

    Full Text Available SUMMARY Introduction Since the 1980s, the use of autologous fat grafting has been growing in plastic surgery. Recently, this procedure has come to be used as a treatment for keloids and hypertrophic scars mainly due to the lack of satisfactory results with other techniques. So far, however, it lacks more consistent scientific evidence to recommend its use. The aim of this study was to review the current state of autologous fat grafting for the treatment of keloids and hypertrophic scars, their benefits and scientific evidences in the literature. Method A review in the Pubmed database was performed using the keywords “fat grafting and scar”, “fat grafting and keloid scar” and “fat grafting and hypertrophic scar.” Inclusion criteria were articles written in English and published in the last 10 years, resulting in 15 studies. Results These articles indicate that autologous fat grafting carried out at sites with pathological scars leads to a reduction of the fibrosis and pain, an increased range of movement in areas of scar contraction, an increase in their flexibility, resulting in a better quality of scars. Conclusion So far, evidences suggest that autologous fat grafting for the treatment of keloids and hypertrophic scars is associated with a better quality of scars, leading to esthetic and functional benefits. However, this review has limitations and these findings should be treated with reservations, since they mostly came from studies with low levels of evidence.

  8. Development of infantile hypertrophic pyloric stenosis in patients treated for oesophageal atresia. A case report

    DEFF Research Database (Denmark)

    Qvist, N; Rasmussen, L; Hansen, L P

    1986-01-01

    Two cases of infantile hypertrophic pyloric stenosis (IHPS) developed in 74 patients treated for oesophageal atresia. Treatment of oesophageal atresia is frequently followed by vomiting and failure to thrive due to gastrooesophageal reflux or anastomotic stricture. The diagnose of IHPS must...

  9. Establishing a Reproducible Hypertrophic Scar following Thermal Injury: A Porcine Model

    Directory of Open Access Journals (Sweden)

    Scott J. Rapp, MD

    2015-02-01

    Conclusions: Deep partial-thickness thermal injury to the back of domestic swine produces an immature hypertrophic scar by 10 weeks following burn with thickness appearing to coincide with the location along the dorsal axis. With minimal pig to pig variation, we describe our technique to provide a testable immature scar model.

  10. Successful treatment of hypertrophic lichen planus with betamethasone under occlusion and TCA-peelings.

    Science.gov (United States)

    Theodosiou, Grigorios; Papageorgiou, Marina; Vakirlis, Efstratios; Mandekou-Lefaki, Ioanna

    2016-09-01

    Hypertrophic lichen planus (HLP) is a variant of lichen planus characterized by marked epidermal hyperplasia and severe pruritus. We present a case of a female patient with HLP and concomitant primary biliary cirrhosis, which responded to topical therapy with betamethasone under occlusion and TCA-peelings. © 2016 Wiley Periodicals, Inc.

  11. Genetic Counseling and Cardiac Care in Predictively Tested Hypertrophic Cardiomyopathy Mutation Carriers: The Patients' Perspective

    NARCIS (Netherlands)

    Christiaans, Imke; van Langen, Irene M.; Birnie, Erwin; Bonsel, Gouke J.; Wilde, Arthur A. M.; Smets, Ellen M. A.

    2009-01-01

    Hypertrophic cardiomyopathy (HCM) is a common hereditary heart disease associated with sudden cardiac death. predictive genetic counseling and testing are performed using adapted Huntington guidelines, that is, psychosocial care and time for reflection are not obligatory and the test result can be

  12. The effect of burn rehabilitation massage therapy on hypertrophic scar after burn: a randomized controlled trial.

    Science.gov (United States)

    Cho, Yoon Soo; Jeon, Jong Hyun; Hong, Aram; Yang, Hyeong Tae; Yim, Haejun; Cho, Yong Suk; Kim, Do-Hern; Hur, Jun; Kim, Jong Hyun; Chun, Wook; Lee, Boung Chul; Seo, Cheong Hoon

    2014-12-01

    To evaluate the effect of burn rehabilitation massage therapy on hypertrophic scar after burn. One hundred and forty-six burn patients with hypertrophic scar(s) were randomly divided into an experimental group and a control group. All patients received standard rehabilitation therapy for hypertrophic scars and 76 patients (massage group) additionally received burn scar rehabilitation massage therapy. Both before and after the treatment, we determined the scores of visual analog scale (VAS) and itching scale and assessed the scar characteristics of thickness, melanin, erythema, transepidermal water loss (TEWL), sebum, and elasticity by using ultrasonography, Mexameter(®), Tewameter(®), Sebumeter(®), and Cutometer(®), respectively. The scores of both VAS and itching scale decreased significantly in both groups, indicating a significant intragroup difference. With regard to the scar characteristics, the massage group showed a significant decrease after treatment in scar thickness, melanin, erythema, TEWL and a significant intergroup difference. In terms of scar elasticity, a significant intergroup difference was noted in immediate distension and gross skin elasticity, while the massage group significant improvement in skin distensibility, immediate distension, immediate retraction, and delayed distension. Our results suggest that burn rehabilitation massage therapy is effective in improving pain, pruritus, and scar characteristics in hypertrophic scars after burn. Copyright © 2014 Elsevier Ltd and ISBI. All rights reserved.

  13. Multivariate-Analysis of Phytoplankton and Related Environmental-Factors, in a Shallow Hypertrophic Lake

    NARCIS (Netherlands)

    Romo, S.; Van Tongeren, O.F.R.

    1995-01-01

    Data on some relevant environmental variables and phytoplankton species composition, collected from the hypertrophic shallow lake Albufera of Valencia (Spain) during 1980-88, were examined using Redundancy Analysis (RDA). The hydrological cycle of the lake is manipulated for rice cultivation in the

  14. Initial results of combined anterior mitral leaflet extension and myectomy in patients with obstructive hypertrophic cardiomyopathy

    NARCIS (Netherlands)

    M.J.M. Kofflard (Marcel); L.A. van Herwerden (Lex); D.J. Waldstein; P.N. Ruygrok (Peter); H. Boersma (Eric); M.A. Taams (Meindert); F.J. ten Cate (Folkert)

    1996-01-01

    textabstractObjectives. The purpose of this study was to describe the clinical and functional results of combined anterior mitral leaflet extension and myectomy in patients with hypertrophic obstructive cardiomyopathy. Background. Septal myectomy is the most commonly performed surgical procedure in

  15. Hypertrophic scars and keloids: a review of the current literature on ...

    African Journals Online (AJOL)

    Conclusion: The growing understanding of the molecular processes of normal and abnormal wound healing is promising for discovery of novel approaches for the management of hypertrophic scars and keloids. Although optimal treatment of these lesions remains undefined, successful healing can be achieved only with ...

  16. Radiographic and ultrasonographic features of hypertrophic feline muscular dystrophy in two cats

    International Nuclear Information System (INIS)

    Berry, C.R.; Gaschen, F.P.; Ackerman, N.

    1992-01-01

    Hypertrophic fellne musculer dystrophy has been reported as an X-linked inherited deficiency of a cytoskeletal myofiber protein called dystrophin. This report deserlbes the radiographic and ultrasonographic abnormalities of two male littermate domestic short-hair cats and reviews the previous reported findings assoclated with hypertrophic feline muscular dystrophy. The thoracic radiographic abnormalities included: progressive cardiomegaly, large convex, scalloped irregularities associated with the vetral aspect of the diaphragm, and variable degrees of esophageal dilation (megaesophagus) with associated cranioventral aspiration pneumonia. Echocardiographic features included: concentric left vetricular wall thickening, increased left ventricular and diastolic and systolic dimensions, and an increase in endocardial echogenicity. Abdominal radiographic abnormalities included: hepatosplenomegaly, peritoneal effusion, renomegaly, adrenal gland mineralization, and paralumbar and diaphragmatic musculature enlargement. Abdomlnal ultrasonographic abnormalities included: irregularly thickened muscular portion of the diaphragm; hypoechogenicity of the liver; peritoneal effusion; hepatosplenomegaly; renomegaly with hyperechoic cortex and medulla; and adrenal gland mineralization. The irregular scalloped appearance of the diaphragm (particularly along the ventral/sternal margin) was a consistenl radiographic abnormlity in the two cats with hypertrophic feline muscular dystrophy after the age of 7 months. This finding was confirmed by ultrasound as a thickened irregular, hyperechoic diaphragm. A diagnosis of hypertrophic feline muscular dystrophy should be strongly suspected if this abnormality is identified

  17. Electrocardiographic features of sarcomere mutation carriers with and without clinically overt hypertrophic cardiomyopathy

    DEFF Research Database (Denmark)

    Lakdawala, Neal K; Thune, Jens Jakob; Maron, Barry J

    2011-01-01

    In hypertrophic cardiomyopathy (HC), electrocardiographic (ECG) changes have been postulated to be an early marker of disease, detectable in sarcomere mutation carriers when left ventricular (LV) wall thickness is still normal. However, the ECG features of mutation carriers have not been fully...

  18. Risk factors for hypertrophic burn scar pain, pruritus, and paresthesia development.

    Science.gov (United States)

    Xiao, Yongqiang; Sun, Yu; Zhu, Banghui; Wang, Kangan; Liang, Pengfei; Liu, Wenjun; Fu, Jinfeng; Zheng, Shiqing; Xiao, Shichu; Xia, Zhaofan

    2018-05-02

    Hypertrophic scar pain, pruritus, and paresthesia symptoms are major and particular concerns for burn patients. However, because no effective and satisfactory methods exist for their alleviation, the clinical treatment for these symptoms is generally considered unsatisfactory. Therefore, their risk factors should be identified and prevented during management. We reviewed the medical records of 129 post-burn hypertrophy scar patients and divided them into two groups for each of three different symptoms based on the University of North Carolina "4P" Scar Scale: patients with scar pain requiring occasional or continuous pharmacological intervention (HSc pain, n=75) vs. patients without such scar pain (No HSc pain, n=54); patients with scar pruritus requiring occasional or continuous pharmacological intervention (HSc pruritus, n=63) vs. patients without such scar pruritus (No HSc pruritus, n=66); patients with scar paresthesia that influenced the patients' daily activities (HSc paresthesia, n=31) vs. patients without such scar paresthesia (No HSc paresthesia, n=98). Three multivariable logistic regression models were built, respectively, to identify the risk factors for hypertrophic burn scar pain, pruritus, and paresthesia development. Multivariable analysis showed that hypertrophic burn scar pain development requiring pharmacological intervention was associated with old age (odds ratio [OR]=1.046; 95% confidence interval [CI], 1.011-1.082, p=0.009), high body mass index(OR=1.242; 95%CI,1.068-1.445, p=0.005), 2-5-mm-thick post-burn hypertrophic scars (OR=3.997; 95%CI, 1.523-10.487; p=0.005), and 6-12-month post-burn hypertrophic scars (OR=4.686; 95%CI; 1.318-16.653; p=0.017). Hypertrophic burn scar pruritus development requiring pharmacological intervention was associated with smoking (OR=3.239; 95%CI, 1.380-7.603; p=0.007), having undergone surgical operation (OR=2.236; 95%CI, 1.001-4.998; p=0.049), and firm scars (OR=3.317; 95%CI, 1.237-8.894; p=0.017). Finally

  19. [Sigmoid septum: A variant of the ventricular hypertrophy or of the hypertrophic cardiomyopathy?].

    Science.gov (United States)

    Gentille-Lorente, Delicia; Salvadó-Usach, Teresa

    2016-01-01

    Sigmoid septum and hypertrophic cardiomyopathy presenting with left ventricular hypertrophy and, although they appear to be different entities, often involve problems in the differential diagnosis. This study was carried out to assess the prevalence and characteristics of the echocardiographic sigmoid septum and its differential findings regarding hypertrophic cardiomyopathy. Descriptive, observational and prospective study. A total of 1,770 patients were studied by echocardiography. Sigmoid septum (focal and isolated hypertrophy of the basal interventricular septum≥13mm in men and ≥12mm in women, exceeding ≥50% of the median septum thickness) was classified as «Type 1» (≤14mm) and «Type 2» (≥15mm). There were 59 cases of sigmoid septum (prevalence of 3.3%): 26 (1.5%) patients with type 1 (50% male) and 33 (1.9%) patients with type 2 (72.7% male); there were 25 (1.4%) cases of hypertrophic cardiomyopathy (76% male). The group with type 2 sigmoid septum differed from hypertrophic cardiomyopathy in: was older (73±10.5years; P<.0001), with more hypertension (84.8%; P<.0001), lower glomerular filtering (73.3±21.4ml/min; P=.007), lower repolarization abnormalities (18.2%; P=.004) and Cornell index (in men, 22.2±11mm; P=.041), more diastolic dysfunction (75%; P=.0089) and in ventricular morphology and fibrosis location in magnetic resonance. Regarding the hypertrophic cardiomyopathy, patients with type 2 sigmoid septum are older and generally hypertensive; otherwise, often they have no clear differences in their clinical, electrocardiographic or echocardiographic characteristics. Therefore, cardiac resonance is helpful in the differential diagnosis. Copyright © 2016 Instituto Nacional de Cardiología Ignacio Chávez. Published by Masson Doyma México S.A. All rights reserved.

  20. Comparative effect and safety of verapamil in keloid and hypertrophic scar treatment: a meta-analysis.

    Science.gov (United States)

    Li, Zhouna; Jin, Zhehu

    2016-01-01

    Keloids and hypertrophic scars are the most common types of pathological scarring. Traditionally, keloids have been considered as a result of aberrant wound healing, involving excessive fibroblast participation that is characterized by hyalinized collagen bundles. However, the usefulness of this characterization has been questioned. In recent years, studies have reported the appropriate use of verapamil for keloids and hypertrophic scars. Searches were conducted on the databases Medline, Embase, Cochrane, PubMed, and China National Knowledge Infrastructure from 2006 to July 2016. State12.0 was used for literature review, data extraction, and meta-analysis. Treatment groups were divided into verapamil and nonverapamil group. Nonverapamil group includes steroids and intense pulsed light (IPL) therapy. Total effective rates include cure rate and effective rate. Cure: skin lesions were completely flattened, became soft and symptoms disappeared. Efficacy: skin lesions subsided, patient significantly reduced symptoms. Inefficient definition of skin was progression free or became worse. Random-effects model was used for the meta-analysis. Six studies that included 331 patients with keloids and hypertrophic scars were analyzed. Analysis of the total effective rate of skin healing was performed. The total effective rates in the two groups were 54.07% (verapamil) and 53.18% (nonverapamil), respectively. The meta-analysis showed that there was no difference between the two groups. We also compared the adverse reactions between the verapamil treatment group and the steroids treatment group in two studies, and the result indicated that the verapamil group showed less adverse reactions. There were no differences between the application of verapamil and nonverapamil group in keloids and hypertrophic scars treatment. Verapamil could act as an effective alternative modality in the prevention and treatment of keloid and hypertrophic scars. A larger number of studies are required to

  1. Duodenal Bulb Mucosa with Hypertrophic Gastric Oxyntic Heterotopia in Patients with Zollinger Ellison Syndrome

    Science.gov (United States)

    Kohan, Emil; Oh, David; Wang, Hank; Hazany, Salar; Ohning, Gordon; Pisegna, Joseph R.

    2009-01-01

    Objectives. Zollinger-Ellison Syndrome (ZES) results in hypersecretion of gastric acid (via gastrinoma) leading to peptic ulcers, diarrhea, and abdominal pain. We describe the novel discovery of hypertrophic, heterotopic gastric mucosa in the proximal duodenal bulb in patients with ZES, which we hypothesize results in an increased incidence of postbulbar ulcers in patients with ZES (a mechanism previously unreported). We determined the incidence of the novel finding of duodenal gastric oxyntic hypertrophic heterotopia (GOH) in patients with ZES. Methods. Seven patients with ZES were enrolled. The diagnosis of ZES was established by hypergastrinemia, gastric acid hypersecretion, and a positive secretin test or based on biopsy specimens (evaluated via tissue staining). Basal acid output (BAO) and baseline gastrin secretion were determined by established methods. Endoscopic examinations with methylene blue staining and biopsy of the gastric and duodenal mucosa were conducted in all patients every 3–6 months for an average of 5 years. Results. The duodenal mucosa demonstrated hypertrophic GOH in 5 out of 7 patients with ZES and an intact stomach and duodenum. Biopsies from the bowel mucosa demonstrated patchy replacement of surface epithelium by gastric-type epithelium with hypertrophic oxyntic glands in the lamina propria in 5 patients. Two of the patients had no evidence of GOH in the duodenal bulb. Patients with GOH had an average serum gastrin level of 1245 pg/mL and BAO of 2.92 mEq/hr versus 724 pg/mL and 0.8 mEq/hr in patients without GOH. Conclusions. This study demonstrated the presence of duodenal mucosa with GOH in 5 out of 7 patients with ZES and an intact stomach and duodenum. The presence of hypertrophic and heterotopic gastric mucosa is proposed to result from increased gastrin levels and may contribute to the increased incidence of postbulbar ulcers in these patients. PMID:19587828

  2. Duodenal Bulb Mucosa with Hypertrophic Gastric Oxyntic Heterotopia in Patients with Zollinger Ellison Syndrome

    Directory of Open Access Journals (Sweden)

    Emil Kohan

    2009-01-01

    Full Text Available Objectives. Zollinger-Ellison Syndrome (ZES results in hypersecretion of gastric acid (via gastrinoma leading to peptic ulcers, diarrhea, and abdominal pain. We describe the novel discovery of hypertrophic, heterotopic gastric mucosa in the proximal duodenal bulb in patients with ZES, which we hypothesize results in an increased incidence of postbulbar ulcers in patients with ZES (a mechanism previously unreported. We determined the incidence of the novel finding of duodenal gastric oxyntic hypertrophic heterotopia (GOH in patients with ZES. Methods. Seven patients with ZES were enrolled. The diagnosis of ZES was established by hypergastrinemia, gastric acid hypersecretion, and a positive secretin test or based on biopsy specimens (evaluated via tissue staining. Basal acid output (BAO and baseline gastrin secretion were determined by established methods. Endoscopic examinations with methylene blue staining and biopsy of the gastric and duodenal mucosa were conducted in all patients every 3–6 months for an average of 5 years. Results. The duodenal mucosa demonstrated hypertrophic GOH in 5 out of 7 patients with ZES and an intact stomach and duodenum. Biopsies from the bowel mucosa demonstrated patchy replacement of surface epithelium by gastric-type epithelium with hypertrophic oxyntic glands in the lamina propria in 5 patients. Two of the patients had no evidence of GOH in the duodenal bulb. Patients with GOH had an average serum gastrin level of 1245 pg/mL and BAO of 2.92 mEq/hr versus 724 pg/mL and 0.8 mEq/hr in patients without GOH. Conclusions. This study demonstrated the presence of duodenal mucosa with GOH in 5 out of 7 patients with ZES and an intact stomach and duodenum. The presence of hypertrophic and heterotopic gastric mucosa is proposed to result from increased gastrin levels and may contribute to the increased incidence of postbulbar ulcers in these patients.

  3. Comparative effect and safety of verapamil in keloid and hypertrophic scar treatment: a meta-analysis

    Directory of Open Access Journals (Sweden)

    Li ZN

    2016-11-01

    Full Text Available Zhouna Li, Zhehu Jin Department of Dermatology, Yanbian University Affiliated hospital, Yanji, Jilin, People’s Republic of China Background: Keloids and hypertrophic scars are the most common types of pathological scarring. Traditionally, keloids have been considered as a result of aberrant wound healing, involving excessive fibroblast participation that is characterized by hyalinized collagen bundles. However, the usefulness of this characterization has been questioned. In recent years, studies have reported the appropriate use of verapamil for keloids and hypertrophic scars.Methods: Searches were conducted on the databases Medline, Embase, Cochrane, PubMed, and China National Knowledge Infrastructure from 2006 to July 2016. State12.0 was used for literature review, data extraction, and meta-analysis. Treatment groups were divided into verapamil and nonverapamil group. Nonverapamil group includes steroids and intense pulsed light (IPL therapy. Total effective rates include cure rate and effective rate. Cure: skin lesions were completely flattened, became soft and symptoms disappeared. Efficacy: skin lesions subsided, patient significantly reduced symptoms. Inefficient definition of skin was progression free or became worse. Random-effects model was used for the meta-analysis.Results: Six studies that included 331 patients with keloids and hypertrophic scars were analyzed. Analysis of the total effective rate of skin healing was performed. The total effective rates in the two groups were 54.07% (verapamil and 53.18% (nonverapamil, respectively. The meta-analysis showed that there was no difference between the two groups. We also compared the adverse reactions between the verapamil treatment group and the steroids treatment group in two studies, and the result indicated that the verapamil group showed less adverse reactions.Conclusion: There were no differences between the application of verapamil and nonverapamil group in keloids and

  4. Hypertrophic Scars: Are Vitamins and Inflammatory Biomarkers Related with the Pathophysiology of Wound Healing?

    Science.gov (United States)

    Correia-Sá, Inês; Serrão, Paula; Marques, Marisa; Vieira-Coelho, Maria A

    2017-12-01

    Hypertrophic scars are a consequence of wound healing. The objective of the present study is to evaluate vitamin D and inflammatory biomarker plasma levels during wound healing. A prospective study was performed in patients (n = 63) submitted to body contouring surgery. Blood samples were collected before (t 0 ) and 5 days after surgery (t 5 ). Blood cell count, protein inflammatory biomarkers, and circulating plasma levels of 25(OH)D, vitamin A and vitamin E were quantified. Six months after surgery, scars were evaluated and classified as normal or hypertrophic. At the end of the study, 73% of the patients developed a normal scar (control group, n = 46) and 27% of the patients presented hypertrophic scars (HT group, n = 17). The patients in the HT group presented higher eosinophil (0.145 × 10 9 /L vs. 0.104 × 10 9 /L, p = 0.028) and basophil count (0.031 × 10 9 /L vs. 0.22 × 10 9 /L, p = 0.049) and C-reactive protein levels (6.12 mg/L vs. 2.30 mg/L, p = 0.015) in t 0 than the patients in the control group. At t 5 , the patients in the HT group showed a decrease in neutrophil (3.144 × 10 9/L vs. 4.03 × 10 9 /L, p = 0.031) and an increase in basophil (0.024 × 10 9 /L vs. 0.015 × 10 9 /L, p = 0.005) and lymphocyte count (1.836 × 10 9 /L vs. 1.557 × 10 9 /L; p = 0.028). Before surgery, vitamin D plasma levels were found to be decreased by almost 50% (23.52 ng/mL vs. 15.46 ng/mL, p = 0.031) in the patients who developed hypertrophic scars. Thirty-one percent of the patients submitted to bariatric surgery had more hypertrophic scars, versus 24% of the patients with no previous bariatric surgery. There is a different systemic inflammatory profile response in the patients during the formation of hypertrophic scars. Vitamin D plasma levels are marked reduced in these patients. Considering the powerful anti-inflammatory effect of vitamin D, these findings could be related.

  5. Biological and Chemical Removal of Primary Cilia Affects Mechanical Activation of Chondrogenesis Markers in Chondroprogenitors and Hypertrophic Chondrocytes.

    Science.gov (United States)

    Deren, Matthew E; Yang, Xu; Guan, Yingjie; Chen, Qian

    2016-02-04

    Chondroprogenitors and hypertrophic chondrocytes, which are the first and last stages of the chondrocyte differentiation process, respectively, are sensitive to mechanical signals. We hypothesize that the mechanical sensitivity of these cells depends on the cell surface primary cilia. To test this hypothesis, we removed the primary cilia by biological means with transfection with intraflagellar transport protein 88 (IFT88) siRNA or by chemical means with chloral hydrate treatment. Transfection of IFT88 siRNA significantly reduced the percentage of ciliated cells in both chondroprogenitor ATDC5 cells as well as primary hypertrophic chondrocytes. Cyclic loading (1 Hz, 10% matrix deformation) of ATDC5 cells in three-dimensional (3D) culture stimulates the mRNA levels of chondrogenesis marker Type II collagen (Col II), hypertrophic chondrocyte marker Type X collagen (Col X), and a molecular regulator of chondrogenesis and chondrocyte hypertrophy bone morphogenetic protein 2 (BMP-2). The reduction of ciliated chondroprogenitors abolishes mechanical stimulation of Col II, Col X, and BMP-2. In contrast, cyclic loading stimulates Col X mRNA levels in hypertrophic chondrocytes, but not those of Col II and BMP-2. Both biological and chemical reduction of ciliated hypertrophic chondrocytes reduced but failed to abolish mechanical stimulation of Col X mRNA levels. Thus, primary cilia play a major role in mechanical stimulation of chondrogenesis and chondrocyte hypertrophy in chondroprogenitor cells and at least a partial role in hypertrophic chondrocytes.

  6. Biological and Chemical Removal of Primary Cilia Affects Mechanical Activation of Chondrogenesis Markers in Chondroprogenitors and Hypertrophic Chondrocytes

    Directory of Open Access Journals (Sweden)

    Matthew E. Deren

    2016-02-01

    Full Text Available Chondroprogenitors and hypertrophic chondrocytes, which are the first and last stages of the chondrocyte differentiation process, respectively, are sensitive to mechanical signals. We hypothesize that the mechanical sensitivity of these cells depends on the cell surface primary cilia. To test this hypothesis, we removed the primary cilia by biological means with transfection with intraflagellar transport protein 88 (IFT88 siRNA or by chemical means with chloral hydrate treatment. Transfection of IFT88 siRNA significantly reduced the percentage of ciliated cells in both chondroprogenitor ATDC5 cells as well as primary hypertrophic chondrocytes. Cyclic loading (1 Hz, 10% matrix deformation of ATDC5 cells in three-dimensional (3D culture stimulates the mRNA levels of chondrogenesis marker Type II collagen (Col II, hypertrophic chondrocyte marker Type X collagen (Col X, and a molecular regulator of chondrogenesis and chondrocyte hypertrophy bone morphogenetic protein 2 (BMP-2. The reduction of ciliated chondroprogenitors abolishes mechanical stimulation of Col II, Col X, and BMP-2. In contrast, cyclic loading stimulates Col X mRNA levels in hypertrophic chondrocytes, but not those of Col II and BMP-2. Both biological and chemical reduction of ciliated hypertrophic chondrocytes reduced but failed to abolish mechanical stimulation of Col X mRNA levels. Thus, primary cilia play a major role in mechanical stimulation of chondrogenesis and chondrocyte hypertrophy in chondroprogenitor cells and at least a partial role in hypertrophic chondrocytes.

  7. Effect of Body Mass Index on Exercise Capacity in Patients With Hypertrophic Cardiomyopathy.

    Science.gov (United States)

    Larsen, Carolyn M; Ball, Caroline A; Hebl, Virginia B; Ong, Kevin C; Siontis, Konstantinos C; Olson, Thomas P; Ackerman, Michael J; Ommen, Steve R; Allison, Thomas G; Geske, Jeffrey B

    2018-01-01

    The objective of this study was to evaluate the relation between body mass index (BMI), exercise capacity, and symptoms in patients with hypertrophic cardiomyopathy (HC) and to utilize results of cardiopulmonary exercise tests (CPX) and transthoracic echocardiograms to understand the mechanism(s) of reduced exercise capacity across body mass index groups. Over a 6-year period, 510 consecutive patients with HC seen at a tertiary referral center underwent (CPX) and a transthoracic echocardiogram. Increasing BMI was associated with decreased exercise capacity as assessed by peak VO 2 (ml/kg/min). However, the prevalence of cardiac impairment did not vary by BMI group. In conclusion, these findings suggest that in some patients with hypertrophic cardiomyopathy, cardiac impairment is not the primary cause of exercise limitation and weight loss may result in improved exercise capacity. Copyright © 2017 Elsevier Inc. All rights reserved.

  8. Citrullinemia type I and hypertrophic pyloric stenosis in a 1-month old male infant

    Directory of Open Access Journals (Sweden)

    Yoona Rhee

    2013-01-01

    Full Text Available Citrullinemia type I (CTLN1 is an inherited urea cycle disorder, now included in most newborn screening panels in the US and Europe. Due to argininosuccinate synthetase deficiency, CTLN1 can lead to recurrent hyperammonemic crisis that may result in permanent neurologic sequelae. Vomiting in patients with urea cycle disorders may either be the result or cause of acute hyperammonemia, particularly if due to an illness that leads to catabolism. Therefore, age-appropriate common etiologies of vomiting must be considered when evaluat- ing these patients. We present a 1-month old male infant with CTLN1 who had a 1-week history of vomiting and was discovered to have hypertrophic pyloric stenosis. This is the first documented case of an infant with CTLN1 who was later diagnosed with hypertrophic pyloric stenosis, and only the second case of concomitant disease.

  9. Critical myocardial perfusion in hypertrophic cardiomyopathy demonstrated with thallium-201 SPECT with a quantitative bullseye map

    International Nuclear Information System (INIS)

    Hunter, G.J.

    1990-01-01

    PURPOSE: A particular problem in hypertrophic cardiomyopathy (HCM) is the need to distinguish between true and apparent ischemia in otherwise normal areas of muscle when these are compared with adjacent hypertrophic muscle. The authors of this paper studied patients with proved HCM to define patterns of perfusion. T1-201 single photon emission CT (SPECT) was performed in 83 HCM patients immediately after stress (dipyridamole, 0.5 mg/kg) and 3 hours later for the redistribution image. The data were analyzed by a normalized quantitative analysis using a local bulls-eye technique. In all patients, the pattern of tracer distribution was different from expected uptake in a normal population. By virtue of the increased microcirculation to hypertrophied muscle, adjacent normal muscle appeared relatively ischemic

  10. Multimodality treatment of hypertrophic scars using long-wave X-radiation

    International Nuclear Information System (INIS)

    Protsenko, T.V.

    1984-01-01

    The paper is concerned with a therapeutic method for hypertrophic scars inc cluding Bucky rays, pyrogenal, vitamins a, e, b 12 , sodium salicylate and dimexide ointment. Multimodality treatment of hypertrophic scars is effective, well tolerated and can be widely used in out-patient clinics. It makes it possible to reduce the period of therapy by 2-3 mos., and the summary dose by 20 000-3000 rad (18.6-27.9 Gy) as compared to common therapy with Wucky rays only Changes in the connective structure of scars in the process of multimodality th herapy are nonspecific and account for some mechanisms of scar regression and reaffirm the efficacy of this therapeutic method

  11. Mitochondrial haplogroups modify the risk of developing hypertrophic cardiomyopathy in a Danish population

    DEFF Research Database (Denmark)

    Hagen, Christian M; Aidt, Frederik H; Hedley, Paula L

    2013-01-01

    Hypertrophic cardiomyopathy (HCM) is a genetic disorder caused by mutations in genes coding for proteins involved in sarcomere function. The disease is associated with mitochondrial dysfunction. Evolutionarily developed variation in mitochondrial DNA (mtDNA), defining mtDNA haplogroups and haplog......Hypertrophic cardiomyopathy (HCM) is a genetic disorder caused by mutations in genes coding for proteins involved in sarcomere function. The disease is associated with mitochondrial dysfunction. Evolutionarily developed variation in mitochondrial DNA (mtDNA), defining mtDNA haplogroups...... factors in the development of HCM. Thus, constitutive differences in mitochondrial function may influence the occurrence and clinical presentation of HCM. This could explain some of the phenotypic variability in HCM. The fact that haplogroup H and J are also modifying factors in ischemic cardiomyopathy...

  12. Hypertrophic Cardiomyopathy Mimicking Acute Anterior Myocardial Infarction Associated with Sudden Cardiac Death

    Directory of Open Access Journals (Sweden)

    Y. Daralammouri

    2012-01-01

    Full Text Available Hypertrophic cardiomyopathy is the most common genetic disease of the heart. We report a rare case of hypertrophic obstructive cardiomyopathy mimicking an acute anterior myocardial infarction associated with sudden cardiac death. The patient presented with acute ST elevation myocardial infarction and significant elevation of cardiac enzymes. Cardiac catheterization showed some atherosclerotic coronary artery disease, without significant stenosis. Echocardiography showed left ventricular hypertrophy with a left ventricular outflow tract obstruction; the pressure gradient at rest was 20 mmHg and became severe with the Valsalva maneuver (100 mmHg. There was no family history of sudden cardiac death. Six days later, the patient suffered a syncope on his way to magnetic resonance imaging. He was successfully resuscitated by ventricular fibrillation.

  13. Progressive Hypertrophic Genital Herpes in an HIV-Infected Woman despite Immune Recovery on Antiretroviral Therapy

    Directory of Open Access Journals (Sweden)

    Mark H. Yudin

    2008-01-01

    Full Text Available Most HIV-infected individuals are coinfected by Herpes simplex virus type 2 (HSV-2. HSV-2 reactivates more frequently in HIV-coinfected individuals with advanced immunosuppression, and may have very unusual clinical presentations, including hypertrophic genital lesions. We report the case of a progressive, hypertrophic HSV-2 lesion in an HIV-coinfected woman, despite near-complete immune restoration on antiretroviral therapy for up to three years. In this case, there was prompt response to topical imiquimod. The immunopathogenesis and clinical presentation of HSV-2 disease in HIV-coinfected individuals are reviewed, with a focus on potential mechanisms for persistent disease despite apparent immune reconstitution. HIV-infected individuals and their care providers should be aware that HSV-2 may cause atypical disease even in the context of near-comlpete immune reconstitution on HAART.

  14. Hypertrophic pachymeningitis: Current criteria for diagnosis and differentiation (Clinical case and review of literature

    Directory of Open Access Journals (Sweden)

    E. G. Mendelevich

    2015-01-01

    Full Text Available The paper describes a 44-year-old male patient with an about 6-year history of hypertrophic pachymeningitis. The major clinical symptoms were characterized by headache, exophthalmos, and blindness in one eye. The data for differential diagnosis of the disease are given. The current literature on the clinical manifestations of hypertrophic pachymeningitis, its differential diagnosis, and the results of magnetic resonance imaging (MRI is reviewed. Diagnostic difficulties at the stage of a clinical observation are due to the nonspecificity of neurological manifestations and the need for a comprehensive examination to detect a somatic disease. MRI can diagnose the disease-specific phenomenon of damage to the meninges, which calls for further careful differentiation. Clinicians must be familiar with alternative differential diagnosis, as a rapid specific therapeutic approach will help avoid long-term or irreversible neurological complications.

  15. Hypertrophic lichen planus as a presenting feature of human immunodeficiency virus infection

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    Kumari Rashmi

    2009-01-01

    Full Text Available Lichen planus (LP is a chronic papulosquamous dermatosis in which both skin and mucous membranes may be involved. To date, there have been only five reports of human immunodeficiency virus (HIV-positive patients with hypertrophic LP. In the present report, we describe a 37-year-old female who presented with widely distributed, hyperpigmented, pruritic scaly lesions involving the face, trunk, and upper and lower extremities for one month. She also had swelling of both lower legs with low grade fever for past one week. She was diagnosed to be a HIV-positive patient who had severe, widespread hypertrophic LP lesions along with acute eruptive lesions of LP. These LP lesions were a presenting feature of HIV infection in our case.

  16. Characterisation of connective tissue from the hypertrophic skeletal muscle of myostatin null mice.

    Science.gov (United States)

    Elashry, Mohamed I; Collins-Hooper, Henry; Vaiyapuri, Sakthivel; Patel, Ketan

    2012-06-01

    Myostatin is a potent inhibitor of muscle development. Genetic deletion of myostatin in mice results in muscle mass increase, with muscles often weighing three times their normal values. Contracting muscle transfers tension to skeletal elements through an elaborate connective tissue network. Therefore, the connective tissue of skeletal muscle is an integral component of the contractile apparatus. Here we examine the connective tissue architecture in myostatin null muscle. We show that the hypertrophic muscle has decreased connective tissue content compared with wild-type muscle. Secondly, we show that the hypertrophic muscle fails to show the normal increase in muscle connective tissue content during ageing. Therefore, genetic deletion of myostatin results in an increase in contractile elements but a decrease in connective tissue content. We propose a model based on the contractile profile of muscle fibres that reconciles this apparent incompatible tissue composition phenotype. © 2012 The Authors. Journal of Anatomy © 2012 Anatomical Society.

  17. Hypertrophic cardiomyopathy and Wolff-Parkinson-White Syndrome with complete auriculoventricular block. A strange association

    International Nuclear Information System (INIS)

    Vallejo, Franco J; Montana, Paula A; Vesga, Carlos; Miranda Antonio; Citelli Jose E; Negrete Alberto; Gil, Efrain

    2007-01-01

    A 22 years old male patient is admitted for a syncope episode. An electrocardiogram shows a Wolff-Parkinson-White pattern and signs of auricular overload with left ventricular hypertrophy and complete auriculoventricular block. The transthoracic echocardiogram is compatible with non-obstructive hypertrophic cardiomyopathy. An electrophysiological study is carried out, finding pre-excitation through an accessory way and infra-His auriculoventricular block. An ablation is performed and a bicameral pacemaker is implanted

  18. Fabry disease mimicking hypertrophic cardiomyopathy: genetic screening needed for establishing the diagnosis in women

    DEFF Research Database (Denmark)

    Havndrup, Ole; Christiansen, Michael; Stoevring, Birgitte

    2010-01-01

    AIMS: Fabry disease, an X-linked storage disorder caused by defective lysosomal enzyme alpha-galactosidase A activity, may resemble sarcomere-gene-associated hypertrophic cardiomyopathy (HCM). The 'cardiac variant' of Fabry disease which only affects the heart may be missed unless specifically te...... therapy, supports systematic testing for Fabry disease. Enzyme measurements are sufficient in men, but genetic testing is needed in women....

  19. Comparative effect and safety of verapamil in keloid and hypertrophic scar treatment: a meta-analysis

    OpenAIRE

    Li ZN; Jin ZH

    2016-01-01

    Zhouna Li, Zhehu Jin Department of Dermatology, Yanbian University Affiliated hospital, Yanji, Jilin, People’s Republic of China Background: Keloids and hypertrophic scars are the most common types of pathological scarring. Traditionally, keloids have been considered as a result of aberrant wound healing, involving excessive fibroblast participation that is characterized by hyalinized collagen bundles. However, the usefulness of this characterization has been questioned. In recent yea...

  20. Comparative effect and safety of verapamil in keloid and hypertrophic scar treatment: a meta-analysis

    OpenAIRE

    Li,Zhouna; Jin,Zhehu

    2016-01-01

    Zhouna Li, Zhehu Jin Department of Dermatology, Yanbian University Affiliated hospital, Yanji, Jilin, People’s Republic of China Background: Keloids and hypertrophic scars are the most common types of pathological scarring. Traditionally, keloids have been considered as a result of aberrant wound healing, involving excessive fibroblast participation that is characterized by hyalinized collagen bundles. However, the usefulness of this characterization has been questioned. In recent...

  1. A single case study of treating hypertrophic lichen planus with Ayurvedic medicine.

    Science.gov (United States)

    Ratha, Kshirod Kumar; Barik, Laxmidhar; Panda, Ashok Kumar; Hazra, Jayram

    2016-01-01

    Ayurvedic medicines are often considered effective for chronic and lifestyle disorders. Hypertrophic lichen planus (HLP) is a rare inflammatory skin condition and develops into squamous cell carcinoma in few cases. It has resemblance with Charma Kushtha mentioned in Ayurvedic classics. Conventional therapy used in this condition is unsatisfactory and is not free from side effects. A case of long-standing systemic steroid-dependent HLP is presented here which was intervened successfully with Ayurvedic modalities.

  2. Papillary thyroid carcinoma treated with radiofrequency ablation in a patient with hypertrophic cardiomyopathy: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Sun, Jian Yi; Liu, Xiao Sun; Zhang, Qing; Hong, Yan Yun; Song, Bin; Teng, Xiao Dong; Yu, Ji Ren [The First Affiliated Hospital, Medical College, Zhejiang University, Hangzhou (China)

    2016-07-15

    Standard therapy has not been established for thyroid cancer when a thyroidectomy is contraindicated due to systemic disease. Herein, we reported a patient who had hypertrophic cardiomyopathy and papillary thyroid carcinoma treated by radiofrequency ablation because of inability to tolerate a thyroidectomy. Radiofrequency ablation can be used to treat thyroid cancer when surgery is not feasible, although the long-term outcome needs further observation.

  3. Papillary Thyroid Carcinoma Treated with Radiofrequency Ablation in a Patient with Hypertrophic Cardiomyopathy: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Sun, Jianyi; Liu, Xiaosun; Zhang, Qing; Hong, Yanyun; Song, Bin [Department of Gastrointestinal and Thyroid Surgery, The First Affiliated Hospital, Medical College, Zhejiang University, Hangzhou 310003 (China); Teng, Xiaodong [Department of Pathology, The First Affiliated Hospital, Medical College, Zhejiang University, Hangzhou 310003 (China); Yu, Jiren [Department of Gastrointestinal and Thyroid Surgery, The First Affiliated Hospital, Medical College, Zhejiang University, Hangzhou 310003 (China)

    2016-11-01

    Standard therapy has not been established for thyroid cancer when a thyroidectomy is contraindicated due to systemic disease. Herein, we reported a patient who had hypertrophic cardiomyopathy and papillary thyroid carcinoma treated by radiofrequency ablation because of inability to tolerate a thyroidectomy. Radiofrequency ablation can be used to treat thyroid cancer when surgery is not feasible, although the long-term outcome needs further observation.

  4. A Systematic Review on the Effect of Mechanical Stretch on Hypertrophic Scars after Burn Injuries

    Directory of Open Access Journals (Sweden)

    Yu-ting Zhang

    2017-06-01

    Conclusion: From extensive literature search, there was no strong evidence indicating the positive effect of mechanical stretch using stretching exercise, massage, or splinting on hypertrophic scars. A firm conclusion cannot be drawn for the discrepancy of outcome measures and varied effectiveness. Most of the included studies lacked objective evaluation or control group for comparison. Further high quality studies with larger sample size and using standardized measurements are needed.

  5. Ultrasound-guided paravertebral block for pyloromyotomy in 3 neonates with congenital hypertrophic pyloric stenosis

    OpenAIRE

    Mata-Gómez, Javier; Guerrero-Domínguez, Rosana; García-Santigosa, Marta; Ontanilla, Antonio

    2015-01-01

    BACKGROUND AND OBJECTIVES: Hypertrophic pyloric stenosis is a relatively common affection of gastrointestinal tract in childhood that results in symptoms, such as projectile vomiting and metabolic disorders that imply a high risk of aspiration during anesthetic induction. In this way, the carrying out of a technique with general anesthesia and intravenous rapid sequence induction, preoxygenation and cricoid pressure are recommended. After the correction of systemic metabolic alkalosis and pH ...

  6. Current management of hypertrophic cardiomyopathy: evidence in pathophysiology, diagnosis and treatment

    International Nuclear Information System (INIS)

    Quesada Mena, Luis Diego

    2013-01-01

    Available literary evidence is reviewed on the current management of hypertrophic cardiomyopathy. The bibliographical search is carried out in physical and online texts, cardiology journals, databases (MEDLINE), original studies, reviews and metaanalysis. Literature in English and Spanish is included from the first descriptions of the disease in the fifties, until the date of the investigation. Clinical management of patients is compared and recommendations published by consensus groups of international associations [es

  7. Inhibition of cyclooxygenase-2 impacts chondrocyte hypertrophic differentiation during endochondral ossification

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    TJM Welting

    2011-12-01

    Full Text Available Skeletogenesis and bone fracture healing involve endochondral ossification, a process during which cartilaginous primordia are gradually replaced by bone tissue. In line with a role for cyclooxygenase-2 (COX-2 in the endochondral ossification process, non-steroidal anti-inflammatory drugs (NSAIDs were reported to negatively affect bone fracture healing due to impaired osteogenesis. However, a role for COX-2 activity in the chondrogenic phase of endochondral ossification has not been addressed before. We show that COX-2 activity fulfils an important regulatory function in chondrocyte hypertrophic differentiation. Our data reveal essential cross-talk between COX-2 and bone morphogenic protein-2 (BMP-2 during chondrocyte hypertrophic differentiation. BMP-2 mediated chondrocyte hypertrophy is associated with increased COX-2 expression and pharmacological inhibition of COX-2 activity by NSAIDs (e.g., Celecoxib decreases hypertrophic differentiation in various chondrogenic models in vitro and in vivo, while leaving early chondrogenic development unaltered. Our findings demonstrate that COX-2 activity is a novel factor partaking in chondrocyte hypertrophy in the context of endochondral ossification and these observations provide a novel etiological perspective on the adverse effects of NSAIDs on bone fracture healing and have important implications for the use of NSAIDs during endochondral skeletal development.

  8. Porous decellularized tissue engineered hypertrophic cartilage as a scaffold for large bone defect healing.

    Science.gov (United States)

    Cunniffe, Gráinne M; Vinardell, Tatiana; Murphy, J Mary; Thompson, Emmet M; Matsiko, Amos; O'Brien, Fergal J; Kelly, Daniel J

    2015-09-01

    Clinical translation of tissue engineered therapeutics is hampered by the significant logistical and regulatory challenges associated with such products, prompting increased interest in the use of decellularized extracellular matrix (ECM) to enhance endogenous regeneration. Most bones develop and heal by endochondral ossification, the replacement of a hypertrophic cartilaginous intermediary with bone. The hypothesis of this study is that a porous scaffold derived from decellularized tissue engineered hypertrophic cartilage will retain the necessary signals to instruct host cells to accelerate endogenous bone regeneration. Cartilage tissue (CT) and hypertrophic cartilage tissue (HT) were engineered using human bone marrow derived mesenchymal stem cells, decellularized and the remaining ECM was freeze-dried to generate porous scaffolds. When implanted subcutaneously in nude mice, only the decellularized HT-derived scaffolds were found to induce vascularization and de novo mineral accumulation. Furthermore, when implanted into critically-sized femoral defects, full bridging was observed in half of the defects treated with HT scaffolds, while no evidence of such bridging was found in empty controls. Host cells which had migrated throughout the scaffold were capable of producing new bone tissue, in contrast to fibrous tissue formation within empty controls. These results demonstrate the capacity of decellularized engineered tissues as 'off-the-shelf' implants to promote tissue regeneration. Copyright © 2015 Acta Materialia Inc. Published by Elsevier Ltd. All rights reserved.

  9. Assessment of chest pain in hypertrophic cardiomyopathy using exercise thallium-201 myocardial scintigraphy

    International Nuclear Information System (INIS)

    Pitcher, D.; Wainwright, R.; Maisey, M.; Curry, P.; Sowton, E.

    1980-01-01

    Exercise thallium-201 myocardial scintigraphy was performed in 23 patients with hypertrophic cardiomyopathy. Eighteen patients presented with chest pain which was a persistent symptom in 11. Selective coronary arteriography was performed in 16 patients and showed normal coronary arteries in 15 and insignificant luminal irregularities in one patient. Eighteen patients had abnormal scintigrams. Three had an abnormal distribution of tracer entirely attributable to asymmetric septal hypertrophy, whereas 15 had discrete tracer uptake defects which could not be explained solely by myocardial hypertrophy. In this latter group of patients three scintigraphic patterns were identified: (1) in 10 patients defects were seen in scintigrams immediately after exercise but not in delayed images obtained four to six hours later. Eight of these patients had chest pain. (2) Four patients had uptake defects seen in both initial and delayed images. One patient had chest pain. (3) In three patients, one of whom had chest pain, tracer defects were seen only in delayed images and were not apparent in the initial scintigrams. Chest pain occurred in eight out of 10 patients with scintigraphic evidence of myocardial ischaemia but was present in only three out of 13 patients with non-ischaemic scintigrams. The value of exercise thallium-201 myocardial imaging as a diagnostic technique in hypertrophic cardiomyopathy appears limited. Scintigraphic evidence of regional myocardial ischaemia in the absence of significant coronary artery disease, however, contributes to an understanding of the mechanism of angina production in patients with hypertrophic cardiomyopathy. (author)

  10. Treatment of hypertrophic scars and keloids using intense pulsed light (IPL).

    Science.gov (United States)

    Erol, O Onur; Gurlek, Ali; Agaoglu, Galip; Topcuoglu, Ela; Oz, Hayat

    2008-11-01

    Keloids and hypertrophic scars are extremely disturbing to patients, both physically and psychologically. This study prospectively assessed the safety and efficacy of intense pulsed light (IPL) on scars originating from burns, trauma, surgery, and acne. Hypertrophic scars in 109 patients, originating from surgical incisions (n = 55), traumatic cuts (traffic accidents) (n = 24), acne scars (n = 6), keloids (n = 5), and burns (n = 19), were treated using an IPL Quantum device. Treatment was administered at 2-4-week intervals, and patients received an average of 8 treatments (range = 6-24). Using digital photographs, Changes in scar appearance were assessed by two physicians who were blinded to the study patients and treatments. The photographs were graded on a scale of 0 to 4 (none, minimal, moderate, good, excellent) for improvement in overall clinical appearance and reduction in height, erythema, and hardness. An overall clinical improvement in the appearance of scars and reductions in height, erythema, and hardness were seen in the majority of the patients (92.5%). Improvement was excellent in 31.2% of the patients, good in 25.7%, moderate in 34%, and minimal in 9.1%. Over half the patients had good or excellent improvement. In the preventive IPL treatment group, 65% had good to excellent improvement in clinical appearance. Patient satisfaction was very high. This study suggests that IPL is effective not only in improving the appearance of hypertrophic scars and keloids regardless of their origin, but also in reducing the height, redness, and hardness of scars.

  11. Molecular and biophysical mechanisms regulating hypertrophic differentiation in chondrocytes and mesenchymal stem cells

    Directory of Open Access Journals (Sweden)

    D Studer

    2012-07-01

    Full Text Available Chondrocyte hypertrophy is one of the key physiological processes involved in the longitudinal growth of long bones, yet the regulation of hypertrophy is also becoming increasingly relevant for clinical application of mesenchymal stem cells (MSCs and screening for drugs to treat hypertrophic osteoarthritis. The extraordinary cell volume increase during hypertrophy is accompanied by an up-regulation of collagen X, matrix metalloproteinases (MMPs, and vascular endothelial growth factor (VEGF, all which are targets of the runt-related transcription factor 2 (Runx2. Many pathways, including parathyroid hormone-related protein (PTHrP/Indian Hedgehog, Wingless/Int (Wnt/β-catenin, and transforming growth factor beta (TGF-β/Sma and Mad Related Family (Smad pathways, can regulate hypertrophy, but factors as diverse as hypoxia, co-culture, epigenetics and biomaterial composition can also potently affect Runx2 expression. Control of hypertrophic differentiation can be exploited both for cartilage repair, where a stable phenotype is desired, but also in bone regeneration, where hypertrophic cartilage could act as a template for endochondral bone formation. We hope this review will motivate the design of novel engineered microenvironments for skeletal regeneration applications.

  12. Benazepril and subclinical feline hypertrophic cardiomyopathy: A prospective, blinded, controlled study

    Science.gov (United States)

    Di Fruscia, Rocky

    2006-01-01

    Abstract Twenty-one cats with hypertrophic cardiomyopathy were enrolled in this study to determine if the administration of benazepril (0.5 mg/kg body weight [BW], PO, q24h) to cats with subclinical hypertrophic cardiomyopathy improves cardiac diastolic function and reverses left ventricular hypertrophy when compared with diltiazem controlled delivery (CD) (10 mg/kg BW, PO, q24h). Cats were evaluated at day 0 and after 3 and 6 months of therapy. In the benazepril group (n = 11), the diastolic transmitral flow of the E and A waves ratio (E/A ratio) increased significantly between 0 and 6 months (P = 0.009) and the thickness of the left ventricular free wall in systole (LVFWs) decreased significantly between 0 and 3 months (P = 0.04). In the diltiazem CD group (n = 5), none of the parameters varied significantly throughout the study. There was no difference between the benazepril and the diltiazem CD group throughout the study. Therefore, the variations observed for the E/A ratio and the LVFWs may have been incidental. Further studies will be needed to establish the role of benazepril in subclinical hypertrophic cardiomyopathy in cat. PMID:16734369

  13. Diagnostic Value of Histological and Microbiological Screening in Etiopathogenesis of Recurrent and Hypertrophic Tonsillitis

    Directory of Open Access Journals (Sweden)

    Uldis Viesturs

    2006-12-01

    Full Text Available Interest in the mechanisms and causes of recurrent tonsillitis is considerable. CD4+CD25+ T-lymphocytes have an important role in the maintenance of immunological tolerance. The aim of our research was to compare the diagnostic value of palatine tonsils histological and microbiological screening in patients with hypertrophic and recurrent tonsillitis. 14 patients with hypertrophic and 10 patients with chronic tonsillitis undergoing tonsillectomy were enrolled in the study. Rapid diagnosis of adenovirus, parainfluenza, influenza A and B, and respiratory syncytial virus infection was made before tonsillectomy by viral antigen detection using the immunofluorescence procedure from tonsils. Herpes simplex and cytomegaloviruses DNA were detected by the polymerase chain reaction. Samples for bacteriological studies were collected using a cotton swab. Immunohistochemical methods were used to evaluate S-100 and TGF-beta1 expression. The obtained results showed that patients with recurrent tonsillitis had less S-100 and TGF-beta1 positive cells in parafollicular regions compared to patients with hypertrophic tonsillitis. In both groups, tonsils were colonized predominantly by gram-positive microorganisms and adenovirus (36% of cases. However, in patients with recurrent tonsillitis, associations of gram-positive, gram-negative bacteria and viruses (40% of cases were observed. To conclude, recurrent tonsillitis is characterized by the breakdown of the immunological tolerance to oral microflora.

  14. Distinguishing hypertrophic cardiomyopathy from athlete's heart physiological remodelling: clinical significance, diagnostic strategies and implications for preparticipation screening.

    Science.gov (United States)

    Maron, B J

    2009-09-01

    Sudden cardiac death in young competitive athletes is an important public health problem, although a relatively low-event-rate phenomenon. The single most common cardiovascular cause of these unexpected catastrophes is hypertrophic cardiomyopathy (HCM), accounting for about one-third of cases. Since the phenotypic expression of HCM is variable, and not uncommonly includes patients with mild and localised left ventricular hypertrophy, the differential diagnosis with physiological remodelling of athlete's heart not uncommonly arises. This review discusses those non-invasive strategies that are useful in distinguishing the benign consequences of systematic athletic training from pathological left ventricular hypertrophy with the potential for sudden cardiac death. Preparticipation screening in healthy general athlete populations may raise the suspicion of HCM, and ultimately lead to definitive diagnosis. However, recently controversy has arisen regarding the most effective and practical strategy for the screening of athletes. European investigators have promoted routine 12-lead ECGs as part of a national mandatory programme distinct from the customary practice in the US which is limited to history and physical examinations. Consensus criteria and recommendations for eligibility and disqualification of athletes with HCM (and other cardiovascular abnormalities) have proved useful to the practising community.

  15. Experimental Study of 5-fluorouracil Encapsulated Ethosomes Combined with CO2 Fractional Laser to Treat Hypertrophic Scar.

    Science.gov (United States)

    Zhang, Zhen; Chen, Jun; Huang, Jun; Wo, Yan; Zhang, Yixin; Chen, Xiangdong

    2018-01-18

    This study is designed to explore permeability of ethosomes encapsulated with 5-florouracil (5-FU) mediated by CO 2 fractional laser on hypertrophic scar tissues. Moreover, therapeutic and duration effect of CO 2 fractional laser combined with 5-FU encapsulated ethosomes in rabbit ear hypertrophic scar model will be evaluated. The permeated amount of 5-FU and retention contents of 5-FU were both determined by high-performance liquid chromatography (HPLC). Fluorescence intensities of ethosomes encapsulated with 5-FU (5E) labeled with Rodanmin 6GO (Rho) were measured by confocal laser scanning microscopy (CLSM). The permeability promotion of 5E labeled with Rho in rabbit ear hypertrophic scar mediated by CO 2 fractional laser was evaluated at 0 h, 6 h, 12 h, 24 h, 3 days and 7 days after the irradiation. The opening rates of the micro-channels were calculated according to CLSM. The therapeutic effect of 5EL was evaluated on rabbit ear hypertrophic scar in vivo. Relative thickness of rabbit ear hypertrophic scar before and after the treatment was measured by caliper method. Scar elevation index (SEI) of rabbit ear hypertrophic scar was measured using H&E staining. The data showed that the penetration amount of 5EL group was higher than 5E group (4.15 ± 2.22 vs. 0.73 ± 0.33; p 5E group (107.61 ± 13.27 vs. 20.73 ± 3.77; p 5E group (24.42 ± 4.37 vs.12.25 ± 1.64; p 5E group at different time points (1, 6, and 24 h). The opening rates of the micro-channels were decreased gradually within 24 h, and micro-channels were closed completely 3 days after the irradiation by CO 2 fractional laser. The relative thickness and SEI of rabbit ear hypertrophic scar after 7 days of treatment in the 5EL group were significantly lower than the 5E group. CO 2 fractional laser combined with topical 5E can be effective in the treatment of hypertrophic scar in vivo and supply a novel therapy method for human hypertrophic scar.

  16. Heart rate turbulence as a marker of myocardial electrical instability in children with hypertrophic cardiomyopathy

    Directory of Open Access Journals (Sweden)

    V. A. Makarova

    2014-01-01

    Full Text Available Heart rate turbulence is a myocardial electrical instability marker used to stratify the risk of sudden cardiac death. Fifty children aged 7 to 17 years with hypertrophic cardiomyopathy were examined. The survey program included standard electrocardiography, Doppler echocardiography, and 24-hour Holter ECG monitoring. Heart rate turbulence parameters, such as turbulence onset and turbulence slope, were analyzed. According to turbulence onset greater than zero, heart rate turbulence impairment was identified in 5 of the 24 patients included in the survey. The abnormal turbulence slope values of less than 6 msec/RR were found in 3 patients. Both parameters were abnormal in 1 patient. Heart rate turbulence impairment was significantly more common in children with the non-obstructive form of hypertrophic cardiomyopathy than in those with its obstructive form (χ2=3,05; p=0,08. All the children with abnormal heart rhythm turbulence values had one or more major risk factors for sudden cardiac death, which significantly exceeds their rates in the normal heart rate turbulence groups (χ2=7,11; p=0,007. The patients with abnormal turbulence onset values were more often found to have syncope (χ2=3,2; p=0,02. One such patient was recorded to have unstable ventricular tachycardia (χ2=10,56; p=0,001. Our findings suggest that heart rate turbulence is an additional predictor of the unfavorable course of hypertrophic cardiomyopathy in children. 

  17. Evaluation of myocardial glucose metabolism in hypertrophic cardiomyopathy using 18F-fluorodeoxyglucose positron emission tomography.

    Directory of Open Access Journals (Sweden)

    Rie Aoyama

    Full Text Available The purposes of this study were to assess the usefulness of myocardial 18F-fluorodeoxyglucose (18F-FDG positron emission tomography (PET/computed tomography (CT for evaluating myocardial metabolic status in hypertrophic cardiomyopathy (HCM and the therapeutic efficacy of alcohol septal ablation (ASA in hypertrophic obstructive cardiomyopathy (HOCM.Thirty HCM patients (64.4±10.5 years, 14 male, 12 hypertrophic non-obstructive cardiomyopathy [HNCM], 16 HOCM, and 2 dilated phase of HCM underwent 18F-FDG-PET/CT. 18F-FDG uptake was semi-quantitatively evaluated using an uptake score in each 17 segment and the entire LV or regional standardized uptake value (SUV.18F-FDG uptake was observed mostly in a hypertrophied myocardium in HNCM patients, whereas 18F-FDG was extensively accumulated beyond the hypertrophied myocardium in HOCM patients. There was a positive correlation between the summed uptake score of 18F-FDG and high-sensitive troponin T level in HNCM patients (r = 0.603, p = 0.049, whereas the score was positively correlated with brain natriuretic peptide level (r = 0.614, p = 0.011 in HOCM patients. In 10 patients who received ASA, the maximum SUV of the entire LV was significantly reduced from 5.6±2.6 to 3.2±2.1 (p = 0.040 after ASA. Reduction of that maximum SUV was particularly significant in the lateral region (from 5.5±2.6 to 2.9 ±2.2, p = 0.024 but not significant in the anteroseptal region (from 4.5±2.6 to 2.9±1.6, p = 0.12.Extensive 18F-FDG uptake beyond the hypertrophied myocardium was observed in HOCM. ASA attenuates 18F-FDG uptake in a remote lateral myocardium.

  18. Mitral valve repair or replacement in hypertrophic obstructive cardiomyopathy: a prospective randomized study.

    Science.gov (United States)

    Bogachev-Prokophiev, Alexander; Afanasyev, Alexander; Zheleznev, Sergey; Fomenko, Michael; Sharifulin, Ravil; Kretov, Eugenie; Karaskov, Alexander

    2017-09-01

    The optimal surgical strategy for concomitant mitral valve intervention during myectomy remains controversial. The purpose of this study was to compare the results of mitral valve replacement versus repair in patients with hypertrophic obstructive cardiomyopathy and severe mitral regurgitation. Between 2010 and 2013, a total of 88 patients with hypertrophic obstructive cardiomyopathy and severe mitral regurgitation were randomly assigned to undergo either mitral valve replacement or repair in addition to extended myectomy. Three patients from the repair group were switched to mitral valve replacement after repair failure. There was 1 early death (2.4%) in the replacement group. The resting left ventricular outflow tract gradient was reduced from 89.1 ± 20.4 to 18.3 ± 5.7 mmHg (P replacement and repair groups, respectively; there was no significant difference between the groups (P = 0.458). At 2-year follow-up, overall survival was 87.2 ± 4.9% and 96.7 ± 3.3% (P = 0.034); freedom from sudden cardiac death was 95.6 ± 3.1% and 96.7 ± 3.3% (P = 0.615); and freedom from thromboembolic events was 91.2 ± 4.2% and 100%, respectively (P = 0.026). Both mitral valve repair and valve replacement in addition to extended myectomy are effective methods of surgical treatment in patients with hypertrophic obstructive cardiomyopathy who have severe mitral regurgitation. The benefits of mitral valve repair are better overall survival and a lower rate of thromboembolic events. ClinicalTrials.gov: NCT02054221. © The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

  19. Detection of myocardial ischemia of hypertrophic cardiomyopathy with gated 99Tcm-MIBI myocardial perfusion imaging

    International Nuclear Information System (INIS)

    Jia Peng; Guo Wanhua; Du Minghua; Gao Ling

    2010-01-01

    Objective: To evaluate the value of gated 99 Tc m -methoxyisobutylisonitrile (MIBI) myocardial perfusion imaging in detection of myocardial ischemia in hypertrophic cardiomyopathy. Methods: Sixty-nine patients with clinically proven hypertrophic cardiomyopathy were divided into 2 groups using coronary angiogram as 'gold standard': positive group (n=19, narrowing ≥ 50%) and negative group (n=50, narrowing 99 Tc m -MIBI myocardial perfusion imaging was performed and positive in all 69 patients (41 males, 28 females, aged 35-75 years). Comparative analysis between the two groups was carried out using t-test. Results: In the positive group, reversible and irreversible perfusion defects were detected in 9 and 10 patients, respectively. Left ventricular ejection fraction (LVEF) increased to (69.1 ± 2.8)% in 8 patients and decreased to (42.8 ± 2.1)% in 11 patients. In the negative group, reversible and irreversible perfusion defects were found in 37 and 13 patients, respectively. LVEF increased to (70.8 ± 4.0)% in 38 patients and decreased to (48.9 ± 2.7)% in 12 patients. The values of ischemic area, severity and extent of perfusion defect, and LVEF were significantly different between the two groups (t=9.28, 16.51, 2.65; P 99 Tc m -MIBI myocardial perfusion imaging is valuable in assessing patients with hypertrophic cardiomyopathy. Detection for the presence or absence of coexisting coronary artery disease and myocardial ischemia has an important prognostic indication and management indication for these patients. (authors)

  20. Clinical evaluation of sup 123 I-BMIPP myocardial scintigraphy in patients with hypertrophic cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Ohtsuki, Katsuichi; Sugihara, Hiroki; Umamoto, Ikuo (Kyoto Prefectural Univ. of Medicine (Japan)) (and others)

    1992-02-01

    {sup 123}I-{beta}-methyl-p-iodophenylpentadecanoic acid ({sup 123}I-BMIPP) myocardial scintigraphy was performed in 13 patients with hypertrophic cardiomyopathy and compared with {sup 201}Tl myocardial scintigraphy performed within 3 months for evaluating the clinical significance of {sup 123}I-BMIPP myocardial scintigraphy. SPECT images were divided into 13 segments and segmental images were visually scored on a 4 (increased tracer uptake) to 0 (severely decreased tracer uptake) scale according to the tracer uptake. In comparison of {sup 123}I-BMIPP early images and {sup 201}Tl perfusion images, mismatches were seen in about 70% of all segments. The number of segments demonstrating lower myocardial uptake of {sup 123}I-BMIPP was larger than that of {sup 201}Tl. In hypertrophic regions, the tracer uptake of {sup 123}I-BMIPP early images was significantly lower than that of {sup 201}Tl images and the lower uptake of {sup 123}I-BMIPP delayed images was more marked. In non-hypertrophic regions, no significant difference was seen between the tracer uptakes of {sup 123}I-BMIPP early images and {sup 201}Tl images but the tracer uptake of {sup 123}I-BMIPP delayed images was significantly lower than that of {sup 201}Tl images. The mismatch between the tracer uptakes of {sup 123}I-BMIPP images and {sup 201}Tl images was thought to be a reflection of disordered myocardial fatty acid metabolism. 'Washout', the difference between the tracer uptakes of {sup 123}I-BMIPP early images and delayed images was also thought to be a reflection of disordered myocardial fatty acid metabolism. These results suggest that {sup 123}I-BMIPP is a promising radiopharmaceutical for evaluating disordered myocardial fatty acid metabolism in patients with HCM. (author).

  1. Clinical evaluation of 123I-BMIPP myocardial scintigraphy in patients with hypertrophic cardiomyopathy

    International Nuclear Information System (INIS)

    Ohtsuki, Katsuichi; Sugihara, Hiroki; Umamoto, Ikuo

    1992-01-01

    123 I-β-methyl-p-iodophenylpentadecanoic acid ( 123 I-BMIPP) myocardial scintigraphy was performed in 13 patients with hypertrophic cardiomyopathy and compared with 201 Tl myocardial scintigraphy performed within 3 months for evaluating the clinical significance of 123 I-BMIPP myocardial scintigraphy. SPECT images were divided into 13 segments and segmental images were visually scored on a 4 (increased tracer uptake) to 0 (severely decreased tracer uptake) scale according to the tracer uptake. In comparison of 123 I-BMIPP early images and 201 Tl perfusion images, mismatches were seen in about 70% of all segments. The number of segments demonstrating lower myocardial uptake of 123 I-BMIPP was larger than that of 201 Tl. In hypertrophic regions, the tracer uptake of 123 I-BMIPP early images was significantly lower than that of 201 Tl images and the lower uptake of 123 I-BMIPP delayed images was more marked. In non-hypertrophic regions, no significant difference was seen between the tracer uptakes of 123 I-BMIPP early images and 201 Tl images but the tracer uptake of 123 I-BMIPP delayed images was significantly lower than that of 201 Tl images. The mismatch between the tracer uptakes of 123 I-BMIPP images and 201 Tl images was thought to be a reflection of disordered myocardial fatty acid metabolism. 'Washout', the difference between the tracer uptakes of 123 I-BMIPP early images and delayed images was also thought to be a reflection of disordered myocardial fatty acid metabolism. These results suggest that 123 I-BMIPP is a promising radiopharmaceutical for evaluating disordered myocardial fatty acid metabolism in patients with HCM. (author)

  2. Desmodium gangeticum root extract attenuates isoproterenol-induced cardiac hypertrophic growth in rats.

    Directory of Open Access Journals (Sweden)

    Divya Hitler

    2014-10-01

    Full Text Available Context: Desmodium gangeticum (L DC (Fabaceae; DG, a medicinal plant that grows in tropical habitats, is widely used to treat various ailments including digestive and inflammatory disorders. Aims: To investigate the possible cardioprotective activity of a DG root extract against isoproterenol (ISO-induced left ventricular cardiac hypertrophy (LVH in adult Wistar rats. Methods: Daily intraperitoneal administration of ISO (10 mg/kg body weight, single injection for 7 days induced LVH in rats. The LVH rats were post-treated orally with DG (100 mg/kg body weight for a period of 30 days. Thereafter, changes in heart weight (HW and body weight (BW, HW/BW ratio, percent of hypertrophy, collagen accumulation, activities of matrix metalloproteinase (MMP -2 and -9, superoxide dismutase (SOD and catalase (CAT enzymes, and the level of an oxidative stress marker, lipid peroxide (LPO, were determined. Results: HW/BW ratio, an indicator of hypertrophic growth, was significantly reduced in DG root post-treated LVH rats as compared with that for the non-treated LVH rats. The altered levels of ventricular LPO, collagen, MMPs-2 and -9, and antioxidant enzymes in the ISO-treated animals reverted back to near normal upon DG treatment. Further, the anti-hypertrophic activity of DG was comparable to that of the standard drug losartan (10 mg/kg. Conclusions: The results of the present study suggest that the aqueous root extract of DG exhibited anti-hypertrophic activity in-vivo by inhibiting ISO-induced ROS generation and MMP activities.

  3. Hypertrophic stimulation increases beta-actin dynamics in adult feline cardiomyocytes.

    Directory of Open Access Journals (Sweden)

    Sundaravadivel Balasubramanian

    2010-07-01

    Full Text Available The myocardium responds to hemodynamic stress through cellular growth and organ hypertrophy. The impact of cytoskeletal elements on this process, however, is not fully understood. While alpha-actin in cardiomyocytes governs muscle contraction in combination with the myosin motor, the exact role of beta-actin has not been established. We hypothesized that in adult cardiomyocytes, as in non-myocytes, beta-actin can facilitate cytoskeletal rearrangement within cytoskeletal structures such as Z-discs. Using a feline right ventricular pressure overload (RVPO model, we measured the level and distribution of beta-actin in normal and pressure overloaded myocardium. Resulting data demonstrated enriched levels of beta-actin and enhanced translocation to the Triton-insoluble cytoskeletal and membrane skeletal complexes. In addition, RVPO in vivo and in vitro hypertrophic stimulation with endothelin (ET or insulin in isolated adult cardiomyocytes enhanced the content of polymerized fraction (F-actin of beta-actin. To determine the localization and dynamics of beta-actin, we adenovirally expressed GFP-tagged beta-actin in isolated adult cardiomyocytes. The ectopically expressed beta-actin-GFP localized to the Z-discs, costameres, and cell termini. Fluorescence recovery after photobleaching (FRAP measurements of beta-actin dynamics revealed that beta-actin at the Z-discs is constantly being exchanged with beta-actin from cytoplasmic pools and that this exchange is faster upon hypertrophic stimulation with ET or insulin. In addition, in electrically stimulated isolated adult cardiomyocytes, while beta-actin overexpression improved cardiomyocyte contractility, immunoneutralization of beta-actin resulted in a reduced contractility suggesting that beta-actin could be important for the contractile function of adult cardiomyocytes. These studies demonstrate the presence and dynamics of beta-actin in the adult cardiomyocyte and reinforce its usefulness in measuring

  4. Increased transient receptor potential vanilloid type 1 (TRPV1) channel expression in hypertrophic heart

    DEFF Research Database (Denmark)

    Thilo, Florian; Liu, Ying; Schulz, Nico

    2010-01-01

    The aim of this study was to compare the expression of transient receptor potential vanilloid type 1 (TRPV1) channels in hypertrophic hearts from transgenic mice showing overexpression of the catalytic subunit alpha of protein phosphatase 2A alpha (PP2Ac alpha) with wild-type mice and with TRPV1-...... alpha transgenic mice compared to wild-type mice and TRPV1-/- mice (8.6±1.3mg/g; 5.4±0.3mg/g; and 5.4±0.4mg/g; respectively; p...

  5. Hypertrophic Cardiomyopathy due to Mitochondrial Disease: Prenatal Diagnosis, Management, and Outcome

    Directory of Open Access Journals (Sweden)

    Lutgardo García-Díaz

    2013-01-01

    Full Text Available A case of prenatally diagnosed fetal hypertrophic cardiomyopathy is reported. The mother was referred to our department at 37 weeks' gestation because of suspected congenital heart disease. Prenatal echocardiography showed biventricular hypertrophy and pericardial effusion, without additional abnormalities. Postnatal echocardiography confirmed prenatal diagnosis. Neonatal EKG showed biventricular hypertrophy and Wolff-Parkinson-White syndrome. Skeletal muscle biopsy was consistent with mitochondrial oxidative phosphorylation defect involving a combined defect of respiratory complexes I and IV. Echocardiographic followup during the first year of life showed progressive regression of hypertrophy and evolution to left ventricular myocardial noncompaction.

  6. Periostal hypertrophic osteopathy of bones (long bones) in colitis ulcerosa in adolescents

    International Nuclear Information System (INIS)

    Bargon, G.; Arlart, I.

    1982-01-01

    The article reports on a 14-year old girl with periostal new formation of bones at the long bones of the lower arms, the femora and the lower legs the individual phalanges and metacarpalia after colitis ulcerosa which had lasted for several years and had progressed stagewise. After a clinically recorded new attack the periostal new formations of bone progressed. Some time after the last attack of colitis the periostal changes in the bones partially receded. The article discusses the hypothetic explanations aiming at interpreting the pathogenesis of hypertrophic osteoarthropathies and periostoses, as given in the literature. (orig.) [de

  7. Periostal hypertrophic osteopathy of bones (long bones) in colitis ulcerosa in adolescents

    Energy Technology Data Exchange (ETDEWEB)

    Bargon, G.; Arlart, I.

    1982-03-01

    The article reports on a 14-year old girl with periostal new formation of bones at the long bones of the lower arms, the femora and the lower legs the individual phalanges and metacarpalia after colitis ulcerosa which had lasted for several years and had progressed stagewise. After a clinically recorded new attack the periostal new formations of bone progressed. Some time after the last attack of colitis the periostal changes in the bones partially receded. The article discusses the hypothetic explanations aiming at interpreting the pathogenesis of hypertrophic osteoarthropathies and periostoses, as given in the literature.

  8. Hypertrophic osteopathy associated with pulmonary metastasis of osteosarcoma in a dog

    International Nuclear Information System (INIS)

    Rahal, S.C.; Mamprim, M.J.; Sequeira, J.L.; Franco, A.P.R.

    2003-01-01

    A 1.8-year-old female German Shepherd dog was presented with lameness, and painful non-edematous swelling of the right front limb. Swelling surrounding soft tissue and periosteal bone formation of the radius, ulna, metacarpals, without evidence of articular involvement were observed in the right front limb by radiographic examinations. Also, pulmonary tumor was observed in radiographic examination. Five months ago, the dog had developed osteosarcoma of the left distal ulna, and the treatment was cisplatin chemotherapy and limb amputation. The final diagnosis was hypertrophic osteopathy associated with pulmonary metastasis of osteosarcoma [pt

  9. Nuclear magnetic resonance imaging in patients with hypertrophic and dilated cardiomyopathy

    International Nuclear Information System (INIS)

    Boisvieux, A.

    1987-01-01

    Patients with hypertrophic and dilated cardiomyopathy and normal subjects were investigated with nuclear magnetic resonance imaging. To evaluate the NMR scanner possibilities, the results were compared with the echocardiographic investigation of the same patients. The capabilities of NMR imaging to provide information about intracardiac anatomy are emphasized. This study is preceded by a description of the physical principles underlying the phenomenon of nuclear magnetic resonance and of the techniques used to obtain NMR images and a review of the clinical use of NMR imaging for cardiac diagnosis [fr

  10. The value of myocardial scintigraphy in hypertrophic cardiomyopathy with angina pectoris

    International Nuclear Information System (INIS)

    Bergen, J.M.; Simons, M.

    1981-01-01

    Myocardial scintigraphy with thallium-201 is a new, non-invasive diagnostic method by means of which on special indications ischaemic heart diseases may be demonstrated. The case history is described of a man with hypertrophic cardiomyopathy and angina pectoris. The electrocardiogram at rest was affected by the cardiomyopathy to such a degree that the interpretation of the ST-T segment during effort was not reliable. Scintigraphy revealed transient ischaemia. A bypass operation was carried out and post-operatively, the improved myocardial perfusion could be confirmed by myocardial scintigraphy. (Auth.)

  11. Hypertrophic Cardiomyopathy After a Single Dose of Dexamethasone in a Preterm Infant

    Directory of Open Access Journals (Sweden)

    Yusuf Kale

    2015-08-01

    Full Text Available Dexamethasone is widely used in preterm infants with severe pulmonary disease. Hypertrophic cardiomyopathy (HCM is a transient side effect observed after multiple doses of dexamethasone. We report a preterm infant with myocardial hypertrophy after a single dose of dexamethasone (0.5 mg/kg used to treat laryngeal edema secondary to prolonged intubation. A benign course was observed without left ventricular outflow tract obstruction and with recovery within 4 weeks. Myocardial effects of dexamethasone may be independent of dose and duration of treatment. The risk/benefit ratio must be carefully considered before using even a single dose of dexamethasone in preterm infants.

  12. Radiofrequency ablation of fast ventricular tachycardia causing an ICD storm in an infant with hypertrophic cardiomyopathy.

    Science.gov (United States)

    Ergul, Yakup; Ozyilmaz, Isa; Bilici, Meki; Ozturk, Erkut; Haydin, Sertaç; Guzeltas, Alper

    2018-04-01

    An implantable cardioverter defibrillator (ICD) storm involves very frequent arrhythmia episodes and ICD shocks, and it is associated with poor short-term and long-term prognosis. Radiofrequency catheter ablation can be used as an effective rescue treatment for patients with an ICD storm. To our knowledge, this is the first report of an infant with hypertrophic cardiomyopathy presenting with an ICD storm and undergoing successful radiofrequency catheter ablation salvage treatment for the fast left posterior fascicular ventricular tachycardia. © 2017 Wiley Periodicals, Inc.

  13. Hypertrophic osteoarthropathy of one leg - a sign of aortic graft infection

    Energy Technology Data Exchange (ETDEWEB)

    Spruijt, S.; Krijgsman, A.A. [Department of Orthopaedic and Trauma Surgery, Elkerliek Hospital, Helmond (Netherlands); Broek, J.A.C. van den [Department of Radiology, Elkerliek Hospital, Helmond (Netherlands); Tutein Nolthenius-Puylaert, M.C.B.J.E. [Department of Pathology, Elkerliek Hospital, Helmond (Netherlands)

    1999-04-01

    We report a rare case of hypertrophic osteoarthropathy (HOA) confined to the right leg secondary to aortic graft infection. The development of HOA exclusively localized to areas distal to a vascular prosthesis may be the presenting manifestation of graft infection and a crucial diagnostic clue in the early detection of vascular graft infection. HOA is diagnosed by its characteristic radiographic and scintigraphic pattern. Most prosthetic, especially aortic, graft infections are uniformly fatal if not treated by aggressive surgical and antibiotic therapy. Recognition of this uncommon association may facilitate an early diagnosis, which usually requires immediate surgical therapy. (orig.) With 6 figs., 28 refs.

  14. Hypertrophic osteoarthropathy of one leg - a sign of aortic graft infection

    International Nuclear Information System (INIS)

    Spruijt, S.; Krijgsman, A.A.; Broek, J.A.C. van den; Tutein Nolthenius-Puylaert, M.C.B.J.E.

    1999-01-01

    We report a rare case of hypertrophic osteoarthropathy (HOA) confined to the right leg secondary to aortic graft infection. The development of HOA exclusively localized to areas distal to a vascular prosthesis may be the presenting manifestation of graft infection and a crucial diagnostic clue in the early detection of vascular graft infection. HOA is diagnosed by its characteristic radiographic and scintigraphic pattern. Most prosthetic, especially aortic, graft infections are uniformly fatal if not treated by aggressive surgical and antibiotic therapy. Recognition of this uncommon association may facilitate an early diagnosis, which usually requires immediate surgical therapy. (orig.)

  15. Echocardiographic evaluation of pre-diagnostic development in young relatives genetically predisposed to hypertrophic cardiomyopathy

    DEFF Research Database (Denmark)

    Jensen, Morten K; Havndrup, Ole; Christiansen, Michael

    2015-01-01

    Identification of the first echocardiographic manifestations of hypertrophic cardiomyopathy may be important for clinical management and our understanding of the pathogenesis. We studied the development of pre-diagnostic echocardiographic changes in young relatives to HCM patients during long...... of relatives with unknown genetic status. Children carrying pathogenic sarcomere gene mutations develop reduced LVEDd and increased E/e' as first pre-diagnostic echocardiographic manifestations during follow-up into adulthood.......-term years follow-up. HCM-relatives not fulfilling the diagnostic criteria for HCM and age of family screening of 11 sarcomere genes, CRYAB, α-GAL, and titin, we evaluated...

  16. Ultrasound-guided paravertebral block for pyloromyotomy in 3 neonates with congenital hypertrophic pyloric stenosis

    Directory of Open Access Journals (Sweden)

    Javier Mata-Gómez

    2015-08-01

    Full Text Available BACKGROUND AND OBJECTIVES: Hypertrophic pyloric stenosis is a relatively common affection of gastrointestinal tract in childhood that results in symptoms, such as projectile vomiting and metabolic disorders that imply a high risk of aspiration during anesthetic induction. In this way, the carrying out of a technique with general anesthesia and intravenous rapid sequence induction, preoxygenation and cricoid pressure are recommended. After the correction of systemic metabolic alkalosis and pH normalization, cerebrospinal fluid can keep a state of metabolic alkalosis. This circumstance, in addition to the residual effect of neuromuscular blocking agents, inhalant anesthetics and opioids could increase the risk of postoperative apnea after a general anesthesia.CASE REPORT: We present the successful management in 3 neonates in those a pyloromyotomy was carried out because they had presented congenital hypertrophic pyloric stenosis. This procedure was done under general anesthesia with orotracheal intubation and rapid sequence induction. Then, ultrasound-guided paravertebral block was performed as analgesic method without the need for administrating opioids within intraoperative period and keeping an appropriate analgesic level.CONCLUSIONS: Local anesthesia has demonstrated to be safe and effective in pediatric practice. We consider the ultrasound-guided paravertebral block with one dose as a possible alternative for other local techniques described, avoiding the use of opioids and neuromuscular blocking agents during general anesthesia, and reducing the risk of central apnea within postoperative period.

  17. Invasive assessment of coronary microvascular dysfunction in hypertrophic cardiomyopathy: the index of microvascular resistance

    International Nuclear Information System (INIS)

    Gutiérrez-Barrios, Alejandro; Camacho-Jurado, Francisco; Díaz-Retamino, Enrique; Gamaza-Chulián, Sergio; Agarrado-Luna, Antonio; Oneto-Otero, Jesús; Del Rio-Lechuga, Ana; Benezet-Mazuecos, Javier

    2015-01-01

    Summary: We present a review of microvascular dysfunction in hypertrophic cardiomyopathy (HCM) and an interesting case of a symptomatic familial HCM patient with inducible ischemia by single photon emission computed tomography. Coronary angiography revealed normal epicardial arteries. Pressure wire measurements of fractional flow reserve (FFR), coronary flow reserve (CFR) and index of microvascular resistance (IMR) demonstrated a significant microcirculatory dysfunction. This is the first such case that documents this abnormality invasively using the IMR. The measurement of IMR, a novel marker of microcirculatory dysfunction, provides novel insights into the pathophysiology of this condition. - Highlights: • Microvascular dysfunction is a common feature in hypertrophic cardiomyopathy (HCM) and represents a strong predictor of unfavorable outcome and cardiovascular mortality. • The index of microvascular resistance (IMR) is a new method for invasively assessing the state of the coronary microcirculation using a single pressure-temperature sensor-tipped coronary wire. • However assessment of IMR in HCM has not been previously reported. We report a case in which microvascular dysfunction is assessed by IMR. This index may be useful in future researches of HCM.

  18. Paquidermoperiostosis (osteoartropatía hipertrófica primaria Pachydermoperiostosis (primary hypertrophic osteoarthropathy

    Directory of Open Access Journals (Sweden)

    Javier A. Cavallasca

    2006-04-01

    Full Text Available La paquidermoperiostosis u osteoartropatía hipertrófica primaria es una rara enfermedad caracterizada por compromiso cutáneo y osteoarticular. Comunicamos dos casos que presentaban hipocratismo digital, uñas en vidrio de reloj, agrandamiento óseo, tumefacción articular y diferentes grados de afectación cutánea, sin otros hallazgos clínicos relevantes. Ambos desconocían antecedentes familiares similares. El estudio radiográfico de las zonas comprometidas mostró periostosis. Con resultados de laboratorio y radiografía de tórax normales, y ausencia de evidencia clínica de otra enfermedad subyacente, se realizó diagnóstico de osteoartropatía hipertrófica primaria.Pachydermoperiostosis or primary hypertrophic osteoarthropathy is a rare disease characterized by cutaneous and osteo-arthicular involvement. We describe two patients with finger clubbing, watch crystal nails, bones thickenings, arthritis and different grades of skin affection, without other clinical manifestations. Both did not know of having relatives with the same alterations. Radiological studies of the affected areas showed periostosis. Because of normal laboratory results and chest radiography plus the absence of other underlying causes, diagnosis of primary hypertrophic osteoarthropathy was made.

  19. Hypertrophic osteopathy associated with mycotic pneumonia in two juvenile elk (Cervus elaphus).

    Science.gov (United States)

    Ferguson, Nicole M; Lévy, Michel; Ramos-Vara, José A; Baird, Debra K; Wu, Ching Ching

    2008-11-01

    Two yearling bull elk (cervus claphus) from the same farm developed anorexia, weight loss, and lameness. On physical examination, both elk were thin and showed diffuse swelling of all lower limbs. Radiographs of the lower limbs showed periosteal thickening of the distal extremities, consistent with hypertrophic osteopathy. Thoracic radiographs indicated the presence of pulmonary nodules. Cytologic evaluations of tracheal washes on both elk were consistent with inflammation. Acid-fast stains on both samples were negative. Because of the poor prognosis, both elk were euthanized. At necropsy, the carpal, metacarpal, tarsal, and metatarsal bones, as well as the radius, ulna, and tibia had thickening of cortical bone. There were multiple encapsulated nodules throughout the lungs, lymph nodes, and kidney, and smaller nodules in the myocardium. On microscopic examination, these nodules contained myriads of hyphae, and immunohistochemistry for Aspergillus sp. was strongly positive. Aspergillus fumigatus was isolated from affected tissue in 1 elk. Necropsy findings in both elk were consistent with disseminated fungal granulomas and periosteal hyperostosis. This case presents the first description of hypertrophic osteopathy in elk. The source of infection was undetermined, but inhalation of spores from contaminated feed or bedding was suspected.

  20. Elastin Is Differentially Regulated by Pressure Therapy in a Porcine Model of Hypertrophic Scar.

    Science.gov (United States)

    Carney, Bonnie C; Liu, Zekun; Alkhalil, Abdulnaser; Travis, Taryn E; Ramella-Roman, Jessica; Moffatt, Lauren T; Shupp, Jeffrey W

    Beneficial effects of pressure therapy for hypertrophic scars have been reported, but the mechanisms of action are not fully understood. This study evaluated elastin and its contribution to scar pliability. The relationship between changes in Vancouver Scar Scale (VSS) scores of pressure-treated scars and differential regulation of elastin was assessed. Hypertrophic scars were created and assessed weekly using VSS and biopsy procurement. Pressure treatment began on day 70 postinjury. Treated scars were compared with untreated shams. Treatment lasted 2 weeks, through day 84, and scars were assessed weekly through day 126. Transcript and protein levels of elastin were quantified. Pressure treatment resulted in lower VSS scores compared with sham-treated scars. Pliability (VSSP) was a key contributor to this difference. At day 70 pretreatment, VSSP = 2. Without treatment, sham-treated scars became less pliable, while pressure-treated scars became more pliable. The percentage of elastin in scars at day 70 was higher than in uninjured skin. Following treatment, the percentage of elastin increased and continued to increase through day 126. Untreated sham scars did not show a similar increase. Quantification of Verhoeff-Van Gieson staining corroborated the findings and immunofluorescence revealed the alignment of elastin fibers. Pressure treatment results in increased protein level expression of elastin compared with sham-untreated scars. These findings further characterize the extracellular matrix's response to the application of pressure as a scar treatment, which will contribute to the refinement of rehabilitation practices and ultimately improvements in functional and psychosocial outcomes for patients.

  1. Percutaneous Septal Ablation in Hypertrophic Obstructive Cardiomyopathy: From Experiment to Standard of Care

    Directory of Open Access Journals (Sweden)

    Lothar Faber

    2014-01-01

    Full Text Available Hypertrophic cardiomyopathy (HCM is one of the more common hereditary cardiac conditions. According to presence or absence of outflow obstruction at rest or with provocation, a more common (about 60–70% obstructive type of the disease (HOCM has to be distinguished from the less common (30–40% nonobstructive phenotype (HNCM. Symptoms include exercise limitation due to dyspnea, angina pectoris, palpitations, or dizziness; occasionally syncope or sudden cardiac death occurs. Correct diagnosis and risk stratification with respect to prophylactic ICD implantation are essential in HCM patient management. Drug therapy in symptomatic patients can be characterized as treatment of heart failure with preserved ejection fraction (HFpEF in HNCM, while symptoms and the obstructive gradient in HOCM can be addressed with beta-blockers, disopyramide, or verapamil. After a short overview on etiology, natural history, and diagnostics in hypertrophic cardiomyopathy, this paper reviews the current treatment options for HOCM with a special focus on percutaneous septal ablation. Literature data and the own series of about 600 cases are discussed, suggesting a largely comparable outcome with respect to procedural mortality, clinical efficacy, and long-term outcome.

  2. Invasive assessment of coronary microvascular dysfunction in hypertrophic cardiomyopathy: the index of microvascular resistance

    Energy Technology Data Exchange (ETDEWEB)

    Gutiérrez-Barrios, Alejandro, E-mail: aleklos@hotmail.com [Cardiology Department, Jerez Hospital, Jerez (Spain); Camacho-Jurado, Francisco [Cardiology Department, Punta Europa Hospital, Algeciras (Spain); Díaz-Retamino, Enrique; Gamaza-Chulián, Sergio; Agarrado-Luna, Antonio; Oneto-Otero, Jesús; Del Rio-Lechuga, Ana; Benezet-Mazuecos, Javier [Cardiology Department, Jerez Hospital, Jerez (Spain)

    2015-10-15

    Summary: We present a review of microvascular dysfunction in hypertrophic cardiomyopathy (HCM) and an interesting case of a symptomatic familial HCM patient with inducible ischemia by single photon emission computed tomography. Coronary angiography revealed normal epicardial arteries. Pressure wire measurements of fractional flow reserve (FFR), coronary flow reserve (CFR) and index of microvascular resistance (IMR) demonstrated a significant microcirculatory dysfunction. This is the first such case that documents this abnormality invasively using the IMR. The measurement of IMR, a novel marker of microcirculatory dysfunction, provides novel insights into the pathophysiology of this condition. - Highlights: • Microvascular dysfunction is a common feature in hypertrophic cardiomyopathy (HCM) and represents a strong predictor of unfavorable outcome and cardiovascular mortality. • The index of microvascular resistance (IMR) is a new method for invasively assessing the state of the coronary microcirculation using a single pressure-temperature sensor-tipped coronary wire. • However assessment of IMR in HCM has not been previously reported. We report a case in which microvascular dysfunction is assessed by IMR. This index may be useful in future researches of HCM.

  3. Up-to-date approach to manage keloids and hypertrophic scars: a useful guide.

    Science.gov (United States)

    Arno, Anna I; Gauglitz, Gerd G; Barret, Juan P; Jeschke, Marc G

    2014-11-01

    Keloids and hypertrophic scars occur anywhere from 30 to 90% of patients, and are characterized by pathologically excessive dermal fibrosis and aberrant wound healing. Both entities have different clinical and histochemical characteristics, and unfortunately still represent a great challenge for clinicians due to lack of efficacious treatments. Current advances in molecular biology and genetics reveal new preventive and therapeutical options which represent a hope to manage this highly prevalent, chronic and disabling problem, with long-term beneficial outcomes and improvement of quality of life. While we wait for these translational clinical products to be marketed, however, it is imperative to know the basics of the currently existing wide array of strategies to deal with excessive scars: from the classical corticotherapy, to the most recent botulinum toxin and lasers. The main aim of this review paper is to offer a useful up-to-date guideline to prevent and treat keloids and hypertrophic scars. Copyright © 2014 Elsevier Ltd and ISBI. All rights reserved.

  4. Left ventricular assist device implantation in a patient who had previously undergone apical myectomy for hypertrophic cardiomyopathy.

    Science.gov (United States)

    Cho, Yang Hyun; Deo, Salil V; Topilsky, Yan; Grogan, Martha A; Park, Soon J

    2012-03-01

    Apical hypertrophy is a rare variant of hypertropic cardiomyopathy. These patients may present with end-stage congestive heart failure subsequent to long standing diastolic dysfunction. We report the technique for left ventricular assist device insertion in a patient with previous apical myectomy for hypertrophic cardiomyopathy. © 2012 Wiley Periodicals, Inc.

  5. Quality of life and psychological distress in hypertrophic cardiomyopathy mutation carriers: A cross-sectional cohort study

    NARCIS (Netherlands)

    Christiaans, Imke; Van Langen, Irene M.; Birnie, Erwin; Bonsel, Gouke J.; Wilde, Arthur A.M.; Smets, Ellen M.A.

    2009-01-01

    Hypertrophic cardiomyopathy (HCM) is a common hereditary heart disease associated with heart failure and sudden death.Quality of life and psychological distress were found to be impaired in HCM patients but have never been assessed in mutationcarriers, with or without manifest HCM. We aimed to

  6. Validation of the HCM Risk-SCD model in patients with hypertrophic cardiomyopathy following alcohol septal ablation

    DEFF Research Database (Denmark)

    Liebregts, Max; Faber, Lothar; Jensen, Morten K

    2018-01-01

    Aims: The HCM Risk-SCD model for prediction of sudden cardiac death (SCD) in hypertrophic cardiomyopathy recommended by the 2014 European Society of Cardiology (ESC) guidelines has not been validated after septal reduction therapy. The aim of this study was to validate the HCM Risk-SCD model...

  7. Prevalence of exercise-induced left ventricular outflow tract obstruction in symptomatic patients with non-obstructive hypertrophic cardiomyopathy.

    LENUS (Irish Health Repository)

    Shah, J S

    2008-10-01

    Resting left ventricular outflow tract obstruction (LVOTO) occurs in 25% of patients with hypertrophic cardiomyopathy (HCM) and is an important cause of symptoms and disease progression. The prevalence and clinical significance of exercise induced LVOTO in patients with symptomatic non-obstructive HCM is uncertain.

  8. Gamma-Secretase Inhibitors Attenuate Neurotrauma and Neurogenic Acute Lung Injury in Rats by Rescuing the Accumulation of Hypertrophic Microglia

    Directory of Open Access Journals (Sweden)

    Hung-Jung Lin

    2017-12-01

    Full Text Available Background/Aims: In response to traumatic brain injury (TBI, activated microglia exhibit changes in their morphology from the resting ramified phenotype toward the activated hypertrophic or amoeboid phenotype. Here, we provide the first description of the mechanism underlying the neuroprotective effects of γ-secretase inhibitors on TBI outcomes in rats. Methods: The neuroprotective effects of γ-secretase inhibitors such as LY411575 or CHF5074 on TBI-induced neurotoxicity were analysed using a neurological motor function evaluation, cerebral contusion assay, immunohistochemical staining for microglia phenotypes, lung injury score and Evans Blue dye extravasation assay of brain and lung oedema. Results: Hypertrophic or amoeboid microglia accumulated in the injured cortex, the blood-brain-barrier was disrupted and neurological deficits and acute lung injury were observed 4 days after TBI in adult rats. However, a subcutaneous injection of LY411575 (5 mg/kg or CHF5074 (30 mg/kg immediately after TBI and once daily for 3 consecutive days post-TBI significantly attenutaed the accumulation of hypertrophic microglia in the injured brain, neurological injury, and neurogenic acute lung injury. Conclusion: Gamma-secretase inhibitors attenuated neurotrauma and neurogenic acute lung injury in rats by reducing the accumulation of hypertrophic microglia in the vicinity of the lesion.

  9. Mismatched regional myocardial uptake between [sup 123]I-BMIPP and [sup 201]Tl SPECT; Comparison between hypertrophic myocardium and dilated myocardium

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    Otsuka, Makoto; Ichiya, Yuichi; Kuwabara, Yasuo; Sasaki, Masayuki; Fukumura, Toshimitsu; Masuda, Kouji; Ejima, Junichi; Tsuda, Yasuo (Kyushu Univ., Fukuoka (Japan). Faculty of Medicine)

    1992-07-01

    The distribution of a new myocardial fatty acid metabolic agent [sup 123]I-BMIPP was compared in 6 each patients with hypertrophic myocardium (4 cases of hypertensive heart disease and 2 of hypertrophic cardiomyopathy) and dilated myocardium (4 of dilated type of valvular heart disease and 2 of dilated cardiomyopathy) with that of [sup 201]Tl. Mismatched regional myocardial uptake between [sup 123]I-BMIPP and [sup 201]Tl SPECT was observed in all of the hypertrophic myocardium, however no but one in the dilated myocardium. Relative increase or decrease in regional BMIPP from the images of 20 min and to those of 4 h was observed in 3 cases of the hypertrophic myocardium. FDG-PET was performed in 2 cases of the hypertrophic myocardium. The distribution of FDG was different from neither those of BMIPP nor Tl in a hypertrophic cardiomyopathy case with the reserved distribution of BMIPP and Tl. Although more investigations are necessary, we concluded that [sup 123]I-BMIPP is a promising radiopharmaceutical for evaluating myocardial fatty acid metabolism in hypertrophic myocardium. (author).

  10. Clinical efficacy of utilizing Ultrapulse CO2 combined with fractional CO2 laser for the treatment of hypertrophic scars in Asians-A prospective clinical evaluation.

    Science.gov (United States)

    Lei, Ying; Li, Shi Feng; Yu, Yi Ling; Tan, Jun; Gold, Michael H

    2017-06-01

    Hypertrophic scarring is seen regularly. Tissue penetration of laser energy into hypertrophic scars using computer defaults from some lasers may be insufficient and penetration not enough. We have developed a treatment with an interrupted laser "drilling" by the Ultrapulse CO 2 (Manual Fractional Technology, MFT) and, a second pass, with fractional CO 2 . The MFT with fractional CO 2 lasers to treat hypertrophic scars is evaluated. A total of 158 patients with hypertrophic scars had three sessions of MFT with fractional CO 2 laser at 3-month intervals. Evaluations made before and 6 months after the 3rd treatment: (1) the Vancouver Scar Scale (VSS), (2) the University of North Carolina (UNC) Scar Scale, and (3) a survey of patient satisfaction. All data were analyzed using a t-test before and after treatment. The VSS score decreased from 9.35 to 3.12 (Plaser drilling by MFT and a fractional CO2 laser had profound effects on the hypertrophic scars treated. It works by increasing the penetration depth of the CO 2 laser in the scar tissue, exerting more precise effects on the hypertrophic scars. MFT combined with fractional CO 2 laser has the potential to be a major advance in the treatment of hypertrophic scars. © 2017 Wiley Periodicals, Inc.

  11. Visualization of living terminal hypertrophic chondrocytes of growth plate cartilage in situ by differential interference contrast microscopy and time-lapse cinematography.

    Science.gov (United States)

    Farnum, C E; Turgai, J; Wilsman, N J

    1990-09-01

    The functional unit within the growth plate consists of a column of chondrocytes that passes through a sequence of phases including proliferation, hypertrophy, and death. It is important to our understanding of the biology of the growth plate to determine if distal hypertrophic cells are viable, highly differentiated cells with the potential of actively controlling terminal events of endochondral ossification prior to their death at the chondro-osseous junction. This study for the first time reports on the visualization of living hypertrophic chondrocytes in situ, including the terminal hypertrophic chondrocyte. Chondrocytes in growth plate explants are visualized using rectified differential interference contrast microscopy. We record and measure, using time-lapse cinematography, the rate of movement of subcellular organelles at the limit of resolution of this light microscopy system. Control experiments to assess viability of hypertrophic chondrocytes include coincubating organ cultures with the intravital dye fluorescein diacetate to assess the integrity of the plasma membrane and cytoplasmic esterases. In this system, all hypertrophic chondrocytes, including the very terminal chondrocyte, exist as rounded, fully hydrated cells. By the criteria of intravital dye staining and organelle movement, distal hypertrophic chondrocytes are identical to chondrocytes in the proliferative and early hypertrophic cell zones.

  12. Extracorporeal shock wave therapy with low-energy flux density inhibits hypertrophic scar formation in an animal model.

    Science.gov (United States)

    Zhao, Jing-Chun; Zhang, Bo-Ru; Hong, Lei; Shi, Kai; Wu, Wei-Wei; Yu, Jia-Ao

    2018-04-01

    Hypertrophic scar is characterized by excessive deposits of collagen during skin wound healing, which could become a challenge to clinicians. This study assessed the effects of the extracorporeal shock wave therapy (ESWT) on hypertrophic scar formation and the underlying gene regu-lation. A rabbit ear hypertrophic scar model was generated and randomly divided into three groups: L-ESWT group to receive L-ESWT (energy flux density of 0.1 mJ/mm2), H-ESWT (energy flux density of 0.2 mJ/mm2) and sham ESWT group (S-ESWT). Hypertrophic scar tissues were then collected and stained with hematoxylin and eosin (H&E) and Masson's trichrome staining, respectively, to assess scar elevation index (SEI), fibroblast density and collagen fiber arrangement. Expression of cell proliferation marker proliferating cell nuclear antigen (PCNA) and α-smooth muscle actin (α-SMA) were assessed using RT-PCR and immunohistochemistry in hypertrophic scar tissues. H&E staining sections showed significant reduction of SEI and fibroblast density in both ESWT treatment groups compared to S-ESWT, but there was no dramatic difference between L-ESWT and H-ESWT groups. Masson's trichrome staining showed that collagen fibers were more slender and broader and oriented in parallel to skin surface after administration of ESWT compared to control tissues. At the gene level, PCNA‑positive fibroblasts and α-SMA-positive myofibroblasts were significantly decreased after L-ESWT or H-ESWT compared to the controls. Furthermore, there was no significant difference in expression of PCNA mRNA between L-ESWT or H-ESWT and S-ESWT, whereas expression of α-SMA mRNA significantly decreased in L-ESWT compared to that of H-ESWT and S-ESWT (P=0.002 and P=0.030, respectively). In conclusion, L-ESWT could be effective on suppression of hypertrophic scar formation by inhibition of scar elevation index and fibroblast density as well as α-SMA expression in hypertrophic scar tissues of the rabbit model.

  13. A Meta-analysis on the correlation between the polymorphism of angiotensin converting enzyme gene and hypertrophic cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Ling CHEN

    2014-01-01

    Full Text Available Objective To systematically investigate the correlation between the polymorphism of angiotensin converting enzyme (ACE gene I/D and hypertrophic cardiomyopathy. Methods The databases, such as PubMed, Embase, OVID, Web of Science, Cochrane library, CNKI, WanFang Data and VIP, were searched to collect the studies on the correlation between ACE I/D polymorphism and hypertrophic cardiomyopathy susceptibility. Studies that met the inclusion criteria were Meta-analyzed using Stata 11.0 software. Results Fifteen articles were collected including 1114 cases and 1648 controls. The Meta-analysis indicated that there was significant correlation between the 4 models of ACE I/D polymorphism and hypertrophic cardiomyopathy susceptibility [D vs I: OR=1.49, 95%CI (1.20, 1.84; DD vs (ID+II: OR=1.56, 95%CI (1.17, 2.08; (DD+ID vs II: OR=1.76, 95%CI (1.30, 2.38; DD vs II: OR=2.20, 95%CI (1.44, 3.37]. In subgroup analysis, the significant difference existed in Asian population, but no significance was found in European population (P<0.05. Conclusions There is a positive correlation between hypertrophic cardiomyopathy and ACE I/D polymorphism in population, and D allele and DD genotype are likely to be the risk factors of hypertrophic cardiomyopathy. But such correlation does not exist in European population. DOI: 10.11855/j.issn.0577-7402.2013.12.07

  14. Identifying unmet clinical need in hypertrophic cardiomyopathy using national electronic health records.

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    Mar Pujades-Rodriguez

    Full Text Available To evaluate unmet clinical need in unselected hypertrophic cardiomyopathy (HCM patients to determine the risk of a wide range of subsequent cardiovascular disease endpoints and safety endpoints relevant for trial design.Population based cohort (CALIBER, linked primary care, hospital and mortality records in England, period 1997-2010, all people diagnosed with HCM were identified and matched by age, sex and general practice with ten randomly selected people without HCM. Random-effects Poisson models were used to assess the associations between HCM and cardiovascular diseases and bleeding.Among 3,290,455 eligible people a diagnosis of hypertrophic cardiomyopathy was found in 4 per 10,000. Forty-one percent of the 1,160 individuals with hypertrophic cardiomyopathy were women and the median age was 57 years. The median follow-up was 4.0 years. Compared to general population controls, people with HCM had higher risk of ventricular arrhythmia (incidence rate ratio = 23.53, [95% confidence interval 12.67-43.72], cardiac arrest or sudden cardiac death (6.33 [3.69-10.85], heart failure (4.31, [3.30-5.62], and atrial fibrillation (3.80 [3.04-4.75]. HCM was also associated with a higher incidence of myocardial infarction ([MI] 1.90 [1.27-2.84] and coronary revascularisation (2.32 [1.46-3.69].The absolute Kaplan-Meier risks at 3 years were 8.8% for the composite endpoint of cardiovascular death or heart failure, 8.4% for the composite of cardiovascular death, stroke or myocardial infarction, and 1.5% for major bleeding.Our study identified major unmet need in HCM and highlighted the importance of implementing improved cardiovascular prevention strategies to increase life-expectancy of the contemporary HCM population. They also show that national electronic health records provide an effective method for identifying outcomes and clinically relevant estimates of composite efficacy and safety endpoints essential for trial design in rare diseases.

  15. Identifying unmet clinical need in hypertrophic cardiomyopathy using national electronic health records.

    Science.gov (United States)

    Pujades-Rodriguez, Mar; Guttmann, Oliver P; Gonzalez-Izquierdo, Arturo; Duyx, Bram; O'Mahony, Constantinos; Elliott, Perry; Hemingway, Harry

    2018-01-01

    To evaluate unmet clinical need in unselected hypertrophic cardiomyopathy (HCM) patients to determine the risk of a wide range of subsequent cardiovascular disease endpoints and safety endpoints relevant for trial design. Population based cohort (CALIBER, linked primary care, hospital and mortality records in England, period 1997-2010), all people diagnosed with HCM were identified and matched by age, sex and general practice with ten randomly selected people without HCM. Random-effects Poisson models were used to assess the associations between HCM and cardiovascular diseases and bleeding. Among 3,290,455 eligible people a diagnosis of hypertrophic cardiomyopathy was found in 4 per 10,000. Forty-one percent of the 1,160 individuals with hypertrophic cardiomyopathy were women and the median age was 57 years. The median follow-up was 4.0 years. Compared to general population controls, people with HCM had higher risk of ventricular arrhythmia (incidence rate ratio = 23.53, [95% confidence interval 12.67-43.72]), cardiac arrest or sudden cardiac death (6.33 [3.69-10.85]), heart failure (4.31, [3.30-5.62]), and atrial fibrillation (3.80 [3.04-4.75]). HCM was also associated with a higher incidence of myocardial infarction ([MI] 1.90 [1.27-2.84]) and coronary revascularisation (2.32 [1.46-3.69]).The absolute Kaplan-Meier risks at 3 years were 8.8% for the composite endpoint of cardiovascular death or heart failure, 8.4% for the composite of cardiovascular death, stroke or myocardial infarction, and 1.5% for major bleeding. Our study identified major unmet need in HCM and highlighted the importance of implementing improved cardiovascular prevention strategies to increase life-expectancy of the contemporary HCM population. They also show that national electronic health records provide an effective method for identifying outcomes and clinically relevant estimates of composite efficacy and safety endpoints essential for trial design in rare diseases.

  16. Bilateral Hypertrophic Olivary Degeneration and Holmes Tremor without Palatal Tremor: An Unusual Association

    Directory of Open Access Journals (Sweden)

    Carlos Cosentino

    2016-07-01

    Full Text Available Background: Lesions in the Guillain–Mollaret triangle or dentate-rubro-olivary pathway may lead to hypertrophic olivary degeneration (HOD, a secondary trans-synaptic degeneration of the inferior olivary nucleus. HOD is usually associated with palatal tremor and rarely with Holmes tremor. Bilateral HOD is a very unusual condition and very few cases are reported. Case Report: We report here two cases of bilateral HOD after two different vascular lesions located at the decussation of superior cerebellar peduncles, thus impairing both central tegmental tracts and interrupting bilaterally the dentate-rubral-olivary pathway. Interestingly, both developed bilateral Holmes tremor but not palatal tremor. Discussion: Lesions in some of the components in the Guillain–Mollaret triangle may develop Holmes tremor with HOD and without palatal tremor. Magnetic resonance imaging is an invaluable tool in these cases. Better understanding of the pathways in this loop is needed.

  17. 5-Fluorouracil-Loaded Transfer some as Theranostics in Dermal Tumor of Hypertrophic Scar Tissue

    International Nuclear Information System (INIS)

    Zhang, Z.; Wang, X.; Chen, X.; Wo, Y.; Zhang, Y.; Biskup, E.

    2015-01-01

    To investigate the ability of transfersomal gel carrying the anti scarring agent (5-FU) to permeate hypertrophic scars in vivo and in vitro, scar permeation studies were performed after the agent was labeled with the fluorescent agent, rhodamine 6GO. Laser con focal microscope was employed to dynamically observe the effects of transfersomal gel carrying 5-FU at different time points. High-performance liquid chromatography (HPLC) was used to analyze the contents of the agent in the scar tissues at different hours after administration. Scar elevation index (SEI) was used to evaluate the changes of the ear scar models in rabbits. Compared with the PBS gel of 5-FU, the transfers omal gel displayed greater permeation rate and depth, as well as a higher content retention of the agent in scar tissues. Local administrations of the agent for some certain periods effectively inhibited the hyperplasia of ear scars in rabbits. Transfersomes can be chosen as a potential transdermal drug delivery system

  18. Isolated papillary muscle hypertrophy: A gap in our knowledge of hypertrophic cardiomyopathy?

    Science.gov (United States)

    Ferreira, Catarina; Delgado, Carlos; Vázquez, María; Trinidad, Carmen; Vilar, Manuel

    2014-06-01

    Increased thickness of left ventricular walls is the predominant characteristic and one of the diagnostic criteria of hypertrophic cardiomyopathy (HCM). This case illustrates an uncommon but important finding of isolated hypertrophy of the papillary muscles (PMs), observed in a young woman in whom an abnormal electrocardiogram was initially detected. During the investigation isolated PM hypertrophy was identified. The structural characteristics of the PMs have received scant attention in this setting and there is little information in the literature on this entity, whose real prevalence and clinical significance remain to be determined. The available information relates solitary PM hypertrophy with an early form or a different pattern of HCM. In this case PM hypertrophy was only detected due to the finding of an abnormal electrocardiogram, which prompted further diagnostic tests and a search for possible etiologies. Copyright © 2013 Sociedade Portuguesa de Cardiologia. Published by Elsevier España. All rights reserved.

  19. Cardiac magnetic resonance and computed tomography in hypertrophic cardiomyopathy: an update

    Energy Technology Data Exchange (ETDEWEB)

    Oliveira, Diogo Costa Leandro de; Assunção, Fernanda Boldrini; Santos, Alair Agusto Sarmet Moreira Damas dos; Nacif, Marcelo Souto, E-mail: diogocloliveira@hotmail.com, E-mail: diogocloliveira@gmail.com [Universidade Federal Fluminense (UFF), Niterói, Rio de Janeiro, RJ (Brazil)

    2016-08-15

    Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiovascular disease and represents the main cause of sudden death in young patients. Cardiac magnetic resonance (CMR) and cardiac computed tomography (CCT) are noninvasive imaging methods with high sensitivity and specificity, useful for the establishment of diagnosis and prognosis of HCM, and for the screening of patients with subclinical phenotypes. The improvement of image analysis by CMR and CCT offers the potential to promote interventions aiming at stopping the natural course of the disease. This study aims to describe the role of RCM and CCT in the diagnosis and prognosis of HCM, and how these methods can be used in the management of these patients. (author)

  20. Feeding of two Cichlidae species (Perciformes in an hypertrophic urban lake

    Directory of Open Access Journals (Sweden)

    Yafe Alejandro

    2002-01-01

    Full Text Available Diet of two cichlid species, Cichlasoma facetum (Jenyns, 1842, and Gymnogeophagus rhabdotus Hensel, 1870, was studied in Rodó Lake, an urban hypertrophic lake in Uruguay. The stomach contents from 192 individuals of C. facetum and 202 of G. rhabdotus, obtained through seasonal sampling in the year 2000, were analyzed. The occurrence frequency and the alimentary importance index of each food item were calculated for each season and size class in both species. Cichlasoma facetum fed upon insects (mainly chironomid larvae and pupae, fish (Cnesterodon decemmaculatus Jenyns, 1842, and vegetals (algae, periphyton and macrophytes debris; large individuals also fed upon the freshwater shrimp Palaemonetes argentinus Nobili, 1901. Gymnogeophagus rhabdotus consumed zooplankton (mainly copepods, vegetals (algae and detritus and Chironomidae larvae in a lesser extent.

  1. Maternal hyperthyroidism after intrauterine insemination due to hypertrophic action of human chorionic gonadotropin: a case report.

    Science.gov (United States)

    Bakas, P; Tzouma, C; Creatsa, M; Boutas, I; Hassiakos, D

    2016-01-01

    To report a rare case of maternal hyperthyroidism after intrauterine insemination due to hypertrophic action of hCG. A 36-year-old woman after successful intrauterine insemination and triplet pregnancy, developed hyperthyroidism with resistance to medical treatment. All signs of hyperthyroidism resolved and the results of thyroid function tests returned to normal without any medication after embryo meiosis. De novo maternal hyperthyroidism may develop during pregnancy as a result of pathological stimulation of the thyroid gland from the high levels of hCG hormone that can be seen in multiple pregnancies. The risk of hyperthyroidism is related to the number of fetuses. Reversibility of symptomatology can be seen after fetal reduction of multiple pregnancies.

  2. Isometric parameters in the monitoring of maximal strength, power, and hypertrophic resistance-training.

    Science.gov (United States)

    Peltonen, Heikki; Walker, Simon; Lähitie, Anuliisa; Häkkinen, Keijo; Avela, Janne

    2018-02-01

    This study monitored strength-training adaptations via isometric parameters throughout 2 × 10 weeks of hypertrophic (HYP I-II) or 10 weeks maximum strength (MS) followed by 10 weeks power (P) training with untrained controls. Trainees performed bilateral isometric leg press tests analyzed for peak force (maximal voluntary contraction (MVC)) and rate of force development (RFD) every 3.5 weeks. These parameters were compared with dynamic performance, voluntary and electrically induced isometric contractions, muscle activity, and cross-sectional area (CSA) in the laboratory before and after 10 and 20 weeks. RFD increased similarly during the first 7 weeks (HYP I, 44% ± 53%; MS, 48% ± 55%, P strength/power training, while MVC cannot distinguish between strength or muscle mass changes. Monitoring RFD provided important information regarding plateaus in RFD improvement, which were observed in dynamic explosive performances after HYP II compared with P.

  3. [Hypertrophic cardiomyopathy showing no 123I-BMIPP myocardial accumulation with type I CD36 deficiency].

    Science.gov (United States)

    Watanabe, K; Miyajima, S; Kusano, Y; Tanabe, N; Hirokawa, Y

    1997-07-01

    A 57 years old male consulted our hospital in complaining chest oppression and short of breath. Familial and dilated phase hypertrophic cardiomyopathy (HCM) was detected by ECG, echocardiography, left ventriculography and left ventricular endomyocardial biopsy. 201T1 SPECT showed regional increased accumulation in the ventricular septum, however, no myocardial accumulation of 123I-beta-methyl-p-iodophenylpentadecanoic acid (123I-BMIPP) was observed. We analyzed CD36 in this patient, and found he had type 1 CD36 deficiency. Myocardial uptake of long-chain fatty acids occurs via a specific transporter, which is homologous with human CD36. We hypothesize that CD36 deficiency, especially type 1 CD36 deficiency, might be one factor of no myocardial 123I-BMIPP uptake.

  4. Alcohol drinking triggers acute myocardial infarction in a case of hypertrophic obstructive cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Po-Chao Hsu

    2011-05-01

    Full Text Available Alcohol ingestion–related increased left ventricular outflow tract (LVOT pressure gradient in hypertrophic obstructive cardiomyopathy (HOCM has been reported in the literature; however, acute myocardial infarction (AMI after alcohol drinking in this patient group is rarely reported. Herein, we report a 68-year-old man with chronic alcoholism suffering from AMI after alcohol drinking. Electrocardiography revealed complete left bundle branch block, and chest X-ray showed acute pulmonary edema. Intubation was done for respiratory failure and intra-aortic balloon pump was also inserted for unstable hemodynamics. However, emergent coronary angiography revealed normal coronary arteries. HOCM was diagnosed by a high pressure gradient over LVOT and systolic anterior motion of mitral valve by echocardiography. This patient became stable under intensive care and medical treatment. This case reminds physicians that alcohol ingestion might cause AMI in HOCM patients because of increased LVOT pressure gradient and decreased coronary perfusion despite normal coronary arteries.

  5. PKA catalytic subunit compartmentation regulates contractile and hypertrophic responses to β-adrenergic signaling

    Science.gov (United States)

    Yang, Jason H.; Polanowska-Grabowska, Renata K.; Smith, Jeffrey S.; Shields, Charles W.; Saucerman, Jeffrey J.

    2014-01-01

    β-adrenergic signaling is spatiotemporally heterogeneous in the cardiac myocyte, conferring exquisite control to sympathetic stimulation. Such heterogeneity drives the formation of protein kinase A (PKA) signaling microdomains, which regulate Ca2+ handling and contractility. Here, we test the hypothesis that the nucleus independently comprises a PKA signaling microdomain regulating myocyte hypertrophy. Spatially-targeted FRET reporters for PKA activity identified slower PKA activation and lower isoproterenol sensitivity in the nucleus (t50 = 10.60±0.68 min; EC50 = 89.00 nmol/L) than in the cytosol (t50 = 3.71±0.25 min; EC50 = 1.22 nmol/L). These differences were not explained by cAMP or AKAP-based compartmentation. A computational model of cytosolic and nuclear PKA activity was developed and predicted that differences in nuclear PKA dynamics and magnitude are regulated by slow PKA catalytic subunit diffusion, while differences in isoproterenol sensitivity are regulated by nuclear expression of protein kinase inhibitor (PKI). These were validated by FRET and immunofluorescence. The model also predicted differential phosphorylation of PKA substrates regulating cell contractility and hypertrophy. Ca2+ and cell hypertrophy measurements validated these predictions and identified higher isoproterenol sensitivity for contractile enhancements (EC50 = 1.84 nmol/L) over cell hypertrophy (EC50 = 85.88 nmol/L). Over-expression of spatially targeted PKA catalytic subunit to the cytosol or nucleus enhanced contractile and hypertrophic responses, respectively. We conclude that restricted PKA catalytic subunit diffusion is an important PKA compartmentation mechanism and the nucleus comprises a novel PKA signaling microdomain, insulating hypertrophic from contractile β-adrenergic signaling responses. PMID:24225179

  6. Annexin A7 deficiency potentiates cardiac NFAT activity promoting hypertrophic signaling

    International Nuclear Information System (INIS)

    Voelkl, Jakob; Alesutan, Ioana; Pakladok, Tatsiana; Viereck, Robert; Feger, Martina; Mia, Sobuj; Schönberger, Tanja; Noegel, Angelika A.; Gawaz, Meinrad; Lang, Florian

    2014-01-01

    Highlights: • Cardiac Anxa7 expression was up-regulated following TAC. • The hypertrophic response following TAC was augmented in Anxa7-deficient mice. • Silencing of Anxa7 increased indicators of HL-1 cardiomyocytes hypertrophy. • Silencing of Anxa7 induced Nfatc1 nuclear translocation. • Silencing of Anxa7 enhanced NFAT-dependent transcriptional activity. - Abstract: Annexin A7 (Anxa7) is a cytoskeletal protein interacting with Ca 2+ signaling which in turn is a crucial factor for cardiac remodeling following cardiac injury. The present study explored whether Anxa7 participates in the regulation of cardiac stress signaling. To this end, mice lacking functional Anxa7 (anxa7 −/− ) and wild-type mice (anxa7 +/+ ) were investigated following pressure overload by transverse aortic constriction (TAC). In addition, HL-1 cardiomyocytes were silenced with Anxa7 siRNA and treated with isoproterenol. Transcript levels were determined by quantitative RT-PCR, transcriptional activity by luciferase reporter assay and protein abundance by Western blotting and confocal microscopy. As a result, TAC treatment increased the mRNA and protein levels of Anxa7 in wild-type mice. Moreover, TAC increased heart weight to body weight ratio and the cardiac mRNA levels of αSka, Nppb, Col1a1, Col3a1 and Rcan1, effects more pronounced in anxa7 −/− mice than in anxa7 +/+ mice. Silencing of Anxa7 in HL-1 cardiomyocytes significantly increased nuclear localization of Nfatc1. Furthermore, Anxa7 silencing increased NFAT-dependent transcriptional activity as well as αSka, Nppb, and Rcan1 mRNA levels both, under control conditions and following β-adrenergic stimulation by isoproterenol. These observations point to an important role of annexin A7 in the regulation of cardiac NFAT activity and hypertrophic response following cardiac stress conditions

  7. Clinical long-term outcome of septal myectomy for obstructive hypertrophic cardiomyopathy in infants.

    Science.gov (United States)

    Schleihauf, Julia; Cleuziou, Julie; Pabst von Ohain, Jelena; Meierhofer, Christian; Stern, Heiko; Shehu, Nerejda; Mkrtchyan, Naira; Kaltenecker, Emanuel; Kühn, Andreas; Nagdyman, Nicole; Hager, Alfred; Seidel, Heide; Lange, Rüdiger; Ewert, Peter; Wolf, Cordula M

    2018-03-01

    Surgical septal myectomy is performed to relieve left ventricular outflow tract narrowing in severe drug-refractory obstructive hypertrophic cardiomyopathy. The objective of this study was to assess the perioperative and long-term clinical outcome of this procedure performed during infancy. Clinical, transthoracic echocardiographic, electrocardiographic, 24-h Holter, cardiopulmonary exercise test and genetic data were extracted by medical record review. A subset of patients underwent additional prospective detailed clinical evaluation including cardiac magnetic resonance imaging with contrast. Surgery was performed in 23 paediatric patients between 1978 and 2015 at the German Heart Centre Munich. Twelve patients had undergone surgery during infancy (≤ 1 year) (Group A), 11 between 1 and 18 years of age (Group B). The underlying genetic diagnosis was Noonan syndrome spectrum and non-syndromic hypertrophic cardiomyopathy. As compared to Group B, patients in Group A showed more concomitant cardiac procedures and received more homologous transfusions. One perioperative death occurred in Group A, and none in Group B. Two patients in Group A but no patient in Group B required redo septal myectomy. The long-term clinical outcome was similar between the 2 groups. One patient in Group B required cardioverter-defibrillator/pacemaker implantation for higher degree atrioventricular block and none in Group A. There was no evidence of differences in myocardial fibrosis between groups on long-term follow-up magnetic resonance imaging. Surgical septal myectomy can be performed safely during infancy with favourable perioperative and long-term clinical outcome but with a trend towards a higher reoperation rate later in life. © The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

  8. Risk and Causes of Death in Patients After Alcohol Septal Ablation for Hypertrophic Obstructive Cardiomyopathy.

    Science.gov (United States)

    Veselka, Josef; Zemánek, David; Jahnlová, Denisa; Krejčí, Jan; Januška, Jaroslav; Dabrowski, Maciej; Bartel, Thomas; Tomašov, Pavol

    2015-10-01

    Because the final myocardial scar might be theoretically associated with an increased risk of sudden cardiac death, the long-term clinical course of patients who undergo alcohol septal ablation (ASA) is still a matter of debate. In this retrospective multicentre study, we report outcomes after ASA, including survival, analysis of causes of deaths, and association between time and cause of death. We enrolled 366 consecutive patients (58 ± 12 years, 54% women) who were treated using ASA and followed-up for 5.1 ± 4.5 years. The in-hospital and 30-day mortality were 0.5% and 0.8%, respectively; the ASA-related morbidity was cause mortality rate was 2.8% per year. The mortality rates of sudden death and sudden death with an appropriate implantable cardioverter-defibrillator (ICD) discharge were 0.4% and 1% per year, respectively. Patients with sudden death or appropriate ICD discharge experienced these mortality events at younger age than patients who died of other hypertrophic obstructive cardiomyopathy-related causes (60.8 years [range, 52-71.5 years] vs 72.4 years [range, 64.2-75.2 years]; P = 0.048). A total of 292 patients (80%) had an outflow gradient ≤ 30 mm Hg, and 327 patients (89%) were in New York Heart Association class ≤ II at the last clinical check-up. ASA had low procedure-related mortality, with subsequent 1% occurrence of sudden mortality events per year and 2.8% mortality rate per year in the long-term follow-up. Patients with sudden death or ICD discharge experienced the mortality events approximately 1 decade earlier than patients who died from other causes not related to hypertrophic cardiomyopathy. Copyright © 2015 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.

  9. Rising CO2 levels will intensify phytoplankton blooms in eutrophic and hypertrophic lakes.

    Directory of Open Access Journals (Sweden)

    Jolanda M H Verspagen

    Full Text Available Harmful algal blooms threaten the water quality of many eutrophic and hypertrophic lakes and cause severe ecological and economic damage worldwide. Dense blooms often deplete the dissolved CO2 concentration and raise pH. Yet, quantitative prediction of the feedbacks between phytoplankton growth, CO2 drawdown and the inorganic carbon chemistry of aquatic ecosystems has received surprisingly little attention. Here, we develop a mathematical model to predict dynamic changes in dissolved inorganic carbon (DIC, pH and alkalinity during phytoplankton bloom development. We tested the model in chemostat experiments with the freshwater cyanobacterium Microcystis aeruginosa at different CO2 levels. The experiments showed that dense blooms sequestered large amounts of atmospheric CO2, not only by their own biomass production but also by inducing a high pH and alkalinity that enhanced the capacity for DIC storage in the system. We used the model to explore how phytoplankton blooms of eutrophic waters will respond to rising CO2 levels. The model predicts that (1 dense phytoplankton blooms in low- and moderately alkaline waters can deplete the dissolved CO2 concentration to limiting levels and raise the pH over a relatively wide range of atmospheric CO2 conditions, (2 rising atmospheric CO2 levels will enhance phytoplankton blooms in low- and moderately alkaline waters with high nutrient loads, and (3 above some threshold, rising atmospheric CO2 will alleviate phytoplankton blooms from carbon limitation, resulting in less intense CO2 depletion and a lesser increase in pH. Sensitivity analysis indicated that the model predictions were qualitatively robust. Quantitatively, the predictions were sensitive to variation in lake depth, DIC input and CO2 gas transfer across the air-water interface, but relatively robust to variation in the carbon uptake mechanisms of phytoplankton. In total, these findings warn that rising CO2 levels may result in a marked

  10. Pathogenesis of depression- and anxiety-like behavior in an animal model of hypertrophic cardiomyopathy.

    Science.gov (United States)

    Dossat, Amanda M; Sanchez-Gonzalez, Marcos A; Koutnik, Andrew P; Leitner, Stefano; Ruiz, Edda L; Griffin, Brittany; Rosenberg, Jens T; Grant, Samuel C; Fincham, Francis D; Pinto, Jose R; Kabbaj, Mohamed

    2017-06-01

    Cardiovascular dysfunction is highly comorbid with mood disorders, such as anxiety and depression. However, the mechanisms linking cardiovascular dysfunction with the core behavioral features of mood disorder remain poorly understood. In this study, we used mice bearing a knock-in sarcomeric mutation, which is exhibited in human hypertrophic cardiomyopathy (HCM), to investigate the influence of HCM over the development of anxiety and depression. We employed behavioral, MRI, and biochemical techniques in young (3-4 mo) and aged adult (7-8 mo) female mice to examine the effects of HCM on the development of anxiety- and depression-like behaviors. We focused on females because in both humans and rodents, they experience a 2-fold increase in mood disorder prevalence vs. males. Our results showed that young and aged HCM mice displayed echocardiographic characteristics of the heart disease condition, yet only aged HCM females displayed anxiety- and depression-like behaviors. Electrocardiographic parameters of sympathetic nervous system activation were increased in aged HCM females vs. controls and correlated with mood disorder-related symptoms. In addition, when compared with controls, aged HCM females exhibited adrenal gland hypertrophy, reduced volume in mood-related brain regions, and reduced hippocampal signaling proteins, such as brain-derived neurotrophic factor and its downstream targets vs. controls. In conclusion, prolonged systemic HCM stress can lead to development of mood disorders, possibly through inducing structural and functional brain changes, and thus, mood disorders in patients with heart disease should not be considered solely a psychologic or situational condition.-Dossat, A. M., Sanchez-Gonzalez, M. A., Koutnik, A. P., Leitner, S., Ruiz, E. L., Griffin, B., Rosenberg, J. T., Grant, S. C., Fincham, F. D., Pinto, J. R. Kabbaj, M. Pathogenesis of depression- and anxiety-like behavior in an animal model of hypertrophic cardiomyopathy. © FASEB.

  11. Hypertrophic remodeling of subcutaneous small resistance arteries in patients with Cushing's syndrome.

    Science.gov (United States)

    Rizzoni, Damiano; Porteri, Enzo; De Ciuceis, Carolina; Rodella, Luigi F; Paiardi, Silvia; Rizzardi, Nicola; Platto, Caterina; Boari, Gianluca E M; Pilu, Annamaria; Tiberio, Guido A M; Giulini, Stefano M; Favero, Gaia; Rezzani, Rita; Rosei, Claudia Agabiti; Bulgari, Giuseppe; Avanzi, Daniele; Rosei, Enrico Agabiti

    2009-12-01

    Structural alterations of small resistance arteries in essential hypertensive patients (EH) are mostly characterized by inward eutrophic remodeling. However, we observed hypertrophic remodeling in patients with renovascular hypertension, in those with acromegaly, as well as in patients with non-insulin-dependent diabetes mellitus, suggesting a relevant effect of humoral growth factors on vascular structure, even independent from the hemodynamic load. Cortisol may stimulate the renin-angiotensin system and may induce cardiac hypertrophy. However, presently no data are available about small artery structure in patients with Cushing's syndrome. We have investigated the structure of sc small resistance arteries in 12 normotensive subjects (NT), in 12 EH subjects, and in eight patients with Cushing's syndrome (CS). Small arteries from sc fat were dissected and mounted on a micromyograph. The normalized internal diameter, media thickness, media to lumen ratio, and the media cross-sectional area were measured, as well as indices of oxidative stress. Demographic variables were similar in the three groups, except for clinic blood pressure. The media to lumen ratio was significantly greater in EH and CS, compared with NT; no difference was observed between EH and CS. The media cross-sectional area was significantly greater in CS compared with EH and with NT. An increased vascular oxidative stress was present in CS, as demonstrated by increased levels of superoxide anions, cyclooxygenase-1 and endothelial nitric oxide synthase in the microvessels. Our results suggest the presence of hypertrophic remodeling in sc small resistance arteries of CS, probably as a consequence of growth-promoting properties of circulating cortisol and/or increased vascular oxidative stress.

  12. Attenuated hypertrophic response to pressure overload in a lamin A/C haploinsufficiency mouse.

    Science.gov (United States)

    Cupesi, Mihaela; Yoshioka, Jun; Gannon, Joseph; Kudinova, Anastacia; Stewart, Colin L; Lammerding, Jan

    2010-06-01

    Inherited mutations cause approximately 30% of all dilated cardiomyopathy cases, with autosomal dominant mutations in the LMNA gene accounting for more than one third of these. The LMNA gene encodes the nuclear envelope proteins lamins A and C, which provide structural support to the nucleus and also play critical roles in transcriptional regulation. Functional deletion of a single allele is sufficient to trigger dilated cardiomyopathy in humans and mice. However, whereas Lmna(-/-) mice develop severe muscular dystrophy and dilated cardiomyopathy and die by 8 weeks of age, heterozygous Lmna(+/-) mice have a much milder phenotype, with changes in ventricular function and morphology only becoming apparent at 1 year of age. Here, we studied 8- to 20-week-old Lmna(+/-) mice and wild-type littermates in a pressure overload model to examine whether increased mechanical load can accelerate or exacerbate myocardial dysfunction in the heterozygotes. While overall survival was similar between genotypes, Lmna(+/-) animals had a significantly attenuated hypertrophic response to pressure overload as evidenced by reduced ventricular mass and myocyte size. Analysis of pressure overload-induced transcriptional changes suggested that the reduced hypertrophy in the Lmna(+/-) mice was accompanied by impaired activation of the mechanosensitive gene Egr-1. In conclusion, our findings provide further support for a critical role of lamins A and C in regulating the cellular response to mechanical stress in cardiomyocytes and demonstrate that haploinsufficiency of lamins A and C alone is sufficient to alter hypertrophic responses and cardiac function in the face of pressure overload in the heart. (c) 2009 Elsevier Ltd. All rights reserved.

  13. Nonsurgical reduction of the interventricular septum in patients with hypertrophic cardiomyopathy.

    Science.gov (United States)

    Shamim, Waqar; Yousufuddin, Mohammed; Wang, Duolao; Henein, Michael; Seggewiss, Hubert; Flather, Marcus; Coats, Andrew J S; Sigwart, Ulrich

    2002-10-24

    In patients with hypertrophic cardiomyopathy and obstruction of the left ventricular outflow tract, nonsurgical reduction of the septum is a treatment option when medical therapy has failed. We investigated the long-term effects of nonsurgical reduction of the septum on functional capacity and electrocardiographic and echocardiographic characteristics. Sixty-four consecutive patients with hypertrophic cardiomyopathy and a mean (+/-SD) age of 48.5+/-17.2 years underwent nonsurgical reduction of the septum by injection of ethanol into the septal perforator branch of the left anterior descending coronary artery. These patients were assessed by exercise testing, electrocardiography, and resting and dobutamine (stress-induced) echocardiography after a mean period of 3.0+/-1.3 years. At follow-up, patients had significant improvements in New York Heart Association class, peak oxygen consumption (from 18.4+/-5.8 to 30.0+/-4.4 ml per kilogram of body weight per minute, P<0.001), and left ventricular outflow tract gradients (resting gradient, from 64+/-36 to 16+/-15 mm Hg; P<0.001; stress-induced gradient, from 132+/-34 to 45+/-19 mm Hg; P<0.001). Procedure-related complications included right bundle-branch block in all patients, complete heart block in 31 patients (48 percent), and significant increases in QRS and corrected QT intervals. Seventeen patients (27 percent) required permanent pacing. R-wave amplitude was significantly decreased (from 32+/-8 to 17+/-7 mV, P<0.001). The dimensions of the left ventricular cavity increased, and the interventricular septal thickness was reduced. Nonsurgical septal reduction leads to sustained improvements in both subjective and objective measures of exercise capacity in association with a persistent reduction in resting and stress-induced left ventricular outflow tract gradients. It is also associated with a high incidence of procedure-related complete heart block, however, often requiring permanent pacing. Copyright 2002

  14. Annexin A7 deficiency potentiates cardiac NFAT activity promoting hypertrophic signaling

    Energy Technology Data Exchange (ETDEWEB)

    Voelkl, Jakob; Alesutan, Ioana; Pakladok, Tatsiana; Viereck, Robert; Feger, Martina; Mia, Sobuj [Department of Physiology, University of Tübingen, Tübingen (Germany); Schönberger, Tanja [Department of Cardiology and Cardiovascular Medicine, University of Tübingen, Tübingen (Germany); Noegel, Angelika A. [Center for Biochemistry, Institute of Biochemistry I, University of Cologne, Köln (Germany); Gawaz, Meinrad [Department of Cardiology and Cardiovascular Medicine, University of Tübingen, Tübingen (Germany); Lang, Florian, E-mail: florian.lang@uni-tuebingen.de [Department of Physiology, University of Tübingen, Tübingen (Germany)

    2014-02-28

    Highlights: • Cardiac Anxa7 expression was up-regulated following TAC. • The hypertrophic response following TAC was augmented in Anxa7-deficient mice. • Silencing of Anxa7 increased indicators of HL-1 cardiomyocytes hypertrophy. • Silencing of Anxa7 induced Nfatc1 nuclear translocation. • Silencing of Anxa7 enhanced NFAT-dependent transcriptional activity. - Abstract: Annexin A7 (Anxa7) is a cytoskeletal protein interacting with Ca{sup 2+} signaling which in turn is a crucial factor for cardiac remodeling following cardiac injury. The present study explored whether Anxa7 participates in the regulation of cardiac stress signaling. To this end, mice lacking functional Anxa7 (anxa7{sup −/−}) and wild-type mice (anxa7{sup +/+}) were investigated following pressure overload by transverse aortic constriction (TAC). In addition, HL-1 cardiomyocytes were silenced with Anxa7 siRNA and treated with isoproterenol. Transcript levels were determined by quantitative RT-PCR, transcriptional activity by luciferase reporter assay and protein abundance by Western blotting and confocal microscopy. As a result, TAC treatment increased the mRNA and protein levels of Anxa7 in wild-type mice. Moreover, TAC increased heart weight to body weight ratio and the cardiac mRNA levels of αSka, Nppb, Col1a1, Col3a1 and Rcan1, effects more pronounced in anxa7{sup −/−} mice than in anxa7{sup +/+} mice. Silencing of Anxa7 in HL-1 cardiomyocytes significantly increased nuclear localization of Nfatc1. Furthermore, Anxa7 silencing increased NFAT-dependent transcriptional activity as well as αSka, Nppb, and Rcan1 mRNA levels both, under control conditions and following β-adrenergic stimulation by isoproterenol. These observations point to an important role of annexin A7 in the regulation of cardiac NFAT activity and hypertrophic response following cardiac stress conditions.

  15. Rising CO2 Levels Will Intensify Phytoplankton Blooms in Eutrophic and Hypertrophic Lakes

    Science.gov (United States)

    Verspagen, Jolanda M. H.; Van de Waal, Dedmer B.; Finke, Jan F.; Visser, Petra M.; Van Donk, Ellen; Huisman, Jef

    2014-01-01

    Harmful algal blooms threaten the water quality of many eutrophic and hypertrophic lakes and cause severe ecological and economic damage worldwide. Dense blooms often deplete the dissolved CO2 concentration and raise pH. Yet, quantitative prediction of the feedbacks between phytoplankton growth, CO2 drawdown and the inorganic carbon chemistry of aquatic ecosystems has received surprisingly little attention. Here, we develop a mathematical model to predict dynamic changes in dissolved inorganic carbon (DIC), pH and alkalinity during phytoplankton bloom development. We tested the model in chemostat experiments with the freshwater cyanobacterium Microcystis aeruginosa at different CO2 levels. The experiments showed that dense blooms sequestered large amounts of atmospheric CO2, not only by their own biomass production but also by inducing a high pH and alkalinity that enhanced the capacity for DIC storage in the system. We used the model to explore how phytoplankton blooms of eutrophic waters will respond to rising CO2 levels. The model predicts that (1) dense phytoplankton blooms in low- and moderately alkaline waters can deplete the dissolved CO2 concentration to limiting levels and raise the pH over a relatively wide range of atmospheric CO2 conditions, (2) rising atmospheric CO2 levels will enhance phytoplankton blooms in low- and moderately alkaline waters with high nutrient loads, and (3) above some threshold, rising atmospheric CO2 will alleviate phytoplankton blooms from carbon limitation, resulting in less intense CO2 depletion and a lesser increase in pH. Sensitivity analysis indicated that the model predictions were qualitatively robust. Quantitatively, the predictions were sensitive to variation in lake depth, DIC input and CO2 gas transfer across the air-water interface, but relatively robust to variation in the carbon uptake mechanisms of phytoplankton. In total, these findings warn that rising CO2 levels may result in a marked intensification of

  16. An International External Validation Study of the 2014 European Society of Cardiology Guideline on Sudden Cardiac Death Prevention in Hypertrophic Cardiomyopathy (Evidence from HCM)

    DEFF Research Database (Denmark)

    O'Mahony, Constantinos; Jichi, Fatima; Ommen, Steve R

    2018-01-01

    Background -Identification of people with hypertrophic cardiomyopathy (HCM) who are at risk of sudden cardiac death (SCD) and require prophylactic implantable cardioverter defibrillator (ICD) is challenging. In 2014, the European Society of Cardiology (ESC) proposed a new risk stratification meth...

  17. Leucine - /sup 14/C transport through erythrocyte cell membrane in newborns with hypertrophic constriction of the pylorus

    Energy Technology Data Exchange (ETDEWEB)

    Stepniewski, M; Janik, A [Akademia Medyczna, Krakow (Poland)

    1980-01-01

    In 12 newborns with hypertrophic constriction of the pylorus the coefficient of the leucine - /sup 14/C distribution in the erythrocyte intracellular twice: the first time during day prior the operation corresponding to advanced malnutrition of the newborns, and the second time seven days after pylorotomy. During the second period the effects of hyponutrition were partially balanced. The obtained data were compared with that noted in 12 healthy newborns and additionally with data of examination done with samples of conserved blood. In newborns with hypertrophic constriction of the pylorus the coefficient of leucine distribution prior the operation was significantly lower than that in the control group and conserved blood. After seven days from operation a significant increase of above coefficient is compared with the control group and erythrocytes in conserved blood has been noted. Above results suggest that disturbances in penetration of leucine through cell membranes of erythrocytes are in association with malnutrition caused by constriction of the pylorus.

  18. Double Chamber Left Ventricle Associated With Severe Form of the Hypertrophic Cardiomyopathy and High Left Intracavitary Pressure

    OpenAIRE

    Bejiqi, Ramush; Retkoceri, Ragip; Bejiqi, Hana; Zeka, Naim; Maloku, Arlinda; Berisha, Majlinda

    2013-01-01

    Double-chambered left ventricle (DCLV) is a rare congenital anomaly, and only a few cases have been reported in the literature, in which a 2-chambered LV is separated by the interventricular septum or an abnormal muscle bundle.We report a case of a girl who was presented at tertiary level for cardiological examination where, during the routine examination systolic murmur was registered. After echocardiographical examination DCLV was confirmed. Anomaly was associated with idiopathic hypertroph...

  19. Significance of left ventricular apical-basal muscle bundle identified by cardiovascular magnetic resonance imaging in patients with hypertrophic cardiomyopathy

    OpenAIRE

    Gruner, Christiane; Chan, Raymond H.; Crean, Andrew; Rakowski, Harry; Rowin, Ethan J.; Care, Melanie; Deva, Djeven; Williams, Lynne; Appelbaum, Evan; Gibson, C. Michael; Lesser, John R.; Haas, Tammy S.; Udelson, James E.; Manning, Warren J.; Siminovitch, Katherine

    2017-01-01

    Aims Cardiovascular magnetic resonance (CMR) has improved diagnostic and management strategies in hypertrophic cardiomyopathy (HCM) by expanding our appreciation for the diverse phenotypic expression. We sought to characterize the prevalence and clinical significance of a recently identified accessory left ventricular (LV) muscle bundle extending from the apex to the basal septum or anterior wall (i.e. apical-basal). Methods and results CMR was performed in 230 genotyped HCM patients (48 ± 15...

  20. Matrix Metalloproteinases Are Differentially Regulated and Responsive to Compression Therapy in a Red Duroc Model of Hypertrophic Scar.

    Science.gov (United States)

    Travis, Taryn E; Ghassemi, Pejhman; Prindeze, Nicholas J; Moffatt, Lauren T; Carney, Bonnie C; Alkhalil, Abdulnaser; Ramella-Roman, Jessica C; Shupp, Jeffrey W

    2018-01-01

    Objective: Proteins of the matrix metalloproteinases family play a vital role in extracellular matrix maintenance and basic physiological processes in tissue homeostasis. The function and activities of matrix metalloproteinases in response to compression therapies have yet to be defined. Here, a swine model of hypertrophic scar was used to profile the transcription of all known 26 matrix metalloproteinases in scars treated with a precise compression dose. Methods: Full-thickness excisional wounds were created. Wounds underwent healing and scar formation. A subset of scars underwent 2 weeks of compression therapy. Biopsy specimens were preserved, and microarrays, reverse transcription-polymerase chain reaction, Western blotting, and immunohistochemistry were performed to characterize the transcription and expression of various matrix metalloproteinase family members. Results: Microarray results showed that 13 of the known 26 matrix metalloproteinases were differentially transcribed in wounds relative to the preinjury skin. The predominant upregulation of these matrix metalloproteinases during early wound-healing stages declined gradually in later stages of wound healing. The use of compression therapy reduced this decline in 10 of the 13 differentially regulated matrix metalloproteinases. Further investigation of MMP7 using reverse transcription-polymerase chain reaction confirmed the effect of compression on transcript levels. Assessment of MMP7 at the protein level using Western blotting and immunohistochemistry was concordant. Conclusions: In a swine model of hypertrophic scar, the application of compression to hypertrophic scar attenuated a trend of decreasing levels of matrix metalloproteinases during the process of hypertrophic wound healing, including MMP7, whose enzyme regulation was confirmed at the protein level.

  1. Comparison of therapeutic response of keloids and hypertrophic scars to cryotherapy plus intralesional steroid and bleomycin tattoo

    Directory of Open Access Journals (Sweden)

    Farahnaz Fatemi

    2005-01-01

    Full Text Available Keloids and hypertrophic scars are abnormal responses of body to skin injuries. Overproduction of compacted fibrous tissue is the basic cause of these lesions. In this study the result of treatment of these skin conditions with bleomycin tattoo are compared with cryotherapy and triamcinolone injection. This study involved 45 patients with hypertrophic scar or keloid. Patients were divided into two groups consecutively. Group A (23 patients was treated with bleomycin tattoo and the group B with cryotherapy and triamcinolone injection. There were four therapeutic sessions one month apart. All patients were followedup for three month after the end of treatment .The therapeutic response was determined as reduction of lesion size or flattening relative to initial size. Therapeutic response was 88.3±14% in group A and 67.4 ±22.5% in group B (p<0.001. In group A 69%, but in group B only 49% of patients were asymptomatic after the end of treatment. In group A there was no relation between therapeutic response and lesion size (p=0.58 but in group B lesions those were smaller (<100mm2 had better therapeutic response than larger ones (p=0.007. It was concluded that bleomycin tattoo is more effective in treatment of hypertrophic scar and keloid than traditional treatment, cryotherapy plus triamcinolone injection especially in larger ones.

  2. Combined pulsed dye laser and fiberoptic Nd-YAG laser for the treatment of hypertrophic port wine stain.

    Science.gov (United States)

    Radmanesh, Mohammed; Radmanesh, Ramin

    2017-10-01

    The hypertrophic Port Wine Stain (PWS) is only partially and superficially treated with the Pulsed dye laser (PDL) because of its limited depth of penetration. We used combined PDL and fiberoptic 1444-nm Nd-YAG laser to treat a case with hypertrophic PWS. After tumescent anesthesia, few holes were made by a 16-gauge needle on different sides of the lesion. The fiberoptic tip of 1444-nm Nd-YAG laser was inserted within the holes and was pushed forward while triggering. In a fan pattern and by a back and forth movement, the subcutaneous and deep dermal areas were coagulated. The skin and outer mucosal surfaces were then treated by PDL. The fiberoptic system used was Accusculpt 1444-nm Nd-YAG laser (Lutronic lasers, South Korea), and the PDL used was 585 nm Nlite system (Chromogenex UK). The parameters used for PDL were fluence = 9 Joules/cm 2 and the spot size was 5 mm. The parameters used for fiberoptic 1444-nm Nd-YAG laser were: Pulse rate = 30 Hz, pulse energy = 300 mJ, power = 6 W, and the total energy = 4000 J for the whole face and mucosa. Little sign of regression and moderate purpura were detected immediately after combined fiberoptic Nd-YAG and PDL therapy. The lesion gradually regressed within 4 months with satisfactory color and volume change. Combined fiberoptic Nd-YAG laser and PDL can be used for the treatment of deeper and superficial layers of hypertrophic PWS.

  3. Pattern of left ventricular hypertrophy seen on transthoracic echo in patients with hypertensive cardiomyopathy when compared with idiopathic hypertrophic cardiomyopathy

    International Nuclear Information System (INIS)

    Mirza, S. J.; Radaideh, G. A.

    2013-01-01

    Objective: To explore the pattern of left ventricular hypertrophy caused by hypertension and to compare it with idiopathic hypertrophic cardiomyopathy. Methods: The retrospective study was conducted at the echocardiography lab of Rashid Hospital, Dubai, from January 2009 to January 2010. Cases of 11 patients with significant left ventricular hypertrophy (septum >15mm) due to underlying hypertension were analysed and compared with 11 cases of idiopathic hypertrophic cardiography (septum >15mm) to assess the two groups with similar baseline echocardiographic features. Minitab software was used for statistical analysis. Results: Although the pattern of hypertrophy in hypertensive patients was more concentric (n=5; 45%), there was also asymmetrical septal hypertrophy in 4 (36%) cases, particularly the elderly with sigmoid shape septum. There was evidence of resting mid-cavity gradient due to reduced left ventricular end-systolic diameter in 4 (36%) cases. Conclusion: Although the equation between hypertension and left ventricular hypertrophy is more concentric, but it can be associated with left ventricular outflow tract obstruction and significant mid-cavity gradients similar to that seen in idiopathic hypertrophic cardiomyopathy. (author)

  4. Autonomous and Non-autonomous Defects Underlie Hypertrophic Cardiomyopathy in BRAF-Mutant hiPSC-Derived Cardiomyocytes

    Directory of Open Access Journals (Sweden)

    Rebecca Josowitz

    2016-09-01

    Full Text Available Germline mutations in BRAF cause cardio-facio-cutaneous syndrome (CFCS, whereby 40% of patients develop hypertrophic cardiomyopathy (HCM. As the role of the RAS/MAPK pathway in HCM pathogenesis is unclear, we generated a human induced pluripotent stem cell (hiPSC model for CFCS from three patients with activating BRAF mutations. By cell sorting for SIRPα and CD90, we generated a method to examine hiPSC-derived cell type-specific phenotypes and cellular interactions underpinning HCM. BRAF-mutant SIRPα+/CD90− cardiomyocytes displayed cellular hypertrophy, pro-hypertrophic gene expression, and intrinsic calcium-handling defects. BRAF-mutant SIRPα−/CD90+ cells, which were fibroblast-like, exhibited a pro-fibrotic phenotype and partially modulated cardiomyocyte hypertrophy through transforming growth factor β (TGFβ paracrine signaling. Inhibition of TGFβ or RAS/MAPK signaling rescued the hypertrophic phenotype. Thus, cell autonomous and non-autonomous defects underlie HCM due to BRAF mutations. TGFβ inhibition may be a useful therapeutic option for patients with HCM due to RASopathies or other etiologies.

  5. Species-specific differences in adaptive phenotypic plasticity in an ecologically relevant trophic trait: hypertrophic lips in Midas cichlid fishes.

    Science.gov (United States)

    Machado-Schiaffino, Gonzalo; Henning, Frederico; Meyer, Axel

    2014-07-01

    The spectacular species richness of cichlids and their diversity in morphology, coloration, and behavior have made them an ideal model for the study of speciation and adaptive evolution. Hypertrophic lips evolved repeatedly and independently in African and Neotropical cichlid radiations. Cichlids with hypertrophic lips forage predominantly in rocky crevices and it has been hypothesized that mechanical stress caused by friction could result in larger lips through phenotypic plasticity. To test the influence of the environment on the size and development of lips, we conducted a series of breeding and feeding experiments on Midas cichlids. Full-sibs of Amphilophus labiatus (thick-lipped) and Amphilophus citrinellus (thin-lipped) each were split into a control group which was fed food from the water column and a treatment group whose food was fixed to substrates. We found strong evidence for phenotypic plasticity on lip area in the thick-lipped species, but not in the thin-lipped species. Intermediate phenotypic values were observed in hybrids from thick- and thin-lipped species reared under "control" conditions. Thus, both a genetic, but also a phenotypic plastic component is involved in the development of hypertrophic lips in Neotropical cichlids. Moreover, species-specific adaptive phenotypic plasticity was found, suggesting that plasticity is selected for in recent thick-lipped species. © 2014 The Author(s). Evolution © 2014 The Society for the Study of Evolution.

  6. Relation between thallium-201/iodine 123-BMIPP subtraction and fluorine 18 deoxyglucose polar maps in patients with hypertrophic cardiomyopathy.

    Science.gov (United States)

    Ito, Y; Hasegawa, S; Yamaguchi, H; Yoshioka, J; Uehara, T; Nishimura, T

    2000-01-01

    Clinical studies have shown discrepancies in the distribution of thallium-201 and iodine 123-beta-methyl-iodophenylpentadecanoic acid (BMIPP) in patients with hypertrophic cardiomyopathy (HCM). Myocardial uptake of fluorine 18 deoxyglucose (FDG) is increased in the hypertrophic area in HCM. We examined whether the distribution of a Tl-201/BMIPP subtraction polar map correlates with that of an FDG polar map. We normalized to maximum count each Tl-201 and BMIPP bull's-eye polar map of 6 volunteers and obtained a standard Tl-201/BMIPP subtraction polar map by subtracting a normalized BMIPP bull's-eye polar map from a normalized Tl-201 bull's-eye polar map. The Tl-201/BMIPP subtraction polar map was then applied to 8 patients with HCM (mean age 65+/-12 years) to evaluate the discrepancy between Tl-201 and BMIPP distribution. We compared the Tl-201/BMIPP subtraction polar map with an FDG polar map. In patients with HCM, the Tl-201/BMIPP subtraction polar map showed a focal uptake pattern in the hypertrophic area similar to that of the FDG polar map. By quantitative analysis, the severity score of the Tl-201/BMIPP subtraction polar map was significantly correlated with the percent dose uptake of the FDG polar map. These results suggest that this new quantitative method may be an alternative to FDG positron emission tomography for the routine evaluation of HCM.

  7. Intralesional triamcinolone alone and in combination with 5-fluorouracil for the treatment of Keloid and Hypertrophic scars

    International Nuclear Information System (INIS)

    Khan, M. A.; Bashir, M. M.; Khan, F. A.

    2014-01-01

    Objective: To compare the use of intralesional triamcinolone acetonide and its combination with 5 flourouracil in the treatment of keloid and hypertrophic scars in terms of reduction in initial height of the scar. Methods: The randomised controlled trial was conducted at the Department of Plastic Surgery, King Edward Medical University, Lahore, from March 2011 to December 2012. It comprised patients of both genders having keloids or hypertrophic scars (1 cm to 5 cm in size) having no history of treatment for the scars in preceding 6 months. Those who were pregnant, planning pregnancy or lactating were excluded. The subjects were divided into two groups: Group A received intralesional triamcinolone acetonide alone; and Group B received triamcinolone acetonide + 5 flourouracil. Eight injections were given at weekly interval. Scars were assessed 4 weeks after the completion of treatment on a five-point scale. SPSS 16 was used for statistical analysis. Results: The 150 subjects in the study were divided into two equal groups of 75(50%) each. Good to excellent results were seen in 51(68%) cases in Group A compared to 63(84%) in Group B. Frequency of complications was 18(24%) and 6(8%) in Group A and Group B respectively. Conclusion: Combination of triamcinolone acetonide and 5 flourouracil is superior to triamcinolone acetonide therapy in the treatment of keloids and hypertrophic scars. (author)

  8. Hypertrophic scarring in cleft lip repair: a comparison of incidence among ethnic groups

    Directory of Open Access Journals (Sweden)

    Soltani AM

    2012-07-01

    Full Text Available Ali M Soltani, Cameron S Francis, Arash Motamed, Ashley L Karatsonyi, Jeffrey A Hammoudeh, Pedro A Sanchez-Lara, John F Reinisch, Mark M UrataDivision of Plastic and Maxillofacial Surgery at Children's Hospital Los Angeles, CA, USA; The Division of Plastic and Reconstructive Surgery at the Keck School of Medicine of the University of Southern California, Los Angeles, CA, USABackground: Although hypertrophic scar (HTS formation following cleft lip repair is relatively common, published rates vary widely, from 1% to nearly 50%. The risk factors associated with HTS formation in cleft patients are not well characterized. The primary aim of this retrospective study of 180 cleft lip repairs is to evaluate the frequency of postoperative HTS among various ethnic groups following cleft lip repair.Methods: A retrospective chart view of patients undergoing primary cleft lip repair over a 16-year period (1990–2005 by the senior surgeon was performed. The primary outcome was the presence of HTS at 1 year postoperatively. Bivariate analysis and multivariable logistic regression were used to evaluate potential risk factors for HTS, including ethnicity, type and laterality of cleft, and gender.Results: One hundred and eighty patients who underwent cleft lip repair were included in the study. The overall rate of postoperative HTS formation was 25%. Ethnicity alone was found to be an independent predictor of HTS formation. Caucasian patients had the lowest rate of HTS formation (11.8% and were used as the reference group. HTS rates were significantly higher in the other ethnicities, 32.2% in Hispanic patients (odds ratio [OR]: 3.51; 95% confidence interval [CI]: 1.53–8.85, and 36.3% for Asian patients (OR 4.27; 95% CI: 1.36–13.70. Sex, cleft type, and cleft laterality were not associated with increased rates of HTS.Conclusions: Differences in ethnic makeup of respective patient populations may be a major factor influencing the wide variability of reported

  9. Hypertrophic Scarring of the Neck Following Ablative Fractional Carbon Dioxide Laser Resurfacing

    Science.gov (United States)

    Avram, Mathew M.; Tope, Whitney D.; Yu, Thomas; Szachowicz, Edward; Nelson, J. Stuart

    2009-01-01

    Background Ablative fractional carbon dioxide (CO2) laser treatments have gained popularity due to their efficacy, shortened downtime, and decreased potential for scarring in comparison to traditional ablative CO2 resurfacing. To date, scarring with fractional CO2 lasers has not been reported. Objective Five patients treated with the same fractional CO2 laser technology for photodamage of the neck were referred to our practices 1–3 months after treatment. Each patient developed scarring. Of the five cases, two are discussed in detail. The first was treated under general anesthesia on the face and anterior neck at a pulse energy of 30 mJ (859 μm depth) with 25% coverage. Eleven days after treatment, three non-healing areas along the horizontal skin folds of the anterior neck were noted. At 2 weeks after CO2 ablative fractional resurfacing, these areas had become thickened. These raised areas were treated with a non-ablative fractionated 1,550 nm laser to modify the wound healing milieu. One week later, distinct firm pale papules in linear arrays with mild hypopigmentation had developed along involved neck skin folds. Skin biopsy was performed. For the second patient, the neck was treated at a pulse energy of 20 mJ (630 μm depth) with 30% coverage of the exposed skin, with a total treatment energy of 5.0 kJ. Minimal crusting was noted on the neck throughout the initial healing phase of 2 weeks. She then experienced tightness on her neck. Approximately 3 weeks after treatment, she developed multiple vertical and horizontal hypertrophic scars (HS). Results Histopathology for the first case confirmed the presence of a hypertrophic scar. The papules in this case completely resolved with mild residual hypopigmentation after treatment with topical corticosteroids. HS failed to resolve in the second case to date after 1 month. Conclusion As with traditional ablative CO2 laser resurfacing, HS is a potential complication of ablative fractional CO2 laser resurfacing

  10. Clinical and Echocardiographic Evaluation of Regional Systolic Function Detected by Tissue Doppler Imaging in Hypertrophic Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    A Sadeghpour

    2009-09-01

    Full Text Available Background: Hypertrophic cardiomyopathy (HCM is the most common type of the genetic cardiovasculardiseases. Regarding to tremendous heterogeneity in the phenotypic expression of HCM, which is generally unrelatedto genotype, we aimed to study, clinical and echocardiographic parameters such as Tissue Doppler Imaging(TDI in various subtypes of HCM patients and evaluate the influence of race and gender in Iranian patients.Methods: Patients with HCM underwent a complete clinical and echocardiographic study including TDI toassess regional systolic contraction( in the 12 segments and early diastolic annular velocity (Em from theseptal mitral annulus.Results: The study comprised 41 patients (20 women, mean age = 41 ± 15 years with mean LVEF 55%±4.8%and mean maximal septal thickness 2.07cm. Considering LVOT gradient>30mmHg, hypertrophic obstructivecardiomyopathy (HOCM was found in 18 (45%. Asymmetric septal hypertrophy (ASH existed in 27 patients(67%, systolic anterior motion of anterior mitral leaflet (SAM in 25 persons (64%. Nineteen patients (46.3%were included in NYHA function class (FC II and 6 (14.7% in FC III or higher. We found syncope in 10(24.4%, chest pain in 4 (9.8%, atrial fibrilation in 14.6 % and ventricular arrhythmias in (17.1% of patients.History of ICD was seen in 7 (17.1% and PPM in 9 cases. Mean E’ velocity was 5.44± 1.65 cm/sec and S velocity5.70± 1.49 cm/sec with significant lower S velocity and E’ in syncope patients. Overall, HOCM patients hadgrade II diastolic dysfunction with E/É >15(17.54±7.46. Majority (25 of cases (61% were categorized in typeIII of HCM. RV involvement was observed in 11 patients (28.2%.No significant differences existed betweenprevalence of syncope and dysrhythmia among HCM and HOCM patients.Conclusion: In our study, we found lower detection of latent HOCM, compared to other studies, suggestive ofinadequate use of appropriate provocative maneuvers such as exercise stress echocardiography and amyl

  11. Fibroma cardíaco mimetizando cardiomiopatia hipertrófica Cardiac fibroma mimicking hypertrophic cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Luís Alberto Dallan

    1989-12-01

    Full Text Available É relatado o caso de paciente com queixa de dor precordial, dispnéia e arritmia desde a adolescência, tratada clinicamente por mais de 10 anos. Nesse período, foi submetida a inúmeros exames ângio e ecocardiográficos, com suspeita inicial de endomiocardiofibrose e, posteriormente, de cardiomiopatia hipertrófica de ventrículo esquerdo. Como houve piora progressiva da sintomatologia e ausência de resposta à medicação, foi encaminhada ao nosso Serviço, onde se diagnosticou fibroma de ventrículo esquerdo. Foi submetida, com sucesso, à ressecção cirúrgica do tumor, sendo realizada reconstrução geométrica do ventrículo esquerdo. Apresenta boa evolução, decorridos dois anos, com remissão completa dos sintomas. Destacamos a dificuldade no diagnóstico diferencial desses tumores benignos e de crescimento lento, com as cardiomiopatias hipertróficas do ventrículo esquerdo.A 33 year-old woman was seen, for the first time, ten years ago, for evaluation of a recurrent chest pain, dyspnea and arrhythmia. She was submitted to echocardiographic studies and a cardiac catheterization. The diagnoses was endomyocardial fibrosis at first, and hypertrophic cardiomyopathy after. Despite treatment with propranolol and quinidine, the episodes of dyspnea and tachyarrhythmias became more frequent and severe, and the patient was guided to our Service. Cardiac re-catheterization, echocardiographic and computed tomography studies identified in traumural cardiac fibroma and the patient was referred for surgical treatment. The cardiac fibroma was successfully resected on extracorporeal bypass and with cardioplegic arrest of the heart. Repair of the heart was accomplished with a patch placed to close the left ventricular cavity. The postoperative course was uncomplicated, and she remains assymptomatic two years later. We have emphazied tha this tumor often produces clinically obscure disease, simulating particularly the left ventricle hypertrophic

  12. Comparative efficacy of intralesional verapamil hydrochloride and triamcinolone acetonide in hypertrophic scars and keloids.

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    Ahuja, Rajeev B; Chatterjee, Pallab

    2014-06-01

    There is not much level 1 evidence based literature to guide management of hypertrophic scars and keloids despite an array of therapeutic modalities at disposal. Intralesional (i/l) triamcinolone injections have remained a gold standard in non surgical management. Sporadic reports on use of i/l verapamil suggest its efficacy. Since verapamil has not found sufficient mention as an effective alternative modality, it was decided to undertake a randomized study which could also address some additional clinical parameters. A randomized, parallel group and observer blinded comparison with 40 patients (48 scars) was carried out to compare the effects of i/l triamcinolone (T) (22 scars) and verapamil injections (V) (26 scars). 1.5 ml was the maximum indicative volume decided in the study protocol for both the drugs (triamcinolone @40 mg/ml and verapamil @ 2.5 mg/ml). Patients included were aged between 15-60 years with scars ranging between 0.5-5 cm (but total area roughly scars under 2 years duration. Patients with keloidal diathesis were excluded. Injections were scheduled every three weeks until complete flattening of the scar or eight sessions, which ever came earlier. No concomitant therapies like massage, silicone gel or pressure garments were used. Scar evaluation at each stage was done by serial photographic records as well as by Vancouver Scar Scale (VSS). Comparative survival analysis between the two drugs was done using Kaplan Meier curves, and VSS scores were analyzed using Wilcoxon test and log rank test. Mean zero VSS scores were achieved with treatments in respect of scar height (T-12 weeks, V-21 weeks), vascularity (T-15 weeks, V-18 weeks) and pliability (T-15 weeks, V-21 weeks). The improvement in scar vascularity and pliability kept pace with decrease in scar height, in both the groups. There was not much difference in the rate of change of scar pigmentation with either drug but almost 60% patients in both the groups regained normal pigmentation. Our

  13. Hypertrophic response to hemodynamic overload: role of load vs. renin-angiotensin system activation

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    Koide, M.; Carabello, B. A.; Conrad, C. C.; Buckley, J. M.; DeFreyte, G.; Barnes, M.; Tomanek, R. J.; Wei, C. C.; Dell'Italia, L. J.; Cooper, G. 4th; hide

    1999-01-01

    Myocardial hypertrophy is one of the basic mechanisms by which the heart compensates for hemodynamic overload. The mechanisms by which hemodynamic overload is transduced by the cardiac muscle cell and translated into cardiac hypertrophy are not completely understood. Candidates include activation of the renin-angiotensin system (RAS) and angiotensin II receptor (AT1) stimulation. In this study, we tested the hypothesis that load, independent of the RAS, is sufficient to stimulate cardiac growth. Four groups of cats were studied: 14 normal controls, 20 pulmonary artery-banded (PAB) cats, 7 PAB cats in whom the AT1 was concomitantly and continuously blocked with losartan, and 8 PAB cats in whom the angiotensin-converting enzyme (ACE) was concomitantly and continuously blocked with captopril. Losartan cats had at least a one-log order increase in the ED50 of the blood pressure response to angiotensin II infusion. Right ventricular (RV) hypertrophy was assessed using the RV mass-to-body weight ratio and ventricular cardiocyte size. RV hemodynamic overload was assessed by measuring RV systolic and diastolic pressures. Neither the extent of RV pressure overload nor RV hypertrophy that resulted from PAB was affected by AT1 blockade with losartan or ACE inhibition with captopril. RV systolic pressure was increased from 21 +/- 3 mmHg in normals to 68 +/- 4 mmHg in PAB, 65 +/- 5 mmHg in PAB plus losartan and 62 +/- 3 mmHg in PAB plus captopril. RV-to-body weight ratio increased from 0.52 +/- 0.04 g/kg in normals to 1.11 +/- 0.06 g/kg in PAB, 1.06 +/- 0.06 g/kg in PAB plus losartan and 1.06 +/- 0.06 g/kg in PAB plus captopril. Thus 1) pharmacological modulation of the RAS with losartan and captopril did not change the extent of the hemodynamic overload or the hypertrophic response induced by PAB; 2) neither RAS activation nor angiotensin II receptor stimulation is an obligatory and necessary component of the signaling pathway that acts as an intermediary coupling load to the

  14. Fatty acid myocardial imaging using iodine-123-beta-methyl-para-iodophenyl pentadecanoic acid (BMIPP) in patients with hypertrophic cardiomyopathy (HCM)

    International Nuclear Information System (INIS)

    Wada, Toyofumi

    1993-01-01

    Myocardial ischemia has been shown to develop during exercise and to play an important role on the pathophysiology in patients with hypertrophic cardiomyopathy (HCM). However, it is known whether or not myocardial ischemia is present under basal condition. BMIPP myocardial imaging has been proven to be useful for the detection of regional abnormalities of fatty acid metabolism induced by myocardial ischemia. We therefore performed BMIPP and Tl myocardial imagings in 18 patients with HCM. Six patients with chest pain syndrome or idiopathic ventricular arrhythmia served as controls. BMIPP and Tl scintigraphies were performed at least five days apart for avoiding cross talk and after overnight fasting. Six control subjects presented BMIPP images similar to Tl images without appreciable discrepancy. Of 18 patients with HCM, 15 manifested segments of reduced BMIPP uptakes as compared with those of Tl images. These segments of reduced BMIPP in patients with HCM were mainly observed in segments of increased Tl uptake, and suggested that myocardial ischemia was present under basal condition. Since the reduction of BMIPP uptake were often localized to parts of segments of increased Tl uptake, small coronary disease seemed to be a mechanism responsible for myocardial ischemia developing during resting condition. Four patients with apical hypertrophy showed reduction of BMIPP uptake in the hypertrophied apical segments. The observation suggests that abnormal myocardial energy metabolism is in progress during resting state, although the condition is generally considered to be benign. (author)

  15. Role of imaging in evaluation of sudden cardiac death risk in hypertrophic cardiomyopathy.

    Science.gov (United States)

    Geske, Jeffrey B; Ommen, Steve R

    2015-09-01

    Hypertrophic cardiomyopathy (HCM) is the most common heritable cardiomyopathy and is associated with sudden cardiac death (SCD) - an uncommon but devastating clinical outcome. This review is designed to assess the role of imaging in established risk factor assessment and its role in emerging SCD risk stratification. Recent publications have highlighted the crucial role of imaging in HCM SCD risk stratification. Left ventricular hypertrophy assessment remains the key imaging determinant of risk. Data continue to emerge on the role of systolic dysfunction, apical aneurysms, left atrial enlargement and left ventricular outflow tract obstruction as markers of risk. Quantitative assessment of delayed myocardial enhancement and T1 mapping on cardiac MRI continue to evolve. Recent multicenter trials have allowed multivariate SCD risk assessment in large HCM cohorts. Given aggregate risk with presence of multiple risk factors, a single parameter should not be used in isolation to determine implantable cardiac defibrillator candidacy. Use of all available imaging data, including cardiac magnetic resonance tissue characterization, allows a comprehensive approach to SCD stratification and implantable cardiac defibrillator decision-making.

  16. Clinical, Radiographic, and Microbiologic Features of Infective Endocarditis in Patients With Hypertrophic Cardiomyopathy.

    Science.gov (United States)

    Sims, Jason R; Anavekar, Nandan S; Bhatia, Subir; O'Horo, John C; Geske, Jeffrey B; Chandrasekaran, Krishnaswamy; Wilson, Walter R; Baddour, Larry M; Gersh, Bernard J; DeSimone, Daniel C

    2018-02-15

    Infective endocarditis (IE) is an infection of the inner lining of the heart with high morbidity and mortality despite medical and surgical advancements in recent decades. Hypertrophic cardiomyopathy (HC) is one of several medical conditions that have been linked to an increased risk of IE, but there is a paucity of data on this association. We therefore sought to define the clinical phenotype of IE in patients with HC at a single tertiary care center. A retrospective cohort of 30 adult patients with HC diagnosed with IE between January 1, 2006 and December 31, 2016 at Mayo Clinic Rochester were identified. Similar rates of aortic (n = 14) and mitral (n = 16) valve involvement by IE were noted (47% vs 53%). This finding persisted even in patients with left-ventricular outflow tract obstruction and systolic anterior motion of the mitral valve. Symptomatic embolic complications occurred in 10 cases (33%). Surgical intervention was performed in 11 cases (37%). One-year mortality was remarkably low at 7%. In conclusion, in the largest single-center cohort of IE complicating HC, there were similar rates of both mitral and aortic valve involvement regardless of the presence of left ventricular outflow tract obstruction, which is contrary to a long-standing tenet regarding the association of HC and IE. Moreover, no "high risk" IE subset was identified based on HC-related parameters. Copyright © 2017 Elsevier Inc. All rights reserved.

  17. The emerging role of cardiovascular MRI for risk stratification in hypertrophic cardiomyopathy

    International Nuclear Information System (INIS)

    Hoey, E.T.D.; Teoh, J.K.; Das, I.; Ganeshan, A.; Simpson, H.; Watkin, R.W.

    2014-01-01

    Hypertrophic cardiomyopathy (HCM) is the most common inheritable cardiovascular disorder. Although many HCM patients remain asymptomatic, sudden death (SD) can occur as the initial manifestation of the disease. It has been hypothesized that myocardial architectural disorganization and scarring represent an unstable electrophysiological substrate that creates susceptibility to malignant ventricular arrhythmias. Cardiovascular magnetic resonance imaging (CMR) is widely used for the diagnosis of HCM, especially in patients with an incomplete or inconclusive echocardiography study. CMR can provide precise non-invasive assessment of biventricular function, wall thickness, and assessment of myocardial fibrosis, using inversion recovery gadolinium-enhanced sequences. CMR is also one of the most promising avenues of research in HCM, and in recent years, has provided many new insights and identified a number of potential adverse prognostic indicators for SD. Future work is still needed to integrate CMR findings into traditional risk assessment algorithms. This paper reviews the evolving role of CMR for risk stratification in HCM including assessment of myocardial hypertrophy, fibrosis and ischaemia

  18. Depigmentation and hypertrophic scars after application of a fluid lactic acid tattoo eraser.

    Science.gov (United States)

    Wollina, Uwe

    2015-05-01

    Tattoo removal is often requested by patients. The gold standard is laser tattoo removal that can be time- and cost-intensive. Therefore, safe alternatives without lasers, pain, and scars would be desirable. We wanted to address safety of chemical tattoo erasers. We report a case of depigmentation and hypertrophic scars after use of a chemical tattoo eraser and searched the literature. Chemical tattoo erasers are not only used by physicians, but also nonmedical professionals such as beauticians, tattoo artists, and others. The case report we observed and other cases from the literature suggest that lactic acid based tattoo erasers are risky. Available safety data are unsufficient to recommend such procedure as an alternative to current laser therapy. Chemical tattoo erasers based on lactic acid may be capable to remove tattoo ink but the procedure bears safety risks of permanent adverse effects. For the safety of patients, better regulations for tattoo erasers need to be implemented. Patients need to be informed about adverse effects by such procedures.

  19. T1 mapping for detection of left ventricular myocardial fibrosis in hypertrophic cardiomyopathy: A preliminary study

    International Nuclear Information System (INIS)

    Lu, Minjie; Zhao, Shihua; Yin, Gang; Jiang, Shiliang; Zhao, Tao; Chen, Xiuyu; Tian, Liangxin; Zhang, Yan; Wei, Yunqing; Liu, Qiong; He, Zuoxiang; Xue, Hui; An, Jing; Shah, Saurabh

    2013-01-01

    Purpose: To investigate the diagnostic value of T1 mapping imaging of evaluating fibrosis in patients with hypertrophic cardiomyopathy (HCM). Materials and methods: 21 subjects with HCM and 18 healthy volunteers underwent conventional late gadolinium enhancement (LGE) imaging and T1 mapping imaging. The region of myocardium in HCM is divided into remote area of LGE, peri-LGE, LGE (halo-like LGE and typical patchy LGE). These regions combined with normal volunteers’ myocardium were calculated by the reduced percent of T1 value (RPTV). Results: The RPTV in healthy volunteers was no significant comparing with that in the remote area of LGE in HCM subjects (3.98 ± 3.19 vs. 3.34 ± 2.75, P > 0.05). There were significant statistical differences in pairwise among the remote area of LGE, peri-LGE, halo-like LGE and typical patchy LGE in the RPTV (P < 0.0001). ROC curves indicated that the T1 mapping imaging has a greater area under the curve comparing with that of traditional LGE imaging (0.975 ± 0.07 vs. 0.753 ± 0.26, P < 0.0001). Conclusions: HCM has a high prevalence of fibrosis and with varying severity. T1 mapping imaging can be a useful method to evaluate the severity of the fibrosis in HCM

  20. Correlation between late gadolinium enhancement and diastolic function in hypertrophic cardiomyopathy assessed by magnetic resonance imaging

    International Nuclear Information System (INIS)

    Motoyasu, Munenobu; Kurita, Tairo; Onishi, Katsuya

    2008-01-01

    Diastolic dysfunction is common in patients with overt hypertrophic cardiomyopathy (HCM). Steady-state cine magnetic resonance imaging (MRI) enables measurement of the diastolic function of the left ventricle (LV), and late gadolinium enhanced MRI can delineate the presence and extent of fibrosis in HCM. The purpose of this study was to determine the relationship between the extent of myocardial fibrosis demonstrated by late gadolinium-enhanced MRI and diastolic dysfunction. Seventeen patients (13 men, mean age 57.7±9.8 years) with HCM were studied. The severity index of late gadolinium enhancement was determined by scoring the extent of enhanced tissue in 30 myocardial segments. The peak filling rate (PFR), LV ejection fraction and LV mass were determined by cine MRI. Contrast-enhanced MRI demonstrated late gadolinium enhancement in 97 of 510 segments (19%) and 13 of the 17 patients (77%). The severity index of late gadolinium enhancement demonstrated a significant negative correlation with PFR (r=-0.86, p<0.01) and with the LV ejection fraction (r=-0.59, p<0.05). No significant correlation was observed between the severity index of late gadolinium enhancement and LV mass (r=0.23, p=0.30). The extent of myocardial fibrosis revealed by late gadolinium-enhanced MRI has a strong relationship to diastolic dysfunction in patients with HCM. (author)

  1. Bilateral brachial plexus blocks in a patient of hypertrophic obstructive cardiomyopathy with hypertensive crisis

    Directory of Open Access Journals (Sweden)

    Rohini V Bhat Pai

    2013-01-01

    Full Text Available Hypertrophic obstructive cardiomyopathy (HOCM is a challenge to anesthesiologists due to the complex pathophysiology involved and various perioperative complications associated with it. We present a 50-year-old man, a known case of HOCM, who successfully underwent emergency haemostasis, and debridement of the traumatically amputated right upper limb and the contused lacerated wound on the left forearm under bilateral brachial plexus blocks. His co-morbidities included hypertension (in hypertensive crisis and diabetes mellitus. He was full stomach and also had an anticipated difficult airway. The management included invasive pressure monitoring and labetalol infusion for emergent control of blood pressure. The regional anaesthesia technique required careful consideration to the dosage of local anaesthetics and staggered performance of brachial plexus blocks on each of the upper limbs to avoid local anaesthetic toxicity. Even though bilateral brachial plexus blocks are rarely indicated, it seemed to be the most appropriate anaesthetic technique in our patient. With careful consideration of the local anaesthetic toxicity and meticulous technique, bilateral brachial plexus blocks can be successfully performed in those patients where general anaesthesia is deemed to be associated with higher risk.

  2. Gastric emptying 16 to 26 years after treatment of infantile hypertrophic pyloric stenosis.

    Science.gov (United States)

    Lüdtke, F E; Bertus, M; Voth, E; Michalski, S; Lepsien, G

    1994-04-01

    Long-term follow-up was performed 16 to 26 years after conservative (group I, n = 18) and operative (group II, n = 38) treatment of 56 patients who had infantile hypertrophic pyloric stenosis (IHPS). The study encompassed the scintigraphic determination of gastric emptying rates for solids and liquids, an interview to obtain medical history and ascertain whether a current disorder of the upper gastrointestinal tract was present, and a clinical examination. Gastric emptying rates were measured on two different days for solids and liquids. The standard solid meal consisted of two scrambled eggs, two slices of toast, and 20 g of margarine. The gastric emptying rate for liquids was measured using 300 mL of apple juice. The scrambled eggs and apple juice were each marked with 2.2 MBq technetium 99m-sulphur-colloid. Two control collectives were used in this study; one group (physicians) served to create a reference curve for gastric emptying, and the other group, with the same age and gender distributions as those of the patients, served to evaluate the frequency of gastrointestinal complaints, by means of a questionnaire. There was no significant rate difference for gastric emptying between the patients treated conservatively or surgically and the controls. No association could be construed between the frequency of gastrointestinal symptoms or disorders and the gastric emptying rates for solids and liquids. The results presented here substantiate that clinically relevant disturbances of stomach motility after IHPS appear to be rare.

  3. Hypertrophic response of the Association of Thyroid Hormone and Exercise in the Heart of Rats

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    Souza, Fernanda Rodrigues de, E-mail: nandaeduca@yahoo.com.br; Resende, Elmiro Santos; Lopes, Leandro; Gonçalves, Alexandre; Chagas, Rafaella; Fidale, Thiago; Rodrigues, Poliana [UFU - Universidade Federal de Uberlândia, Uberlândia, MG (Brazil)

    2014-02-15

    Cardiac hypertrophy is a component of cardiac remodeling occurring in response to an increase of the activity or functional overload of the heart. Assess hypertrophic response of the association of thyroid hormone and exercise in the rat heart. We used 37 Wistar rats, male, adults were randomly divided into four groups: control, hormone (TH), exercise (E), thyroid hormone and exercise (H + E); the group received daily hormone levothyroxine sodium by gavage at a dose of 20 μg thyroid hormone/100g body weight, the exercise group took swimming five times a week, with additional weight corresponding to 20% of body weight for six weeks; in group H + E were applied simultaneously TH treatment groups and E. The statistics used was analysis of variance, where appropriate, by Tukey test and Pearson correlation test. The T4 was greater in groups TH and H + E. The total weight of the heart was greater in patients who received thyroid hormone and left ventricular weight was greater in the TH group. The transverse diameter of cardiomyocytes increased in groups TH, E and H + E. The percentage of collagen was greater in groups E and H + E Correlation analysis between variables showed distinct responses. The association of thyroid hormone with high-intensity exercise produced cardiac hypertrophy, and generated a standard hypertrophy not directly correlated to the degree of fibrosis.

  4. Single-Coil Defibrillator Leads Yield Satisfactory Defibrillation Safety Margin in Hypertrophic Cardiomyopathy.

    Science.gov (United States)

    Okamura, Hideo; Friedman, Paul A; Inoue, Yuko; Noda, Takashi; Aiba, Takeshi; Yasuda, Satoshi; Ogawa, Hisao; Kamakura, Shiro; Kusano, Kengo; Espinosa, Raul E

    2016-09-23

    Single-coil defibrillator leads have gained favor because of their potential ease of extraction. However, a high defibrillation threshold remains a concern in patients with hypertrophic cardiomyopathy (HCM), and in many cases, dual-coil leads have been used for this patient group. There is little data on using single-coil leads for HCM patients. We evaluated 20 patients with HCM who received an implantable cardioverter-defibrillator (ICD) on the left side in combination with a dual-coil lead. Two sets of defibrillation tests were performed in each patient, one with the superior vena cava (SVC) coil "on" and one with the SVC coil "off". ICDs were programmed to deliver 25 joules (J) for the first attempt followed by maximum energy (35 J or 40 J). Shock impedance and shock pulse width at 25 J in each setting as well as the results of the shock were analyzed. All 25-J shocks in both settings successfully terminated ventricular fibrillation. However, shock impedance and pulse width increased substantially with the SVC coil programmed "off" compared with "on" (66.4±6.1 ohm and 14.0±1.3 ms "off" vs. 41.9±5.0 ohm and 9.3±0.8 ms "on", Psatisfactory safety margin for 35-J devices. Single-coil leads appear appropriate for left-sided implantation in this patient group. (Circ J 2016; 80: 2199-2203).

  5. Ventricular premature contraction in hypertrophic cardiomyopathy and essential hypertension with left ventricular hypertrophy

    International Nuclear Information System (INIS)

    Kobiki, Naoki

    1989-01-01

    In order to investigate the relationship of different morbid states of the hypertrophied myocardium to the appearance of ventricular premature contraction (VPC), we compared the VPC findings from Holter ECG with those of UCG and stress thallium-201 myocardial SPECT scintigraphy (stress scinti) in 31 patients with hypertrophic cardiomyopathy (HCM) and 20 with essential hypertension (HT). The HCM patients consisted of 21 with asymmetric hypertrophy (ASH), 3 with symmetric hypertrophy (SH), and 7 with apical hypertrophy (APH). We recognized positive findings on the stress scinti such as fixed perfusion defect (FD) or reversible perfusion defect (RD) in 11 patients (ASH 10, APH 1) out of 31 patients with HCM (35%). Positive findings were observed in only one patient out of 20 with HT (5%). We recognized a high grade VPC (grade 4a and 4b of Lown's criteria) in 8 of 11 scinti positive patients with HCM (ASH 7, APH 1)(73%), while high grade VPC appeared in 5 (all of them are ASH) out of 20 scinti negative patients with HCM (25%). Therefore, these findings suggest that high grade VPCs in HCM occur in relation to a myocardial perfusion defect. (author)

  6. Coexistent coronary artery disease or myocardial bridging in patients with hypertrophic cardiomyopathy using coronary CT angiography

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Jae Hwan; Chun, Eun Ju; Kim, Yeo Koon; Yoo, Jin Young; Choi, Sang Il; Choi, Dong Ju [Seoul National University Bundang Hospital, Seongnam (Korea, Republic of)

    2015-07-15

    To evaluate the prevalence of coexistent coronary artery disease (CAD) or myocardial bridging (MB) in patients with hypertrophic cardiomyopathy (HCM) using coronary CT angiography (CCTA) and assess the role of CCTA. The prevalence of obstructive CAD (> 50% luminal reduction) and MB (partial and full encasement) were assessed in 150 patients with HCM diagnosed by clinical findings, electrocardiography, and echocardiography of 19588 consecutive patients who underwent CCTA for suspected CAD. The overall feasibility of coronary artery visualization was 98.9% with CCTA. In patients with HCM, the prevalence of obstructive CAD and MB (14.7% partial and 28.0% full encasement) were 23.3% and 42.7%, respectively. Age, hypertension, family history of premature CAD, Framingham risk score and severe chest pain were associated with CAD, whereas male gender and septal type were associated with MB (all p < 0.05). In comparison to invasive coronary angiography (n = 37), the diagnostic accuracy of CCTA for the detection of CAD and full encasement MB was 89.2% and 86.5%, respectively. One-quarter of patients with HCM had coexistent obstructive CAD or full encasement MB. CCTA can be a feasible and accurate noninvasive imaging modality for the detection of CAD and MB in patients with HCM.

  7. High resolution melting: improvements in the genetic diagnosis of hypertrophic cardiomyopathy in a Portuguese cohort

    Directory of Open Access Journals (Sweden)

    Santos Susana

    2012-03-01

    Full Text Available Abstract Background Hypertrophic Cardiomyopathy (HCM is a complex myocardial disorder with a recognized genetic heterogeneity. The elevated number of genes and mutations involved in HCM limits a gene-based diagnosis that should be considered of most importance for basic research and clinical medicine. Methodology In this report, we evaluated High Resolution Melting (HRM robustness, regarding HCM genetic testing, by means of analyzing 28 HCM-associated genes, including the most frequent 4 HCM-associated sarcomere genes, as well as 24 genes with lower reported HCM-phenotype association. We analyzed 80 Portuguese individuals with clinical phenotype of HCM allowing simultaneously a better characterization of this disease in the Portuguese population. Results HRM technology allowed us to identify 60 mutated alleles in 72 HCM patients: 49 missense mutations, 3 nonsense mutations, one 1-bp deletion, one 5-bp deletion, one in frame 3-bp deletion, one insertion/deletion, 3 splice mutations, one 5'UTR mutation in MYH7, MYBPC3, TNNT2, TNNI3, CSRP3, MYH6 and MYL2 genes. Significantly 22 are novel gene mutations. Conclusions HRM was proven to be a technique with high sensitivity and a low false positive ratio allowing a rapid, innovative and low cost genotyping of HCM. In a short return, HRM as a gene scanning technique could be a cost-effective gene-based diagnosis for an accurate HCM genetic diagnosis and hopefully providing new insights into genotype/phenotype correlations.

  8. Changes in left atrial deformation in hypertrophic cardiomyopathy: Evaluation by vector velocity imaging

    Directory of Open Access Journals (Sweden)

    Hala Mahfouz Badran

    2012-12-01

    Full Text Available Objectives: Hypertrophic cardiomyopathy (HCM represents a generalized myopathic process affecting both ventricular and atrial myocardium. We assessed the global and regional left atrial (LA function and its relation to left ventricular (LV mechanics and clinical status in patients with HCM using Vector Velocity Imaging (VVI. Methods: VVI of the LA and LV was acquired from apical four- and two-chamber views of 108 HCM patients (age 40±19years, 56.5% men and 33 healthy subjects, all had normal LV systolic function. The LA subendocardium was traced to obtain atrial volumes, ejection fraction, velocities, and strain (ɛ/strain rate (SR measurements. Results: Left atrial reservoir (ɛsys,SRsys and conduit (early diastolic SRe function were significantly reduced in HCM compared to controls (P-1.8s-1 was 81% sensitive and 30% specific, SRa>-1.5s-1 was 73% sensitive and 40% specific. By multivariate analysis global LVɛsys and LV septal thickness are independent predictors for LAɛsys, while end systolic diameter is the only independent predictor for SRsys, P<.001. Conclusion: Left atrial reservoir and conduit function as measured by VVI were significantly impaired while contractile function was preserved among HCM patients. Left atrial deformation was greatly influenced by LV mechanics and correlated to severity of phenotype.

  9. Changes in left atrial deformation in hypertrophic cardiomyopathy: Evaluation by vector velocity imaging

    Science.gov (United States)

    Badran, Hala Mahfouz; Soltan, Ghada; Hassan, Hesham; Nazmy, Ahmed; Faheem, Naglaa; Saadan, Haythem; Yacoub, Magdi H.

    2012-01-01

    Abstract: Objectives: Hypertrophic cardiomyopathy (HCM) represents a generalized myopathic process affecting both ventricular and atrial myocardium. We assessed the global and regional left atrial (LA) function and its relation to left ventricular (LV) mechanics and clinical status in patients with HCM using Vector Velocity Imaging (VVI). Methods: VVI of the LA and LV was acquired from apical four- and two-chamber views of 108 HCM patients (age 40 ± 19years, 56.5% men) and 33 healthy subjects, all had normal LV systolic function. The LA subendocardium was traced to obtain atrial volumes, ejection fraction, velocities, and strain (ϵ)/strain rate (SR) measurements. Results: Left atrial reservoir (ϵsys,SRsys) and conduit (early diastolic SRe) function were significantly reduced in HCM compared to controls (P  − 1.8s− 1 was 81% sensitive and 30% specific, SRa> − 1.5s− 1 was 73% sensitive and 40% specific. By multivariate analysis global LVϵsys and LV septal thickness are independent predictors for LAϵsys, while end systolic diameter is the only independent predictor for SRsys, P < .001. Conclusion: Left atrial reservoir and conduit function as measured by VVI were significantly impaired while contractile function was preserved among HCM patients. Left atrial deformation was greatly influenced by LV mechanics and correlated to severity of phenotype. PMID:24688992

  10. The Genetic and Molecular Bases for Hypertrophic Cardiomyopathy: The Role for Calcium Sensitization.

    Science.gov (United States)

    Ren, Xianfeng; Hensley, Nadia; Brady, Mary Beth; Gao, Wei Dong

    2018-02-01

    Hypertrophic cardiomyopathy (HCM) affects millions of people around the world as one of the most common genetic heart disorders and leads to cardiac ischemia, heart failure, dysfunction of other organ systems, and increased risk for sudden unexpected cardiac deaths. HCM can be caused by single-point mutations, insertion or deletion mutations, or truncation of cardiac myofilament proteins. The molecular mechanism that leads to disease progression and presentation is still poorly understood, despite decades of investigations. However, recent research has made dramatic advances in the understanding of HCM disease development. Studies have shown that increased calcium sensitivity is a universal feature in HCM. At the molecular level, increased crossbridge force (or power) generation resulting in hypercontractility is the prominent feature. Thus, calcium sensitization/hypercontractility is emerging as the primary stimulus for HCM disease development and phenotypic expression. Cross-bridge inhibition has been shown to halt HCM presentation, and myofilament desensitization appears to reduce lethal arrhythmias in animal models of HCM. These advances in basic research will continue to deepen the knowledge of HCM pathogenesis and are beginning to revolutionize the management of HCM. Copyright © 2018 Elsevier Inc. All rights reserved.

  11. Metastatic anal sac carcinoma with hypercalcaemia and associated hypertrophic osteopathy in a dog

    Directory of Open Access Journals (Sweden)

    A. Giuliano

    2015-05-01

    Full Text Available A seven-year-old male neutered Irish setter was treated for a metastatic anal sac adenocarcinoma (ASAC and hypercalcaemia by complete surgical excision of the primary tumour and partial excision of the sublumbar lymph nodes. Further enlargement of the sublumbar lymph nodes was linked to recurrent hypercalcaemia 3 months after surgical treatment. Medical treatment with Toceranib and Clodronate showed modest results in the treatment of the tumour and the hypercalcaemia. Radiotherapy of the sublumbar lymph nodes and later concurrent carboplatin chemotherapy resulted in partial tumour remission with marked reduction in size of the lymph nodes and normalization of the calcaemia. Unfortunately, concurrently with subsequent relapse of the hypercalaemia, the dog developed hypertrophic osteopathy (HO and lumbar spinal metastasis and the dog was euthanized. To the authors’ knowledge, this is the second case of metastatic apocrine gland carcinoma of the anal sac associated with HO and the first case that describe the development of HO late in the stage of the disease.

  12. Hypertrophic osteoarthropathy in a young adult male from Berber, Sudan (2nd-3rd century CE).

    Science.gov (United States)

    Binder, M; Saad, M

    2017-09-01

    Hypertrophic osteoarthropathy (HOA) is a pathological condition characterised by extensive periosteal new bone formation (NBF) on the diaphyses of the long bones, metacarpal and metatarsal bones. In modern clinical contexts, the secondary form of the disease is common and most often occurs secondary to intra-thoracic cancer and other forms of chronic pulmonary disease. Paleopathological evidence for HOA on the other hand has only occasionally been reported. Here we report a young adult male from the Meriotic cemetery at Berber in Sudan (2nd-3rd century CE) displaying widespread NBF on the diaphyses of the upper and lower limb bones, metacarpal and metatarsal bones, as well as the pelvis and scapulae. While several pathological conditions have to be considered as differential diagnostic options for NBF in the post-cranial skeleton, HOA is the most likely diagnosis, based on the distribution of the changes observed in this individual, as well as their macroscopic and radiographic characteristics. A chronic pulmonary condition as indicated by NBF on the visceral side of the ribs may represent the underlying cause for the HOA. This individual represents the first paleopathological case of HOA reported from an archaeological site in Africa. Copyright © 2017 Elsevier Inc. All rights reserved.

  13. Hypertrophic remodeling and increased arterial stiffness in patients with intracranial aneurysms.

    Science.gov (United States)

    Maltete, David; Bellien, Jeremy; Cabrejo, Lucie; Iacob, Michele; Proust, François; Mihout, Bruno; Thuillez, Christian; Guegan-Massardier, Evelyne; Joannides, Robinson

    2010-08-01

    Because an underlying arteriopathy might contribute to the development of intracranial aneurysms (IAs), we assessed the elastic properties of proximal conduit arteries in patients with IA. In 27 patients with previous ruptured IA and 27 control subjects matched for age, gender and BMI, we determined arterial pressure, internal diameter, intima-media thickness (IMT), circumferential wall stress (CWS) and elastic modulus (wall stiffness) in common carotid arteries using applanation tonometry and echotracking. Moreover, carotid augmentation index (AIx, arterial wave reflections) and carotid-to-femoral pulse wave velocity (PWV, aortic stiffness) were assessed. Compared with controls, patients with IA exhibited higher brachial and carotid systolic and diastolic blood pressures, with similar brachial but higher carotid artery pulse pressure (35 + or - 6mm Hg vs. 41 + or - 8mm Hg, P=0.014). Moreover, patients have higher PWV (7.8 + or - 1.2ms(-1) vs. 8.3 + or - 1.1ms(-1), P=0.048) and AIx (15.8 + or - 10.8% vs. 21.1 + or - 8.5%, PIA display a particular carotid artery phenotype with an exaggerated hypertrophic remodeling and altered elastic properties. Thus, a systemic arteriopathy might contribute, together with the arterial wall fatiguing effect of the increased pulsatile stress, to the pathogenesis of IA. Copyright 2010 Elsevier Ireland Ltd. All rights reserved.

  14. Changing trends in the management of infantile hypertrophic pyloric stenosis--an audit over 11 years.

    LENUS (Irish Health Repository)

    Doyle, D

    2012-02-03

    BACKGROUND: This article is a follow-up to an audit performed by the Department of Surgery and published in the Irish Journal of Medical Science in 1996. This audit reviewed all cases of Infantile Hypertrophic Pyloric Stenosis (IHPS) operated on over 22 years up to 1991. AIMS: We aim to demonstrate that radiologic investigations, namely barium meal and ultrasound, have been increasingly employed in the diagnosis of IHPS. In addition, ultrasound is now the investigation of choice. METHODS: We have reviewed all cases of IHPS, at the same institution, over the subsequent 11 years, with reference to any radiological investigations performed. In the previous study, the diagnosis of IHPS was made clinically in 92.6% with the remainder diagnosed radiologically. RESULTS: Over 11 years, 157 patients were diagnosed with IHPS. Male to female ratio was 4.06:1. Median age was four weeks (range 1-18 weeks).Twenty-four per cent had a barium meal, 36% had an ultrasound and 13% had both performed. CONCLUSION: We conclude a change in practice in the management of IHPS with radiology, particularly ultrasound, playing an increasing role.

  15. Analysis of diet optimization models for enabling conditions for hypertrophic muscle enlargement in athletes

    Directory of Open Access Journals (Sweden)

    L. Matijević

    2013-01-01

    Full Text Available In this study mathematical models were created and used in diet optimization for an athlete – recreational bodybuilder for pretournament period. The main aim was to determine weekly menus that can enable conditions for the hypertrophic muscle enlargement and to reduce the fat mass in a body. Each daily offer was planned to contain six to seven meals but with respect to several user’s personal demands. Optimal carbohydrates, fat and protein ratio in diet for enabling hypertrophy, recommended in literature, was found to be 43:30:27 and was chosen as the target in this research. Variables included in models were presented dishes and constraints, observed values of the offers; price, mass of consumed food, energy, water and content of different nutrients. The general idea was to create the models and to compare different programs in solving a problem. LINDO and MS Excel were recognized as widely spread and were chosen for model testing and examination. Both programs were suggested weekly menus that were acceptable to the user and were met all recommendations and demands. Weekly menus were analysed and compared. Sensitivity tests from both programs were used to detect possible critical points in the menu. Used programs produced slightly different results but still with very high correlation between proposed weekly intakes (R2=0.99856, p<0.05 so both can be successfully used in the pretournament period of bodybuilding and recommended for this complex task.

  16. Hypertrophic cardiomyopathy with midventricular obstruction and apical aneurysm formation in a single family: case report

    Directory of Open Access Journals (Sweden)

    Paraskevaidis Stylianos

    2009-06-01

    Full Text Available Abstract Background Hypertrophic cardiomyopathy (HCM is an extremely heterogeneous disease. An under recognized and very often missed subgroup within this broad spectrum concerns patients with left ventricular (LV apical aneurysms in the absence of coronary artery disease. Case presentation We describe a case of HCM with midventricular obstruction and apical aneurysm formation in 3 patients coming from a single family. This HCM pattern was detected by 2D-echocardiography and confirmed by cardiac magnetic resonance imaging. A cardioverter defibrillator was implanted in one of the patients because of non-sustained ventricular tachycardia detected in 24-h Holter monitoring and an abrupt drop in systolic blood pressure during maximal exercise test. The defibrillator activated 8 months after implantation by suppression of a ventricular tachycardia providing anti-tachycardia pacing. The patient died due to refractory heart failure 2 years after initial evaluation. The rest of the patients are stable after a 2.5-y follow-up period. Conclusion The detection of apical aneurysm by echocardiography in HCM patients may be complicated. Ventricular tachycardia arising from the scarred aneurysm wall may often occur predisposing to sudden death.

  17. Effect of Left Ventricular Outflow Tract Obstruction on Left Atrial Mechanics in Hypertrophic Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Lynne K. Williams

    2015-01-01

    Full Text Available Left atrial (LA volumes are known to be increased in hypertrophic cardiomyopathy (HCM and are a predictor of adverse outcome. In addition, LA function is impaired and is presumed to be due to left ventricular (LV diastolic dysfunction as a result of hypertrophy and myocardial fibrosis. In the current study, we assess the incremental effect of outflow tract obstruction (and concomitant mitral regurgitation on LA function as assessed by LA strain. Patients with HCM (50 obstructive, 50 nonobstructive were compared to 50 normal controls. A subset of obstructive patients who had undergone septal myectomy was also studied. Utilising feature-tracking software applied to cardiovascular magnetic resonance images, LA volumes and functional parameters were calculated. LA volumes were significantly elevated and LA ejection fraction and strain were significantly reduced in patients with HCM compared with controls and were significantly more affected in patients with obstruction. LA volumes and function were significantly improved after septal myectomy. LVOT obstruction and mitral regurgitation appear to further impair LA mechanics. Septal myectomy results in a significant reduction in LA volumes, paralleled by an improvement in function.

  18. Ferulic Acid Promotes Hypertrophic Growth of Fast Skeletal Muscle in Zebrafish Model.

    Science.gov (United States)

    Wen, Ya; Ushio, Hideki

    2017-09-26

    As a widely distributed and natural existing antioxidant, ferulic acid and its functions have been extensively studied in recent decades. In the present study, hypertrophic growth of fast skeletal myofibers was observed in adult zebrafish after ferulic acid administration for 30 days, being reflected in increased body weight, body mass index (BMI), and muscle mass, along with an enlarged cross-sectional area of myofibers. qRT-PCR analyses demonstrated the up-regulation of relative mRNA expression levels of myogenic transcriptional factors (MyoD, myogenin and serum response factor (SRF)) and their target genes encoding sarcomeric unit proteins involved in muscular hypertrophy (skeletal alpha-actin, myosin heavy chain, tropomyosin, and troponin I). Western blot analyses detected a higher phosphorylated level of zTOR (zebrafish target of rapamycin), p70S6K, and 4E-BP1, which suggests an enhanced translation efficiency and protein synthesis capacity of fast skeletal muscle myofibers. These changes in transcription and translation finally converge and lead to higher protein contents in myofibers, as confirmed by elevated levels of myosin heavy chain (MyHC), and an increased muscle mass. To the best of our knowledge, these findings have been reported for the first time in vivo and suggest potential applications of ferulic acid as functional food additives and dietary supplements owing to its ability to promote muscle growth.

  19. Localized hypertrophic neuropathy of the sciatic nerve in children: MRI findings

    International Nuclear Information System (INIS)

    Roux, Adrien; Treguier, Catherine; Bruneau, Bertrand; Marin, Franck; Gandon, Yves; Gauvrit, Jean-Yves; Riffaud, Laurent; Violas, Philippe; Michel, Anne

    2012-01-01

    Localized hypertrophic neuropathy (LHN) of the sciatic nerve in children is a rare condition characterized by a painless neurological deficit in the sciatic nerve territory. To demonstrate the role of MRI using a specific protocol and describe the primary findings in LHN. Imaging in four children (age 2 years to 12 years) is presented. All children presented with lower limb asymmetry. Three had a steppage gait. LHN was confirmed by electrophysiological studies and by MRI of the whole sciatic nerve with a dedicated protocol covering the lumbar spine and the lower limb. There were four direct MRI findings: (1) linear and focal hypertrophy with progressive enlargement of a peripheral nerve or plexus diameter, (2) abnormal hyperintensity of the nerve on T2-weighted images, (3) preserved fascicular configuration, and (4) variable enhancement after intravenous gadolinium administration. In addition there were atrophy and fatty infiltration of innervated muscles. MRI was helpful for determining the extent of lesions and in excluding peripheral nerve compression or tumour. MRI of the whole sciatic nerve is the method of choice for diagnosing LHN of the sciatic nerve. (orig.)

  20. Development of hypertrophic osteodystrophy and antibody response in a litter of vaccinated Weimaraner puppies.

    Science.gov (United States)

    Harrus, S; Waner, T; Aizenberg; Safra, N; Mosenco, A; Radoshitsky, M; Bark, H

    2002-01-01

    Two different vaccination protocols were compared with regard to the development of hypertrophic osteodystrophy (HOD) (also termed metaphyseal osteopathy) and effectiveness of immunisation in a litter of 10 Weimaraner puppies. Five puppies (group 1) were vaccinated with a modified live canine parvovirus vaccine (CPV) and then two weeks later with a trivalent vaccine containing modified live canine distemper virus and adenovirus type 2 combined with a Leptospira bacterin (DHL). The CPV and DHL vaccine protocols were administered a further two times, at two-week intervals. Group 2 was vaccinated with three consecutive multivalent vaccines containing modified live canine distemper virus, canine parvovirus, parainfluenza and adenovirus type 2 combined with a Leptospira bacterin, at four-week intervals. All puppies were first vaccinated at the age of eight weeks. Three dogs in group 1 developed HOD, while all five dogs in group 2 developed HOD during the study period. Dogs in group 2 had more episodes of HOD than those in group 1. Dogs in group 1 developed higher antibody titres to canine distemper virus and parvovirus compared with those in group 2. Only two out of the 10 dogs developed protective antibody titres to parvovirus. The results of this study suggest that the two different vaccination protocols affected the pattern of appearance of HOD and immunisation in this litter of Weimaraner puppies. The results obtained and the previously reported data suggest that a larger controlled study is needed to further elucidate the effect of different vaccination protocols on HOD and immunisation in Weimaraner puppies.

  1. Effect of PPAR γ activators on hypertrophic cardiac myocytes in vitro

    International Nuclear Information System (INIS)

    Wu Shimin; Zhou Xin; Ye Ping; Wang Qiong; Gao Yue; Liu Yongxue

    2004-01-01

    Objective: To investigate the effects of peroxisome proliferator-activated receptor γ (PPAR γ) activators pioglitazone and 15-deoxy-Δ 12,14 prostaglandin J 2 (15d-PGJ 2 ) on hypertrophic cardiac myocytes (MC) of neonatal rats in vitro. Methods; With the stimulation of angiotensin II(Ang II), a model of hypertrophy of MC was established. With the method of reverse transcription-polymerase chain reaction (RT-PCR), mRNA expression of atrial natriuretic peptide (ANP) and brain natriuretic peptide (BNP) was amplified; with the aid of NIH Image J software the surface area of MC was analyzed and with 3 H-leucine incorporation, the synthesizing rate of protein in MC was measured. Results: Increases in surface area of MC, mRNA expression of ANP and BNP and 3 H-leucine incorporation in MC were observed in the model of cardiac hypertrophy. Pioglitazone and 15d-PGJ 2 , two kinds of PPAR γ activators, inhibited the above changes in a dose-dependent manner. Conclusion: It is suggested that PPAR γ activators inhibit hypertrophy of cardiac myocytes and PPAR γ-dependent pathway be involved in the inhibitory course

  2. Localized hypertrophic neuropathy of the sciatic nerve in children: MRI findings

    Energy Technology Data Exchange (ETDEWEB)

    Roux, Adrien; Treguier, Catherine; Bruneau, Bertrand; Marin, Franck; Gandon, Yves; Gauvrit, Jean-Yves [University Hospital, Department of Radiology, Hopital Sud, 16 Boulevard de Bulgarie, BP 90347, Rennes cedex 2 (France); Riffaud, Laurent [University Hospital, Department of Pediatric Neurosurgery, Hopital Sud, Rennes (France); Violas, Philippe [University Hospital, Department of Pediatric Surgery, Hopital Sud, Rennes (France); Michel, Anne [University Hospital, Department of Neurological Functional Explorations, Hopital Sud, Rennes (France)

    2012-08-15

    Localized hypertrophic neuropathy (LHN) of the sciatic nerve in children is a rare condition characterized by a painless neurological deficit in the sciatic nerve territory. To demonstrate the role of MRI using a specific protocol and describe the primary findings in LHN. Imaging in four children (age 2 years to 12 years) is presented. All children presented with lower limb asymmetry. Three had a steppage gait. LHN was confirmed by electrophysiological studies and by MRI of the whole sciatic nerve with a dedicated protocol covering the lumbar spine and the lower limb. There were four direct MRI findings: (1) linear and focal hypertrophy with progressive enlargement of a peripheral nerve or plexus diameter, (2) abnormal hyperintensity of the nerve on T2-weighted images, (3) preserved fascicular configuration, and (4) variable enhancement after intravenous gadolinium administration. In addition there were atrophy and fatty infiltration of innervated muscles. MRI was helpful for determining the extent of lesions and in excluding peripheral nerve compression or tumour. MRI of the whole sciatic nerve is the method of choice for diagnosing LHN of the sciatic nerve. (orig.)

  3. Cardiovascular magnetic resonance imaging in hypertrophic cardiomyopathy: Current state of the art.

    Science.gov (United States)

    Kamal, Muhammad Umar; Riaz, Irbaz Bin; Janardhanan, Rajesh

    2016-01-01

    Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiomyopathy with a prevalence of 1:500 (0.2%) in the general population. Sudden cardiac death (SCD) is the most feared presentation of HCM. Therefore, it is essential to identify individuals at high risk in order to prevent SCD. The absence of conventional risk factors does not nullify the risk of HCM related SCD. Although echocardiography is currently the most widely used imaging modality, cardiac magnetic resonance (CMR) allows detailed characterization of the HCM phenotype, which makes it possible to differentiate HCM from other causes of left ventricular hypertrophy. CMR has the potential to further refine risk stratification. Late gadolinium enhancement (LGE) on CMR is a high-risk feature and there is emerging data to suggest that the presence of LGE should be employed as a marker for major adverse outcomes such as SCD, arrhythmias, systolic and diastolic heart failure. Hence, LGE on CMR may be considered an additional risk factor for SCD in HCM patients and should be incorporated in decision-making for implant-able cardioverter defibrillator implantation to aid primary prevention. Novel markers such as the extent of myocardial fibrosis on CMR must be accounted for comprehensive risk stratifica-tion of HCM patients. The purpose of this review is to discuss the current status and emerging role of CMR in HCM.

  4. Correlation between myocardial Thallium-201 kinetics, myocardial lactate metabolism and coronary angiographic findings in hypertrophic cardiomyopathy

    International Nuclear Information System (INIS)

    Hanrath, P.; Mathey, D.; Thiel, U.; Kupper, W.; Bleifeld, W.; Mantz, R.; Vorbringer, H.; Schneider, C.

    1980-01-01

    In 20 patients with idiopathic hypertrophic obstructive and nonobstructive cardiomyopathy (IHSS), biphasic Thallium-201 myocardial scintigraphy was performed. Regional myocardial Thallium-201 kinetics of these patients were analysed by a semiquantitative computerized method and compared with those of 6 normal subjects. In 12 of 18 with IHSS and no coronary artery disease 26 regions of interest with irreversible and 6 regions of interest with reversible Thallium-201 defects could be detected. Most of the Thallium-201 defects were localized in the interventricular septum. The defects were not related to the age of the patients and there was no relationship between the occurence of reversible Thallium-201 defects and pathological myocardial lactate extraction rate during maximal atrial pacing measured in 14 patients. The percentage (6.8%) of irreversible defect regions in patients with LV outflow obstruction at rest (n = 13) was more than twice higher compared to those (n = 5, 3.2%) without LV outflow obstruction or no provocative pressure gradient, resp. These data suggest that IHSS often associated with regional ischemic myocardial fibrosis despite normal coronary arteries. Therefore in these patients Thallium scintigraphy cannot be used as a noninvasive screening method to exclude or prove coronary artery disease. (orig.) [de

  5. Myocarditis caused by Feline Immunodeficiency Virus in Five Cats with Hypertrophic Cardiomyopathy.

    Science.gov (United States)

    Rolim, V Machado; Casagrande, R Assis; Wouters, A Terezinha Barth; Driemeier, D; Pavarini, S Petinatti

    2016-01-01

    Viral infections have been implicated as the cause of cardiomyopathy in several mammalian species. This study describes hypertrophic cardiomyopathy (HCM) and myocarditis associated with feline immunodeficiency virus (FIV) infection in five cats aged between 1 and 4 years. Clinical manifestations included dyspnoea in four animals, one of which also exhibited restlessness. One animal showed only lethargy, anorexia and vomiting. Necropsy examination revealed marked cardiomegaly, marked left ventricular hypertrophy and pallor of the myocardium and epicardium in all animals. Microscopical and immunohistochemical examination showed multifocal infiltration of the myocardium with T lymphocytes and fewer macrophages, neutrophils and plasma cells. An intense immunoreaction for FIV antigen in the cytoplasm and nucleus of lymphocytes and the cytoplasm of some macrophages was observed via immunohistochemistry (IHC). IHC did not reveal the presence of antigen from feline calicivirus, coronavirus, feline leukaemia virus, feline parvovirus, Chlamydia spp. or Toxoplasma gondii. The results demonstrate the occurrence of FIV infection in inflammatory cells in the myocardium of five cats with myocarditis and HCM. Copyright © 2015 Elsevier Ltd. All rights reserved.

  6. Focal status epilepticus as a manifestation of idiopathic hypertrophic cranial pachymeningitis.

    Science.gov (United States)

    Navalpotro-Gómez, Irene; Vivanco-Hidalgo, Rosa María; Cuadrado-Godia, Elisa; Medrano-Martorell, Santiago; Alameda-Quitllet, Francisco; Villalba-Martínez, Gloria; Roquer, Jaume

    2016-08-15

    Idiopathic hypertrophic cranial pachymeningitis (IHCP) is an uncommon disease of unknown etiology characterized by thickening of the cerebral dura mater with possible associated inflammation. The most frequently described clinical symptoms include headache, cranial nerve palsy, and cerebellar dysfunction. Epilepsy and/or status epilepticus as main presentation is very uncommon. Two consecutive cases are presented of patients manifesting focal status epilepticus secondary to IHCP, with clinical, laboratory [blood test and cerebrospinal fluid (CSF) analysis], neuroradiologic [magnetic resonance imaging (MRI) at 3 Tesla and digital subtraction angiography (DSA)], and therapeutic data. One patient underwent meningeal biopsy; pathology findings are also included. Corticosteroid therapy resulted in clinical improvement in both cases, and neuroimaging showed decreased abnormal morphology, compared to initial findings. In the diagnostic approach to focal status epilepticus or epilepsy, IHCP must be considered a potential, although extremely infrequent, cause. Anti-inflammatory treatment is an effective addition to antiepileptic drug therapy in patients with IHCP. Copyright © 2016 Elsevier B.V. All rights reserved.

  7. Evaluation and reformulation of the maximum peak height algorithm (MPH) and application in a hypertrophic lagoon

    Science.gov (United States)

    Pitarch, Jaime; Ruiz-Verdú, Antonio; Sendra, María. D.; Santoleri, Rosalia

    2017-02-01

    We studied the performance of the MERIS maximum peak height (MPH) algorithm in the retrieval of chlorophyll-a concentration (CHL), using a matchup data set of Bottom-of-Rayleigh Reflectances (BRR) and CHL from a hypertrophic lake (Albufera de Valencia). The MPH algorithm produced a slight underestimation of CHL in the pixels classified as cyanobacteria (83% of the total) and a strong overestimation in those classified as eukaryotic phytoplankton (17%). In situ biomass data showed that the binary classification of MPH was not appropriate for mixed phytoplankton populations, producing also unrealistic discontinuities in the CHL maps. We recalibrated MPH using our matchup data set and found that a single calibration curve of third degree fitted equally well to all matchups regardless of how they were classified. As a modification to the former approach, we incorporated the Phycocyanin Index (PCI) in the formula, thus taking into account the gradient of phytoplankton composition, which reduced the CHL retrieval errors. By using in situ biomass data, we also proved that PCI was indeed an indicator of cyanobacterial dominance. We applied our recalibration of the MPH algorithm to the whole MERIS data set (2002-2012). Results highlight the usefulness of the MPH algorithm as a tool to monitor eutrophication. The relevance of this fact is higher since MPH does not require a complete atmospheric correction, which often fails over such waters. An adequate flagging or correction of sun glint is advisable though, since the MPH algorithm was sensitive to sun glint.

  8. Regional left ventricular contractile dynamics in hypertrophic cardiomyopathy estimated by magnetic resonance imaging

    International Nuclear Information System (INIS)

    Sato, Tetsuya

    1994-01-01

    To assess the regional myocardial function in hypertrophic cardiomyopathy (HCM), I examined the systolic wall thickening (%WT) and percent change of segmental wall area (%AR) using cine magnetic resonance imaging in 23 normal volunteers (G1) and 40 patients (G2) with HCM. Short axis images of the left ventricle were recorded at the base and the apex, and were divided into 5 segments, and %WT and %AR were measured for each segment. There were no significant differences in %WT and %AR among the segments in G1, while %WT of the posterior septum, posterior and lateral segments in the apex were higher than the corresponding segments of the base. Wall segments of G2 were classified into 3 groups according to the end-diastolic wall thickness: G2a, ≤12 mm; G2b, 12 15. At each slice level, %WT and %AR were highest in G2a and lowest in G2c. These findings suggest that myocardial shortening in normal subjects is higher in the apex than in the base, and, in HCM, regional myocardial function decreases in association with an advance of hypertrophy, with a possible compensatory increased wall function of normal segments. (author)

  9. Inhibition effects of a negative electret 5-FU patch on the growth of a hypertrophic scar

    Science.gov (United States)

    Wang, YUAN; Lili, XU; Ping, HUANG; Xiaoqiang, AN; Lili, CUI; Jian, JIANG

    2018-05-01

    In this study, the hypertrophic scar (HS) model in rats was established. 5-fluorouracil (5-FU) patch, ‑1000 V and ‑2000 V polypropylene (PP) electret 5-FU patches were prepared and applied onto the wound. The in vitro permeation experiment was performed using the Franz diffusion cell system to determine the permeation cumulative amount and retention amount of 5-FU through/in scar skin. The inhibition effect of negative electret on growth of HS was studied by hematoxylin-eosin (HE) staining, Masson staining and the immunohistologicall methods. The permeation study indicated that a negative electret could enhance the permeation and retention of 5-FU through and in scar skin respectively. HE staining and Masson staining indicated a better effect for ‑1000 V and ‑2000 V electret 5-FU patches on HS inhibition after 28 d post-wounding compared with 5-FU patch. The immunohistological study showed much more reduced expressions of collegan type I, collegan type III, TGF-β1 and HSP47 in scar tissue after application of negative electret 5-FU patches than those of 5-FU patch. A negative electret 5-FU patch may be advantageous for HS treatment.

  10. [CHRONIC PERIODONTITIS WITH SYMPTOMATIC HYPERTROPHIC GINGIVITIS: CASE REPORT AND REVIEW OF THE LITERATURE].

    Science.gov (United States)

    Shinkevich, V; Udaltsova, K; Pisarenko, E; Kolomiets, S; Khmil, T

    2015-12-01

    Gingivitis in traditional national dentistry referred to independent diseases or symptomatic condition in periodontitis and classified morphologically. The diagnostic features of the diseases are characteristic, but the clinical presentation of symptomatic gingivitis and patterns of bone destructions may vary between patients. Successful treatment of the disease depends from proper diagnosis and advanced disease stages, but for symptomatic gingivitis that accompanying chronic periodontitis, protocols include surgical excision. Despite of the high prevalence of chronic generalized periodontitis, its active treatment often start in severe destruction and bone loss (2-3 stage severity). Today etiotropic antimicrobial therapy is real way to control microbial biofilm and has solid evidence base. Applying of etiotropic antimicrobial therapy as systemic azithromycin with timely treatment of mild to moderate periodontal and bone destruction may reduce severe periodontitis incidence of and treatment-related complications in the future. This paper attempts to describe the clinical diagnostic features and the current treatment options along with a suggested protocol for comprehensive management of chronic generalized periodontitis and hypertrophic gingivitis patient with case reports and a brief review.

  11. Metabolic crosstalk between the heart and liver impacts familial hypertrophic cardiomyopathy.

    Science.gov (United States)

    Magida, Jason A; Leinwand, Leslie A

    2014-04-01

    Familial hypertrophic cardiomyopathy (HCM) is largely caused by dominant mutations in genes encoding cardiac sarcomeric proteins, and it is etiologically distinct from secondary cardiomyopathies resulting from pressure/volume overload and neurohormonal or inflammatory stimuli. Here, we demonstrate that decreased left ventricular contractile function in male, but not female, HCM mice is associated with reduced fatty acid translocase (CD36) and AMP-activated protein kinase (AMPK) activity. As a result, the levels of myocardial ATP and triglyceride (TG) content are reduced, while the levels of oleic acid and TG in circulating very low density lipoproteins (VLDLs) and liver are increased. With time, these metabolic changes culminate in enhanced glucose production in male HCM mice. Remarkably, restoration of ventricular TG and ATP deficits via AMPK agonism as well as inhibition of gluconeogenesis improves ventricular architecture and function. These data underscore the importance of the systemic effects of a primary genetic heart disease to other organs and provide insight into potentially novel therapeutic interventions for HCM.

  12. Effect of botulinum toxin type A on transforming growth factor beta1 in fibroblasts derived from hypertrophic scar: a preliminary report.

    Science.gov (United States)

    Xiao, Zhibo; Zhang, Fengmin; Lin, Weibin; Zhang, Miaobo; Liu, Ying

    2010-08-01

    Hypertrophic scar is a common dermal disease. Numerous treatments are currently available but they do not always yield excellent therapeutic results. Hence, alternatives are needed. Recent basic and clinical research has shown that botulinum toxin type A (BTXA) has antihypertrophic scar properties but the molecular mechanism for this action is unknown. The aim of this study was to explore the effect of BTXA on transforming growth factor beta1 (TGF-beta1) in fibroblasts derived from hypertrophic scar and further elucidate its actual mechanism. Fibroblasts were isolated from tissue specimens of hypertrophic scar. Fibroblasts were treated with BTXA and the difference in proliferation between treated and nontreated cells was analyzed through the MTT method from the first to the fifth day after treatment. Proteins of TGF-beta1 were checked using ELISA in fibroblasts with BTXA and without BTXA from the first to the fifth day. The growth of the fibroblast treated with BTXA was obviously slower than that of the fibroblast without BTXA treatment (p < 0.01), which showed that BTXA effectively inhibited the growth of fibroblasts. Proteins of TGF-beta1 between fibroblasts with BTXA and fibroblasts without BTXA are statistically significant (p < 0.01). These results suggest that BTXA effectively inhibited the growth of fibroblasts derived from hypertrophic scar and in turn caused a decrease in TGF-beta1 protein, indicating that BTXA-based therapies for hypertrophic scar are promising and worth investigating further.

  13. Comparison of Maximal Wall Thickness in Hypertrophic Cardiomyopathy Differs Between Magnetic Resonance Imaging and Transthoracic Echocardiography.

    Science.gov (United States)

    Bois, John P; Geske, Jeffrey B; Foley, Thomas A; Ommen, Steve R; Pellikka, Patricia A

    2017-02-15

    Left ventricular (LV) wall thickness is a prognostic marker in hypertrophic cardiomyopathy (HC). LV wall thickness ≥30 mm (massive hypertrophy) is independently associated with sudden cardiac death. Presence of massive hypertrophy is used to guide decision making for cardiac defibrillator implantation. We sought to determine whether measurements of maximal LV wall thickness differ between cardiac magnetic resonance imaging (MRI) and transthoracic echocardiography (TTE). Consecutive patients were studied who had HC without previous septal ablation or myectomy and underwent both cardiac MRI and TTE at a single tertiary referral center. Reported maximal LV wall thickness was compared between the imaging techniques. Patients with ≥1 technique reporting massive hypertrophy received subset analysis. In total, 618 patients were evaluated from January 1, 2003, to December 21, 2012 (mean [SD] age, 53 [15] years; 381 men [62%]). In 75 patients (12%), reported maximal LV wall thickness was identical between MRI and TTE. Median difference in reported maximal LV wall thickness between the techniques was 3 mm (maximum difference, 17 mm). Of the 63 patients with ≥1 technique measuring maximal LV wall thickness ≥30 mm, 44 patients (70%) had discrepant classification regarding massive hypertrophy. MRI identified 52 patients (83%) with massive hypertrophy; TTE, 30 patients (48%). Although guidelines recommend MRI or TTE imaging to assess cardiac anatomy in HC, this study shows discrepancy between the techniques for maximal reported LV wall thickness assessment. In conclusion, because this measure clinically affects prognosis and therapeutic decision making, efforts to resolve these discrepancies are critical. Copyright © 2016 Elsevier Inc. All rights reserved.

  14. Prevalence and Impact of Coexistent Bicuspid Aortic Valve in Hypertrophic Cardiomyopathy.

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    Padang, Ratnasari; Gersh, Bernard J; Ommen, Steve R; Geske, Jeffrey B

    2018-01-01

    The association between bicuspid aortic valve (BAV) and hypertrophic cardiomyopathy (HCM) has been reported but its true prevalence is unknown. This study investigated the prevalence and clinical impact of coexistent BAV in a large referral HCM population. Retrospective analysis of 3765 echocardiograms between 2004 and 2014 in 2640 consecutive patients with HCM was performed to assess for BAV. Patients with coexistent conditions were studied. Twenty-three patients (0.9%) were identified with coexisting BAV and HCM. Mean age was 52±16years, 18 males (78%), 16 with NYHA functional class I/II at initial evaluation (70%). A family history of HCM was present in five patients (22%); none had a family history of BAV or aortopathy. Maximal left ventricular wall thickness was 24±6mm; the majority had either reverse curve or sigmoid septal morphology. Moderate or greater aortic valve dysfunction was present in seven patients (30%), BAV-related aortopathy in 18 patients (78%) and dynamic left ventricular outflow tract (LVOT) obstruction in nine patients (39%). Three patients had combined LVOT obstruction and aortic stenosis. Median time from diagnosis of BAV or HCM to last follow-up was 11±12.5years. At last follow-up, 22% had undergone BAV-related surgeries, 30% had septal reduction therapy (SRT), and 17% had combined SRT and BAV-related surgeries. Overall survival was 95% at 10 years. This study reported a 0.9% prevalence of BAV among HCM population, similar to the general population. Aortopathy and LVOT obstruction were common, necessitating cardiac interventions in over one-third of cases. Long-term survival appeared favourable. Copyright © 2017 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.

  15. Platelet Function Analyzer 100 and Brain Natriuretic Peptide as Biomarkers in Obstructive Hypertrophic Cardiomyopathy.

    Science.gov (United States)

    Blackshear, Joseph L; Safford, Robert E; Thomas, Colleen S; Bos, J Martijn; Ackerman, Michael J; Geske, Jeffrey B; Ommen, Steve R; Shapiro, Brian P; Johns, Gretchen S

    2018-03-15

    To test dual blood biomarkers compared with electrocardiogram (ECG) for hypertrophic cardiomyopathy (HC) screening, we performed 3 analyses and cut-point assessments. First, we measured platelet function analyzer (PFA)-100 (n = 99) and normalized B-type natriuretic peptide (BNP) or NT-proBNP (BNP/upper limit of normal [ULN], n = 92) in 64 patients with HC and 29 normal controls (NCs). Second, from the regression equation between PFA and gradient (r = 0.77), we derived estimated PFA in a population of 189 patients with functional class I HC in whom measured BNP/ULN and ECG were available, and calculated single and dual biomarker sensitivity and specificity compared with ECG. Finally, we compared BNP/ULN in class I patients based on mutation and familial history status. In 42 patients with obstructive HC versus NCs, there was a slight overlap of PFA and BNP/ULN, but for the product of PFA × BNP/ULN, there was near-complete separation of values. Among patients with class I obstructive HC, estimated PFA × BNP/ULN had a sensitivity of 93% and a specificity of 100%; in latent and nonobstructive HC, sensitivity dropped to 61% and 72%; for ECG in obstructive, latent, and nonobstructive HC, sensitivity was 71%, 34%, and 67%. Functional class I patients with positive (n = 28) and negative (n = 36) sarcomere mutations and a positive (n = 71) or a negative (n = 109) family history had significant elevations of BNP/ULN versus NC, with no between-group differences. In conclusion, PFA and BNP were highly associated with obstructive HC and could potentially be used for screening; BNP was not uniquely elevated in patients with familial versus nonfamilial or mutation-positive versus mutation-negative HC. Copyright © 2017 Elsevier Inc. All rights reserved.

  16. Does septal thickness influence outcome of myectomy for hypertrophic obstructive cardiomyopathy?

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    Nguyen, Anita; Schaff, Hartzell V; Nishimura, Rick A; Dearani, Joseph A; Geske, Jeffrey B; Lahr, Brian D; Ommen, Steve R

    2018-03-01

    Patients with hypertrophic obstructive cardiomyopathy and basal septal thickness 21 mm, n = 505), were performed with the Kruskal-Wallis and the Pearson χ2 tests and semiparametric analysis of covariance. Median group ages were 57, 57 and 54 years (P = 0.007); men comprised 50.4%, 56.7% and 62.0%, respectively (P = 0.003). Intrinsic MV disease was present in 5.9%, 5.2% and 4.6%, respectively (P = 0.80). All patients underwent transaortic septal myectomy. Additional mitral procedures were performed in 7.6%, 7.8% and 8.1%, respectively (P = 0.90). Reasons for MV surgery included intrinsic MV disease (66.7%), residual mitral regurgitation (30.8%) and residual gradient (2.6%). All groups had postoperative gradient relief (median reduction: 51, 54 and 50 mmHg; P = 0.11). Ventricular septal defect occurred in 4 patients (0.3%), and risk did not differ by group (P = 0.24). Adequate relief of left ventricular outflow tract obstruction can be achieved via transaortic septal myectomy without concomitant MV procedures when septal thickness is < 18 mm, and the risk of ventricular septal defect is minimal. Concomitant MV repair/replacement should be reserved for patients with intrinsic MV disease or inadequate relief of mitral regurgitation/left ventricular outflow tract obstruction following adequate extended septal myectomy. © The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

  17. Implantable Cardioverter-defibrillator Therapy for Hypertrophic Cardiomyopathy: Usefulness in Primary and Secondary Prevention.

    Science.gov (United States)

    Sarrias, Axel; Galve, Enrique; Sabaté, Xavier; Moya, Àngel; Anguera, Ignacio; Núñez, Elaine; Villuendas, Roger; Alcalde, Óscar; García-Dorado, David

    2015-06-01

    Hypertrophic cardiomyopathy is a frequent cause of sudden death. Clinical practice guidelines indicate defibrillator implantation for primary prevention in patients with 1 or more risk factors and for secondary prevention in patients with a history of aborted sudden death or sustained ventricular arrhythmias. The aim of the present study was to analyze the follow-up of patients who received an implantable defibrillator following the current guidelines in nonreferral centers for this disease. This retrospective observational study included all patients who underwent defibrillator implantation between January 1996 and December 2012 in 3 centers in the province of Barcelona. The study included 69 patients (mean age [standard deviation], 44.8 [17] years; 79.3% men), 48 in primary prevention and 21 in secondary prevention. The mean number of risk factors per patient was 1.8 in the primary prevention group and 0.5 in the secondary prevention group (P=.029). The median follow-up duration was 40.5 months. The appropriate therapy rate was 32.7/100 patient-years in secondary prevention and 1.7/100 patient-years in primary prevention (P<.001). Overall mortality was 10.1%. Implant-related complications were experienced by 8.7% of patients, and 13% had inappropriate defibrillator discharges. In patients with a defibrillator for primary prevention, the appropriate therapy rate is extremely low, indicating the low predictive power of the current risk stratification criteria. Copyright © 2014 Sociedad Española de Cardiología. Published by Elsevier España, S.L.U. All rights reserved.

  18. CASE OF DIAGNOSIS OF APICAL FORM OF HYPERTROPHIC CARDIOMYOPATHY WITH A PATIENT WITH PROGRESSIVE ANGINA CLINIC

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    N. S. Krylova

    2015-01-01

    Full Text Available Objective of work: to describe the apical form of hypertrophic cardiomyopathy (AFHC developing under the "mask" of the ischemic heart disease not diagnosed for a long period.Materials and methods. Patient B., 73 y.o., female, was brought to the cardiology department with complains of severe pressing pain behind the breastbone caused with no apparent reason and lasting for over 4 hours. The following examination of the patient was performed: electrocardiography (ECG, echocardiography (EchoCG, Holter ECG monitoring, coronary angiography (CAG, ventriculography.Results. The final diagnosis for the patient was set on the basis of the following readings: ECG data (basic rhythm – atrial fibrillation, left ventricle (LV hypertrophy, negative T-waves in leads V1–6, ST segment depression up to 1–2 mm in leads V4–6, EchoCG (hypertrophy of apical segments of the LV with decreasing of its cavity, moderate dilatation of the left atrium, intraventricular obstruction in the apical third of the LV with the maximum pressure gradient of up to 48 mm Hg., CAG (stenotic lesions of coronary arteries were found, ventriculography (LV volume is not increased, no violations of local contractility, narrowing of the LV cavity in the lower third is observed with thinning in the apex, which indicatesexpressed apical hypertrophy of the LV myocard. AFHC, apical form with moderate obstruction in the lower third of the left ventricle. Stress angina syndrome. CAG and ventriculography were main diagnostic methods that allowed setting the final diagnosis.Conclusion. The clinical case sets forth the peculiarities of diagnostics, therapy, and post-therapy management of patients with this form of AFHC.

  19. Abnormal myocardial capillary density in apical hypertrophic cardiomyopathy can be assessed by myocardial contrast echocardiography

    International Nuclear Information System (INIS)

    Moon, Jeonggeun; Cho, In-Jeong; Shim, Chi-Young; Ha, Jong-Won; Jang, Yangsoo; Chung, Namsik; Rim, Se-Joong

    2010-01-01

    Myocardial ischemia and dysfunction can occur in hypertrophic cardiomyopathy (HCM) because of the high muscle-to-blood ratio, even without significant coronary artery disease. Microbubbles reside only in the intravascular space and myocardial video-intensity during systole results mostly from microbubbles within capillaries. The hypothesis explored in the present study was that an abnormal capillary density in apical HCM (ApHCM) can be demonstrated using myocardial contrast echocardiography (MCE). The 56 patients were investigated (31 males, age 58±9 years; 33 ApHCM, 9 hypertensive left ventricular hypertrophy [LVH], 14 controls). MCE was performed with low-mechanical-index power modulation imaging. Tissue Doppler imaging to assess myocardial contractile function was obtained at the mitral annulus (S'), and 99m Tc-MIBI single photon emission computed tomography (SPECT) was also performed. All ApHCM patients exhibited perfusion defects at the hypertrophied segments in the systolic phase during MCE, whereas SPECT showed normal or rather increased perfusion at those sites. The cyclic variation of video-intensity was exaggerated in ApHCM when compared with the LVH or control group (% of [systolic video-intensity]/[diastolic video-intensity]: 33.0±12.3%, 88.3±19.2% and 79.4±13.9%, respectively [P<0.05]). Concurrently, MCE cyclic variation and perfusion defect size were related to decreased S' (P<0.05 for all). A perfusion defect at the hypertrophied segment, representing abnormal myocardial capillary density, was observed in ApHCM patients during MCE. The extent of MCE cyclic variation and the perfusion defect size both correlate with decreased myocardial contractile property in ApHCM. (author)

  20. Quantification and significance of diffuse myocardial fibrosis and diastolic dysfunction in childhood hypertrophic cardiomyopathy.

    Science.gov (United States)

    Hussain, Tarique; Dragulescu, Andreea; Benson, Lee; Yoo, Shi-Joon; Meng, Howard; Windram, Jonathan; Wong, Derek; Greiser, Andreas; Friedberg, Mark; Mertens, Luc; Seed, Michael; Redington, Andrew; Grosse-Wortmann, Lars

    2015-06-01

    The purpose of this study was to evaluate the presence of diffuse myocardial fibrosis in children and adolescents with hypertrophic cardiomyopathy (HCM) and to assess associations with echocardiographic and clinical parameters of disease. While a common end point in adults with HCM, it is unclear whether diffuse myocardial fibrosis occurs early in the disease. Cardiac magnetic resonance (CMR) estimation of myocardial post-contrast longitudinal relaxation time (T1) is an increasingly used method to estimate diffuse fibrosis. T1 measurements were taken using standard multi-breath-hold spoiled gradient echo phase-sensitive inversion-recovery CMR before and 15 min after the injection of gadolinium. The tissue-blood partition coefficient was calculated as a function of the ratio of T1 change of myocardium compared with blood. An echocardiogram and blood brain natriuretic peptide (BNP) levels were obtained on the day of the CMR. Twelve controls (mean age 12.8 years; 7 male) and 28 patients with HCM (mean age 12.8 years; 21 male) participated. The partition coefficient for both septal (0.27 ± 0.17 vs. 0.13 ± 0.09; p = 0.03) and lateral walls (0.22 ± 0.09 vs. 0.07 ± 0.10; p 100 pg/ml) had raised lateral wall coefficients (0.27 ± 0.07 vs. 0.20 ± 0.07; p = 0.03), as did those with traditional risk factors for sudden death (0.27 ± 0.06 vs. 0.18 ± 0.08; p = 0.007). Diffuse fibrosis, measured by the partition coefficient technique, is demonstrable in children and adolescents with HCM. Markers of fibrosis show an association with symptoms and raised serum BNP. Further study of the prognostic implication of this technique in young patients with HCM is warranted.

  1. Cardiac troponin and tropomyosin: structural and cellular perspectives to unveil the Hypertrophic Cardiomyopathy phenotype

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    Mayra de A. Marques

    2016-09-01

    Full Text Available Inherited myopathies affect both skeletal and cardiac muscle and are commonly associated with genetic dysfunctions, leading to the production of anomalous proteins. In cardiomyopathies, mutations frequently occur in sarcomeric genes, but the cause-effect scenario between genetic alterations and pathological processes remains elusive. Hypertrophic cardiomyopathy (HCM was the first cardiac disease associated with a genetic background. Since the discovery of the first mutation in the β-myosin heavy chain, more than 1,400 new mutations in 11 sarcomeric genes have been reported, awarding HCM the title of the disease of the sarcomere. The most common macroscopic phenotypes are left ventricle and interventricular septal thickening, but because the clinical profile of this disease is quite heterogeneous, these phenotypes are not suitable for an accurate diagnosis. The development of genomic approaches for clinical investigation allows for diagnostic progress and understanding at the molecular level. Meanwhile, the lack of accurate in vivo models to better comprehend the cellular events triggered by this pathology has become a challenge. Notwithstanding, the imbalance of Ca2+ concentrations, altered signaling pathways, induction of apoptotic factors, and heart remodeling leading to abnormal anatomy have already been reported. Of note, a misbalance of signaling biomolecules, such as kinases and tumor suppressors (e.g., Akt and p53, seems to participate in apoptotic and fibrotic events. In HCM, structural and cellular information about defective sarcomeric proteins and their altered interactome is emerging but still represents a bottleneck for developing new concepts in basic research and for future therapeutic interventions. This review focuses on the structural and cellular alterations triggered by HCM-causing mutations in troponin and tropomyosin proteins and how structural biology can aid in the discovery of new platforms for therapeutics. We

  2. Factors Influencing the Phenotypic Expression of Hypertrophic Cardiomyopathy in Genetic Carriers.

    Science.gov (United States)

    Pérez-Sánchez, Inmaculada; Romero-Puche, Antonio José; García-Molina Sáez, Esperanza; Sabater-Molina, María; López-Ayala, José María; Muñoz-Esparza, Carmen; López-Cuenca, David; de la Morena, Gonzalo; Castro-García, Francisco José; Gimeno-Blanes, Juan Ramón

    2018-03-01

    Hypertrophic cardiomyopathy (HCM) is a disorder with variable expression. It is mainly caused by mutations in sarcomeric genes but the phenotype could be modulated by other factors. The aim of this study was to determine whether factors such as sex, systemic hypertension, or physical activity are modifiers of disease severity and to establish their role in age-related penetrance of HCM. We evaluated 272 individuals (mean age 49 ± 17 years, 57% males) from 72 families with causative mutations. The relationship between sex, hypertension, physical activity, and left ventricular hypertrophy was studied. The proportion of affected individuals increased with age. Men developed the disease 12.5 years earlier than women (adjusted median, 95%CI, -17.52 to -6.48; P < .001). Hypertensive patients were diagnosed with HCM later (10.8 years of delay) than normotensive patients (adjusted median, 95%CI, 6.28-17.09; P < .001). Individuals who performed physical activity were diagnosed with HCM significantly earlier (7.3 years, adjusted median, 95%CI, -14.49 to -1.51; P = .016). Sex, hypertension, and the degree of physical activity were not significantly associated with the severity of left ventricular hypertrophy. Adjusted survival both free from sudden death and from the combined event were not influenced by any of the exploratory variables. Men and athletes who are carriers of sarcomeric mutations are diagnosed earlier than women and sedentary individuals. Hypertensive carriers of sarcomeric mutations have a delayed diagnosis. Sex, hypertension, and physical activity are not associated with disease severity in carriers of HCM causative mutations. Copyright © 2017 Sociedad Española de Cardiología. Published by Elsevier España, S.L.U. All rights reserved.

  3. Identification of high risk patients with hypertrophic cardiomyopathy in a northern Greek population

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    Karvounis Charalambos

    2009-07-01

    Full Text Available Abstract Background The percentage of hypertrophic cardiomyopathy (HCM patients who are in high risk for Sudden Death (SD constitutes only a minority of all HCM population but the incidence of SD in this subset is high (at least 5% annually. The identification of this small but important proportion of high risk HCM patients has been the clue in the clinical evaluation of these patients. Methods Our study cohort consisted from 123 patients with HCM who are currently followed up in our Institution. Five clinical risk factors were assessed: a family history of premature SD, unexplained syncope, Non Sustained Ventricular Tachycardia (NSVT on 24-h ECG monitoring, Abnormal Blood Pressure Response (ABPR during upright exercise testing and Maximum left ventricular Wall Thickness (MWT ≥30 mm. The purpose of our study was the identification of high risk HCM patients coming from Northern Greece. Results Fifteen patients (12.2% of the whole cohort had MWT ≥ 30 mm, 30 patients (24.4% had an ABPR to exercise, 17 patients (13.8% had episodes of NSVT in 24-h Holter monitoring, 17 patients (13.8% suffered from syncope, and 8 patients (6.5% had a positive family history of premature SD. Data analysis revealed that 74 patients (60.1% had none risk factor. Twenty four patients (19.5% had 1 risk factor, 17 patients (13.8% had 2 risk factors, 4 patients (3.25% had 3 risk factors, and 4 patients (3.25% had 4 risk factors, while none patient had 5 risk factors. Twenty five patients (20.3% had 2 or more risk factors. Conclusion This study for the first time confirms that, although a 60% of patients with HCM coming from a regional Greek population are in low risk for SD, a substantial proportion (almost 20% carries a high risk for SD justifying prophylactic therapy with amiodaron or ICD implantation.

  4. Physical activity in hypertrophic cardiomyopathy: prevalence of inactivity and perceived barriers

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    Sweeting, Joanna; Ingles, Jodie; Timperio, Anna; Patterson, Jillian; Ball, Kylie; Semsarian, Christopher

    2016-01-01

    Objectives This study aimed to determine the prevalence of physical inactivity and perceived barriers to physical activity among individuals with hypertrophic cardiomyopathy (HCM), and to determine potential demographic, clinical and health-related factors influencing likelihood of meeting physical activity guidelines. Methods This was a cross-sectional study of consecutive patients (n=198) with HCM attending a specialist HCM centre from July 2014 to November 2015. The primary outcome measure was physical activity (minutes per day), as measured by self-report (International Physical Activity Questionnaire (IPAQ)) and objective means (ActiGraph accelerometer). For both, participants were classified as meeting guidelines if they did at least 150 min per week of physical activity. Quality of life (Short Form-36 V.2, SF-36v2), barriers to exercise and clinical–demographic data were also collected. Results In total, 54.8% of participants did not meet physical activity recommendations based on IPAQ, and 12.7% did not meet guidelines based on accelerometer data. The most commonly identified barriers to exercise were ‘pain interferes with my exercise’ (33%) and ‘I have an injury/disability that stops me’ (29%). Independent factors associated with meeting guidelines included older age (OR 0.66, 95% CI 0.51 to 0.85, p=0.002), higher education level (OR 2.31, 95% CI 1.08 to 4.93, p=0.03), better physical quality of life (OR 1.05, 95% CI 1.0 to 1.09, p=0.05) and more reported barriers (OR 0.71, 95% CI 0.56 to 0.91, p=0.01). Conclusions More than half of the patients with HCM did not meet minimum physical activity recommendations. Several barriers to exercise among individuals with HCM exist, and provide the basis for targeted interventions to promote physical activity and improve overall health in patients with HCM. PMID:27547438

  5. Adverse events in families with hypertrophic or dilated cardiomyopathy and mutations in the MYBPC3 gene

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    Lehrke Stephanie

    2008-10-01

    Full Text Available Abstract Background Mutations in MYBPC3 encoding myosin binding protein C belong to the most frequent causes of hypertrophic cardiomyopathy (HCM and may also lead to dilated cardiomyopathy (DCM. MYBPC3 mutations initially were considered to cause a benign form of HCM. The aim of this study was to examine the clinical outcome of patients and their relatives with 18 different MYBPC3 mutations. Methods 87 patients with HCM and 71 patients with DCM were screened for MYBPC3 mutations by denaturing gradient gel electrophoresis and sequencing. Close relatives of mutation carriers were genotyped for the respective mutation. Relatives with mutation were then evaluated by echocardiography and magnetic resonance imaging. A detailed family history regarding adverse clinical events was recorded. Results In 16 HCM (18.4% and two DCM (2.8% index patients a mutation was detected. Seven mutations were novel. Mutation carriers exhibited no additional mutations in genes MYH7, TNNT2, TNNI3, ACTC and TPM1. Including relatives of twelve families, a total number of 42 mutation carriers was identified of which eleven (26.2% had at least one adverse event. Considering the twelve families and six single patients with mutations, 45 individuals with cardiomyopathy and nine with borderline phenotype were identified. Among the 45 patients, 23 (51.1% suffered from an adverse event. In eleven patients of seven families an unexplained sudden death was reported at the age between 13 and 67 years. Stroke or a transient ischemic attack occurred in six patients of five families. At least one adverse event occurred in eleven of twelve families. Conclusion MYBPC3 mutations can be associated with cardiac events such as progressive heart failure, stroke and sudden death even at younger age. Therefore, patients with MYBPC3 mutations require thorough clinical risk assessment.

  6. The Differential Hormonal Milieu of Morning versus Evening May Have an Impact on Muscle Hypertrophic Potential.

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    Simon D Burley

    Full Text Available Substantial gains in muscle strength and hypertrophy are clearly associated with the routine performance of resistance training. What is less evident is the optimal timing of the resistance training stimulus to elicit these significant functional and structural skeletal muscle changes. Therefore, this investigation determined the impact of a single bout of resistance training performed either in the morning or evening upon acute anabolic signalling (insulin-like growth factor-binding protein-3 (IGFBP-3, myogenic index and differentiation and catabolic processes (cortisol. Twenty-four male participants (age 21.4±1.9yrs, mass 83.7±13.7kg with no sustained resistance training experience were allocated to a resistance exercise group (REP. Sixteen of the 24 participants were randomly selected to perform an additional non-exercising control group (CP protocol. REP performed two bouts of resistance exercise (80% 1RM in the morning (AM: 0800 hrs and evening (PM: 1800 hrs, with the sessions separated by a minimum of 72 hours. Venous blood was collected immediately prior to, and 5 min after, each resistance exercise and control sessions. Serum cortisol and IGFBP-3 levels, myogenic index, myotube width, were determined at each sampling period. All data are reported as mean ± SEM, statistical significance was set at P≤0.05. As expected a significant reduction in evening cortisol concentration was observed at pre (AM: 98.4±10.5, PM: 49.8±4.4 ng/ml, P0.05. Timing of resistance training regimen in the evening appears to augment some markers of hypertrophic potential, with elevated IGFBP-3, suppressed cortisol and a superior cellular environment. Further investigation, to further elucidate the time course of peak anabolic signalling in morning vs evening training conditions, are timely.

  7. The Differential Hormonal Milieu of Morning versus Evening May Have an Impact on Muscle Hypertrophic Potential.

    Science.gov (United States)

    Burley, Simon D; Whittingham-Dowd, Jayde; Allen, Jeremy; Grosset, Jean-Francois; Onambele-Pearson, Gladys L

    2016-01-01

    Substantial gains in muscle strength and hypertrophy are clearly associated with the routine performance of resistance training. What is less evident is the optimal timing of the resistance training stimulus to elicit these significant functional and structural skeletal muscle changes. Therefore, this investigation determined the impact of a single bout of resistance training performed either in the morning or evening upon acute anabolic signalling (insulin-like growth factor-binding protein-3 (IGFBP-3), myogenic index and differentiation) and catabolic processes (cortisol). Twenty-four male participants (age 21.4±1.9yrs, mass 83.7±13.7kg) with no sustained resistance training experience were allocated to a resistance exercise group (REP). Sixteen of the 24 participants were randomly selected to perform an additional non-exercising control group (CP) protocol. REP performed two bouts of resistance exercise (80% 1RM) in the morning (AM: 0800 hrs) and evening (PM: 1800 hrs), with the sessions separated by a minimum of 72 hours. Venous blood was collected immediately prior to, and 5 min after, each resistance exercise and control sessions. Serum cortisol and IGFBP-3 levels, myogenic index, myotube width, were determined at each sampling period. All data are reported as mean ± SEM, statistical significance was set at P≤0.05. As expected a significant reduction in evening cortisol concentration was observed at pre (AM: 98.4±10.5, PM: 49.8±4.4 ng/ml, P0.05). Timing of resistance training regimen in the evening appears to augment some markers of hypertrophic potential, with elevated IGFBP-3, suppressed cortisol and a superior cellular environment. Further investigation, to further elucidate the time course of peak anabolic signalling in morning vs evening training conditions, are timely.

  8. Positron computed tomography for myocardial uptake of N-13 ammonia in patients with hypertrophic cardiomyopathy

    International Nuclear Information System (INIS)

    Yoshida, Katsuya; Himi, Toshiharu; Imai, Hitoshi; Shukuya, Masaki; Masuda, Yoshiaki; Inagaki, Yoshiaki; Yamasaki, Toshiro; Tateno, Yukio.

    1985-01-01

    In the present study, positron computed tomography (PCT) was used to evaluate the myocardial uptake of N-13 ammonia in patients with hypertrophic cardiomyopathy (HCM). Eight subjects including two normal persons, four patients with HCM, and two with old myocardial farction (OMI) were selected for the study. N-13 ammonia was administered intravenously as a bolus and, commencing with the tracer injection, serial 30-second PCT scans were performed. The results were summarized as follows: 1. The first scan exhibiting cardiac blood pool images revealed a reduced left ventricular cavity in the HCM subjects. 2. After clearance of N-13 from the cardiopulmonary vasculature, the left ventricular myocardium was clearly visualized and an increased myocardial mass with characteristic morphology was demonstrated in the HCM subjects. 3. Detailed analysis of the time-activity curves of the blood pool and myocardium derived from these serial scan images disclosed two uptake phases in the uptake mode of N-13 ammonia. In the initial phase within three minutes, the myocardial uptake of N-13 was rapid in the normal and OMI subjects, whereas its significant delay was observed in the HCM subjects. This may reflect an abnormal initial extraction of N-13 ammonia in the HCM patients compared with the other subjects. 4. Subsequently, in the second phase, which was characterized by a gradual increase of N-13 in the myocardium, the HCM subjects revealed higher uptake ratios than did the others. This may indicate an increased extraction of metabolites of N-13 ammonia during the second phase. These preliminary results undersore the usefulness of dynamic PCT with N-13 ammonia for the assessment of HCM. (author)

  9. Echocaridography, electrocardiography, and radiography of cats with dilatation cardiomyopathy, hypertrophic cardiomyopathy, and hyperyroidism

    International Nuclear Information System (INIS)

    Moise, N.S.; Dietze, A.E.; Mezza, L.E.; Strickland, D.; Erb, H.N.; Edwards, N.J.

    1986-01-01

    The echocardiographic, ECG, and radiographic findings of sequentially examined cats with dilatation cardiomyopathy (DCM, n = 7), hypertrophic cardiomyopathy (HCM, n = 8), and hyperthyroidism (HT, n = 20) were compared with those of healthy control cats (n = 11). Cats with DCM were easily differentiated from healthy cats by echocardiography and from cats with HCM and HT by a dilated left ventricle at end-diastole with a mean +/- SD of 2.20 +/- 0.36 cm, reduced fractional shortening (2.9% +/- 3.7%), reduced aortic amplitude (0.07 +/- 0.05 cm), reduced left ventricular wall amplitude (0.09 +/- 0.09 cm), and increased E-point septal separation (0.83 +/- 0.29 cm). The cats with HCM were most consistently recognized echocardiographically by increased left ventricular wall thickness at end-diastole (0.75 +/- 0.12 cm). Some cats with HT had abnormal echocardiograms with left ventricular wall hypertrophy. These cats could usually be differentiated from the cats with HCM because of normal or increased ventricular wall amplitude, aortic amplitude, or percentage of thickening of the left ventricular wall and interventricular septum. Left atrial enlargement (left atrial diameter greater than 1.57 cm or left atrium/aorta greater than 1.75) was commonly detected by the echocardiogram in cats with DCM, HCM, or HT. The echocardiogram was helpful in differentiating the type of cardiomyopathy (DCM, HCM, or HT) when plain thoracic radiographs indicated that cardiomegaly existed. The ECG may have indicated incorrectly that there was left ventricular enlargement in some cats with HT, and it did not indicate consistently that left ventricular enlargement existed when present in cats with DCM or HCM. The ECG was a poor indicator of left atrial enlargement in all cats

  10. The association between brain natriuretic peptide and tissue Doppler parameters in children with hypertrophic cardiomyopathy

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    Taliha Öner

    2016-01-01

    Full Text Available In this study, we investigated the association between brain natriuretic peptide (BNP levels and tissue Doppler imaging measurements and also screening for deadly mutations in patients with hypertrophic cardiomyopathy (HCM. We enrolled 20 patients diagnosed with HCM (age:10.7±5 years (1-17, 85% male, weight:42.25±23.10 kg, height:141.80±32.45 cm and 20 age, gender and body weight-matched control subjects. We performed electrocardiography, transthoracic echocardiography, and tissue Doppler echocardiography in each group, as well as genetic tests (for Arg403Gln, Arg453Cys, Arg719Trp and Arg719Gln mutations in MYH7 Exons 13, 14, 19 and BNP in the patients. The patients were divided into two groups according to the presence (Group 1 or absence (Group 2 of left ventricular (LV outflow tract obstruction. QTc dispersion and the LV ejection fraction and left atrial (LA volume index were increased in Group 1. The LA volume index and the mitral and septal E/Ea ratio and septum Z-score were increased while the mitral lateral annulus and septal annulus Ea wave velocities and the mitral and tricuspid E/A ratio were decreased in patients with high levels of BNP compared to those with normal BNP levels. There were no mutations that are associated with increased risk of sudden death found in patients included in this study. In the light of our data, we conclude that such parameters BNP levels above the 98 pg/mL, septal thickness Z-score ˃6, and higher mitral and septal E/Ea ratios can be used for management of patients with HCM according to life-threatening conditions.

  11. Hypertrophic Cardiomyopathy: A Vicious Cycle Triggered by Sarcomere Mutations and Secondary Disease Hits.

    Science.gov (United States)

    Wijnker, Paul J M; Sequeira, Vasco; Kuster, Diederik W D; Velden, Jolanda van der

    2018-04-11

    Hypertrophic cardiomyopathy (HCM) is a cardiac genetic disease characterized by left ventricular hypertrophy, diastolic dysfunction, and myocardial disarray. Disease onset occurs between 20 and 50 years of age, thus affecting patients in the prime of their life. HCM is caused by mutations in sarcomere proteins, the contractile building blocks of the heart. Despite increased knowledge of causal mutations, the exact path from genetic defect leading to cardiomyopathy is complex and involves additional disease hits. Recent Advances: Laboratory-based studies indicate that HCM development not only depends on the primary sarcomere impairment caused by the mutation but also on secondary disease-related alterations in the heart. Here we propose a vicious mutation-induced disease cycle, in which a mutation-induced energy depletion alters cellular metabolism with increased mitochondrial work, which triggers secondary disease modifiers that will worsen disease and ultimately lead to end-stage HCM. Evidence shows excessive cellular reactive oxygen species (ROS) in HCM patients and HCM animal models. Oxidative stress markers are increased in the heart (oxidized proteins, DNA, and lipids) and serum of HCM patients. In addition, increased mitochondrial ROS production and changes in endogenous antioxidants are reported in HCM. Mutant sarcomeric protein may drive excessive levels of cardiac ROS via changes in cardiac efficiency and metabolism, mitochondrial activation and/or dysfunction, impaired protein quality control, and microvascular dysfunction. Interventions restoring metabolism, mitochondrial function, and improved ROS balance may be promising therapeutic approaches. We discuss the effects of current HCM pharmacological therapies and potential future therapies to prevent and reverse HCM. Antioxid. Redox Signal. 00, 000-000.

  12. Prevalence, clinical significance, and natural history of left ventricular apical aneurysms in hypertrophic cardiomyopathy.

    Science.gov (United States)

    Maron, Martin S; Finley, John J; Bos, J Martijn; Hauser, Thomas H; Manning, Warren J; Haas, Tammy S; Lesser, John R; Udelson, James E; Ackerman, Michael J; Maron, Barry J

    2008-10-07

    Hypertrophic cardiomyopathy (HCM) is the most common genetic heart disease characterized by a diverse clinical and phenotypic spectrum. This study reports the prevalence, morphology, clinical course, and management of an underrecognized subgroup of HCM patients with left ventricular apical aneurysms. Of 1299 HCM patients, 28 (2%) were identified with left ventricular apical aneurysms, including a pair of identical twins. Aneurysms were recognized at a wide age range (26 to 83 years), including 12 patients (43%) who were rims, and were associated with transmural (and often more extensive) myocardial scarring identified by late gadolinium enhancement cardiovascular magnetic resonance. Apical aneurysms were recognized by echocardiography in only 16 of 28 patients (57%) but by cardiovascular magnetic resonance in the 12 patients undetected by echocardiography. Left ventricular chamber morphology varied; however, 19 patients (68%) showed an "hourglass" contour, with midventricular hypertrophy producing muscular narrowing and intracavitary gradients in 9 patients (74+/-42 mm Hg). Sarcomeric protein missense mutations known to cause other phenotypic expressions of HCM were present in 3 patients. Over 4.1+/-3.7 years of follow-up, 12 patients (43%) with left ventricular apical aneurysms experienced adverse disease complications (event rate, 10.5%/y), including sudden death, appropriate implantable cardioverter-defibrillator discharges, nonfatal thromboembolic stroke, and progressive heart failure and death. Patients with left ventricular apical aneurysms represent an underappreciated subset in the heterogeneous HCM disease spectrum with important clinical implications, often requiring a high index of suspicion and cardiovascular magnetic resonance for identification. Apical aneurysms in HCM are associated with substantial cardiovascular morbidity and mortality and raise novel treatment considerations.

  13. Spatial and temporal distribution of coloured dissolved organic matter in a hypertrophic freshwater lagoon

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    Diana Vaičiūtė

    2015-05-01

    Full Text Available A dataset of 224 Medium Resolution Imaging Spectrometer (MERIS full resolution satellite images were processed to retrieve the concentration of coloured dissolved organic matter (CDOM in a hypertrophic estuary (Curonian Lagoon, Lithuania and Russia. Images covered a period of 7 months, spanning from the ice melting (March to the late summer (September of 7 consecutive years (2005-2011. The aim of the study was to analyse the spatial and temporal variations of CDOM, by focusing on the main regulating factors (riverine discharge, sea-lagoon water exchange, water temperature, chlorophyll a, wind in a large estuary. The working hypothesis is that CDOM distribution may reveal distinct, site specific seasonal patterns. Our results demonstrated that CDOM concentrations at the whole lagoon level were elevated (1.5-4 m-1 and slightly but significantly higher in spring (1.50 m-1 on average compared to the summer (1.45 m-1 on average. This is due to very different flow of CDOM-rich freshwater from the main lagoon tributary in spring compared to summer. They also highlight macroscopic differences among areas within the lagoon, depending on season, suggesting a complex regulation of CDOM in this system. Significant factors explaining observed differences are the dilution of lagoon water with CDOM-poor brackish water, regeneration of large amounts of dissolved organic matter from sediments and combinations of uptake/release from phytoplankton. CDOM and its variations are understudied due to inherent methodological and analytical difficulties. However, this pool has a demonstrated relevant role in the biogeochemistry of aquatic environments. We speculate that the dissolved organic pool in the Curonian Lagoon has a mainly allochthonous origin in the high discharge period and an autochthonous origin in the summer, algal bloom period. Both positive and negative relationships between CDOM and phytoplankton suggest that pelagic microalgae may act as a source or as

  14. Characteristic systolic waveform of left ventricular longitudinal strain rate in patients with hypertrophic cardiomyopathy.

    Science.gov (United States)

    Okada, Kazunori; Kaga, Sanae; Mikami, Taisei; Masauzi, Nobuo; Abe, Ayumu; Nakabachi, Masahiro; Yokoyama, Shinobu; Nishino, Hisao; Ichikawa, Ayako; Nishida, Mutsumi; Murai, Daisuke; Hayashi, Taichi; Shimizu, Chikara; Iwano, Hiroyuki; Yamada, Satoshi; Tsutsui, Hiroyuki

    2017-05-01

    We analyzed the waveform of systolic strain and strain-rate curves to find a characteristic left ventricular (LV) myocardial contraction pattern in patients with hypertrophic cardiomyopathy (HCM), and evaluated the utility of these parameters for the differentiation of HCM and LV hypertrophy secondary to hypertension (HT). From global strain and strain-rate curves in the longitudinal and circumferential directions, the time from mitral valve closure to the peak strains (T-LS and T-CS, respectively) and the peak systolic strain rates (T-LSSR and T-CSSR, respectively) were measured in 34 patients with HCM, 30 patients with HT, and 25 control subjects. The systolic strain-rate waveform was classified into 3 patterns ("V", "W", and "√" pattern). In the HCM group, T-LS was prolonged, but T-LSSR was shortened; consequently, T-LSSR/T-LS ratio was distinctly lower than in the HT and control groups. The "√" pattern of longitudinal strain-rate waveform was more frequently seen in the HCM group (74 %) than in the control (4 %) and HT (20 %) groups. Similar but less distinct results were obtained in the circumferential direction. To differentiate HCM from HT, the sensitivity and specificity of the T-LSSR/T-LS ratio patients with HCM, a reduced T-LSSR/T-LS ratio and a characteristic "√"-shaped waveform of LV systolic strain rate was seen, especially in the longitudinal direction. The timing and waveform analyses of systolic strain rate may be useful to distinguish between HCM and HT.

  15. Merits and pitfalls of genetic testing in a hypertrophic cardiomyopathy clinic.

    Science.gov (United States)

    Arad, Michael; Monserrat, Lorenzo; Haron-Khun, Shiraz; Seidman, Jonathan G; Seidman, Christine E; Arbustini, Eloisa; Glikson, Michael; Freimark, Dov

    2014-11-01

    Hypertrophic cardiomyopathy (HCM) is a familial disease with autosomal dominant inheritance and age-dependent penetrance, caused primarily by mutations of sarcomere genes. Because the clinical variability of HCM is related to its genetic heterogeneity, genetic studies may improve the diagnosis and prognostic evaluation in HCM. To analyze the impact of genetic diagnosis on the clinical management of HCM. Genetic studies were performed for either research or clinical reasons. Once the disease-causing mutation was identified, the management plan was reevaluated. Family members were invited to receive genetic counseling and encouraged to be tested for the mutation. Ten mutations in sarcomere protein genes were identified in 9 probands: 2 novel and 8 previously described. Advanced heart failure or sudden death in a young person prompted the genetic study in 8 of the 9 families. Of 98 relatives available for genotyping, only 53 (54%) agreed to be tested. The compliance was higher in families with sudden death and lower in what appeared to be sporadic HCM or elderly-onset disease. Among the healthy we identified 9 carriers and 19 non-carriers. In 6 individuals the test result resolved an uncertainty about "possible HCM." In several cases the genetic result was also used for family planning and played a role in decisions on cardioverter-defibrillator implantation. Recurrence of a same mutation in different families created an opportunity to apply the information from the literature for risk stratification of individual patients. We suggest that the clinical context determines the indication for genetic testing and interpretation of the results.

  16. Phenotypic expression in hypertrophic cardiomyopathy and late gadolinium enhancement on cardiac magnetic resonance.

    Science.gov (United States)

    Caetano, Francisca; Botelho, Ana; Trigo, Joana; Silva, Joana; Almeida, Inês; Venâncio, Margarida; Pais, João; Sanches, Conceição; Leitão Marques, António

    2014-05-01

    The prognostic value of late gadolinium enhancement (LGE) for risk stratification of hypertrophic cardiomyopathy (HCM) patients is the subject of disagreement. We set out to examine the association between clinical and morphological variables, risk factors for sudden cardiac death and LGE in HCM patients. From a population of 78 patients with HCM, we studied 53 who underwent cardiac magnetic resonance. They were divided into two groups according to the presence or absence of LGE. Ventricular arrhythmias and morbidity and mortality during follow-up were analyzed. Patients with LGE were younger at the time of diagnosis (p=0.046) and more often had a family history of sudden death (p=0.008) and known coronary artery disease (p=0.086). On echocardiography they had greater maximum wall thickness (p=0.007) and left atrial area (p=0.037) and volume (p=0.035), and more often presented a restrictive pattern of diastolic dysfunction (p=0.011) with a higher E/É ratio (p=0.003) and left ventricular systolic dysfunction (p=0.038). Cardiac magnetic resonance supported the association between LGE and previous echocardiographic findings: greater left atrial area (p=0.029) and maximum wall thickness (p<0.001) and lower left ventricular ejection fraction (p=0.056). Patients with LGE more often had an implantable cardioverter-defibrillator (ICD) (p=0.015). At follow-up, no differences were found in the frequency of ventricular arrhythmias, appropriate ICD therapies or mortality. The presence of LGE emerges as a risk marker, associated with the classical predictors of sudden cardiac death in this population. However, larger studies are required to confirm its independent association with clinical events. Copyright © 2013 Sociedade Portuguesa de Cardiologia. Published by Elsevier España. All rights reserved.

  17. Prognostic significance of radionuclide-assessed diastolic function in hypertrophic cardiomyopathy

    International Nuclear Information System (INIS)

    Chikamori, T.; Dickie, S.; Poloniecki, J.D.; Myers, M.J.; Lavender, J.P.; McKenna, W.J.

    1990-01-01

    To evaluate the prognostic significance of diastolic function in hypertrophic cardiomyopathy (HC), technetium-99m gated equilibrium radionuclide angiography, acquired in list mode, was performed in 161 patients. Five diastolic indexes were calculated. During 3.0 +/- 1.9 years, 13 patients had disease-related deaths. With univariate analysis, these patients were younger (29 +/- 20 vs 42 +/- 16 years; p less than 0.05), had a higher incidence of syncope (p less than 0.025), dyspnea (p less than 0.001), reduced peak filling rate (2.9 +/- 0.9 vs 3.4 +/- 1.0 end-diastolic volume/s; p = 0.09) with increased relative filling volume during the rapid filling period (80 +/- 7 vs 75 +/- 12%; p = 0.06) and decreased atrial contribution (17 +/- 7 vs 22 +/- 11%; p = 0.07). Stepwise discriminant analysis revealed that young age at diagnosis, syncope at diagnosis, reduced peak ejection rate, positive family history, reduced peak filling rate, increased relative filling volume by peak filling rate and concentric left ventricular hypertrophy were the most statistically significant (p = 0.0001) predictors of disease-related death (sensitivity 92%, specificity 76%, accuracy 77%, positive predictive value 25%). Discriminant analysis excluding the diastolic indexes, however, showed similar predictability (sensitivity 92%, specificity 76%, accuracy 78%, positive predictive value 26%). To obtain more homogeneous groups for analysis, patients were classified as survivors or electrically unstable, including sudden death, out-of-hospital ventricular fibrillation and nonsustained ventricular tachycardia during 48-hour ambulatory electrocardiography, and heart failure death or cardiac transplant

  18. Late enhanced computed tomography in Hypertrophic Cardiomyopathy enables accurate left-ventricular volumetry

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    Langer, Christoph; Lutz, M.; Kuehl, C.; Frey, N. [Christian-Albrechts-Universitaet Kiel, Department of Cardiology, Angiology and Critical Care Medicine, University Medical Center Schleswig-Holstein (Germany); Partner Site Hamburg/Kiel/Luebeck, DZHK (German Centre for Cardiovascular Research), Kiel (Germany); Both, M.; Sattler, B.; Jansen, O; Schaefer, P. [Christian-Albrechts-Universitaet Kiel, Department of Diagnostic Radiology, University Medical Center Schleswig-Holstein (Germany); Harders, H.; Eden, M. [Christian-Albrechts-Universitaet Kiel, Department of Cardiology, Angiology and Critical Care Medicine, University Medical Center Schleswig-Holstein (Germany)

    2014-10-15

    Late enhancement (LE) multi-slice computed tomography (leMDCT) was introduced for the visualization of (intra-) myocardial fibrosis in Hypertrophic Cardiomyopathy (HCM). LE is associated with adverse cardiac events. This analysis focuses on leMDCT derived LV muscle mass (LV-MM) which may be related to LE resulting in LE proportion for potential risk stratification in HCM. N=26 HCM-patients underwent leMDCT (64-slice-CT) and cardiovascular magnetic resonance (CMR). In leMDCT iodine contrast (Iopromid, 350 mg/mL; 150mL) was injected 7 minutes before imaging. Reconstructed short cardiac axis views served for planimetry. The study group was divided into three groups of varying LV-contrast. LeMDCT was correlated with CMR. The mean age was 64.2 ± 14 years. The groups of varying contrast differed in weight and body mass index (p < 0.05). In the group with good LV-contrast assessment of LV-MM resulted in 147.4 ± 64.8 g in leMDCT vs. 147.1 ± 65.9 in CMR (p > 0.05). In the group with sufficient contrast LV-MM appeared with 172 ± 30.8 g in leMDCT vs. 165.9 ± 37.8 in CMR (p > 0.05). Overall intra-/inter-observer variability of semiautomatic assessment of LV-MM showed an accuracy of 0.9 ± 8.6 g and 0.8 ± 9.2 g in leMDCT. All leMDCT-measures correlated well with CMR (r > 0.9). LeMDCT primarily performed for LE-visualization in HCM allows for accurate LV-volumetry including LV-MM in > 90 % of the cases. (orig.)

  19. Recent Advances in the Molecular Genetics of Familial Hypertrophic Cardiomyopathy in South Asian Descendants

    Directory of Open Access Journals (Sweden)

    Jessica Kraker

    2016-10-01

    Full Text Available The South Asian population, numbered at 1.8 billion, is estimated to comprise around 20% of the global population and 1% of the American population, and has one of the highest rates of cardiovascular disease. While South Asians show increased classical risk factors for developing heart failure, the role of population-specific genetic risk factors has not yet been examined for this group. Hypertrophic cardiomyopathy (HCM is one of the major cardiac genetic disorders among South Asians, leading to contractile dysfunction, heart failure, and sudden cardiac death. This disease displays autosomal dominant inheritance, and it is associated with a large number of variants in both sarcomeric and non-sarcomeric proteins. South Asians, a population with large ethnic diversity, potentially carries region-specific polymorphisms. There is high variability in disease penetrance and phenotypic expression of variants associated with HCM. Thus, extensive studies are required to decipher pathogenicity and the physiological mechanisms of these variants, as well as the contribution of modifier genes and environmental factors to disease phenotypes. Conducting genotype-phenotype correlation studies will lead to improved understanding of HCM and, consequently, improved treatment options for this high-risk population. The objective of this review is to report the history of cardiovascular disease and HCM in South Asians, present previously published pathogenic variants, and introduce current efforts to study HCM using induced pluripotent stem cell-derived cardiomyocytes, next-generation sequencing, and gene editing technologies. The authors ultimately hope that this review will stimulate further research, drive novel discoveries, and contribute to the development of personalized medicine with the aim of expanding therapeutic strategies for HCM.

  20. Effect of substrate on periphyton communities and relationships among food web components in shallow hypertrophic lake

    Directory of Open Access Journals (Sweden)

    Tomasz Mieczan

    2012-07-01

    Full Text Available We studied the role of natural (common reed and artificial substrata (bamboo in structuring the abundance and taxonomic composition of periphyton assemblages. Investigations were conducted in a shallow, hypertrophic lake situated in the area of Polesie Lubelskie (Eastern Poland. Periphyton communities (algae, ciliates, small metazoa and chironomids on both types of substratum were sampled monthly, from May to November of 2007. Water samples for chemical analysis were collected together with biological samples. We selected the group of ten environmental variables which are the most important in determining the habitat conditions in highly eutrophic lakes: temperature, Secchi disc visibility, conductivity, dissolved oxygen, periphytic chlorophyll-a, N-NO3, N-NH4, TP, P-PO4 and total organic carbon (TOC. The abundances of periphytic algae, ciliates, metazoa and chironomids were significantly affected by season and substrate. On natural substrata, in all studied months, periphyton communities showed higher abundances. The results of PCA analysis confirmed the distinction between periphyton communities on natural and artificial substrata. The Monte Carlo permutation test showed that the periphyton communities on common reed were the most significantly affected by temperature, N-NO3, Secchi disc visibility and TOC. The communities on artificial substrata were significantly influenced by temperature, P-PO4 and TOC. On natural substrata biomass of periphytic algae was significantly negatively correlated with abundances of all groups of potential grazers (ciliates, metazoa, chironomids. On artificial substrata the relations between components of periphytic food web were stronger; correlation coefficients between algae, protists and chironomids were significant at P<0.01. The results of analysis indicate that periphytic algae can play an important role as food source for higher trophic levels. These interactions are less significant on natural (reed

  1. Long-term outcomes of septal reduction for obstructive hypertrophic cardiomyopathy.

    Science.gov (United States)

    Sedehi, Daniel; Finocchiaro, Gherardo; Tibayan, Yen; Chi, Jeffrey; Pavlovic, Aleksandra; Kim, Young M; Tibayan, Frederick A; Reitz, Bruce A; Robbins, Robert C; Woo, Joseph; Ha, Richard; Lee, David P; Ashley, Euan A

    2015-07-01

    Surgical myectomy and alcohol septal ablation (ASA) aim to decrease left ventricular outflow tract (LVOT) gradient in hypertrophic cardiomyopathy (HCM). Outcome of myectomy beyond 10 years has rarely been described. We describe 20 years of follow-up of surgical myectomy and 5 years of follow-up for ASA performed for obstructive HCM. We studied 171 patients who underwent myectomy for symptomatic LVOT obstruction between 1972 and 2006. In addition, we studied 52 patients who underwent ASA for the same indication and who declined surgery. Follow-up of New York Heart Association (NYHA) functional class, echocardiographic data, and vital status were obtained from patient records. Mortality rates were compared with expected mortality rates of age- and sex-matched populations. Surgical myectomy improved NYHA class (2.74±0.65 to 1.54±0.74, phistorical data from non-operated HCM patients. Survival after ASA at 2 and 5 years was 97.8% and 94.7%, respectively. Short-term (5 year) survival after ASA (SMR=0.61, p=0.48) was comparable to that of the general population. Long-term follow-up of septal reduction strategies in obstructive HCM reveals that surgical myectomy and ASA are effective for symptom relief and LVOT gradient reduction and are associated with favorable survival. While overall prognosis for the community HCM population is similar to the general population, the need for surgical myectomy may identify a sub-group with poorer long-term prognosis. We await long-term outcomes of more extensive myectomy approaches adopted in the past 10 years at major institutions. Copyright © 2015. Published by Elsevier Ltd.

  2. Clinical Spectrum and Management of Heart Failure in Hypertrophic Cardiomyopathy.

    Science.gov (United States)

    Maron, Barry J; Rowin, Ethan J; Udelson, James E; Maron, Martin S

    2018-05-01

    Heart failure (HF), characterized by excessive exertional dyspnea, is a common complication within the broad clinical spectrum of hypertrophic cardiomyopathy (HCM). HF has become an increasingly prominent management issue with the reduction in sudden deaths due to use of implantable defibrillators in this disease. Exertional dyspnea ranges in severity from mild to severe (New York Heart Association functional classes II to IV) and not uncommonly becomes refractory to medical management, leading to progressive disability, but largely in the absence of pulmonary congestion and volume overload requiring hospitalization. HCM-related HF is most commonly due to dynamic mechanical impedance to left ventricular outflow produced by mitral valve systolic anterior motion, leading to high intracavity pressures. Surgical septal myectomy with low operative mortality (<1%) produces HF reversal and symptom relief in 90% to 95% of patients, while also conveying a survival benefit. Exercise echocardiography has assumed an important role in the evaluation of patients with HCM, i.e., by identifying candidates for septal reduction therapy with refractory HF when outflow gradients are present only with physiological exercise, distinguishing highly symptomatic nonobstructive patients as heart transplant candidates, and predicting future development of progressive HF. Notably, mortality directly attributable to HF has become exceedingly uncommon in HCM (<0.5%/year) in contrast with HF in non-HCM diseases (by 20-fold). In conclusion, HF in HCM is associated with diverse and complex pathophysiology, but a substantially more favorable prognosis than conventional non-HCM HF, and highly amenable to effective treatment options in the vast majority of patients. Copyright © 2018 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

  3. Myocardial perfusion abnormality and chest pain in patients with hypertrophic cardiomyopathy

    International Nuclear Information System (INIS)

    Narita, Michihiro; Kurihara, Tadashi; Murano, Kenichi; Usami, Masahisa

    1991-01-01

    To investigate the role of myocardial ischemia in the development of chest pain in patients with hypertrophic cardiomyopathy (HCM), exercise stress (Ex) redistribution myocardial single photon emission CT's (SPECT's) with thallium-201 (Tl) were obtained in 27 patients with HCM. In all patients, coronary arteries were normal arteriographically. Patients were classified into NYHA Class I, II and III according to the frequency and severity of the chest pain during daily life. In these 3 groups, age, sex and intraventricular septal thickness measured by echocardiography were not different. Types of myocardial perfusion obtained by myocardial SPECT's were divided into 5: (1) normal perfusion, (2) no perfusion defect with abnormal myocardial Tl washout rate (WOR) during 3 hours (<30%) [Def(-)/WORabn], (3) reversible perfusion defect (RD), (4) fixed defect with abnormal WOR (FD/WORabn), and (5) fixed defect with normal WOR (FD/WORnl). In 14 patients of Class I, 9 patients (64%) showed normal perfusion but the rest showed perfusion abnormality (def(-)/WORabn in 3 and RD in 2). In Class II and III, all patients showed perfusion abnormalities of RD, FD/WORabn or FD/WORnl. As the functional class progressed from Class II to III, the ratio of fixed defect (both WORnl and WORabn) to RD increased, but it was not statistically significant. In 2 patients in whom Ex SPECT's were repeated because of the progression of the chest pain, the severity of the perfusion abnormality also progressed. Perfusion abnormalities were observed most frequently in anterior (35%), then inferior/posterior (20%) and septal wall (18%). The frequency of Ex induced ECG abnormalities (ST-depression or T wave changes) increased as the NYHA Class progressed (Class III vs I p<0.05). These findings suggested the following: chest pain in patients with HCM relates to the myocardial ischemia which may originate in the myocardial small arteries, and when the lesions progress myocardial necrosis may ensue. (author)

  4. Bilateral hypertrophic olivary nucleus degeneration on magnetic resonance imaging in children with Leigh and Leigh-like syndrome.

    Science.gov (United States)

    Bindu, P S; Taly, A B; Sonam, K; Govindaraju, C; Arvinda, H R; Gayathri, N; Bharath, M M Srinivas; Ranjith, D; Nagappa, M; Sinha, S; Khan, N A; Thangaraj, K

    2014-02-01

    Bilateral hypertrophic olivary degeneration on brain MRI has been reported in a few metabolic, genetic and neurodegenerative disorders, including mitochondrial disorders. In this report, we sought to analyse whether bilateral symmetrical inferior olivary nucleus hypertrophy is specifically associated with mitochondrial disorders in children. This retrospective study included 125 children (mean age, 7.6 ± 5 years; male:female, 2.6:1) diagnosed with various metabolic and genetic disorders during 2005-2012. The routine MRI sequences (T1 weighted, T2 weighted and fluid-attenuated inversion-recovery sequences) were analysed for the presence of bilateral symmetrical olivary hypertrophy and central tegmental tract or dentate nuclei signal changes. The other imaging findings and the final diagnoses were noted. The cohort included patients with Leigh and Leigh-like syndrome (n = 25), other mitochondrial diseases (n = 25), Wilson disease (n = 40), Type 1 glutaric aciduria (n = 14), maple syrup urine disease (n = 13), giant axonal neuropathy (n = 5) and L-2 hydroxy glutaric aciduria (n = 3). Bilateral inferior olivary nucleus hypertrophy was noted in 10 patients, all of whom belonged to the Leigh and Leigh-like syndrome group. Bilateral hypertrophic olivary degeneration on MRI is relatively often, but not routinely, seen in children with Leigh and Leigh-like syndrome. Early detection of this finding by radiologists and physicians may facilitate targeted metabolic testing in these children. This article highlights the occurrence of bilateral hypertrophic olivary nucleus degeneration on MRI in children with Leigh and Leigh-like syndrome, compared with other metabolic disorders.

  5. Hypertrophic changes of the teres minor muscle in rotator cuff tears: quantitative evaluation by magnetic resonance imaging.

    Science.gov (United States)

    Kikukawa, Kenshi; Ide, Junji; Kikuchi, Ken; Morita, Makoto; Mizuta, Hiroshi; Ogata, Hiroomi

    2014-12-01

    Few reports have assessed the teres minor (TM) muscle in rotator cuff tears. This study aimed to quantitatively analyze the morphologic changes of the TM muscle in patients with or without rotator cuff tears by magnetic resonance imaging (MRI). This retrospective study consisted of 279 subjects classified on the basis of interpretations of conventional MRI observations into 6 groups: no cuff tear; partial-thickness supraspinatus (SSP) tear; full-thickness SSP tear; SSP and subscapularis tears; SSP and infraspinatus (ISP) tears; and SSP, ISP, and subscapularis tears. With use of ImageJ software (National Institutes of Health, Bethesda, MD, USA) for oblique sagittal MRI, we measured the areas of ISP, TM, and anatomic external rotation (ISP + TM) muscles on the most lateral side in which the scapular spine was in contact with the scapular body. The occupational ratios of the TM muscle area to the anatomic external rotation muscle area were calculated. Ratios above the maximum of the 95% confidence intervals of the occupational ratio in the no-tear group were defined as hypertrophy of the TM muscle. Occupational ratios of the TM muscle in the no-tear group followed a normal distribution, and ratios >0.288 were defined as hypertrophic. Hypertrophic changes of the TM muscle were confirmed in rotator cuff tears involving the ISP tendon. A negative correlation was found between the occupational ratios of TM and ISP (P muscle appeared hypertrophic in rotator cuff tears involving the ISP, and the progression of ISP muscle atrophy seemed to induce the development of this compensatory hypertrophy. Copyright © 2014 Journal of Shoulder and Elbow Surgery Board of Trustees. Published by Elsevier Inc. All rights reserved.

  6. Eccentric apical hypertrophic cardiomyopathy unmasked by multimodality imaging: an uncommon but missed cause of out of hospital cardiac arrest

    Science.gov (United States)

    Towe, Eric; Sharma, Saurabh; Geske, Jeffrey; Ackerman, Michael J

    2015-01-01

    A woman in her late 50s experienced a witnessed, sudden out of hospital cardiac arrest. Initial workup included coronary angiography, transthoracic echocardiogram and a CT scan of the chest to rule out pulmonary embolus. She was subsequently discharged home without an implantable cardioverter defibrillator (ICD) or a life vest. On follow-up at another facility, an ICD was placed and a Holter monitor showed no ventricular ectopy. Further transthoracic echocardiographic images were obtained, which were suggestive of apical hypertrophic cardiomyopathy. A limited transthoracic echocardiogram with contrast was performed, which did not elucidate the hypertrophy. However, eccentric left ventricular apical wall hypertrophy was visualised by a coronary CT scan. PMID:26153133

  7. Hypertrophic osteoarthropathy in association with pulmonary tuberculosis: a case report; Osteoartropatia hipertrofica associada a tuberculose pulmonar: relato de caso

    Energy Technology Data Exchange (ETDEWEB)

    Moreira, Luiza Beatriz Melo; Marchiori, Edson [Universidade Federal Fluminense, Niteroi, RJ (Brazil). Dept. de Radiologia]. E-mail: edmarchiori@zipmail.com.br; Melo, Alessandro Severo Alves de [Hospital Universitario Antonio Pedro, Niteroi, RJ (Brazil). Servico de Radiologia; Pinto, Ana Lucia de Araujo [Hospital Universitario Antonio Pedro, Niteroi, RJ (Brazil). Servico de Pneumologia; Monteiro, Nicolau Pedro [Universidade Federal Fluminense, Niteroi, RJ (Brazil). Dept. de Medicina Clinica

    2002-02-01

    In this article the authors report a case of a patient with hypertrophic osteoarthropathy associated with pulmonary tuberculosis. This disorder is a rare complication of tuberculosis and may be associated with severe and fulminant disease. The imaging methods used in the diagnosis of this disease included lower limbs radiographs that showed bilateral periosteal reaction, and chest radiographs and computed tomography that revealed a mass in the upper lobe of the right lung. Although a fine needle aspiration was suggestive of tuberculosis, the definitive diagnosis was made after thoracotomy and histopathological examination of the mass. The patient presented symptomatic and radiological improvement after treatment of tuberculosis. (author)

  8. A Rare Coincidence of Infantile Hypertrophic Pyloric Stenosis and Esophageal Atresia with TracheoEsophageal Fistula: A Case Report

    Directory of Open Access Journals (Sweden)

    Yesim Coskun

    2017-10-01

    Full Text Available The coincidence of Infantile Hypertrophic Pyloric Stenosis (IHPS and Esophageal Atresia (EA with Tracheo-Esophageal Fistula (TEF is rare. Although, vomiting and regurgitation in operated cases of EA and TEF are attributed to Gastroesophageal Reflux (GER and the stricture of the anastomosis, it may be also associated with IHPS as well. We report a case of 3-hour-old female infant, who had EA and TEF operation and diagnosed to have IHPS at 9th week of age. Early diagnosis and treatment can prevent complications.

  9. Incomplete form of Primary Hypertrophic Osteoarthropathy (Touraine-Solente-Gole Syndrome) Masquerading as Polyartrhalgia Diagnosed in Technetium-99m-Methylene Diphosphonate Scintigraphy: An Interesting Case Report.

    Science.gov (United States)

    Sivathapandi, Thangalakshmi; Amalachandran, Jaykanth; Simon, Shelley; Elangovan, Indirani

    2018-01-01

    The primary hypertrophic osteoarthropathy (PHOA) (pachydermoperiostosis) is a rare genetic/hereditary disease characterized by skin changes (pachydermia), clubbing of fingers and periosteal thickening (periostitis) with sub-periosteal new bone formation. Here we describe a case of an adolescent male who presented with clubbing and polyarthralgia. On evaluation with scintigraphy and SPECT-CT, he was diagnosed to have incomplete form of PHOA(skeletal manifestations without skin changes). The identification of incomplete form of primary hypertrophic osteoarthropathy which can be easily misdiagnosed as rheumatoid arthritis is discussed here.

  10. Wnt7b can replace Ihh to induce hypertrophic cartilage vascularization but not osteoblast differentiation during endochondral bone development.

    Science.gov (United States)

    Joeng, Kyu Sang; Long, Fanxin

    2014-01-01

    Indian hedgehog (Ihh) is an essential signal that regulates endochondral bone development. We have previously shown that Wnt7b promotes osteoblast differentiation during mouse embryogenesis, and that its expression in the perichondrium is dependent on Ihh signaling. To test the hypothesis that Wnt7b may mediate some aspects of Ihh function during endochondral bone development, we activated Wnt7b expression from the R26-Wnt7b allele with Col2-Cre in the Ihh(-/-) mouse. Artificial expression of Wnt7b rescued vascularization of the hypertrophic cartilage in the Ihh(-/-) mouse, but failed to restore orthotopic osteoblast differentiation in the perichondrium. Similarly, Wnt7b did not recover Ihh-dependent perichondral bone formation in the Ihh(-/-); Gli3(-/-) embryo. Interestingly, Wnt7b induced bone formation at the diaphyseal region of long bones in the absence of Ihh, possibly due to increased vascularization in the area. Thus, Ihh-dependent expression of Wnt7b in the perichondrium may contribute to vascularization of the hypertrophic cartilage during endochondral bone development.

  11. Early detection of myocardial dysfunction using two-dimensional speckle tracking echocardiography in a young cat with hypertrophic cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Ryohei Suzuki

    2018-02-01

    Full Text Available Case summary A 5-month-old intact female Scottish Fold cat was presented for cardiac evaluation. Careful auscultation detected a slight systolic murmur (Levine I/VI. The findings of electrocardiography, thoracic radiography, non-invasive blood pressure measurements and conventional echocardiographic studies were unremarkable. However, two-dimensional speckle tracking echocardiography revealed abnormalities in myocardial deformations, including decreased early-to-late diastolic strain rate ratios in longitudinal, radial and circumferential directions, and deteriorated segmental systolic longitudinal strain. At the follow-up examinations, the cat exhibited echocardiographic left ventricular hypertrophy and was diagnosed with hypertrophic cardiomyopathy using conventional echocardiography. Relevance and novel information This is the first report on the use of two-dimensional speckle tracking echocardiography for the early detection of myocardial dysfunction in a cat with hypertrophic cardiomyopathy; the myocardial dysfunction was detected before the development of hypertrophy. The findings from this case suggest that two-dimensional speckle tracking echocardiography can be useful for myocardial assessment when conventional echocardiographic and Doppler findings are ambiguous.

  12. The role of γ-ray-induced fibroblast apoptosis in inhibiting biliary duct hypertrophic scar formation in dogs

    International Nuclear Information System (INIS)

    He Guijin; Zhang Hong; Gao Xinyi; Xu Shuhe; Gao Hong; Jiang Weiguo; Jiangtao; Dai Xianwei; Ma Kai

    2005-01-01

    Objective: To investigate the role of γ-ray-induced fibroblast apoptosis in the inhibition of biliary duct hypertrophic scar formation in dogs. Methods: γ-radiation-induced apoptotic fibroblast cells were analysed by using transmission electron microscopy and DNA from frozen biliary duct tissue was extracted with phenol chloroform. DNA ladder profile after extraction of RNA was observed, and apoptosis cells in paraffinem-bedded biliary duct tissue sections were examined used immuno-histochemical method. Dog biliary duct cross-sections were stained with hematoxylin-erosin, Masson's trichrome, and Verhoeff-van Giesen stains. Muscle formation area, lumen circumference, and stenosis degree were determined by a computer-assisted image analysis system. Results: 103 Pd radioactive stent significantly inhibited fibroblast proliferation. The features of fibroblast apoptosis (e.g, apoptic bodies, DNA ladder band) could be seen in the 103 Pd radioactive stent group. The fibroblast apoptotic rate was significantly increased in the 103 Pd radioactive stent group than in the control group (P 103 Pd radioactive stent significantly reduced biliary muscular formation. Conclusion: 103 Pd radioactive stent could have the effect of inhibiting the proliferation of scar-forming fibroblast, and thus could be used for treatment and (or) prevention of hypertrophic scar formation in biliary duct. (authors)

  13. IGF-1 Gene Transfer to Human Synovial MSCs Promotes Their Chondrogenic Differentiation Potential without Induction of the Hypertrophic Phenotype

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    Yasutoshi Ikeda

    2017-01-01

    Full Text Available Mesenchymal stem cell- (MSC- based therapy is a promising treatment for cartilage. However, repair tissue in general fails to regenerate an original hyaline-like tissue. In this study, we focused on increasing the expression levels for insulin-like growth factor-1 (IGF-1 to improve repair tissue quality. The IGF-1 gene was introduced into human synovial MSCs with a lentiviral vector and examined the levels of gene expression and morphological status of MSCs under chondrogenic differentiation condition using pellet cultures. The size of the pellets derived from IGF-1-MSCs were significantly larger than those of the control group. The abundance of glycosaminoglycan (GAG was also significantly higher in the IGF-1-MSC group. The histology of the IGF-1-induced pellets demonstrated similarities to hyaline cartilage without exhibiting features of a hypertrophic chondrocyte phenotype. Expression levels for the Col2A1 gene and protein were significantly higher in the IGF-1 pellets than in the control pellets, but expression levels for Col10, MMP-13, ALP, and Osterix were not higher. Thus, IGF-1 gene transfer to human synovial MSCs led to an improved chondrogenic differentiation capacity without the detectable induction of a hypertrophic or osteogenic phenotype.

  14. Is myocardial fatty acid metabolism different between hypertrophic cardiomyopathy and hypertensive hypertrophy?

    International Nuclear Information System (INIS)

    Narita, Michihiro; Kurihara, Tadashi; Usami, Masahisa; Honda, Minoru

    1994-01-01

    To investigate characteristics of fatty acid metabolism in hypertrophic cardiomyopathy (HCM), we performed myocardial imaging with 123 I-iodophenyl-3-methylpentadecanoic acid (BMIPP) in 24 HCM patients, 13 patients with hypertensive hypertrophy (HT) and 10 normal subjects. Rest myocardial imaging with 123 I-BMIPP was obtained at 20 minutes and 3 hours after 123 I-BMIPP injection. Rest 201 Tl imaging was also performed. In addition to ordinary tomography, whole body imaging was performed to calculate %Uptake (percentage of cardiac uptake of the isotope to total injected dose). As global indexes of fatty acid metabolism, we calculated two parameters; Uptake Ratio (%Uptake of 123 I-BMIPP normalized by myocardial perfusion) and WOR (percent reduction of myocardial 123 I-BMIPP within 3 hours). Regional abnormality was evaluated by visual assessment of ordinary tomograms and by BMIPP/Tl map. BMIPP/Tl map was made from Bull's-eye maps of 123 I-BMIPP and 201 Tl, and it represented 123 I-BMIPP uptake normalized by myocardial perfusion of each pixel which constructed the image. %Uptake of 123 I-BMIPP was not different among three groups. Uptake Ratio was significantly (p HT (1.03±0.08)>HCM (0.87±0.09). WOR of 123 I-BMIPP was accerelated in HCM (12.7±4.7%) and HT (10.2±2.9%) compared with normal (5.1±3.1%) (p 123 I-BMIPP distribution was found in 17 of 24 patients (71%) including 3 patients with equivocal abnormality. In HT patients, only equivocal abnormality was observed in 23%. In BMIPP/Tl map, abnormality was observed in 92% of HCM and 8% of HT. Although global myocardial fatty acid metabolism was equally disturbed both in HCM and HT, regional abnormality of fatty acid metabolism was observed preferetially in HCM. This indicated myocardial fatty acid metabolism was not identical between HCM and HT. (author)

  15. Reproducibility of in-vivo diffusion tensor cardiovascular magnetic resonance in hypertrophic cardiomyopathy

    Directory of Open Access Journals (Sweden)

    McGill Laura-Ann

    2012-12-01

    Full Text Available Abstract Background Myocardial disarray is an important histological feature of hypertrophic cardiomyopathy (HCM which has been studied post-mortem, but its in-vivo prevalence and extent is unknown. Cardiac Diffusion Tensor Imaging (cDTI provides information on mean intravoxel myocyte orientation and potentially myocardial disarray. Recent technical advances have improved in-vivo cDTI, and the aim of this study was to assess the interstudy reproducibility of quantitative in-vivo cDTI in patients with HCM. Methods and results A stimulated-echo single-shot-EPI sequence with zonal excitation and parallel imaging was implemented. Ten patients with HCM were each scanned on 2 different days. For each scan 3 short axis mid-ventricular slices were acquired with cDTI at end systole. Fractional anisotropy (FA, mean diffusivity (MD, and helix angle (HA maps were created using a cDTI post-processing platform developed in-house. The mean ± SD global FA was 0.613 ± 0.044, MD was 0.750 ± 0.154 × 10-3 mm2/s and HA was epicardium −34.3 ± 7.6°, mesocardium 3.5 ± 6.9° and endocardium 38.9 ± 8.1°. Comparison of initial and repeat studies showed global interstudy reproducibility for FA (SD = ± 0.045, Coefficient of Variation (CoV = 7.2%, MD (SD = ± 0.135 × 10-3 mm2/s, CoV = 18.6% and HA (epicardium SD = ± 4.8°; mesocardium SD = ± 3.4°; endocardium SD = ± 2.9°. Reproducibility of FA was superior to MD (p = 0.003. Global MD was significantly higher in the septum than the reference lateral wall (0.784 ± 0.188 vs 0.750 ± 0.154 x10-3 mm2/s, p  Conclusions To the best of our knowledge, this is the first study to assess the interstudy reproducibility of DTI in the human HCM heart in-vivo and the largest cDTI study in HCM to date. Our results show good reproducibility of FA, MD and HA which indicates that current technology yields robust in-vivo measurements that have potential clinical value. The

  16. Role of late gadolinium enhancement cardiovascular magnetic resonance in the risk stratification of hypertrophic cardiomyopathy.

    Science.gov (United States)

    Ismail, Tevfik F; Jabbour, Andrew; Gulati, Ankur; Mallorie, Amy; Raza, Sadaf; Cowling, Thomas E; Das, Bibek; Khwaja, Jahanzaib; Alpendurada, Francisco D; Wage, Ricardo; Roughton, Michael; McKenna, William J; Moon, James C; Varnava, Amanda; Shakespeare, Carl; Cowie, Martin R; Cook, Stuart A; Elliott, Perry; O'Hanlon, Rory; Pennell, Dudley J; Prasad, Sanjay K

    2014-12-01

    Myocardial fibrosis identified by late gadolinium enhancement (LGE) cardiovascular magnetic resonance (CMR) in patients with hypertrophic cardiomyopathy (HCM) is associated with adverse cardiovascular events, but its value as an independent risk factor for sudden cardiac death (SCD) is unknown. We investigated the role of LGE-CMR in the risk stratification of HCM. We conducted a prospective cohort study in a tertiary referral centre. Consecutive patients with HCM (n=711, median age 56.3 years, IQR 46.7-66.6; 70.0% male) underwent LGE-CMR and were followed for a median 3.5 years. The primary end point was SCD or aborted SCD. Overall, 471 patients (66.2%) had myocardial fibrosis (median 5.9% of left ventricular mass, IQR: 2.2-13.3). Twenty-two (3.1%) reached the primary end point. The extent but not the presence of fibrosis was a significant univariable predictor of the primary end point (HR per 5% LGE: 1.24, 95% CI 1.06 to 1.45; p=0.007 and HR for LGE: 2.69, 95% CI 0.91 to 7.97; p=0.073, respectively). However, on multivariable analysis, only LV-EF remained statistically significant (HR: 0.92, 95% CI 0.89 to 0.95; p<0.001). For the secondary outcome of cardiovascular mortality/aborted SCD, the presence and the amount of fibrosis were significant predictors on univariable but not multivariable analysis after adjusting for LV-EF and non-sustained ventricular tachycardia. The amount of myocardial fibrosis was a strong univariable predictor of SCD risk. However, this effect was not maintained after adjusting for LV-EF. Further work is required to elucidate the interrelationship between fibrosis and traditional predictors of outcome in HCM. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

  17. Intraoperative Diagnosis of Anderson-Fabry Disease in Patients With Obstructive Hypertrophic Cardiomyopathy Undergoing Surgical Myectomy.

    Science.gov (United States)

    Cecchi, Franco; Iascone, Maria; Maurizi, Niccolò; Pezzoli, Laura; Binaco, Irene; Biagini, Elena; Fibbi, Maria Laura; Olivotto, Iacopo; Pieruzzi, Federico; Fruntelata, Ana; Dorobantu, Lucian; Rapezzi, Claudio; Ferrazzi, Paolo

    2017-10-01

    Diagnostic screening for Anderson-Fabry cardiomyopathy (AFC) is performed in the presence of specific clinical red flags in patients with hypertrophic cardiomyopathy (HCM) older than 25 years. However, left ventricular outflow tract obstruction (LVOTO) has been traditionally considered an exclusion criteria for AFC. To examine a series of patients diagnosed with HCM and severe basal LVOTO undergoing myectomy in whom the diagnosis of AFC was suspected by the cardiac surgeon intraoperatively and confirmed by histological and genetic examinations. This retrospective analysis of patients undergoing surgical septal reduction strategies was conducted in 3 European tertiary referral centers for HCM from July 2013 to December 2016. Patients with a clinical diagnosis of obstructive HCM referred for surgical management of LVOTO were observed for at least 18 months after the procedure (mean [SD] follow-up, 33 [14] months). Etiology of patients with HCM who underwent surgical myectomy. From 2013, 235 consecutive patients with a clinical diagnosis of HCM underwent septal myectomy. The cardiac surgeon suspected a storage disease in 3 patients (1.3%) while inspecting their heart samples extracted from myectomy. The mean (SD) age at diagnosis for these 3 patients was 42 (4) years; all were male. None of the 3 patients presented with extracardiac features suggestive of AFC. All patients showed asymmetrical left ventricular hypertrophy, with maximal left ventricular thickness in the basal septum (19-31 mm), severe basal LVOTO (70-120 mm Hg), and left atrial dilatation (44-57 mm). Only 1 patient presented with late gadolinium enhancement on cardiovascular magnetic resonance at the right ventricle insertion site. The mean (SD) age at surgical procedure was 63 (5) years. On tactile sensation, the surgeon felt a spongy consistency of the surgical samples, different from the usual stony-elastic consistency typical of classic HCM, and this prompted histological examinations. Histology

  18. Lower energy radial shock wave therapy improves characteristics of hypertrophic scar in a rabbit ear model.

    Science.gov (United States)

    Zhao, Jing-Chun; Zhang, Bo-Ru; Shi, Kai; Wang, Jian; Yu, Qing-Hua; Yu, Jia-Ao

    2018-01-01

    The aim of the present study was to investigate the effects of radial extracorporeal shock wave therapy (rESWT) on scar characteristics and transforming growth factor (TGF)-β1/Smad signaling in order to explore a potential modality for the treatment of hypertrophic scars (HS). The HS model was generated in rabbit ears, then rabbits were randomly divided into 3 groups: Lower (L)-ESWT [treated with rESWT with lower energy flux density (EFD) of 0.1 mJ/mm 2 ], higher (H)-ESWT (treated with a higher EFD of 0.18 mJ/mm 2 ) and the sham ESWT group (S-ESWT; no ESWT treatment). Scar characteristics (wrinkles, texture, diameter, area, volume of elevation, hemoglobin and melanin) were assessed using the Antera 3D ® system. The protein and mRNA expression of TGF-β1, Smad2, Smad3 and Smad7 was assessed by enzyme-linked immunosorbent assay and reverse transcription-quantitative polymerase chain reaction, respectively. The Antera 3D ® results indicated that wrinkles and hemoglobin of the HS were significantly improved in both of the rESWT groups when compared with the S-ESWT group. However, these changes appeared much earlier in the L-ESWT group than the H-ESWT. Scar texture was also improved in the L-ESWT group. However, rESWT did not influence HS diameter, area, volume of elevation or melanin levels. rESWT had no effect on TGF-β1 or Smad7 expression in either of rESWT groups. Although no difference was observed in Smad2 mRNA expression in the L-ESWT group, the Smad3 mRNA and protein expression significantly decreased when compared with the H-ESWT and S-ESWT groups. By contrast, Smad2 and Smad3 mRNA expression were upregulated in the H-ESWT group. These results demonstrated that rESWT with 0.1 mJ/mm 2 EFD improved some characteristics of the HS tissue. Downregulation of Smad3 expression may underlie this inhibitory effect. Inhibition of the TGF-β1/Smad signal transduction pathway may be a potential therapeutic target for the management of HS.

  19. [Early hypertrophic scar after surgery on the nasal region: value of long-acting corticosteroid injections].

    Science.gov (United States)

    Amici, J-M

    2014-01-01

    "Pincushioning" is a complication of post-surgical scarring following use of transposition flaps particularly when surgery is performed on the nasal region. The transposition flap technique is very useful for the repair of certain defects of the tip of the nose, the medial canthus or of the ala nasi. The aim of this study is to define the clinical characteristics of this scarring dystrophy, which we propose to call "early hypertrophy scarring", to clarify the nature thereof and to assess the efficacy of intralesional injection of corticosteroids at the first signs of hypertrophy. A prospective, open, non-comparative, single-centre study examined the clinical and histological characteristics of early hypertrophy scarring and the effectiveness of therapy with one or two injections of corticosteroids performed on the 15th day post-operatively and optionally repeated at D45 depending on the outcome. From January 2011 to January 2013, 12 consecutive patients with early hypertrophy scarring were included (ten men and two women - mean age: 64 years). All had undergone surgery for basal cell carcinoma under local anaesthesia with one-stage repair by means of a rhombic flap or a bilobed flap located in the nasal area. Scars were injected strictly intra-lesionally with triamcinolone acetate (40 mg/1 mL) until whitening occurred. A single injection was performed in three cases of rhombic flap while a second injection was given at D45 in the remaining nine cases. Complete regression of the early hypertrophy scarring was obtained in ten of the 12 patients by D90. Incomplete regression was observed but with a marked improvement in the other two patients. Early hypertrophy scarring is distinguished by its clinical characteristics of hypertrophic or keloid scars. Biopsy performed in two cases showed the fibrous but non-fatty nature of early hypertrophy scarring. Biomechanical factors particular to the nasal region and the transposition flap technique could account for the early

  20. Heart rate variability analysis based on time–frequency representation and entropies in hypertrophic cardiomyopathy patients

    International Nuclear Information System (INIS)

    Clariá, F; Vallverdú, M; Caminal, P; Baranowski, R; Chojnowska, L

    2008-01-01

    In hypertrophic cardiomyopathy (HCM) patients there is an increased risk of premature death, which can occur with little or no warning. Furthermore, classification for sudden cardiac death on patients with HCM is very difficult. The aim of our study was to improve the prognostic value of heart rate variability (HRV) in HCM patients, giving insight into changes of the autonomic nervous system. In this way, the suitability of linear and nonlinear measures was studied to assess the HRV. These measures were based on time–frequency representation (TFR) and on Shannon and Rényi entropies, and compared with traditional HRV measures. Holter recordings of 64 patients with HCM and 55 healthy subjects were analyzed. The HCM patients consisted of two groups: 13 high risk patients, after aborted sudden cardiac death (SCD); 51 low risk patients, without SCD. Five-hour RR signals, corresponding to the sleep period of the subjects, were considered for the analysis as a comparable standard situation. These RR signals were filtered in the three frequency bands: very low frequency band (VLF, 0–0.04 Hz), low frequency band (LF, 0.04–0.15 Hz) and high frequency band (HF, 0.15–0.45 Hz). TFR variables based on instantaneous frequency and energy functions were able to classify HCM patients and healthy subjects (control group). Results revealed that measures obtained from TFR analysis of the HRV better classified the groups of subjects than traditional HRV parameters. However, results showed that nonlinear measures improved group classification. It was observed that entropies calculated in the HF band showed the highest statistically significant levels comparing the HCM group and the control group, p-value < 0.0005. The values of entropy measures calculated in the HCM group presented lower values, indicating a decreasing of complexity, than those calculated from the control group. Moreover, similar behavior was observed comparing high and low risk of premature death, the values of

  1. Significant regional heterogeneity of coronary flow reserve in paediatric hypertrophic cardiomyopathy

    International Nuclear Information System (INIS)

    Tadamura, E.; Kudoh, T.; Kubo, S.; Konishi, J.; Motooka, M.; Nohara, R.; Matsumori, A.; Sasayama, S.; Matsuda, T.; Tamaki, N.

    2000-01-01

    Previous studies have indicated that cardiac events in young patients with hypertrophic cardiomyopathy (HCM) are related to ischaemia rather than to arrhythmia. We measured coronary flow reserve in paediatric HCM and compared the values with those in adult HCM. We studied 12 patients with HCM including six paediatric ( 20 years old: mean 62 years), and six healthy young adults (mean 29 years) as controls. Every patient underwent magnetic resonance imaging (MRI) for anatomical assessment. Myocardial blood flow at rest and after dipyridamole infusion was measured with dynamic nitrogen-13 ammonia positron emission tomography (PET). Partial volume effect was corrected for using the anatomical data obtained with MRI. In adult patients with HCM, coronary flow reserve in the hypertrophied septal region was not significantly different from that in the non-hypertrophied lateral wall (1.38±0.29 vs 1.77±0.39, respectively). In the paediatric patients, coronary flow reserve in the hypertrophied septal region was significantly lower than in the non-hypertrophied lateral wall (0.84±0.33 vs 2.74±0.90, respectively, P<0.01). In addition, coronary flow reserve in adult patients was lower than in control subjects both in the septal wall (1.38±0.29 vs 2.94±0.35, respectively, P<0.0001) and in the lateral wall (1.77±0.39 vs 2.85±0.69, respectively, P<0.05). In contrast, coronary flow reserve in paediatric patients was not significantly different from that in control subjects in the lateral wall (2.74±0.90 vs 2.85±0.69, respectively), while absolute reduction of myocardial blood flow was noted after pharmacological vasodilatation in the hypertrophied septal region. In conclusion, significant regional differences of coronary flow reserve were present in the paediatric patients with HCM. These results suggest that paediatric patients with HCM intrinsically have the potential to experience significant regional ischaemia even in the absence of coronary stenosis. (orig.)

  2. MYBPC3 hypertrophic cardiomyopathy can be detected by using advanced ECG in children and young adults.

    Science.gov (United States)

    Fernlund, E; Liuba, P; Carlson, J; Platonov, P G; Schlegel, T T

    2016-01-01

    The conventional ECG is commonly used to screen for hypertrophic cardiomyopathy (HCM), but up to 25% of adults and possibly larger percentages of children with HCM have no distinctive abnormalities on the conventional ECG, whereas 5 to 15% of healthy young athletes do. Recently, a 5-min resting advanced 12-lead ECG test ("A-ECG score") showed superiority to pooled criteria from the strictly conventional ECG in correctly identifying adult HCM. The purpose of this study was to evaluate whether in children and young adults, A-ECG scoring could detect echocardiographic HCM associated with the MYBPC3 genetic mutation with greater sensitivity than conventional ECG criteria and distinguish healthy young controls and athletes from persons with MYBPC3 HCM with greater specificity. Five-minute 12-lead ECGs were obtained from 15 young patients (mean age 13.2years, range 0-30years) with MYBPC3 mutation and phenotypic HCM. The conventional and A-ECG results of these patients were compared to those of 198 healthy children and young adults (mean age 13.2, range 1month-30years) with unremarkable echocardiograms, and to those of 36 young endurance-trained athletes, 20 of whom had athletic (physiologic) left ventricular hypertrophy. Compared with commonly used, age-specific pooled criteria from the conventional ECG, a retrospectively generated A-ECG score incorporating results from just 2 derived vectorcardiographic parameters (spatial QRS-T angle and the change in the vectorcardiographic QRS azimuth angle from the second to the third eighth of the QRS interval) increased the sensitivity of ECG for identifying MYBPC3 HCM from 46% to 87% (pyoung endurance-trained athletes (100% vs. 69% for conventional ECG criteria, pyoung adults, a 2-parameter 12-lead A-ECG score is retrospectively significantly more sensitive and specific than pooled, age-specific conventional ECG criteria for detecting MYBPC3-HCM and in distinguishing such patients from healthy controls, including endurance

  3. The use of silicone occlusive sheeting (Sil-K) and silicone occlusive gel (epiderm) in the prevention of hypertrophic scar formation

    NARCIS (Netherlands)

    Niessen, FB; Spauwen, PHM; Robinson, PH; Fidler, [No Value; Kon, M

    The development of hypertrophic scars and keloids is an unsolved problem in the process of found healing. For this reason, a successful treatment to prevent excessive scar formation still has not been found. Over the last decade, however, a promising new treatment has been introduced. Silicone

  4. Ultra-structural changes and expression of chondrogenic and hypertrophic genes during chondrogenic differentiation of mesenchymal stromal cells in alginate beads

    Directory of Open Access Journals (Sweden)

    Havva Dashtdar

    2016-03-01

    Full Text Available Chondrogenic differentiation of mesenchymal stromal cells (MSCs in the form of pellet culture and encapsulation in alginate beads has been widely used as conventional model for in vitro chondrogenesis. However, comparative characterization between differentiation, hypertrophic markers, cell adhesion molecule and ultrastructural changes during alginate and pellet culture has not been described. Hence, the present study was conducted comparing MSCs cultured in pellet and alginate beads with monolayer culture. qPCR was performed to assess the expression of chondrogenic, hypertrophic, and cell adhesion molecule genes, whereas transmission electron microscopy (TEM was used to assess the ultrastructural changes. In addition, immunocytochemistry for Collagen type II and aggrecan and glycosaminoglycan (GAG analysis were performed. Our results indicate that pellet and alginate bead cultures were necessary for chondrogenic differentiation of MSC. It also indicates that cultures using alginate bead demonstrated significantly higher (p < 0.05 chondrogenic but lower hypertrophic (p < 0.05 gene expressions as compared with pellet cultures. N-cadherin and N-CAM1 expression were up-regulated in second and third weeks of culture and were comparable between the alginate bead and pellet culture groups, respectively. TEM images demonstrated ultrastructural changes resembling cell death in pellet cultures. Our results indicate that using alginate beads, MSCs express higher chondrogenic but lower hypertrophic gene expression. Enhanced production of extracellular matrix and cell adhesion molecules was also observed in this group. These findings suggest that alginate bead culture may serve as a superior chondrogenic model, whereas pellet culture is more appropriate as a hypertrophic model of chondrogenesis.

  5. Case Report: A case of hypertrophic lupus erythematosus with negative CD123 staining and absence of transepidermal elimination of elastin [v2; ref status: indexed, http://f1000r.es/3n7

    Directory of Open Access Journals (Sweden)

    Matthew Hughes

    2014-06-01

    Full Text Available We report the case of a 49-year-old male with clinical and histological findings consistent with hypertrophic lupus erythematosus (HLE. HLE must be clinically and histologically differentiated from keratoacanthoma, hypertrophic lichen planus, squamous cell carcinoma and plaque type psoriasis. CD123 positivity and transepidermal elimination of elastin have recently been reported as tools to distinguish HLE. Interestingly, in this case, biopsies of two separate lesions failed to reveal these two features. The etiology of this discrepancy is unknown and further studies are needed to clarify the utility of CD123 positivity and transepidermal elimination of elastin in the diagnosis of hypertrophic lupus erythematosus.

  6. Myosin-binding Protein C Compound Heterozygous Variant Effect on the Phenotypic Expression of Hypertrophic Cardiomyopathy.

    Science.gov (United States)

    Rafael, Julianny Freitas; Cruz, Fernando Eugênio Dos Santos; Carvalho, Antônio Carlos Campos de; Gottlieb, Ilan; Cazelli, José Guilherme; Siciliano, Ana Paula; Dias, Glauber Monteiro

    2017-04-01

    Hypertrophic cardiomyopathy (HCM) is an autosomal dominant genetic disease caused by mutations in genes encoding sarcomere proteins. It is the major cause of sudden cardiac death in young high-level athletes. Studies have demonstrated a poorer prognosis when associated with specific mutations. The association between HCM genotype and phenotype has been the subject of several studies since the discovery of the genetic nature of the disease. This study shows the effect of a MYBPC3 compound variant on the phenotypic HCM expression. A family in which a young man had a clinical diagnosis of HCM underwent clinical and genetic investigations. The coding regions of the MYH7, MYBPC3 and TNNT2 genes were sequenced and analyzed. The proband present a malignant manifestation of the disease, and is the only one to express HCM in his family. The genetic analysis through direct sequencing of the three main genes related to this disease identified a compound heterozygous variant (p.E542Q and p.D610H) in MYBPC3. A family analysis indicated that the p.E542Q and p.D610H alleles have paternal and maternal origin, respectively. No family member carrier of one of the variant alleles manifested clinical signs of HCM. We suggest that the MYBPC3-biallelic heterozygous expression of p.E542Q and p.D610H may cause the severe disease phenotype seen in the proband. Resumo A cardiomiopatia hipertrófica (CMH) é uma doença autossômica dominante causada por mutações em genes que codificam as proteínas dos sarcômeros. É a principal causa de morte súbita cardíaca em atletas jovens de alto nível. Estudos têm demonstrado um pior prognóstico associado a mutações específicas. A associação entre genótipo e fenótipo em CMH tem sido objeto de diversos estudos desde a descoberta da origem genética dessa doença. Este trabalho apresenta o efeito de uma mutação composta em MYBPC3 na expressão fenotípica da CMH. Uma família na qual um jovem tem o diagnóstico clínico de CMH foi

  7. Perioperative management of an elderly patient of hypertrophic obstructive cardiomyopathy for knee arthroplasty and the role of peripheral nerve blocks

    Directory of Open Access Journals (Sweden)

    Sunny Rupal

    2013-01-01

    Full Text Available This case report exemplifies how the anaesthetic technique of general anesthesia with continuous bilateral femoral nerve block for bilateral knee arthroplasty was well chosen for the management of perioperative complications in an elderly patient with hypertrophic obstructive cardiomyopathy (HOCM. A 69-year-old female patient of HOCM was scheduled for bilateral total knee replacement. Echocardiography revealed severe left ventricular outflow tract obstruction with peak systolic gradient of 56 mmHg. The surgery was conducted under general anaesthesia with invasive monitoring and bilateral continuous femoral nerve blocks for postoperative analgesia. Postoperatively, she developed pulmonary oedema due to the liberal administration of fluids. This complication was successfully managed without interrupting the management of pain. Management of patients with HOCM for noncardiac surgery requires knowledge of variable presentation of two forms of disease. Also, this case report highlights the practical advantage of continuous femoral nerve block (CFNBs over epidural anaesthesia.

  8. Genotype phenotype correlations of cardiac beta-myosin heavy chain mutations in Indian patients with hypertrophic and dilated cardiomyopathy

    DEFF Research Database (Denmark)

    Rai, Taranjit Singh; Ahmad, Shamim; Bahl, Ajay

    2009-01-01

    The aim of the current study was to determine the frequency of mutations in the beta-myosin heavy chain gene (MYH7) in a cohort of hypertrophic cardiomyopathy (HCM) and dilated cardiomyopathy (DCM) and their families, and to investigate correlations between genotype and phenotype. About 130...... consecutive patients diagnosed with HCM or DCM (69 with HCM and 61 with DCM) attending the cardiology clinic of Post Graduate Institute of Medical Education and Research were screened for mutations in the MYH7 gene. The control group for genetic studies consisted of 100 healthy subjects. We report 14...... mutations in 6 probands (5 probands in HCM and 1 proband in DCM) and their family members. Out of these 6 mutations, 3 are new and are being reported for the first time. One known mutation (p.Gly716Arg) was found to be "de novo" which resulted in severe asymmetric septal hypertrophy (31 mm) and resulted...

  9. Intraoperative Two- and Three-Dimensional Transesophageal Echocardiography in Combined Myectomy-Mitral Operations for Hypertrophic Cardiomyopathy.

    Science.gov (United States)

    Nampiaparampil, Robert G; Swistel, Daniel G; Schlame, Michael; Saric, Muhamed; Sherrid, Mark V

    2018-03-01

    Transesophageal echocardiography is essential in guiding the surgical approach for patients with obstructive hypertrophic cardiomyopathy. Septal hypertrophy, elongated mitral valve leaflets, and abnormalities of the subvalvular apparatus are prominent features, all of which may contribute to left ventricular outflow tract obstruction. Surgery aims to alleviate the obstruction via an extended myectomy, often with an intervention on the mitral valve and subvalvular apparatus. The goal of intraoperative echocardiography is to assess the anatomic pathology and pathophysiology in order to achieve a safe intraoperative course and a successful repair. This guide summarizes the systematic evaluation of these patients to determine the best surgical plan. Copyright © 2017 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.

  10. Recolonization of macrozoobenthos on defaunated sediments in a hypertrophic brackish lagoon: effects of sulfide removal and sediment grain size.

    Science.gov (United States)

    Kanaya, Gen

    2014-04-01

    Influences of sediment types on recolonization of estuarine macrozoobenthos were tested using enclosures in a hypertrophic lagoon. Three types of azoic sediment, sand (S), sulfide-rich mud (M), and mud removed of sulfide through iron addition (MFe), were set in field for 35 days during a hypoxic period. A total of 14 taxa including opportunistic polychaetes and amphipods occurred. Infaunal community in S treatment was characterized by highest diversity, total density and biomass, and population density of five dominant taxa, while those parameters were lowest in M treatment. Sulfide removal in MFe treatment achieved much higher density, biomass, and population densities of several taxa in the sediment. Multivariate analyses demonstrated that the established community structure was unique to each treatment. These imply that dissolved sulfide level as well as sediment grain size is a key determinant for the community composition and recolonization speed of early colonists in estuarine soft-bottom habitats. Copyright © 2014 Elsevier Ltd. All rights reserved.

  11. Imaging and histology in the diagnosis of multiple papillary fibroelastomas in a patient with hypertrophic obstructive cardiomyopathy. Case report.

    Science.gov (United States)

    Ionescu, Alin Alexandru; Radulescu, Bogdan; Herlea, Vlad; Miclea, Ioan; Parepa, Irinel; Bubenek, Serban; Popescu, Bogdan Alexandru; Ginghina, Carmen; Jurcut, Ruxandra

    2017-11-29

    Papillary fibroelastomas (PFEs) are one of the most frequent primary cardiac tumors and occur more often in patients with hypertrophic obstructive cardiomyopathy (HOCM). PFEs have been linked to an increased risk of neurological events. We report a case of a 59-year-old woman with HOCM in whom echocardiography (transthoracic and transesophageal, using 2D and 3D techniques) revealed multiple masses in various locations in the left cardiac chambers. Surgical excision of the cardiac tumors and aortic valve replacement was performed and the pathologic report confirmed the diagnosis of PFEs. Patient followup using ultrasonography is crucial since recurrence is a possibility. Current cardiac ultrasound techniques are essential for diagnosing and for guiding the management of these conditions.

  12. Effect of amiodarone-induced hyperthyroidism on left ventricular outflow obstruction after septal myectomy for hypertrophic cardiomyopathy.

    Science.gov (United States)

    Pokorney, Sean D; Stone, Neil J; Passman, Rod; Oyer, David; Rigolin, Vera H; Bonow, Robert O

    2010-12-01

    Patients with obstructive hypertrophic cardiomyopathy who undergo septal myectomy are at risk for developing postoperative atrial fibrillation. Amiodarone is effective in treating this arrhythmia but is associated with multiple adverse effects, often with delayed onset. A novel case is described of a patient who developed type 2 amiodarone-induced hyperthyroidism that presented as recurrence of outflow obstruction after septal myectomy. The patient's symptoms and echocardiographic findings of outflow obstruction resolved substantially with the treatment of the amiodarone-induced hyperthyroidism. Amiodarone-induced hyperthyroidism of delayed onset can be a subtle diagnosis, requiring a high index of suspicion. In conclusion, recognition of this diagnosis in patients with recurrence of outflow obstruction by symptoms and cardiac imaging after septal myectomy may avoid unnecessary repeat surgical intervention. Copyright © 2010 Elsevier Inc. All rights reserved.

  13. Human Engineered Cardiac Tissues Created Using Induced Pluripotent Stem Cells Reveal Functional Characteristics of BRAF-Mediated Hypertrophic Cardiomyopathy.

    Directory of Open Access Journals (Sweden)

    Timothy J Cashman

    Full Text Available Hypertrophic cardiomyopathy (HCM is a leading cause of sudden cardiac death that often goes undetected in the general population. HCM is also prevalent in patients with cardio-facio-cutaneous syndrome (CFCS, which is a genetic disorder characterized by aberrant signaling in the RAS/MAPK signaling cascade. Understanding the mechanisms of HCM development in such RASopathies may lead to novel therapeutic strategies, but relevant experimental models of the human condition are lacking. Therefore, the objective of this study was to develop the first 3D human engineered cardiac tissue (hECT model of HCM. The hECTs were created using human cardiomyocytes obtained by directed differentiation of induced pluripotent stem cells derived from a patient with CFCS due to an activating BRAF mutation. The mutant myocytes were directly conjugated at a 3:1 ratio with a stromal cell population to create a tissue of defined composition. Compared to healthy patient control hECTs, BRAF-hECTs displayed a hypertrophic phenotype by culture day 6, with significantly increased tissue size, twitch force, and atrial natriuretic peptide (ANP gene expression. Twitch characteristics reflected increased contraction and relaxation rates and shorter twitch duration in BRAF-hECTs, which also had a significantly higher maximum capture rate and lower excitation threshold during electrical pacing, consistent with a more arrhythmogenic substrate. By culture day 11, twitch force was no longer different between BRAF and wild-type hECTs, revealing a temporal aspect of disease modeling with tissue engineering. Principal component analysis identified diastolic force as a key factor that changed from day 6 to day 11, supported by a higher passive stiffness in day 11 BRAF-hECTs. In summary, human engineered cardiac tissues created from BRAF mutant cells recapitulated, for the first time, key aspects of the HCM phenotype, offering a new in vitro model for studying intrinsic mechanisms and

  14. 3D-printed transparent facemasks in the treatment of facial hypertrophic scars of young children with burns.

    Science.gov (United States)

    Wei, Yating; Li-Tsang, Cecilia W P; Liu, Jun; Xie, Lihua; Yue, Shukai

    2017-05-01

    Facial burns could create serious scar problems resulting disfigurement particularly on children. The conventional methods of producing transparent face masks for scar control remains complex and require dexterous skills of experienced clinician and patients' compliance during fitting. In this study, we adopted a portable 3D scanning and Computer-Aided Design (CAD) to produce 3D-printed transparent facemasks. Its efficacy was tested on two children with facial burns resulting hypertrophic scars. This study adopted a longitudinal case follow up research design. Two children with facial burns were recruited in the study upon consent. Their facial features were scanned with a portable 3D scanner and then edited and converted to the target files: the customized printable facemask files. The transparent facemask was directly printed out on the transparent biocompatible material followed by adding the medical grade silicone gel to provide extra pressure on the scar site. The facemasks were fitted to the patients with elastic straps connecting the printed anchoring bolts. Both children and family were instructed to wear the facemask for at least 20h per day and they were assessed before treatment, one month and three months after treatment on the facial scar conditions. At the one-month and three-month assessments after treatment, a decrease in average scar thickness was shown and the facial appearance was satisfactory. The 3D-printed facemasks were well fitted on both patients. The treatment was well-tolerated and no complication was reported. 3D-printed transparent facemask is convenient and efficient to fabricate, and is suitable for treating pediatric facial hypertrophic scars after burn. Copyright © 2016 Elsevier Ltd and ISBI. All rights reserved.

  15. Mitochondrial Dysfunctions Contribute to Hypertrophic Cardiomyopathy in Patient iPSC-Derived Cardiomyocytes with MT-RNR2 Mutation

    Directory of Open Access Journals (Sweden)

    Shishi Li

    2018-03-01

    Full Text Available Summary: Hypertrophic cardiomyopathy (HCM is the most common cause of sudden cardiac death in young individuals. A potential role of mtDNA mutations in HCM is known. However, the underlying molecular mechanisms linking mtDNA mutations to HCM remain poorly understood due to lack of cell and animal models. Here, we generated induced pluripotent stem cell-derived cardiomyocytes (HCM-iPSC-CMs from human patients in a maternally inherited HCM family who carry the m.2336T>C mutation in the mitochondrial 16S rRNA gene (MT-RNR2. The results showed that the m.2336T>C mutation resulted in mitochondrial dysfunctions and ultrastructure defects by decreasing the stability of 16S rRNA, which led to reduced levels of mitochondrial proteins. The ATP/ADP ratio and mitochondrial membrane potential were also reduced, thereby elevating the intracellular Ca2+ concentration, which was associated with numerous HCM-specific electrophysiological abnormalities. Our findings therefore provide an innovative insight into the pathogenesis of maternally inherited HCM. : In this article, Yan Q, Liu Z, Huang W, and colleagues show that patient-specific iPSCs as well as their derived cardiomyocytes carrying the m.2336T>C mutation in MT-RNR2 were generated to understand the pathogenic mechanism of maternally inherited HCM. MT-RNR2 mutation resulted in mitochondrial dysfunctions and ultrastructure defects, which induced abnormal Ca2+ homeostasis, then HCM-specific cellular and electrophysiological characteristics in iPSC-CMs. Keywords: mitochondrion, hypertrophic cardiomyopathy, induced pluripotent stem cells, MT-RNR2, maternal inheritance

  16. Clinical features and prognostic implications of familial hypertrophic cardiomyopathy related to the cardiac myosin-binding protein C gene.

    Science.gov (United States)

    Charron, P; Dubourg, O; Desnos, M; Bennaceur, M; Carrier, L; Camproux, A C; Isnard, R; Hagege, A; Langlard, J M; Bonne, G; Richard, P; Hainque, B; Bouhour, J B; Schwartz, K; Komajda, M

    1998-06-09

    Little information is available on phenotype-genotype correlations in familial hypertrophic cardiomyopathy that are related to the cardiac myosin binding protein C (MYBPC3) gene. The aim of this study was to perform this type of analysis. We studied 76 genetically affected subjects from nine families with seven recently identified mutations (SASint20, SDSint7, SDSint23, branch point int23, Glu542Gln, a deletion in exon 25, and a duplication/deletion in exon 33) in the MYBPC3 gene. Detailed clinical, ECG, and echocardiographic parameters were analyzed. An intergene analysis was performed by comparing the MYBPC3 group to seven mutations in the beta-myosin heavy-chain gene (beta-MHC) group (n=52). There was no significant phenotypic difference among the different mutations in the MYBPC3 gene. However, in the MYBPC3 group compared with the beta-MHC group, (1) prognosis was significantly better (P<0.0001), and no deaths occurred before the age of 40 years; (2) the age at onset of symptoms was delayed (41+/-19 versus 35+/-17 years, P<0.002); and (3) before 30 years of age, the phenotype was particularly mild because penetrance was low (41% versus 62%), maximal wall thicknesses lower (12+/-4 versus 16+/-7 mm, P<0.03), and abnormal T waves less frequent (9% versus 45%, P<0.02). These results are consistent with specific clinical features related to the MYBPC3 gene: onset of the disease appears delayed and the prognosis is better than that associated with the beta-MHC gene. These findings could be particularly important for the purpose of clinical management and genetic counseling in familial hypertrophic cardiomyopathy.

  17. Osteoartropatia hipertrófica primária (paquidermoperiostose: relato de casos em dois irmãos Primary hypertrophic osteoarthropathy (pachydermoperiostosis: case report of two sibling

    Directory of Open Access Journals (Sweden)

    Tarcísio Nunes Carvalho

    2004-04-01

    Full Text Available Neste artigo apresentamos dois irmãos acometidos pela osteoartropatia hipertrófica primária (paquidermoperiostose. Esta representa somente 3% a 5% de todos os casos de osteoartropatia hipertrófica. Nos nossos pacientes as alterações nos ossos longos são evidenciadas em radiografias convencionais, constando-se em neoformação óssea periosteal e alargamento das diáfises.We report a case of two siblings with primary hypertrophic osteoarthropathy (pachydermoperiostosis. Pachydermoperiostosis represents only 3 to 5 per cent of all cases of hypertrophic osteoarthropathy. These patients presented long bone changes on conventional x-ray films including periosteal new bone formation and expansion of the diaphysis.

  18. Successful anaesthetic management of a case of hypertrophic obstructive cardiomyopathy posted for elective caesarean section using epidural anaesthesia with 0.75% Ropivacaine

    Directory of Open Access Journals (Sweden)

    Anjali R Bhure

    2011-01-01

    Full Text Available Hypertrophic obstructive cardiomyopathy (HOCM is a complex cardiovascular disorder with autosomal dominant inheritance and an incidence of 0.1-0.5% in pregnant females. Anaesthetic management of a pregnant female with HOCM posted for elective caesarean section is a challenge, as even minor hemodynamic insults may lead to life-threatening complications. We report successful management of one such patient using epidural anaesthesia with 0.75% Ropivacaine.

  19. Canine hypertrophic osteopathy associated with extra-thoracic lesions Osteopatia hipertrófica canina associada com lesões extratorácicas

    OpenAIRE

    Selwyn Arlington Headley; Eduardo Alcântara Ribeiro; Gustavo José Von G. dos Santos; Carlos Maia Bettini; Ewaldo Mattos Júnior

    2005-01-01

    Canine hypertrophic osteopathy is described in a dog that presented extra-thoracic lesions, mainly in the liver. Hepatic lesions were characterized by necrosis, hemorrhage, severe hydropic degeneration of centrolobular hepatocytes, proliferation of epithelial cells of bile ducts, and mild biliary stasis. The disease syndrome was diagnosed based on clinical signs, radiological evaluation, and inspection of macerated bones.Osteopatia hipertrófica canina é descrita em um cão que apresentou lesõe...

  20. A New Animal Model for Investigation of Mechanical Unloading in Hypertrophic and Failing Hearts: Combination of Transverse Aortic Constriction and Heterotopic Heart Transplantation.

    Directory of Open Access Journals (Sweden)

    Andreas Schaefer

    Full Text Available Previous small animal models for simulation of mechanical unloading are solely performed in healthy or infarcted hearts, not representing the pathophysiology of hypertrophic and dilated hearts emerging in heart failure patients. In this article, we present a new and economic small animal model to investigate mechanical unloading in hypertrophic and failing hearts: the combination of transverse aortic constriction (TAC and heterotopic heart transplantation (hHTx in rats.To induce cardiac hypertrophy and failure in rat hearts, three-week old rats underwent TAC procedure. Three and six weeks after TAC, hHTx with hypertrophic and failing hearts in Lewis rats was performed to induce mechanical unloading. After 14 days of mechanical unloading animals were euthanatized and grafts were explanted for further investigations.50 TAC procedures were performed with a survival of 92% (46/50. When compared to healthy rats left ventricular surface decreased to 5.8±1.0 mm² (vs. 9.6± 2.4 mm² (p = 0.001 after three weeks with a fractional shortening (FS of 23.7± 4.3% vs. 28.2± 1.5% (p = 0.01. Six weeks later, systolic function decreased to 17.1± 3.2% vs. 28.2± 1.5% (p = 0.0001 and left ventricular inner surface increased to 19.9±1.1 mm² (p = 0.0001. Intraoperative graft survival during hHTx was 80% with 46 performed procedures (37/46. All transplanted organs survived two weeks of mechanical unloading.Combination of TAC and hHTx in rats offers an economic and reproducible small animal model enabling serial examination of mechanical unloading in a truly hypertrophic and failing heart, representing the typical pressure overloaded and dilated LV, occurring in patients with moderate to severe heart failure.

  1. Comparing Two Methods of Cryotherapy and Intense Pulsed Light with Triamcinolone Injection in the Treatment of Keloid and Hypertrophic Scars: A Clinical Trial

    OpenAIRE

    Meymandi, Simin Shamsi; Moosazadeh, Mahmood; Rezazadeh, Azadeh

    2016-01-01

    Objectives Keloid and hypertrophic scars are abnormal manifestations of wounds that occur following skin injuries in the form of local proliferation of fibroblasts and increased production of collagen. There are several ways to cure these scars; treatment must be selected based on the nature of the scars. In this clinical trial, two methods?cryotherapy and intense pulsed light (IPL)?are compared in the treatment of scars, and the results are presented in terms of improvement level, complicati...

  2. Myocardial Impairment Detected by Late Gadolinium Enhancement in Hypertrophic Cardiomyopathy: Comparison with 99mTc-MIBI/Tetrofosmin and 123I-BMIPP SPECT

    OpenAIRE

    Hashimura, Hiromi; Kiso, Keisuke; Yamada, Naoaki; Kono, Atsushi; Morita, Yoshiaki; Fukushima, Kazuto; Higashi, Masahiro; Noguchi Teruo; Ishibashi-Ueda, Hatsue; Naito, Hiroaki; Sugimura, Kazuro

    2013-01-01

    Purpose: Myocardial fibrosis is considered to be an important factor in myocardial dysfunction and sudden cardiac death in hypertrophic cardiomyopathy (HCM). The purpose of this study was to compare myocardial fibrosis detected by late gadolinium enhancement (LGE) on cardiac MRI with myocardial perfusion and fatty acid metabolism assessed by single photon emission computed tomography in HCM.Materials and Methods: We retrospectively evaluated 20 consecutive HCM patients (female, 7; mean age, 5...

  3. Microplasma radiofrequency technology combined with triamcinolone improved the therapeutic effect on Chinese patients with hypertrophic scar and reduced the risk of tissue atrophy.

    Science.gov (United States)

    Yu, Shui; Li, Hengjin

    2016-01-01

    The current study aimed to assess the value of microplasma radiofrequency technology combined with triamcinolone for the therapy of Chinese patients with hypertrophic scar. A total of 120 participants with hypertrophic scars were enrolled in the current study. Participants were divided into two groups based on sex, and then randomly and evenly divided into four groups (Groups A, B, C, and D). Participants in Group A received microplasma radiofrequency technology combined with triamcinolone. Participants in Group B received microplasma radiofrequency technology combined with normal saline. Participants in Groups C and D received triamcinolone (40 and 10 mg/mL) injected directly into scar. Experienced physicians evaluated the condition of scars according to the Vancouver Scar Scale 1 month before and after the therapy. There was no difference in age, sex, area, height and location of scars, and Vancouver Scar Scale scores before the therapy between any groups (P>0.05 for all). Vancouver Scar Scale scores after the therapy were significantly lower than those before the therapy in all groups (P0.05 for all). Incidences of tissue atrophy after the therapy were significantly lower in Groups A and B than in Group C (P0.05 for all). Microplasma radiofrequency technology combined with triamcinolone improved the therapeutic effect on Chinese patients with hypertrophic scar and reduced the risk of tissue atrophy compared with the use of either microplasma radiofrequency technology or triamcinolone injection alone.

  4. Norepinephrine-induced apoptotic and hypertrophic responses in H9c2 cardiac myoblasts are characterized by different repertoire of reactive oxygen species generation

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    Anita Thakur

    2015-08-01

    Full Text Available Despite recent advances, the role of ROS in mediating hypertrophic and apoptotic responses in cardiac myocytes elicited by norepinephrine (NE is rather poorly understood. We demonstrate through our experiments that H9c2 cardiac myoblasts treated with 2 µM NE (hypertrophic dose generate DCFH-DA positive ROS only for 2 h; while those treated with 100 µM NE (apoptotic dose sustains generation for 48 h, followed by apoptosis. Though the levels of DCFH fluorescence were comparable at early time points in the two treatment sets, its quenching by DPI, catalase and MnTmPyP suggested the existence of a different repertoire of ROS. Both doses of NE also induced moderate levels of H2O2 but with different kinetics. Sustained but intermittent generation of highly reactive species detectable by HPF was seen in both treatment sets but no peroxynitrite was generated in either conditions. Sustained generation of hydroxyl radicals with no appreciable differences were noticed in both treatment sets. Nevertheless, despite similar profile of ROS generation between the two conditions, extensive DNA damage as evident from the increase in 8-OH-dG content, formation of γ-H2AX and PARP cleavage was seen only in cells treated with the higher dose of NE. We therefore conclude that hypertrophic and apoptotic doses of NE generate distinct but comparable repertoire of ROS/RNS leading to two very distinct downstream responses.

  5. Role of quantitative myocardial positron emission tomography for risk stratification in patients with hypertrophic cardiomyopathy: a 2016 reappraisal

    Energy Technology Data Exchange (ETDEWEB)

    Castagnoli, Helga; Passeri, Alessandro; Berti, Valentina; Sciagra, Roberto [University of Florence, Department of Experimental and Clinical Biomedical Sciences - Nuclear Medicine Unit, Firenze (Italy); Ferrantini, Cecilia; Coppini, Raffaele; Baldini, Katia; Cecchi, Franco; Olivotto, Iacopo [Careggi University Hospital, Referral Center for Myocardial Diseases and Genetic Diagnostics Unit, Florence (Italy)

    2016-12-15

    Myocardial blood flow <1.1 mL/min/g following dipyridamole (Dip-MBF) assessed by positron emission tomography (PET) was identified in 2003 as an important outcome predictor in hypertrophic cardiomyopathy (HCM), based on scans performed in the 90s. However, such extreme Dip-MBF impairment is rarely observed in contemporary cohorts. We, therefore, reassessed the Dip-MBF threshold defining high-risk HCM patients. Dip-MBF was measured using {sup 13}N-ammonia in 100 HCM consecutive patients, prospectively enrolled and followed for 4.0 ± 2.2 years. Outcome was assessed based on tertiles of Dip-MBF. The study end-point was a combination of cardiovascular death, progression to severe functional limitation, cardioembolic stroke, life-threatening ventricular arrhythmias. Global Dip-MBF was 1.95 ± 0.85, ranging from 0.7 to 5.9 mL/min/g. Dip-MBF tertile cut-off values were: 0.73 to 1.53 mL/min/g (lowest), 1.54 to 2.13 mL/min/g (middle), and 2.14 to 5.89 mL/min/g (highest). During follow-up, lowest tertile Dip-MBF was associated with sevenfold independent risk of unfavorable outcome compared to the other two tertiles. Dip-MBF 1.35 mL/min/g was identified as the best threshold for outcome prediction. Regional perfusion analysis showed that all cardiac deaths (n = 4) occurred in patients in the lowest tertile of lateral wall Dip-MBF (≤1.72 mL/min/g); septal Dip-MBF was not predictive. Dip-MBF confirms its role as potent predictor of outcome in HCM. However, the threshold for prediction in a contemporary cohort is higher than that reported in earlier studies. Dip-MBF impairment in the lateral wall, possibly reflecting diffuse disease extending to non-hypertrophic regions, is a sensitive predictor of mortality in HCM. (orig.)

  6. Efficacy and Safety of Fractional CO2 Laser Resurfacing in Non-hypertrophic Traumatic and Burn Scars

    Science.gov (United States)

    Majid, Imran; Imran, Saher

    2015-01-01

    Background: Fractional photothermolysis is one of the most effective treatment options used to resurface scars of different aetiologies. Aim: To assess the efficacy and safety of fractional CO2 laser resurfacing treatment in the management of non-hypertrophic traumatic and burn scars. Materials and Methods: Twenty-five patients affected by non-hypertrophic traumatic and burn scars were treated with four sessions of fractional CO2 laser resurfacing treatment at 6-weekly intervals. Patients were photographed at each visit and finally, 3 months after the end of treatment schedule. Response to treatment was assessed clinically as well as by comparing the initial photograph of the patient with the one taken at the last follow-up visit 3-months after the final treatment session. Changes in skin texture, surface irregularity and pigmentation were assessed on a quartile grading scale and scored individually from 0 to 4. A mean of the three individual scores was calculated and the response was labelled as ‘excellent’ if the mean score achieved was >2. A score of 1-2 was labeled as good response while a score below 1 was labeled as ‘poor’ response. The subjective satisfaction of each patient with the treatment offered was also assessed at the last follow-up visit. Results: The commonest site of scarring treated was the face followed by hands. Response to treatment was rated as excellent in 60% (15/25) patients while 24% (6/25) and 16% (4/25) patients were labeled as good and poor responders, respectively. Skin texture showed better response than other variables with average score of 2.44. Linear post-traumatic scars were seen to respond less than other morphological types. Majority of the patients (19 out of 25) were highly satisfied with the treatment offered. No long-term adverse effects were noted in any patient. Conclusions: Fractional photothermolysis with a fractional CO2 laser gives excellent results in patients with post-burn scars with minimal adverse

  7. Myocardial energy metabolism during global ischemia and reperfusion in SHR hypertrophic rat heart assessed by 31P-NMR

    International Nuclear Information System (INIS)

    Shirotani, Hitoshi; Oka, Hiroshi; Katayama, Osamu; Nishioka, Takazumi; Oku, Hidetaka

    1983-01-01

    An experiment regarding myocardial ischemia and reperfusion was performed under various conditions in SHR hypertrophic and WKY non-hypertrophic rat hearts. An effect of cardioplegia was evaluated in the following 4 conditions, that is, Group 1: hypothermia only, Group 2: hypothermia with intermittent infusion of GIK solution, Group 3: hypothermia with intermittent infusion of cold blood cardioplegia, Group 4: hypothermia with intermittent infusion of cold blood cardioplegia and administration of coenzyme Q 10 prior to isolation of the heart. 1) In WKY heart, ATP contents after 90 minutes myocardial ischemia at 15 0 C decreased to 25% in Group 1,42% in Group 2,52% in Group 3 and 62% in Group 4, and the contents after 30 minutes reperfusion increased to 42, 50, 60 and 75%, respectively. On the other hand, in SHR heart, ATP contents decreased to 22, 38, 40 and 41% but no trend of recovery was present. 2) Creatine phosphate content in SHR heart was 50% of that in WKY heart during isolated perfusion. Creatine phosphate decreased to zero after 30 minutes myocardial ischemia. In WKY heart, the content was recovered to over 100% by 30 minutes reperfusion after 90 minutes myocardial ischemia in all groups. On the contrary, in SHR heart, the contents increased to only 10, 15, 22 and 41%, in 4 groups, respectively. 3) In WKY heart, pH fell to 6.2, 6.7, 6.8 and 6.8, in 4 groups, respectively, a fter 90 minutes myocardial ischemia, and returned to the preischemic value of 7.2 after 30 minutes reperfusion in all groups. In SHR heart, pH fell to 6.1 in group 1, 6.3 in group 2, 6.4 in group 3 and 6.7 in group 4 after 90 minutes myocardial ischemia and the values returned to 6.5, 6.6, 6.7 and 6.8, respectively, after 30 minutes reperfusion. The latter values were lower than preischemic value of 7.0. (J.P.N.)

  8. Myocardial energy metabolism during global ischemia and reperfusion in SHR hypertrophic rat heart assessed by /sup 31/P-NMR

    Energy Technology Data Exchange (ETDEWEB)

    Shirotani, Hitoshi; Oka, Hiroshi; Katayama, Osamu; Nishioka, Takazumi; Oku, Hidetaka [Kinki Univ., Higashi-Osaka, Osaka (Japan)

    1983-12-01

    An experiment regarding myocardial ischemia and reperfusion was performed under various conditions in SHR hypertrophic and WKY non-hypertrophic rat hearts. An effect of cardioplegia was evaluated in the following 4 conditions, that is, Group 1: hypothermia only, Group 2: hypothermia with intermittent infusion of GIK solution, Group 3: hypothermia with intermittent infusion of cold blood cardioplegia, Group 4: hypothermia with intermittent infusion of cold blood cardioplegia and administration of coenzyme Q/sub 10/ prior to isolation of the heart. 1) In WKY heart, ATP contents after 90 minutes myocardial ischemia at 15/sup 0/ C decreased to 25% in Group 1,42% in Group 2,52% in Group 3 and 62% in Group 4, and the contents after 30 minutes reperfusion increased to 42, 50, 60 and 75%, respectively. On the other hand, in SHR heart, ATP contents decreased to 22, 38, 40 and 41% but no trend of recovery was present. 2) Creatine phosphate content in SHR heart was 50% of that in WKY heart during isolated perfusion. Creatine phosphate decreased to zero after 30 minutes myocardial ischemia. In WKY heart, the content was recovered to over 100% by 30 minutes reperfusion after 90 minutes myocardial ischemia in all groups. On the contrary, in SHR heart, the contents increased to only 10, 15, 22 and 41%, in 4 groups, respectively. 3) In WKY heart, pH fell to 6.2, 6.7, 6.8 and 6.8, in 4 groups, respectively, a fter 90 minutes myocardial ischemia, and returned to the preischemic value of 7.2 after 30 minutes reperfusion in all groups. In SHR heart, pH fell to 6.1 in group 1, 6.3 in group 2, 6.4 in group 3 and 6.7 in group 4 after 90 minutes myocardial ischemia and the values returned to 6.5, 6.6, 6.7 and 6.8, respectively, after 30 minutes reperfusion. The latter values were lower than preischemic value of 7.0.

  9. Role of quantitative myocardial positron emission tomography for risk stratification in patients with hypertrophic cardiomyopathy: a 2016 reappraisal

    International Nuclear Information System (INIS)

    Castagnoli, Helga; Passeri, Alessandro; Berti, Valentina; Sciagra, Roberto; Ferrantini, Cecilia; Coppini, Raffaele; Baldini, Katia; Cecchi, Franco; Olivotto, Iacopo

    2016-01-01

    Myocardial blood flow <1.1 mL/min/g following dipyridamole (Dip-MBF) assessed by positron emission tomography (PET) was identified in 2003 as an important outcome predictor in hypertrophic cardiomyopathy (HCM), based on scans performed in the 90s. However, such extreme Dip-MBF impairment is rarely observed in contemporary cohorts. We, therefore, reassessed the Dip-MBF threshold defining high-risk HCM patients. Dip-MBF was measured using "1"3N-ammonia in 100 HCM consecutive patients, prospectively enrolled and followed for 4.0 ± 2.2 years. Outcome was assessed based on tertiles of Dip-MBF. The study end-point was a combination of cardiovascular death, progression to severe functional limitation, cardioembolic stroke, life-threatening ventricular arrhythmias. Global Dip-MBF was 1.95 ± 0.85, ranging from 0.7 to 5.9 mL/min/g. Dip-MBF tertile cut-off values were: 0.73 to 1.53 mL/min/g (lowest), 1.54 to 2.13 mL/min/g (middle), and 2.14 to 5.89 mL/min/g (highest). During follow-up, lowest tertile Dip-MBF was associated with sevenfold independent risk of unfavorable outcome compared to the other two tertiles. Dip-MBF 1.35 mL/min/g was identified as the best threshold for outcome prediction. Regional perfusion analysis showed that all cardiac deaths (n = 4) occurred in patients in the lowest tertile of lateral wall Dip-MBF (≤1.72 mL/min/g); septal Dip-MBF was not predictive. Dip-MBF confirms its role as potent predictor of outcome in HCM. However, the threshold for prediction in a contemporary cohort is higher than that reported in earlier studies. Dip-MBF impairment in the lateral wall, possibly reflecting diffuse disease extending to non-hypertrophic regions, is a sensitive predictor of mortality in HCM. (orig.)

  10. Myocardial scarring on cardiovascular magnetic resonance in asymptomatic or minimally symptomatic patients with “pure” apical hypertrophic cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Kim Kyung-Hee

    2012-07-01

    Full Text Available Abstract Background Late gadolinium enhancement (LGE cardiovascular magnetic resonance (CMR enables state-of-the-art in vivo evaluations of myocardial fibrosis. Although LGE patterns have been well described in asymmetrical septal hypertrophy, conflicting results have been reported regarding the characteristics of LGE in apical hypertrophic cardiomyopathy (ApHCM. This study was undertaken to determine 1 the frequency and distribution of LGE and 2 its prognostic implication in ApHCM. Methods Forty patients with asymptomatic or minimally symptomatic pure ApHCM (age, 60.2 ± 10.4 years, 31 men were prospectively enrolled. LGE images were acquired using the inversion recovery segmented spoiled-gradient echo and phase-sensitive inversion recovery sequence, and analyzed using a 17-segment model. Summing the planimetered LGE areas in all short axis slices yielded the total volume of late enhancement, which was subsequently presented as a proportion of total LV myocardium (% LGE. Results Mean maximal apical wall thickness was 17.9±2.3mm, and mean left ventricular (LV ejection fraction was 67.7 ± 8.0%. All but one patient presented with electrocardiographic negative T wave inversion in anterolateral leads, with a mean maximum negative T wave of 7.2 ± 4.7mm. Nine patients (22.5% had giant negative T waves, defined as the amplitude of ≥10mm, in electrocardiogram. LGE was detected in 130 segments of 30 patients (75.0%, occupying 4.9 ± 5.5% of LV myocardium. LGE was mainly detected at the junction between left and right ventricles in 12 (30% and at the apex in 28 (70%, although LGE-positive areas were widely distributed, and not limited to the apex. Focal LGE at the non-hypertrophic LV segments was found in some ApHCM patients, even without LGE of hypertrophied apical segments. Over the 2-year follow-up, there was no one achieving the study end-point, defined as all-cause death, sudden cardiac death and hospitalization for heart failure

  11. Efficacy and safety of fractional CO 2 laser resurfacing in non-hypertrophic traumatic and burn scars

    Directory of Open Access Journals (Sweden)

    Imran Majid

    2015-01-01

    Full Text Available Background: Fractional photothermolysis is one of the most effective treatment options used to resurface scars of different aetiologies. Aim: To assess the efficacy and safety of fractional CO 2 laser resurfacing treatment in the management of non-hypertrophic traumatic and burn scars. Materials and Methods: Twenty-five patients affected by non-hypertrophic traumatic and burn scars were treated with four sessions of fractional CO 2 laser resurfacing treatment at 6-weekly intervals. Patients were photographed at each visit and finally, 3 months after the end of treatment schedule. Response to treatment was assessed clinically as well as by comparing the initial photograph of the patient with the one taken at the last follow-up visit 3-months after the final treatment session. Changes in skin texture, surface irregularity and pigmentation were assessed on a quartile grading scale and scored individually from 0 to 4. A mean of the three individual scores was calculated and the response was labelled as ′excellent′ if the mean score achieved was >2. A score of 1-2 was labeled as good response while a score below 1 was labeled as ′poor′ response. The subjective satisfaction of each patient with the treatment offered was also assessed at the last follow-up visit. Results: The commonest site of scarring treated was the face followed by hands. Response to treatment was rated as excellent in 60% (15/25 patients while 24% (6/25 and 16% (4/25 patients were labeled as good and poor responders, respectively. Skin texture showed better response than other variables with average score of 2.44. Linear post-traumatic scars were seen to respond less than other morphological types. Majority of the patients (19 out of 25 were highly satisfied with the treatment offered. No long-term adverse effects were noted in any patient. Conclusions: Fractional photothermolysis with a fractional CO 2 laser gives excellent results in patients with post-burn scars with

  12. Compensatory role of the NBCn1 sodium/bicarbonate cotransporter on Ca2+-induced mitochondrial swelling in hypertrophic hearts.

    Science.gov (United States)

    Vargas, Lorena A; Velasquez, Fernanda Carrizo; Alvarez, Bernardo V

    2017-03-01

    NBC Na + /HCO 3 - cotransporter (NBCn1) and NHE1 Na + /H + exchanger have been associated with cardiac disorders and recently located in coronary endothelial cells (CEC) and cardiomyocytes mitochondria, respectively. Mitochondrial NHE1 blockade delays permeability transition pore (MPTP) opening and reduces superoxide levels, two critical events exacerbated in cells of diseased hearts. Conversely, activation of NBCn1 prevented apoptosis in CEC subjected to ischemic stress. We characterized the role of the NHE1 and NBCn1 transporters in heart mitochondria from hypertrophic (SHR) and control (Wistar) rats. Expression of NHE1 was analyzed in left ventricular mitochondrial lysates (LVML), by immunoblots. NHE1 expression increased by ~40% in SHR compared to control (P < 0.05, n = 4). To examine NHE1-mediated Na + /H + exchange activity in cardiac hypertrophy, mitochondria were loaded with BCECF-AM dye and the maximal rate of pHm change measured after the addition of 50 mM NaCl. SHR mitochondria had greater changes in pHm compared to Wistar, 0.10 ± 0.01 vs. 0.06 ± 0.01, respectively (P < 0.05, n = 5). In addition, mitochondrial suspensions from SHR and control myocardium were exposed to 200 μM CaCl 2 to induce MPTP opening (light-scattering decrease, LSD) and swelling. Surprisingly, SHR rats showed smaller LSD and a reduction in mitochondrial swelling, 67 ± 10% (n = 15), compared to control, 100 ± 8% (n = 13). NBC inhibition with S0859 (1 μM) significantly increased swelling in both control 139 ± 10% (n = 8) and SHR 115 ± 10% (n = 4). Finally, NBCn1 Na + /HCO 3 - cotransporter increased by twofold its expression in SHR LVML, compared to normal (P < 0.05, n = 5). We conclude that increased NBCn1 activity may play a compensatory role in hypertrophic hearts, protecting mitochondria from Ca 2+ -induced MPTP opening and swelling.

  13. Spatial and temporal variations in bacterial macromolecule labeling with [methyl-3H]thymidine in a hypertrophic lake

    International Nuclear Information System (INIS)

    Robarts, R.D.; Wicks, R.J.; Sephton, L.M.

    1986-01-01

    The incorporation of [methyl- 3 H]thymidine into three macromolecular fractions, designated as DNA, RNA, and protein, by bacteria from Hartbeespoort Dam, South Africa, was measured over 1 year by acid-base hydrolysis procedures. Samples were collected at 10 m, which was at least 5 m beneath the euphotic zone. On four occasions, samples were concurrently collected at the surface. Approximately 80% of the label was incorporated into bacterial DNA in surface samples. At 10 m, total incorporation of label into bacterial macromolecules was correlated to bacterial utilization of glucose. The labeling of DNA, which ranged between 0 and 78% of total macromolecule incorporation, was inversely related to glucose uptake, total thymidine incorporation, and euphotic zone algal production. With decreased DNA labeling, increasing proportions of label were found in the RNA fraction and proteins. Enzymatic digestion followed by chromatographic separation of macromolecule fragments indicated that DNA and proteins were labeled while RNA was not. The RNA fraction may represent labeled lipids or other macromolecules or both. The data demonstrated a close coupling between phytoplankton production and heterotrophic bacterial activity in this hypertrophic lake but also confirmed the need for the routine extraction and purification of DNA during [methyl- 3 H]thymidine studies of aquatic bacterial production

  14. A Pregnancy with Severe Hypertrophic Obstructive Cardiomyopathy after Surgery for an Implantable Cardioverter Defibrillator: A Case Report and Literature Review

    Directory of Open Access Journals (Sweden)

    Takashi Mitsui

    2016-01-01

    Full Text Available Hypertrophic obstructive cardiomyopathy (HOCM is cardiac hypertrophy of ventricular myocardium with left ventricular outflow tract obstruction. We report a pregnancy with HOCM after defibrillator implantation surgery. The patient was a 33-year-old nulligravida and was categorized as New York Heart Association class II. Her brain natriuretic peptide (BNP level was 724.6 pg/dL at preconception. She received careful pregnancy management. However, because frequent uterine contractions were observed at 25 weeks and 6 days of pregnancy, she was hospitalized, and magnesium sulfate was started as a tocolytic agent. At 27 weeks and 5 days of pregnancy, she had respiratory discomfort and orthopnea with a sudden decrease in peripheral oxygen saturation. Cardiac ultrasonography showed a worsened condition of HOCM and her BNP level was 1418.0 pg/mL. We performed an emergent cesarean section and she delivered a boy weighing 999 g. The Apgar score was 8 and 9 points at 1 and 5 minutes, respectively. The mother’s heart failure quickly improved after birth and she was discharged at 10 days postoperatively. Fluctuations in circulatory dynamics during pregnancy may sometimes exacerbate heart disease. Therefore, the risks should be fully explained and careful assessment of cardiac function should be performed during pregnancy in patients with severe HOCM.

  15. A mutation in the β-myosin rod associated with hypertrophic cardiomyopathy has an unexpected molecular phenotype

    International Nuclear Information System (INIS)

    Armel, Thomas Z.; Leinwand, Leslie A.

    2010-01-01

    Hypertrophic cardiomyopathy (HCM) is a common, autosomal dominant disorder primarily characterized by left ventricular hypertrophy and is the leading cause of sudden cardiac death in youth. HCM is caused by mutations in several sarcomeric proteins, with mutations in MYH7, encoding β-MyHC, being the most common. While many mutations in the globular head region of the protein have been reported and studied, analysis of HCM-causing mutations in the β-MyHC rod domain has not yet been reported. To address this question, we performed an array of biochemical and biophysical assays to determine how the HCM-causing E1356K mutation affects the structure, stability, and function of the β-MyHC rod. Surprisingly, the E1356K mutation appears to thermodynamically destabilize the protein, rather than alter the charge profile know to be essential for muscle filament assembly. This thermodynamic instability appears to be responsible for the decreased ability of the protein to form filaments and may be responsible for the HCM phenotype seen in patients.

  16. A mutation in the {beta}-myosin rod associated with hypertrophic cardiomyopathy has an unexpected molecular phenotype

    Energy Technology Data Exchange (ETDEWEB)

    Armel, Thomas Z. [Department of Molecular, Cellular, and Developmental Biology, University of Colorado, Boulder, CO 80309 (United States); Leinwand, Leslie A., E-mail: leslie.leinwand@colorado.edu [Department of Molecular, Cellular, and Developmental Biology, University of Colorado, Boulder, CO 80309 (United States)

    2010-01-01

    Hypertrophic cardiomyopathy (HCM) is a common, autosomal dominant disorder primarily characterized by left ventricular hypertrophy and is the leading cause of sudden cardiac death in youth. HCM is caused by mutations in several sarcomeric proteins, with mutations in MYH7, encoding {beta}-MyHC, being the most common. While many mutations in the globular head region of the protein have been reported and studied, analysis of HCM-causing mutations in the {beta}-MyHC rod domain has not yet been reported. To address this question, we performed an array of biochemical and biophysical assays to determine how the HCM-causing E1356K mutation affects the structure, stability, and function of the {beta}-MyHC rod. Surprisingly, the E1356K mutation appears to thermodynamically destabilize the protein, rather than alter the charge profile know to be essential for muscle filament assembly. This thermodynamic instability appears to be responsible for the decreased ability of the protein to form filaments and may be responsible for the HCM phenotype seen in patients.

  17. Myocardial glucose metabolism is different between hypertrophic cardiomyopathy and hypertensive heart disease associated with asymmetrical septal hypertrophy

    International Nuclear Information System (INIS)

    Shiba, Nobuyuki; Kagaya, Yutaka; Ishide, Nobumasa; Takeyama, Daiya; Yamane, Yuriko; Chida, Masanobu; Otani, Hiroki; Shirato, Kunio; Ido, Tatsuo.

    1997-01-01

    Myocardial glucose metabolism has been shown to be heterogeneous in patients with hypertrophic cardiomyopathy (HCM). We tested the hypothesis that myocardial glucose metabolism differs between patients with HCM and those with hypertensive heart disease (HHD) associated with asymmetrical septal hypertrophy. We studied 12 patients with HCM, 7 HHD patients associated with asymmetrical septal hypertrophy using 18 F 2-deoxyglucose (FDG) and positron emission tomography. We calculated % FDG fractional uptake in the interventricular septum and posterolateral wall. Heterogeneity of FDG uptake was evaluated by % interregional coefficient of variation of FDG fractional uptake in each wall segment. In both the interventricular septum and posterolateral wall, % FDG fractional uptake was not significantly different between the two groups. The % interregional coefficient of variation for both interventricular septum (10.6±1.6 vs. 4.1±0.5, p<0.01) and posterolateral wall (5.9±0.7 vs. 3.8±0.5, p< 0.05) was significantly larger in patients with HCM than in HHD patients associated with asymmetrical septal hypertrophy. Echocardiography demonstrated that the degree of asymmetrical septal hypertrophy was similar between the two groups. These results suggest that myocardial glucose metabolism may be more heterogeneous in patients with HCM compared to HHD patients associated with asymmetrical septal hypertrophy, although the left ventricular shape is similar. The difference in the heterogeneity might have resulted from differences in the pathogeneses of the two diseases. (author)

  18. Correlation between myocardial fibrosis and the occurrence of atrial fibrillation in hypertrophic cardiomyopathy: A cardiac magnetic resonance imaging study

    Energy Technology Data Exchange (ETDEWEB)

    Pujadas, S., E-mail: sandrapujadas@gmail.co [Cardiac Imaging Unit, Cardiology Department, Hospital de la Santa Creu i Sant Pau, Av. Pare M Claret 167, 08025 Barcelona (Spain); Vidal-Perez, R. [Cardiac Imaging Unit, Cardiology Department, Hospital de la Santa Creu i Sant Pau, Av. Pare M Claret 167, 08025 Barcelona (Spain); Hidalgo, A. [Radiology Department, Hospital de la Santa Creu i Sant Pau, Av. Pare M Claret 167, 08025 Barcelona (Spain); Leta, R.; Carreras, F.; Barros, A. [Cardiac Imaging Unit, Cardiology Department, Hospital de la Santa Creu i Sant Pau, Av. Pare M Claret 167, 08025 Barcelona (Spain); Bayes-Genis, A. [Cardiomyopathy and Cardiac Transplant Unit, Cardiology Department, Hospital de la Santa Creu i Sant Pau, Av. Pare M Claret 167, 08025 Barcelona (Spain); Subirana, M.T. [Congenital Heart Disease Unit, Hospital de la Santa Creu i Sant Pau, Av. Pare M Claret 167, 08025 Barcelona (Spain); Pons-Llado, Guillem [Cardiac Imaging Unit, Cardiology Department, Hospital de la Santa Creu i Sant Pau, Av. Pare M Claret 167, 08025 Barcelona (Spain)

    2010-08-15

    Cardiac magnetic resonance imaging (CMR) in hypertrophic cardiomyopathy (HCM) often shows delayed contrast enhancement (DE) representing regions of focal myocardial fibrosis. Atrial fibrillation (AF) is a commonly reported complication of HCM. We determined the relationship between the presence of left ventricular myocardial fibrosis (LVMF) detected by DE-CMR and the occurrence AF in a series of patients with HCM. 67 patients with HCM (47 males; mean age 50.1 {+-} 18.5 years) were studied by CMR measuring mass of LVMF, left ventricular mass, volume and function, and left atrial (LA) area. AF was present in 17 (25%) patients. LVMF was observed in 57% of patients. AF was significantly more frequent in patients who also showed LVMF, compared with the group without LVMF (42.1% vs. 3.4%, respectively; p < 0.0001). LA size was larger in patients showing DE (LA area: 37.4 {+-} 11.1 vs. 25.9 {+-} 6.8 cm{sup 2}; respectively, p = 0.0001). AF in HCM is related with myocardial fibrosis detected by DE-CMR and dilatation of the LA. This fact adds to the proven adverse prognostic value of myocardial fibrosis in HCM, thus, reinforcing the usefulness of this technique in the assessment of these patients.

  19. Assessment of pathophysiology based on the left ventricular shape in five patients with midventricular obstructive hypertrophic cardiomyopathy

    International Nuclear Information System (INIS)

    Aoki, Mayumi; Uekita, Kazumi; Obata, Hiromi; Makiguchi, Noriko; Mitsuoka, Takao; Kikuchi, Kenjiro

    2007-01-01

    The pathophysiology of midventricular obstructive hypertrophic cardiomyopathy (MVO) is unknown. Patients with MVO and MVO-like cardiomyopathy were classified into three groups based on the cardioimaging morphological characteristics of the left ventricle to investigate their complications and treatment. Four patients with MVO and one patient with disease-like MVO were admitted in our hospital from 1999 to 2005. Group A consisted of one patient with indications of pressure gradient at mid-ventricle without apical aneurysm, Group B consisted of three patients with indications of pressure gradient and apical aneurysm, and Group C consisted of one patient with hour-glass appearance with apical aneurysm and decreased left ventricular systolic function without pressure gradient. The diagnosis was established during examination for sustained ventricular tachycardia (SVT, three patients), paroxysmal atrial fibrillation (one patient), and coronary artery disease (one patient). Cardiogenic embolization was observed in all cases which originated from atrial fibrillation (one case) and apical aneurysm (two cases). No embolic event occurred in any patient after warfarin therapy. SVT occurred in patients in Groups B and C. SVT refractory to beta-blocker and mexiletine was treated by amiodarone. Apical aneurysmectomy and cryoablation could prevent recurrent SVT with drug resistance. Four of the five patients with MVO had arrhythmia (atrial fibrillation, SVT) and three had cardiogenic embolization. MVO could be classified into three groups depending on the morphological characteristics and complications. Treatment of MVO should be based on these characteristics. (author)

  20. Correlation between myocardial fibrosis and the occurrence of atrial fibrillation in hypertrophic cardiomyopathy: A cardiac magnetic resonance imaging study

    International Nuclear Information System (INIS)

    Pujadas, S.; Vidal-Perez, R.; Hidalgo, A.; Leta, R.; Carreras, F.; Barros, A.; Bayes-Genis, A.; Subirana, M.T.; Pons-Llado, Guillem

    2010-01-01

    Cardiac magnetic resonance imaging (CMR) in hypertrophic cardiomyopathy (HCM) often shows delayed contrast enhancement (DE) representing regions of focal myocardial fibrosis. Atrial fibrillation (AF) is a commonly reported complication of HCM. We determined the relationship between the presence of left ventricular myocardial fibrosis (LVMF) detected by DE-CMR and the occurrence AF in a series of patients with HCM. 67 patients with HCM (47 males; mean age 50.1 ± 18.5 years) were studied by CMR measuring mass of LVMF, left ventricular mass, volume and function, and left atrial (LA) area. AF was present in 17 (25%) patients. LVMF was observed in 57% of patients. AF was significantly more frequent in patients who also showed LVMF, compared with the group without LVMF (42.1% vs. 3.4%, respectively; p 2 ; respectively, p = 0.0001). AF in HCM is related with myocardial fibrosis detected by DE-CMR and dilatation of the LA. This fact adds to the proven adverse prognostic value of myocardial fibrosis in HCM, thus, reinforcing the usefulness of this technique in the assessment of these patients.

  1. Serum levels of innate immunity cytokines are elevated in dogs with metaphyseal osteopathy (hypertrophic osteodytrophy) during active disease and remission.

    Science.gov (United States)

    Safra, Noa; Hitchens, Peta L; Maverakis, Emanual; Mitra, Anupam; Korff, Courtney; Johnson, Eric; Kol, Amir; Bannasch, Michael J; Pedersen, Niels C; Bannasch, Danika L

    2016-10-15

    Metaphyseal osteopathy (MO) (hypertrophic osteodystrophy) is a developmental disorder of unexplained etiology affecting dogs during rapid growth. Affected dogs experience relapsing episodes of lytic/sclerotic metaphyseal lesions and systemic inflammation. MO is rare in the general dog population; however, some breeds (Weimaraner, Great Dane and Irish Setter) have a much higher incidence, supporting a hereditary etiology. Autoinflammatory childhood disorders of parallel presentation such as chronic recurrent multifocal osteomyelitis (CRMO), and deficiency of interleukin-1 receptor antagonist (DIRA), involve impaired innate immunity pathways and aberrant cytokine production. Given the similarities between these diseases, we hypothesize that MO is an autoinflammatory disease mediated by cytokines involved in innate immunity. To characterize immune dysregulation in MO dogs we measured serum levels of inflammatory markers in 26 MO and 102 control dogs. MO dogs had significantly higher levels (pg/ml) of serum Interleukin-1beta (IL-1β), IL-18, IL-6, Granulocyte-macrophage colony stimulating factor (GM-CSF), C-X-C motif chemokine 10 (CXCL10), tumor necrosis factor (TNF), and IL-10. Notably, recovered MO dogs were not different from dogs during active MO disease, providing a suggestive mechanism for disease predisposition. This is the first documentation of elevated immune markers in MO dogs, uncovering an immune profile similar to comparable autoinflammatory disorders in children. Copyright © 2016 Elsevier B.V. All rights reserved.

  2. Vascular Endothelial Growth Factor Is Associated with the Morphologic and Functional Parameters in Patients with Hypertrophic Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Radek Pudil

    2015-01-01

    Full Text Available Background. Hypertrophic cardiomyopathy (HCM is mostly autosomal dominant disease of the myocardium, which is characterized by myocardial hypertrophy. Vascular endothelial growth factor (VEGF is involved in myocyte function, growth, and survival. The aim of study was to analyze the clinical significance of VEGF in structural and functional changes in patient with HCM. Methods. In a group of 21 patients with nonobstructive HCM, we assessed serum VEGF and analyzed its association with morphological and functional parameters. Compared to healthy controls, serum VEGF was increased: 199 (IQR: 120.4–260.8 ng/L versus 20 (IQR: 14.8–37.7 ng/L, P<0.001. VEGF levels were associated with left atrium diameter (r=0.51, P=0.01, left ventricle ejection fraction (r=-0.56, P=0.01, fractional shortening (r=-0.54, P=0.02, left ventricular mass (r=0.61, P=0.03, LV mass index (r=0.46, P=0.04, vena cava inferior diameter (r=0.65, P=0.01, and peak gradient of tricuspid regurgitation (r=0.46, P=0.03. Conclusions. Increased VEGF level is associated with structural and functional parameters in patients with HCM and serves as a potential tool for diagnostic process of these patients.

  3. Short-limb dwarfism and hypertrophic cardiomyopathy in a patient with paternal isodisomy 14: 45,XYidic(14)(p11)

    Energy Technology Data Exchange (ETDEWEB)

    Walter, C.A.; Moore, C.M.; Kaye, C.I. [Univ. of Texas Health Science Center, San Antonio, TX (United States)] [and others

    1996-11-11

    Uniparental disomy (UPD) has been shown to result in specific disorders either due to imprinting and/or homozygosity of mutant alleles. Here we present the findings in a child with paternal UPD14. Ultrasound evaluation was performed at 30 weeks of gestation because of abnormally large uterine size. Pertinent ultrasound findings included polyhydramnios, short limbs, abnormal position of hands, small thorax, and nonvisualization of the fetal stomach. Postnatally the infant was found to have a low birth weight, short birth length, contractures, short limbs, and a small thorax with upslanting ribs. Assisted ventilation and gastrostomy were required. At age 6 months, the infant required hospitalization for hypertrophic cardiomyopathy which responded to Atenolol{reg_sign}. Initial cytogenetic studies demonstrated an apparently balanced de novo Robertsonian translocation involving chromosomes 14 and a karyotype designation of 45,XY,t(14q14q). No indication of mosaicism for trisomy 14 was observed in metaphase spreads prepared from peripheral blood lymphocytes or skin-derived fibroblasts. C-band and fluorescence in situ hybridization results demonstrated that the chromosome was dicentric. DNA analyses showed paternal uniparental isodisomy for chromosome 14. Based on the cytogenetic and DNA results a final karyotype designation of 45,XY,idic(14)(p11) was assigned to this infant with paternal isodisomy of chromosome 14. 41 refs., 5 figs., 2 tabs.

  4. Scoring of late gadolinium enhancement in cardiac magnetic resonance imaging can predict cardiac events in patients with hypertrophic cardiomyopathy

    International Nuclear Information System (INIS)

    Nojiri, Ayumi; Hongo, Kenichi; Kawai, Makoto; Komukai, Kimiaki; Sakuma, Toru; Taniguchi, Ikuo; Yoshimura, Michihiro

    2011-01-01

    Late gadolinium enhancement (LGE) of cardiac magnetic resonance imaging (MRI) represents myocardial fibrosis and may be related to the clinical outcome of various heart diseases. This study evaluated the relationship between LGE and cardiac events in hypertrophic cardiomyopathy (HCM) using a new scoring method. This study retrospectively followed 46 HCM patients without heart failure symptoms for 3.8±1.8 years. Gadolinium-enhanced cardiac MRI was performed in all patients. Cardiac events including newly developed heart failure or ventricular tachyarrhythmia were evaluated during the follow-up period. We evaluated the predictive factors to identify the patients with cardiac events. None of the risk factors reported to be related to poor outcome or the existence of LGE alone could predict cardiac events, which might be due to the small number of subjects investigated in this study. A new scoring method for LGE-positive areas (LGE score) was applied and higher LGE score can predict cardiac events in this study population. The proposed LGE score for cardiac MRI is considered to be a potentially valid method for assessing cardiac events in HCM patients. (author)

  5. Kaempferol inhibits fibroblast collagen synthesis, proliferation and activation in hypertrophic scar via targeting TGF-β receptor type I.

    Science.gov (United States)

    Li, Hongwei; Yang, Liu; Zhang, Yuebing; Gao, Zhigang

    2016-10-01

    Hypertrophic scar (HPS) formation is a debilitating condition that results in pain, esthetic symptom and loss of tissue function. So far, no satisfactory therapeutic approach has been available for HPS treatment. In this study, we discovered that a natural small molecule, kaempferol, could significantly inhibit HPS formation in a mechanical load-induced mouse model. Our results also demonstrated that kaempferol remarkably attenuated collagen synthesis, proliferation and activation of fibroblasts in vitro and in vivo. Western blot analysis further revealed that kaempferol significantly down-regulated Smad2 and Smad3 phosphorylation in a dose-dependent manner. At last, we found that such bioactivity of kaempferol which resulted from the inhibition of TGF-β1/Smads signaling was induced by the selective binding of kaempferol to TGF-β receptor type I (TGFβRI). These findings suggest that kaempferol could be developed into a promising agent for the treatment of HPS or other fibroproliferative disorders. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

  6. Exercise-induced ST-segment depression and myocardial ischemia in patients with hypertrophic cardiomyopathy. Myocardial scintigraphic study

    International Nuclear Information System (INIS)

    Miyai, Nobuyuki; Kawasaki, Tatsuya; Taniguchi, Takuya; Kamitani, Tadaaki; Kawasaki, Shingo; Sugihara, Hiroki

    2005-01-01

    Patients with hypertrophic cardiomyopathy (HCM) sometimes develop myocardial ischemia during exercise in the absence of coronary lesions. The relationship between myocardial ischemia and ST-segment depression was investigated during exercise testing in patients with HCM. Regional hypoperfusion and/or transient left ventricular cavity dilation, a parameter of subendocardial hypoperfusion, were assessed on exercise 99 m Tc-tetrofosmin myocardial scintigraphy in 42 patients with non-obstructive HCM. The scintigraphic results were further correlated with the ST-segment responses to exercise. Regional hypoperfusion or transient left ventricular cavity dilation were observed in 19 (45%) or 16 (38%) patients with HCM, respectively. The incidence of ST-segment depression ≥0.1 mV during exercise testing was similar in HCM patients with regional hypoperfusion, with transient left ventricular cavity dilation, and without hypoperfusion (42%, 38%, 38%, p=0.95). Furthermore, exercise-induced ST-segment depression ≥0.1 mV occurred similarly irrespective of symptoms, exercise tolerance, the degree or the site of hypertrophy, or the presence or absence of resting ST-segment depression. ST-segment depression during exercise testing was common in patients with HCM, but seems to be an unreliable marker of myocardial ischemia as assessed by exercise scintigraphy. (author)

  7. Effects of Dredging and Lanthanum-Modified Clay on Water Quality Variables in an Enclosure Study in a Hypertrophic Pond

    Directory of Open Access Journals (Sweden)

    Miquel Lürling Guido Waajen

    2017-05-01

    Full Text Available An enclosure experiment was conducted between July and September 2009 to compare the effectiveness of a phosphate fixative, the lanthanum-modified bentonite clay Phoslock® (LMB, dredging, and their combination in controlling eutrophication in a hypertrophic urban pond in Heesch, The Netherlands. In total, 25 water quality variables were monitored. Multivariate analysis revealed that the combination LMB-treated and dredged enclosures deviated most from the pond (reference and the controls, and showed the strongest eutrophication reduction. Overall, dredging significantly increased transparency, lowered turbidity, and improved the oxygen conditions in the enclosures compared to non-dredged ones. Nonetheless, one dredged enclosure deviated dramatically from the others, which might reflect methodological issues with dredging. The LMB treatment appeared to be less effective at mitigating eutrophication than dredging, and phosphate concentrations even increased during the experiment in the LMB-treated enclosures. Chemical equilibrium modeling suggested that humic substances could have formed complexes with lanthanum (La from the LMB, rendering it unavailable for intercepting P over the course of the enclosure experiment. Residual lanthanum concentrations in combination dredging and LMB treatments exceeded the Dutch standard 10-fold. Total zooplankton abundance, and particularly Cladocera, increased in all enclosures over the course of the experiment. The limited effect of LMB in the enclosure experiment and the violation of the Dutch La standard when combined with dredging disqualify LMB as an intervention agent in the restoration of the pond.

  8. Use of Myocardial T1 Mapping at 3.0 T to Differentiate Anderson-Fabry Disease from Hypertrophic Cardiomyopathy.

    Science.gov (United States)

    Karur, Gauri R; Robison, Sean; Iwanochko, Robert M; Morel, Chantal F; Crean, Andrew M; Thavendiranathan, Paaladinesh; Nguyen, Elsie T; Mathur, Shobhit; Wasim, Syed; Hanneman, Kate

    2018-04-24

    Purpose To compare left ventricular (LV) and right ventricular (RV) 3.0-T cardiac magnetic resonance (MR) imaging T1 values in Anderson-Fabry disease (AFD) and hypertrophic cardiomyopathy (HCM) and evaluate the diagnostic value of native T1 values beyond age, sex, and conventional imaging features. Materials and Methods For this prospective study, 30 patients with gene-positive AFD (37% male; mean age ± standard deviation, 45.0 years ± 14.1) and 30 patients with HCM (57% male; mean age, 49.3 years ± 13.5) were prospectively recruited between June 2016 and September 2017 to undergo cardiac MR imaging T1 mapping with a modified Look-Locker inversion recovery (MOLLI) acquisition scheme at 3.0 T (repetition time msec/echo time msec, 280/1.12; section thickness, 8 mm). LV and RV T1 values were evaluated. Statistical analysis included independent samples t test, receiver operating characteristic curve analysis, multivariable logistic regression, and likelihood ratio test. Results Septal LV, global LV, and RV native T1 values were significantly lower in AFD compared with those in HCM (1161 msec ± 47 vs 1296 msec ± 55, respectively [P 3.0 T are significantly lower in patients with AFD compared with those with HCM and provide independent and incremental diagnostic value beyond age, sex, and conventional imaging features. © RSNA, 2018.

  9. Comparison of Valsalva Maneuver, Amyl Nitrite, and Exercise Echocardiography to Demonstrate Latent Left Ventricular Outflow Obstruction in Hypertrophic Cardiomyopathy.

    Science.gov (United States)

    Ayoub, Chadi; Geske, Jeffrey B; Larsen, Carolyn M; Scott, Christopher G; Klarich, Kyle W; Pellikka, Patricia A

    2017-12-15

    Guidelines recommend exercise stress echocardiogram (ESE) for patients with hypertrophic cardiomyopathy (HC) if a 50 mm Hg gradient is not present at rest or provoked with Valsalva or amyl nitrite, to direct medical and surgical management. However, no study has directly compared all 3 methods. We sought to evaluate efficacy and degree of provocation of left ventricular outflow gradients by ESE, and compare with Valsalva and amyl nitrite. In patients with HC between 2002 and 2015, resting echocardiograms and ESEs within 1 year were retrospectively reviewed. Gradients elicited by each provocation method were compared. Rest and ESE were available in 97 patients (mean age 54 ± 18 years, 57% male); 78 underwent Valsalva maneuver and 41 amyl nitrite provocation. Median gradients (interquartile range) were 10 mm Hg (7,19) at rest, 16 mm Hg (9,34) with Valsalva, 23 mm Hg (13,49) with amyl nitrite, and 26 mm Hg (13,58) with ESE. ESE and amyl nitrite were able to provoke obstruction (≥30 mm Hg) and severe obstruction (≥50 mm Hg) more frequently than Valsalva. In patients with resting gradient <30 mm Hg (n = 83), provocation maneuvers demonstrated dynamic obstruction in 51%; in those with Valsalva gradient <30 mm Hg (n = 57), ESE or amyl nitrite provoked a gradient in 44%; and in those with amyl nitrite gradient <30 mm Hg (n = 20), ESE provoked a gradient in 29%. No demographic or baseline echocardiographic parameter predicted provocable obstruction. In conclusion, ESE is clinically useful; however, different provocation maneuvers may be effective in different patients with HC, and all maneuvers may be required to provoke dynamic obstruction in symptomatic patients. Copyright © 2017 Elsevier Inc. All rights reserved.

  10. Atrial Fibrillation in Hypertrophic Cardiomyopathy: Prevalence, Clinical Correlations, and Mortality in a Large High‐Risk Population

    Science.gov (United States)

    Siontis, Konstantinos C.; Geske, Jeffrey B.; Ong, Kevin; Nishimura, Rick A.; Ommen, Steve R.; Gersh, Bernard J.

    2014-01-01

    Background Atrial fibrillation (AF) is a common sequela of hypertrophic cardiomyopathy (HCM), but evidence on its prevalence, risk factors, and effect on mortality is sparse. We sought to evaluate the prevalence of AF, identify clinical and echocardiographic correlates, and assess its effect on mortality in a large high‐risk HCM population. Methods and Results We identified HCM patients who underwent evaluation at our institution from 1975 to 2012. AF was defined by known history (either chronic or paroxysmal), electrocardiogram, or Holter monitoring at index visit. We examined clinical and echocardiographic variables in association with AF. The effect of AF on overall and cause‐specific mortality was evaluated with multivariate Cox proportional hazards models. Of 3673 patients with HCM, 650 (18%) had AF. Patients with AF were older and more symptomatic (P<0.001). AF was less common among patients with obstructive HCM phenotype and was associated with larger left atria, higher E/e’ ratios, and worse cardiopulmonary exercise tolerance (all P values<0.001). During median (interquartile range) follow‐up of 4.1 (0.2 to 10) years, 1069 (29%) patients died. Patients with AF had worse survival compared to those without AF (P<0.001). In multivariate analysis adjusted for established risk factors of mortality in HCM, the hazard ratio (95% confidence interval) for the effect of AF on overall mortality was 1.48 (1.27 to 1.71). AF did not have an effect on sudden or nonsudden cardiac death. Conclusions In this large referral HCM population, approximately 1 in 5 patients had AF. AF was a strong predictor of mortality, even after adjustment for established risk factors. PMID:24965028

  11. High Incidence of De Novo and Subclinical Atrial Fibrillation in Patients With Hypertrophic Cardiomyopathy and Cardiac Rhythm Management Device.

    Science.gov (United States)

    Wilke, Iris; Witzel, Katrin; Münch, Julia; Pecha, Simon; Blankenberg, Stephan; Reichenspurner, Hermann; Willems, Stephan; Patten, Monica; Aydin, Ali

    2016-07-01

    Atrial fibrillation (AF) is an important prognostic parameter in patients with hypertrophic cardiomyopathy (HCM). Though cardiac rhythm management (CRM) devices (e.g., ICD, pacemaker or implantable loop recorder) can detect subclinical AF, data describing the incidence of AF are rare. We therefore investigated the incidence and clinical impact of de novo and subclinical AF detected by CRM devices in patients with HCM. In our retrospective single-center study, we included patients with HCM and need for CRM devices. The primary endpoint of the study was the incidence of clinical and subclinical de novo AF. During follow-up, patients were screened for adverse events like stroke, ventricular arrhythmia, heart failure, or death. From 192 HCM patients, 44 patients received a CRM device (38 ICDs, 5 pacemakers, 1 implantable loop recorder). In 14 of these patients (32%), AF had been documented before device implantation. Thirty (68%) patients were free from AF at the time of implantation. During a median follow-up of 595 days (interquartile range, 367-890 days), de novo AF was recorded in 16 of these 30 patients (53%). Fourteen (88%) of the 16 patients with de novo AF were free from any clinical symptoms, so these patients were classified to have subclinical AF. In logistic regression analysis, age was the only significant predictor for an increased risk of AF. AF is common in patients with HCM who need a CRM device. More than 50% of these patients develop de novo AF that was predominantly subclinical in our cohort. © 2016 Wiley Periodicals, Inc.

  12. Long-Term Outcomes of Hypertrophic Cardiomyopathy Diagnosed During Childhood: Results from a National Population-Based Study.

    Science.gov (United States)

    Alexander, Peta M A; Nugent, Alan W; Daubeney, Piers E F; Lee, Katherine J; Sleeper, Lynn A; Schuster, Tibor; Turner, Christian; Davis, Andrew M; Semsarian, Chris; Colan, Steven D; Robertson, Terry; Ramsay, James; Justo, Robert; Sholler, Gary F; King, Ingrid; Weintraub, Robert G

    2018-02-28

    Background -Late survival and symptomatic status of children with hypertrophic cardiomyopathy (HCM) have not been well defined. We examined long-term outcomes for pediatric HCM. Methods -The National Australian Childhood Cardiomyopathy Study is a longitudinal population-based cohort study of children (0-10 years) diagnosed with cardiomyopathy between 1987 and 1996. The primary study end-point was time to death or cardiac transplantation. Results -There were 80 patients with HCM with median age at diagnosis of 0.48 (Inter-quartile range [IQR] 0.1, 2.5) years. Freedom from death/transplantation (95% confidence interval [CI]) was 86 (77-92)% one year after presentation, 80 (69-87)% at 10 years and 78 (67-86)% at 20 years. From multivariable analyses, risk factors for death/transplantation included symmetric left ventricular hypertrophy at the time of diagnosis (hazard ratio [HR] 4.20 95%CI 1.60, 11.05 p=0.004), Noonan syndrome (HR 2.88, 95%CI 1.02, 8.08, p=0.045), higher posterior wall thickness z-score (HR 1.45, 95%CI 1.22, 1.73, p<0.001) and lower fractional shortening z-score (HR 0.84, 95%CI 0.74, 0.95, p=0.005) during follow-up. Nineteen (23%) subjects underwent left ventricular myectomy. At median 15.7 years' follow-up, 27 (42%) of 63 survivors were treated with beta-blocker and 13 (21%) had an implantable cardioverter-defibrillator. Conclusions -The highest risk of death or transplantation for children with HCM is within one year post-diagnosis, with low attrition rates thereafter. Many subjects receive medical, surgical or device therapy.

  13. Recognition of regional hypertrophy in hypertrophic cardiomyopathy using thallium-201 emission-computed tomography: comparison with two-dimensional echocardiography

    International Nuclear Information System (INIS)

    Suzuki, Y.; Kadota, K.; Nohara, R.

    1984-01-01

    The configuration of the hypertrophied myocardium was evaluated by thallium-201 emission-computed tomography and 2-dimensional (2-D) sector scan in 10 patients with obstructive hypertrophic cardiomyopathy (HC), 10 with nonobstructive HC with giant negative T waves and 10 with concentric left ventricular (LV) hypertrophy. Thallium-201 myocardial imaging was reconstructed into multiple 12-mm-thick slices in 3 planes. The thickness ratio of the ventricular septum and the LV posterior wall in the short-axis plane and the ratio of the ventricular septum and the apical wall in the long-axis plane were analyzed. In the patients with obstructive HC the ventricular septal wall thickness index was increased, and the ratio of septal to posterior wall thickness index (1.45 +/- 0.23) was greater than that in the patients with nonobstructive HC with giant negative T waves or in those with concentric LV hypertrophy (1.03 +/- 0.20 and 0.98 +/- 0.11, respectively; p less than 0.01 for each). In the patients with nonobstructive HC with giant negative T waves, increased apical wall thickness with apical cavity obliteration was characteristic, and the ratio of ventricular septal to apical wall thickness index (0.66 +/- 0.14) was less than that in the patients with obstructive HC or in those with concentric LV hypertrophy (1.46 +/- 0.38 and 1.04 +/- 0.09, respectively; p less than 0.001 for each). In contrast, technically satisfactory 2-D sector scanning (83%) demonstrated various configurations of the hypertrophied ventricularseptum, but could not detect apical hypertrophy in 4 of the 10 patients with nonobstructive HC with giant negative T waves whose LV cineangiograms demonstrated apical hypertrophy. Thus, thallium-201 emission-computed tomography is useful in evaluating the characteristics of LV hypertrophy and assists 2-D sector scan, especially in patients with apical hypertrophy in HC

  14. Focalized contractile impairment at hypertrophied myocardium proven in consideration of wall stress in patients with hypertrophic cardiomyopathy

    International Nuclear Information System (INIS)

    Yamazaki, Tadashi; Suzuki, Jun-ichi; Shimamoto, Ryoichi; Tsuji, Taeko; Ohmoto, Yuki; Toyo-oka, Teruhiko; Omata, Masao; Ohtomo, Kuni; Nagai, Ryozo

    2006-01-01

    In hypertrophic cardiomyopathy (HCM) a hyperkinetic state is sometimes observed in spite of impaired systolic function in the hypertrophied myocardium. The aim of the present study was to determine the mechanism of this paradox. Seventeen patients with HCM and 10 normal subjects underwent cine magnetic resonance (MR) imaging to measure percent systolic wall thickening and percent fractional shortening. The ratio of systolic radial wall stress of the LV at the hypertrophied myocardium over that at the nonhypertrophied myocardium was evaluated to describe the focal advantageous condition for wall thickening. The ratio was 0.66±0.36 at the start of contraction and 0.78±0.31 at early-systole, indicating consistently smaller radial wall stress at the hypertrophied myocardium. Although the condition for contraction was favorable (a ratio less than 1.00), percent systolic wall thickening at the hypertrophied myocardium (23.0±11.8%) was smaller than that at the nonhypertrophied myocardium (70.5±32.3%). Smaller end-diastolic dimension (HCM group; 45.2±4.2 mm, reference group; 48.9±4.1 mm, P=0.04) with a statistically identical value of systolic decrease in intraventricular dimension (HCM group; 19.7±3.9 mm, reference group; 18.9±3.2 mm, P=0.60) yielded high percent fractional shortening in patients with HCM (43.5±7.6%). Although contractile impairment was proven at the hypertrophied region with low radial wall stress in the HCM group, the smaller end-diastolic dimension in this group resulted in high percent fractional shortening. (author)

  15. Using H2O2 treatments for the degradation of cyanobacteria and microcystins in a shallow hypertrophic reservoir.

    Science.gov (United States)

    Papadimitriou, Theodoti; Kormas, Konstantinos; Dionysiou, Dionysios D; Laspidou, Chrysi

    2016-11-01

    Toxins produced by cyanobacteria in freshwater ecosystems constitute a serious health risk worldwide for humans that may use the affected water bodies for recreation, drinking water, and/or irrigation. Cyanotoxins have also been deemed responsible for loss of animal life in many places around the world. This paper explores the effect of H 2 O 2 treatments on cyanobacteria and microcystins in natural samples from a hypertrophic reservoir in microcosm experiments. According to the results, cyanobacteria were more easily affected by H 2 O 2 than by other phytoplanktonic groups. This was shown by the increase in the fractions of chlorophyll-a (a proxy for phytoplankton) and chlorophyll-b (a proxy for green algae) over total phytoplankton pigments and the decrease in the fraction of phycocyanin (a proxy for cyanobacteria) over total phytoplankton pigments. Thus, while an overall increase in phytoplankton occurred, a preferential decrease in cyanobacteria was observed with H 2 O 2 treatments over a few hours. Moreover, significant degradation of total microcystins was observed under H 2 O 2 treatments, while more microcystins were degraded when UV radiation was used in combination with H 2 O 2 . The combination of H 2 O 2 and ultraviolet (UV) treatment in natural samples resulted in total microcystin concentrations that were below the World Health Organization limit for safe consumption of drinking water of 1 μg/L. Although further investigation into the effects of H 2 O 2 addition on ecosystem function must be performed, our results show that the application of H 2 O 2 could be a promising method for the degradation of microcystins in reservoirs and the reduction of public health risks related to the occurrence of harmful algal blooms.

  16. A novel approach to select differential pathways associated with hypertrophic cardiomyopathy based on gene co‑expression analysis.

    Science.gov (United States)

    Chen, Xiao-Min; Feng, Ming-Jun; Shen, Cai-Jie; He, Bin; Du, Xian-Feng; Yu, Yi-Bo; Liu, Jing; Chu, Hui-Min

    2017-07-01

    The present study was designed to develop a novel method for identifying significant pathways associated with human hypertrophic cardiomyopathy (HCM), based on gene co‑expression analysis. The microarray dataset associated with HCM (E‑GEOD‑36961) was obtained from the European Molecular Biology Laboratory‑European Bioinformatics Institute database. Informative pathways were selected based on the Reactome pathway database and screening treatments. An empirical Bayes method was utilized to construct co‑expression networks for informative pathways, and a weight value was assigned to each pathway. Differential pathways were extracted based on weight threshold, which was calculated using a random model. In order to assess whether the co‑expression method was feasible, it was compared with traditional pathway enrichment analysis of differentially expressed genes, which were identified using the significance analysis of microarrays package. A total of 1,074 informative pathways were screened out for subsequent investigations and their weight values were also obtained. According to the threshold of weight value of 0.01057, 447 differential pathways, including folding of actin by chaperonin containing T‑complex protein 1 (CCT)/T‑complex protein 1 ring complex (TRiC), purine ribonucleoside monophosphate biosynthesis and ubiquinol biosynthesis, were obtained. Compared with traditional pathway enrichment analysis, the number of pathways obtained from the co‑expression approach was increased. The results of the present study demonstrated that this method may be useful to predict marker pathways for HCM. The pathways of folding of actin by CCT/TRiC and purine ribonucleoside monophosphate biosynthesis may provide evidence of the underlying molecular mechanisms of HCM, and offer novel therapeutic directions for HCM.

  17. Rare Variants in Genes Encoding MuRF1 and MuRF2 Are Modifiers of Hypertrophic Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Ming Su

    2014-05-01

    Full Text Available Modifier genes contribute to the diverse clinical manifestations of hypertrophic cardiomyopathy (HCM, but are still largely unknown. Muscle ring finger (MuRF proteins are a class of muscle-specific ubiquitin E3-ligases that appear to modulate cardiac mass and function by regulating the ubiquitin-proteasome system. In this study we screened all the three members of the MuRF family, MuRF1, MuRF2 and MuRF3, in 594 unrelated HCM patients and 307 healthy controls by targeted resequencing. Identified rare variants were confirmed by capillary Sanger sequencing. The prevalence of rare variants in both MuRF1 and MuRF2 in HCM patients was higher than that in control subjects (MuRF1 13/594 (2.2% vs. 1/307 (0.3%, p = 0.04; MuRF2 22/594 (3.7% vs. 2/307 (0.7%; p = 0.007. Patients with rare variants in MuRF1 or MuRF2 were younger (p = 0.04 and had greater maximum left ventricular wall thickness (p = 0.006 than those without such variants. Mutations in genes encoding sarcomere proteins were present in 19 (55.9% of the 34 HCM patients with rare variants in MuRF1 and MuRF2. These data strongly supported that rare variants in MuRF1 and MuRF2 are associated with higher penetrance and more severe clinical manifestations of HCM. The findings suggest that dysregulation of the ubiquitin-proteasome system contributes to the pathogenesis of HCM.

  18. Extent of late gadolinium enhancement at right ventricular insertion points in patients with hypertrophic cardiomyopathy: relation with diastolic dysfunction

    Energy Technology Data Exchange (ETDEWEB)

    Zhu, Yinsu [Seoul National University Hospital, Department of Radiology, Seoul (Korea, Republic of); The First Affiliated Hospital of Nanjing Medical University, Department of Radiology, Nanjing, Jiangsu (China); Park, Eun-Ah; Lee, Whal; Chu, Ajung; Chung, Jin Wook; Park, Jae Hyung [Seoul National University Hospital, Department of Radiology, Seoul (Korea, Republic of); Kim, Hyung-Kwan [Seoul National University Hospital, Division of Cardiology, Department of Internal Medicine, Seoul (Korea, Republic of)

    2015-04-01

    Our aim was to examine the association between the extent of late gadolinium enhancement (LGE) at right ventricular insertion points (RVIP) and left ventricular (LV) functional parameters in patients with hypertrophic cardiomyopathy (HCM). Sixty-one HCM patients underwent echocardiography and cardiovascular magnetic resonance (CMR) within one week. Mitral annular velocities (E/E') were obtained from echocardiography; LV ejection fraction (EF), LV mass index, LV wall maximal thickness, and left atrial volume index (LAVI) were obtained from MR. LGE extent was quantified (proportion of total LV myocardial mass) according to location: % RVIP-LGE and % non-RVIP-LGE. Although LGE was commonly present in both apical (74 %) and non-apical HCMs (88 %) (p = 0.163), RVIP-LGE was more frequent (86 % vs. 47 %, p = 0.002) in non-apical HCMs in which E/E' was significantly higher (19.23 ± 8.40 vs. 13.13 ± 5.06, p = 0.009). In addition, RVIP-LGE extent was associated with LV diastolic dysfunction (r = 0.45, p < 0.001 for E/E'; r = 0.53, p < 0.001 for LAVI) and lower LVEF (r = -0.42, p = 0.001). There was no correlation between non-RVIP-LGE extent and other parameters. Multiple linear regression analysis revealed RVIP-LGE extent as an independent predictor of E/E' (β = 0.45, p < 0.001) and LAVI in HCM patients (β = 0.53, p < 0.001). The extent of LGE at RVIPs in HCM patients is associated with increased estimated LV filling pressure and chronic diastolic burden. (orig.)

  19. Mechanical dispersion and global longitudinal strain by speckle tracking echocardiography: Predictors of appropriate implantable cardioverter defibrillator therapy in hypertrophic cardiomyopathy.

    Science.gov (United States)

    Candan, Ozkan; Gecmen, Cetin; Bayam, Emrah; Guner, Ahmet; Celik, Mehmet; Doğan, Cem

    2017-06-01

    In this study, we investigated whether mechanical dispersion which reflects electrical abnormality and other echocardiographic and clinic parameters predict appropriate ICD shock in patients undergone ICD implantation for hypertrophic cardiomyopathy. Sixty-three patients who received ICD implantation for primary or secondary prevention were included in the study. Patients' clinical, electrocardiographic, 2D classic, and speckle tracking echocardiographic data were collected. Mechanical dispersion was defined as the standard deviation of time to peak negative strain in 18 left ventricular segments. Appropriate ICD therapy was defined as cardioversion or defibrillation due to ventricular tachycardia or fibrillation. Patients were divided into two groups as occurrence or the absence of appropriate ICD therapy. A total of 17 (26.9%) patients were observed to have an appropriate ICD therapy during follow-up periods. In patients who performed appropriate ICD therapy, a larger left atrial volume index, higher sudden cardiac death (SCD)-Risk Score, longer mechanical dispersion, and decreased global longitudinal peak strain (GLPS) were observed. In multivariate logistic regression analysis, including (GLPS, mechanical dispersion, LAVi, and SCD-Risk Score) was used to determine independent predictors of occurrence of appropriate ICD therapy during the follow-up. Mechanical dispersion, GLPS, and SCD-Risk Score were found to be independent predictors of occurrence of appropriate ICD therapy. Mechanical dispersion, GLPS, and SCD-Risk Score were found to be predictive for appropriate ICD therapy in patients receiving ICD implantation. Readily measurable mechanical dispersion and GLPS could be helpful to distinguish patients at high risk who could optimally benefit from ICD therapy. © 2017, Wiley Periodicals, Inc.

  20. Hypertrophic Cardiomyopathy in Children, Adolescents, and Young Adults Associated With Low Cardiovascular Mortality With Contemporary Management Strategies.

    Science.gov (United States)

    Maron, Barry J; Rowin, Ethan J; Casey, Susan A; Lesser, John R; Garberich, Ross F; McGriff, Deepa M; Maron, Martin S

    2016-01-05

    Youthful age has been considered the time of greatest risk for patients with hypertrophic cardiomyopathy (HCM), largely because of the possibility of sudden death. The last 2 decades have witnessed more reliable identification of at-risk patients and utilization of implantable cardioverter-defibrillators for prevention of sudden death, and other contemporary treatment options. Whether such management advances have significantly altered the considerable mortality rate for young HCM patients remains unresolved. We studied long-term outcome in 474 consecutive HCM patients between 7 and 29 years of age presenting at 2 referral institutions. Over 7.1±5.1 years of follow-up (6.0 [3.0, 10.0]), 452 patients (95%) survived, with 95% experiencing no or mild symptoms. HCM-related death occurred in 18 patients (3%; 0.54%/y): arrhythmic sudden death (n=12), progressive heart failure and heart transplant complications (n=5), or postoperatively (n=1). In contrast, aborted life-threatening events occurred in 63 other high-risk patients (13%) with implantable cardioverter-defibrillator interventions for ventricular tachyarrhythmias (n=31), resuscitated out-of-hospital cardiac arrest (n=20), or heart transplant for advanced heart failure (n=12), 1.8%/y, 3-fold higher than HCM mortality. Five- and 10-year survival (considering only HCM deaths) was high (97% and 94%, respectively), virtually identical to that reported in middle-aged adult HCM patients (98% and 94%, P=0.23). In a large hospital-based cohort of young HCM patients, representing an age group considered at greatest risk, low mortality rates can be achieved with the application of contemporary cardiovascular treatment strategies, largely because of reliable identification of high-risk patients who benefited from implantable cardioverter-defibrillators for sudden death prevention, thereby creating the opportunity for extended longevity and good quality of life. © 2015 American Heart Association, Inc.

  1. [Early diffuse hypertrophic osteitis recurrence. Unexpected and consternating development after a large évidement cavity. Apropos of 15 cases].

    Science.gov (United States)

    Fleury, P; Basset, J M; Candau, P; Bré, M; Despreaux, G; Fabre, A; Saliba, N

    1985-01-01

    The authors report 15 cases collected over a period of 13 years (1972 - 1984) of a particularly serious eventuality affecting certain evacuation cavities: Early recurrence of diffuse hypertrophic osteitis (E.R.D.H.O.). This is a rare complication, occurring in 1.5% of cases of chronic otitis. However, the fact of having operated upon 7 cases in 1984 alone, whilst during the previous 12 years, only 8 had been seen, raised the alarm. The classical pattern of this complication occurs in 3 stages, each involving surgical operation: at the outset, chronic otitis with cholesteatoma or osteitis, most often mixed, for which an "inadequate" surgical procedure is performed. then, after a variable period which may exceed 10 years, a very large evacuation cavity was created by the authors. finally, 3rd and final stage: within 6 months, this cavity filled progressively and, at operation, the authors discovered a cavity virtually completely filled with a "fantastic" "moist sugar" osteitis, requiring a new evacuation cavity, even larger than that created before. In all cases, a successful result was obtained within a varying period, sometimes accompanied by spectacular improvement in general condition. The most plausible etiopathogenic hypothesis advanced to attempt to explain this phenomenon is that of secondary infection by a varied microbial flora, including, on the one hand, aerobic organisms usually discovered in such cases (pseudomonas pyocyaneus, proteus mirabilis) and, secondly, this being of essential importance, "anaerobic" bacteria, the bacteriological diagnosis of which requires application of a strict protocol. The authors have taken three decisions following their bibliographic investigations.(ABSTRACT TRUNCATED AT 250 WORDS)

  2. Longitudinal analysis of osteogenic and angiogenic signaling factors in healing models mimicking atrophic and hypertrophic non-unions in rats.

    Directory of Open Access Journals (Sweden)

    Susann Minkwitz

    Full Text Available Impaired bone healing can have devastating consequences for the patient. Clinically relevant animal models are necessary to understand the pathology of impaired bone healing. In this study, two impaired healing models, a hypertrophic and an atrophic non-union, were compared to physiological bone healing in rats. The aim was to provide detailed information about differences in gene expression, vascularization and histology during the healing process. The change from a closed fracture (healing control group to an open osteotomy (hypertrophy group led to prolonged healing with reduced mineralized bridging after 42 days. RT-PCR data revealed higher gene expression of most tested osteogenic and angiogenic factors in the hypertrophy group at day 14. After 42 days a significant reduction of gene expression was seen for Bmp4 and Bambi in this group. The inhibition of angiogenesis by Fumagillin (atrophy group decreased the formation of new blood vessels and led to a non-healing situation with diminished chondrogenesis. RT-PCR results showed an attempt towards overcoming the early perturbance by significant up regulation of the angiogenic regulators Vegfa, Angiopoietin 2 and Fgf1 at day 7 and a further continuous increase of Fgf1, -2 and Angiopoietin 2 over time. However µCT angiograms showed incomplete recovery after 42 days. Furthermore, lower expression values were detected for the Bmps at day 14 and 21. The Bmp antagonists Dan and Twsg1 tended to be higher expressed in the atrophy group at day 42. In conclusion, the investigated animal models are suitable models to mimic human fracture healing complications and can be used for longitudinal studies. Analyzing osteogenic and angiogenic signaling patterns, clear changes in expression were identified between these three healing models, revealing the importance of a coordinated interplay of different factors to allow successful bone healing.

  3. Fabry Disease in Families With Hypertrophic Cardiomyopathy: Clinical Manifestations in the Classic and Later-Onset Phenotypes.

    Science.gov (United States)

    Adalsteinsdottir, Berglind; Palsson, Runolfur; Desnick, Robert J; Gardarsdottir, Marianna; Teekakirikul, Polakit; Maron, Martin; Appelbaum, Evan; Neisius, Ulf; Maron, Barry J; Burke, Michael A; Chen, Brenden; Pagant, Silvere; Madsen, Christoffer V; Danielsen, Ragnar; Arngrimsson, Reynir; Feldt-Rasmussen, Ulla; Seidman, Jonathan G; Seidman, Christine E; Gunnarsson, Gunnar Th

    2017-08-01

    The screening of Icelandic patients clinically diagnosed with hypertrophic cardiomyopathy resulted in identification of 8 individuals from 2 families with X-linked Fabry disease (FD) caused by GLA (α-galactosidase A gene) mutations encoding p.D322E (family A) or p.I232T (family B). Familial screening of at-risk relatives identified mutations in 16 family A members (8 men and 8 heterozygotes) and 25 family B members (10 men and 15 heterozygotes). Clinical assessments, α-galactosidase A (α-GalA) activities, glycosphingolipid substrate levels, and in vitro mutation expression were used to categorize p.D322E as a classic FD mutation and p.I232T as a later-onset FD mutation. In vitro expression revealed that p.D322E and p.I232T had α-GalA activities of 1.4% and 14.9% of the mean wild-type activity, respectively. Family A men had markedly decreased α-GalA activity and childhood-onset classic manifestations, except for angiokeratoma and cornea verticillata. Family B men had residual α-GalA activity and developed FD manifestations in adulthood. Despite these differences, all family A and family B men >30 years of age had left ventricular hypertrophy, which was mainly asymmetrical, and had similar late gadolinium enhancement patterns. Ischemic stroke and severe white matter lesions were more frequent among family A men, but neither family A nor family B men had overt renal disease. Family A and family B heterozygotes had less severe or no clinical manifestations. Men with classic or later-onset FD caused by GLA missense mutations developed prominent and similar cardiovascular disease at similar ages, despite markedly different α-GalA activities. © 2017 American Heart Association, Inc.

  4. Mechanisms of pro-arrhythmic abnormalities in ventricular repolarisation and anti-arrhythmic therapies in human hypertrophic cardiomyopathy.

    Science.gov (United States)

    Passini, Elisa; Mincholé, Ana; Coppini, Raffaele; Cerbai, Elisabetta; Rodriguez, Blanca; Severi, Stefano; Bueno-Orovio, Alfonso

    2016-07-01

    Hypertrophic cardiomyopathy (HCM) is a cause of sudden arrhythmic death, but the understanding of its pro-arrhythmic mechanisms and an effective pharmacological treatment are lacking. HCM electrophysiological remodelling includes both increased inward and reduced outward currents, but their role in promoting repolarisation abnormalities remains unknown. The goal of this study is to identify key ionic mechanisms driving repolarisation abnormalities in human HCM, and to evaluate anti-arrhythmic effects of single and multichannel inward current blocks. Experimental ionic current, action potential (AP) and Ca(2+)-transient (CaT) recordings were used to construct populations of human non-diseased and HCM AP models (n=9118), accounting for inter-subject variability. Simulations were conducted for several degrees of selective and combined inward current block. Simulated HCM cardiomyocytes exhibited prolonged AP and CaT, diastolic Ca(2+) overload and decreased CaT amplitude, in agreement with experiments. Repolarisation abnormalities in HCM models were consistently driven by L-type Ca(2+) current (ICaL) re-activation, and ICaL block was the most effective intervention to normalise repolarisation and diastolic Ca(2+), but compromised CaT amplitude. Late Na(+) current (INaL) block partially abolished repolarisation abnormalities, with small impact on CaT. Na(+)/Ca(2+) exchanger (INCX) block effectively restored repolarisation and CaT amplitude, but increased Ca(2+) overload. Multichannel block increased efficacy in normalising repolarisation, AP biomarkers and CaT amplitude compared to selective block. Experimentally-calibrated populations of human AP models identify ICaL re-activation as the key mechanism for repolarisation abnormalities in HCM, and combined INCX, INaL and ICaL block as effective anti-arrhythmic therapies also able to partially reverse the HCM electrophysiological phenotype. Copyright © 2015 The Authors. Published by Elsevier Ltd.. All rights reserved.

  5. Contribution of mitral valve leaflet length and septal wall thickness to outflow tract obstruction in patients with hypertrophic cardiomyopathy.

    Science.gov (United States)

    Morant, Kareem; Mikami, Yoko; Nevis, Immaculate; McCarty, David; Stirrat, John; Scholl, David; Rajchl, Martin; Giannoccaro, Peter; Kolman, Louis; Heydari, Bobby; Lydell, Carmen; Howarth, Andrew; Grant, Andrew; White, James A

    2017-08-01

    We sought to examine whether elongation of the mitral valve leaflets in patients with hypertrophic cardiomyopathy (HCM) is synergistic to septal wall thickness (SWT) in the development of left ventricular outflow tract obstruction (LVOTO). HCM is a common genetic cardiac disease characterized by asymmetric septal hypertrophy and predisposition towards LVOTO. It has been reported that elongation of the mitral valve leaflets may be a primary phenotypic feature and contribute to LVOTO. However, the relative contribution of this finding versus SWT has not been studied. 152 patients (76 with HCM and 76 non-diseased age, race and BSA-matched controls) and 18 young, healthy volunteers were studied. SWT and the anterior mitral valve leaflet length (AMVLL) were measured using cine MRI. The combined contribution of these variables (SWT × AMVLL) was described as the Septal Anterior Leaflet Product (SALP). Peak LVOT pressure gradient was determined by Doppler interrogation and defined as "obstructive" if ≥ 30 mmHg. Patients with HCM were confirmed to have increased AMVLL compared with controls and volunteers (p SALP were significantly higher in patients with LVOTO (N = 17) versus without. SALP showed modest improvement in predictive accuracy for LVOTO (AUC = 0.81) among the HCM population versus SWT alone (AUC = 0.77). However, in isolated patients this variable identified patients with LVOTO despite modest SWT. Elongation of the AMVLL is a primary phenotypic feature of HCM. While incremental contributions to LVOTO appear modest at a population level, specific patients may have dominant contribution to LVOTO. The combined marker of SALP allows for maintained identification of such patients despite modest increases in SWT.

  6. OSTEOARTROPATIA HIPERTRÓFICA ASSOCIADA A TUBERCULOSE PULMONAR Hypertrophic osteoarthropathy in association with pulmonary tuberculosis: a case report

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    Luiza Beatriz Melo Moreira

    2002-01-01

    Full Text Available Neste artigo é descrito um caso de osteoartropatia hipertrófica associada a tuberculose pulmonar. Esta alteração é uma complicação rara da tuberculose e pode estar associada à forma grave e fulminante da doença. Entre os métodos de imagem utilizados no diagnóstico estavam radiografias dos membros inferiores, que mostravam reação periosteal bilateralmente, e radiografias e tomografia computadorizada do tórax, que evidenciaram massa no lobo superior do pulmão direito. Embora a punção aspirativa por agulha fina tivesse sido sugestiva de tuberculose, o diagnóstico definitivo foi dado pelo exame histopatológico da massa, após a realização de toracotomia. O paciente evoluiu com melhora sintomática e radiológica após o tratamento com drogas tuberculostáticas.In this article the authors report a case of a patient with hypertrophic osteoarthropathy associated with pulmonary tuberculosis. This disorder is a rare complication of tuberculosis and may be associated with severe and fulminant disease. The imaging methods used in the diagnosis of this disease included lower limbs radiographies that showed bilateral periosteal reaction, and chest radiographies and computed tomography that revealed a mass in the upper lobe of the right lung. Although a fine needle aspiration was suggestive of tuberculosis, the definitive diagnosis was made after thoracotomy and hystopathological examination of the mass. The patient presented symptomatic and radiological improvement after treatment of tuberculosis.

  7. Missense Variant in MAPK Inactivator PTPN5 Is Associated with Decreased Severity of Post-Burn Hypertrophic Scarring.

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    Ravi F Sood

    Full Text Available Hypertrophic scarring (HTS is hypothesized to have a genetic mechanism, yet its genetic determinants are largely unknown. The mitogen-activated protein kinase (MAPK pathways are important mediators of inflammatory signaling, and experimental evidence implicates MAPKs in HTS formation. We hypothesized that single-nucleotide polymorphisms (SNPs in MAPK-pathway genes would be associated with severity of post-burn HTS.We analyzed data from a prospective-cohort genome-wide association study of post-burn HTS. We included subjects with deep-partial-thickness burns admitted to our center who provided blood for genotyping and had at least one Vancouver Scar Scale (VSS assessment. After adjusting for HTS risk factors and population stratification, we tested MAPK-pathway gene SNPs for association with the four VSS variables in a joint regression model. In addition to individual-SNP analysis, we performed gene-based association testing.Our study population consisted of 538 adults (median age 40 years who were predominantly White (76% males (71% admitted to our center from 2007-2014 with small-to-moderate-sized burns (median burn size 6% total body surface area. Of 2,146 SNPs tested, a rare missense variant in the PTPN5 gene (rs56234898; minor allele frequency 1.5% was significantly associated with decreased severity of post-burn HTS (P = 1.3×10-6. In gene-based analysis, PTPN5 (P = 1.2×10-5 showed a significant association and BDNF (P = 9.5×10-4 a borderline-significant association with HTS severity.We report PTPN5 as a novel genetic locus associated with HTS severity. PTPN5 is a MAPK inhibitor expressed in neurons, suggesting a potential role for neurotrophic factors and neuroinflammatory signaling in HTS pathophysiology.

  8. Novel insights on the relationship between T-tubular defects and contractile dysfunction in a mouse model of hypertrophic cardiomyopathy.

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    Crocini, C; Ferrantini, C; Scardigli, M; Coppini, R; Mazzoni, L; Lazzeri, E; Pioner, J M; Scellini, B; Guo, A; Song, L S; Yan, P; Loew, L M; Tardiff, J; Tesi, C; Vanzi, F; Cerbai, E; Pavone, F S; Sacconi, L; Poggesi, C

    2016-02-01

    Abnormalities of cardiomyocyte Ca(2+) homeostasis and excitation-contraction (E-C) coupling are early events in the pathogenesis of hypertrophic cardiomyopathy (HCM) and concomitant determinants of the diastolic dysfunction and arrhythmias typical of the disease. T-tubule remodelling has been reported to occur in HCM but little is known about its role in the E-C coupling alterations of HCM. Here, the role of T-tubule remodelling in the electro-mechanical dysfunction associated to HCM is investigated in the Δ160E cTnT mouse model that expresses a clinically-relevant HCM mutation. Contractile function of intact ventricular trabeculae is assessed in Δ160E mice and wild-type siblings. As compared with wild-type, Δ160E trabeculae show prolonged kinetics of force development and relaxation, blunted force-frequency response with reduced active tension at high stimulation frequency, and increased occurrence of spontaneous contractions. Consistently, prolonged Ca(2+) transient in terms of rise and duration are also observed in Δ160E trabeculae and isolated cardiomyocytes. Confocal imaging in cells isolated from Δ160E mice reveals significant, though modest, remodelling of T-tubular architecture. A two-photon random access microscope is employed to dissect the spatio-temporal relationship between T-tubular electrical activity and local Ca(2+) release in isolated cardiomyocytes. In Δ160E cardiomyocytes, a significant number of T-tubules (>20%) fails to propagate action potentials, with consequent delay of local Ca(2+) release. At variance with wild-type, we also observe significantly increased variability of local Ca(2+) transient rise as well as higher Ca(2+)-spark frequency. Although T-tubule structural remodelling in Δ160E myocytes is modest, T-tubule functional defects determine non-homogeneous Ca(2+) release and delayed myofilament activation that significantly contribute to mechanical dysfunction. Copyright © 2015 The Authors. Published by Elsevier Ltd.. All

  9. Erythrocyte creatine as a marker of intravascular hemolysis due to left ventricular outflow tract obstruction in hypertrophic cardiomyopathy.

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    Kubo, Toru; Okumiya, Toshika; Baba, Yuichi; Hirota, Takayoshi; Tanioka, Katsutoshi; Yamasaki, Naohito; Sugiura, Tetsuro; Doi, Yoshinori L; Kitaoka, Hiroaki

    2016-03-01

    Erythrocyte creatine, a marker of erythrocyte age that increases with shortening of erythrocyte survival, has been reported to be a quantitative and reliable marker for intravascular hemolysis. We hypothesized that hemolysis could also occur due to intraventricular obstruction in patients with hypertrophic cardiomyopathy (HCM). The purpose of this study was to examine the presence of subclinical hemolysis and the relation between intravascular hemolysis and intraventricular pressure gradient (IVPG). We measured erythrocyte creatine in 92 HCM patients. Twelve patients had left ventricular outflow tract obstruction (LVOTO), 4 had midventricular obstruction (MVO), and the remaining 76 were non-obstructive. Erythrocyte creatine levels ranged from 0.92 to 4.36μmol/g hemoglobin. Higher levels of erythrocyte creatine were associated with higher IVPG (r=0.437, pcreatine levels are high (≥1.8μmol/g hemoglobin), subclinical hemolysis is considered to be present. Half of LVOTO patients and no MVO patients showed high erythrocyte creatine levels. Although non-obstructive patients did not show significant intraventricular obstruction at rest, some showed high erythrocyte creatine levels. When LVOT-PG was measured during the strain phase of the Valsalva maneuver in 20 non-obstructive patients, 7 of those 20 patients showed LVOTO. In the 20 patients, there was no relation between erythrocyte creatine levels and LVOT-PG before the Valsalva maneuver (r=0.125, p=0.600), whereas there was a significant correlation between erythrocyte creatine and LVOT-PG provoked by the Valsalva maneuver (r=0.695, p=0.001). There is biochemical evidence of subclinical hemolysis in patients with HCM, and this hemolysis seems to be associated with LVOTO provoked by daily physical activities. Copyright © 2015 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.

  10. MYBPC3 mutations are associated with a reduced super-relaxed state in patients with hypertrophic cardiomyopathy.

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    James W McNamara

    Full Text Available The "super-relaxed state" (SRX of myosin represents a 'reserve' of motors in the heart. Myosin heads in the SRX are bound to the thick filament and have a very low ATPase rate. Changes in the SRX are likely to modulate cardiac contractility. We previously demonstrated that the SRX is significantly reduced in mouse cardiomyocytes lacking cardiac myosin binding protein-C (cMyBP-C. Here, we report the effect of mutations in the cMyBP-C gene (MYBPC3 using samples from human patients with hypertrophic cardiomyopathy (HCM. Left ventricular (LV samples from 11 HCM patients were obtained following myectomy surgery to relieve LV outflow tract obstruction. HCM samples were genotyped as either MYBPC3 mutation positive (MYBPC3mut or negative (HCMsmn and were compared to eight non-failing donor hearts. Compared to donors, only MYBPC3mut samples display a significantly diminished SRX, characterised by a decrease in both the number of myosin heads in the SRX and the lifetime of ATP turnover. These changes were not observed in HCMsmn samples. There was a positive correlation (p < 0.01 between the expression of cMyBP-C and the proportion of myosin heads in the SRX state, suggesting cMyBP-C modulates and maintains the SRX. Phosphorylation of the myosin regulatory light chain in MYBPC3mut samples was significantly decreased compared to the other groups, suggesting a potential mechanism to compensate for the diminished SRX. We conclude that by altering both contractility and sarcomeric energy requirements, a reduced SRX may be an important disease mechanism in patients with MYBPC3 mutations.

  11. Divergent effects of adrenaline in human induced pluripotent stem cell-derived cardiomyocytes obtained from hypertrophic cardiomyopathy

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    Chandra Prajapati

    2018-02-01

    Full Text Available Hypertrophic cardiomyopathy (HCM is a common inherited cardiac disease that affects the heart muscle with diverse clinical outcomes. HCM can cause sudden cardiac death (SCD during or immediately after mild to rigorous physical activity in young patients. However, the mechanism causing SCD as a result of exercise remains unknown, but exercise-induced ventricular arrhythmias are thought to be responsible for this fatal consequence. To understand the disease mechanism behind HCM in a better way, we generated patient-specific induced pluripotent stem cell-derived cardiomyocytes (hiPSC-CMs from HCM patients carrying either the MYBPC3-Gln1061X or TPM1-Asp175Asn mutation. We extensively investigated the effects of low to high concentrations of adrenaline on action potential characteristics, and the occurrence of arrhythmias in the presence of various concentrations of adrenaline and in wash-out condition. We classified and quantified different types of arrhythmias observed in hiPSC-CMs, and found that the occurrence of arrhythmias was dependent on concentrations of adrenaline and positions of mutations in genes causing HCM. In addition, we observed ventricular tachycardia types of arrhythmias in hiPSC-CMs carrying the TPM1-Asp175Asn mutation. We additionally examined the antiarrhythmic potency of bisoprolol in HCM-specific hiPSC-CMs. However, bisoprolol could not reduce the occurrence of arrhythmias during administration or during the wash-out condition of adrenaline in HCM-specific hiPSC-CMs. Our study demonstrates hiPSC-CMs as a promising tool for studying HCM. The experimental design used in this study could be suitable and beneficial for studying other components and drugs related to cardiac disease in general.

  12. Assessing blood flow, microvasculature, erythema and redness in hypertrophic scars: A cross sectional study showing different features that require precise definitions.

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    Jaspers, M E H; Stekelenburg, C M; Simons, J M; Brouwer, K M; Vlig, M; van den Kerckhove, E; Middelkoop, E; van Zuijlen, P P M

    2017-08-01

    In hypertrophic scar assessment, laser Doppler imaging (LDI), colorimetry and subjective assessment (POSAS) can be used to evaluate blood flow, erythema and redness, respectively. In addition, the microvasculature (i.e. presence of microvessels) can be determined by immunohistochemistry. These measurement techniques are frequently used in clinical practice and/or in research to evaluate treatment response and monitor scar development. However, until now it has not been tested to what extent the outcomes of these techniques are associated, whilst the outcome terms are frequently used interchangeably or replaced by the umbrella term 'vascularization'. This is confusing, as every technique seems to measure a specific feature. Therefore, we evaluated the correlations of the four measurement techniques. We included 32 consecutive patients, aged ≥18 years, who underwent elective resection of a hypertrophic scar. Pre-operatively, we performed LDI (measuring blood flow), colorimetry (measuring erythema) and the POSAS (subjective redness) within the predefined scar area of interest (∼1.5cm). Subsequently, the scar was excised and the area of interest was sent for immunohistochemistry, to determine the presence of microvessels. Only a statistically significant correlation was found between erythema values (colorimetry) and subjective redness assessment (POSAS) (r=0.403, p=0.030). We found no correlations between the outcomes of LDI, immunohistochemistry and colorimetry. Blood flow, the presence of microvessels and erythema appear to be different hypertrophic scar features because they show an absence of correlation. Therefore, in the field of scar assessment, these outcome terms cannot be used interchangeably. In addition, we conclude that the term 'vascularization' does not seem appropriate to serve as an umbrella term. The use of precise definitions in research as well as in clinical practice is recommended. Copyright © 2017 Elsevier Ltd and ISBI. All rights reserved.

  13. Glycogen synthase kinase 3 (GSK3) in the heart: a point of integration in hypertrophic signalling and a therapeutic target? A critical analysis.

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    Sugden, P H; Fuller, S J; Weiss, S C; Clerk, A

    2008-03-01

    Glycogen synthase kinase 3 (GSK3, of which there are two isoforms, GSK3alpha and GSK3beta) was originally characterized in the context of regulation of glycogen metabolism, though it is now known to regulate many other cellular processes. Phosphorylation of GSK3alpha(Ser21) and GSK3beta(Ser9) inhibits their activity. In the heart, emphasis has been placed particularly on GSK3beta, rather than GSK3alpha. Importantly, catalytically-active GSK3 generally restrains gene expression and, in the heart, catalytically-active GSK3 has been implicated in anti-hypertrophic signalling. Inhibition of GSK3 results in changes in the activities of transcription and translation factors in the heart and promotes hypertrophic responses, and it is generally assumed that signal transduction from hypertrophic stimuli to GSK3 passes primarily through protein kinase B/Akt (PKB/Akt). However, recent data suggest that the situation is far more complex. We review evidence pertaining to the role of GSK3 in the myocardium and discuss effects of genetic manipulation of GSK3 activity in vivo. We also discuss the signalling pathways potentially regulating GSK3 activity and propose that, depending on the stimulus, phosphorylation of GSK3 is independent of PKB/Akt. Potential GSK3 substrates studied in relation to myocardial hypertrophy include nuclear factors of activated T cells, beta-catenin, GATA4, myocardin, CREB, and eukaryotic initiation factor 2Bvarepsilon. These and other transcription factor substrates putatively important in the heart are considered. We discuss whether cardiac pathologies could be treated by therapeutic intervention at the GSK3 level but conclude that any intervention would be premature without greater understanding of the precise role of GSK3 in cardiac processes.

  14. Echocardiography and cardiac MRI in mutation-negative hypertrophic cardiomyopathy in an older patient: a case defining the need for ICD.

    Science.gov (United States)

    Rodriguez, Fatima; Degnan, Kathleen O; Seidman, Christine E; Mangion, Judy R

    2014-08-01

    We report the case of a 67-year-old man with hypertrophic cardiomyopathy who presented for a second opinion about implantable cardio-defibrillator (ICD) placement after a witnessed syncopal episode. Despite his older age, being mutation-negative, and having a maximal septal thickness of 2.2 cm on echocardiography, he demonstrated rapid progression of myocardial fibrosis on cardiac MRI, correlating to ventricular tachyarrhythmias and syncope. We review the role of echocardiography and cardiac MRI in optimizing medical care for such patients who may not otherwise meet criteria for an ICD placement or further interventions. © 2014, Wiley Periodicals, Inc.

  15. Motorcycle Parts

    Science.gov (United States)

    1993-01-01

    An article in NASA Tech Briefs describing a vacuum bagging process for forming composite parts helped a small Oklahoma Company to improve its manufacturing process. President of Performance Extremes, Larry Ortega, and his partners make motorcycle parts from carbon/epoxy to reduce weight. Using vacuum bags, parts have a better surface and fewer voids inside. When heat used in the vacuum bag process caused deformation upon cooling, a solution found in another tech brief solved the problem. A metal plate inside the vacuum bag made for more even heat transfer. A third article described a simple procedure for repairing loose connector pins, which the company has also utilized.

  16. Combined 595-nm and 1,064-nm laser irradiation of recalcitrant and hypertrophic port-wine stains in children and adults.

    Science.gov (United States)

    Alster, Tina S; Tanzi, Elizabeth L

    2009-06-01

    Although pulsed dye laser (PDL) treatment of port-wine stain (PWS) has long been proven safe and effective, incomplete clearance of these vascular malformations can be problematic. In addition, advanced PWS with deeper coloration and tissue hypertrophy can be particularly difficult to treat because of the superficial dermal penetration of 585- to 595-nm light. The purpose of this study was to evaluate the safety and efficacy of a novel device that delivers sequential pulses of 595- and 1,064-nm wavelengths in the treatment of recalcitrant and hypertrophic PWS. Twenty-five children and adults (skin phototypes I-III) with recalcitrant or hypertrophic PWS showing incomplete clearance after 10 prior PDL treatments were included in the study. Successive treatments using a 595-nm PDL and a 1,064-nm neodymium-doped yttrium-aluminum-garnet (Nd:YAG) laser were delivered at 6- to 8-week intervals. Two masked assessors determined clinical improvement of treatment areas using independent evaluation of comparative photographs at baseline and 3 months after treatment using a standard quartile grading scale. The use of dual 595-/1,064-nm wavelengths provided continued improvement of PWS that were previously recalcitrant to ongoing PDL therapy. Side effects were limited to transient erythema, edema, and mild purpura. Rare vesicle formation was observed, with no subsequent scarring or undesirable pigmentary changes. The novel dual 595-nm PDL and 1,064-nm Nd:YAG laser is an effective treatment for PWS that are recalcitrant to PDL therapy alone.

  17. Absence of collagen IX accelerates hypertrophic differentiation in the embryonic mouse spine through a disturbance of the Ihh-PTHrP feedback loop.

    Science.gov (United States)

    Kamper, Matthias; Paulsson, Mats; Zaucke, Frank

    2017-02-01

    Collagen IX (Col IX) is a component of the cartilage extracellular matrix and contributes to its structural integrity. Polymorphisms in the genes encoding the Col IX ɑ2- and ɑ3-chains are associated with early onset of disc degeneration. Col IX-deficient mice already display changes in the spine at the newborn stage and premature disc degeneration starting at 6 months of age. To determine the role of Col IX in early spine development and to identify molecular mechanisms underlying disc degeneration, the embryonic development of the spine was analyzed in Col IX -/- mice. Histological staining was used to show tissue morphology at different time points. Localization of extracellular matrix proteins as well as components of signaling pathways were analyzed by immunohistochemistry. Developing vertebral bodies of Col IX -/- mice were smaller and already appeared more compact at E12.5. At E15.5, vertebral bodies of Col IX -/- mice revealed an increased number of hypertrophic chondrocytes as well as enhanced staining for the terminal differentiation markers alkaline phosphatase and collagen X. This correlates with an imbalance in the Ihh-PTHrP signaling pathway at this time point, reflected by an increase of Ihh and a concomitant decrease of PTHrP expression. An accelerated hypertrophic differentiation caused by a disturbed Ihh-PTHrP signaling pathway may lead to a higher bone mineral density in the vertebral bodies of newborn Col IX -/- mice and, as a result, to the early onset of disc degeneration.

  18. [Left atrial function and left atrial appendage flow velocity in hypertrophic cardiomyopathy: comparison of patients with and without paroxysmal atrial fibrillation].

    Science.gov (United States)

    Akasaka, K; Kawashima, E; Shiokoshi, T; Ishii, Y; Hasebe, N; Kikuchi, K

    1998-07-01

    The involvement of left atrial (LA) appendage flow velocity in reduced left atrial function was investigated in 24 patients with hypertrophic cardiomyopathy, who retained sinus rhythm at the examination. Patients were divided into 11 with a history of paroxysmal atrial fibrillation [PAf(+)] and 13 without such history [PAf(-)]. Transthoracic echocardiography was performed to evaluate LA fractional shortening (LA%FS) and mean velocity of circumferential LA fiber shortening (LAmVcf), as contractile functions of the left atrium at the phase of active atrial contraction. Transesophageal echocardiographic Doppler examination was performed in all patients to measure the LA appendage velocity. In all patients, significant positive correlations were observed between the LA appendage velocity and LA%FS (r = 0.50, p fibrillation were significantly lower than in those without (0.84 +/- 0.15 vs 1.28 +/- 0.37 circ/sec, 44 +/- 12 vs 65 +/- 20 cm/sec, both p fibrillation. These results indicate that there is a close relationship between LA appendage velocity and LA contractile function in patients with hypertrophic cardiomyopathy with paroxysmal atrial fibrilation, and these patients have potential risk of cerebral infarction.

  19. Comparing Two Methods of Cryotherapy and Intense Pulsed Light with Triamcinolone Injection in the Treatment of Keloid and Hypertrophic Scars: A Clinical Trial.

    Science.gov (United States)

    Meymandi, Simin Shamsi; Moosazadeh, Mahmood; Rezazadeh, Azadeh

    2016-10-01

    Keloid and hypertrophic scars are abnormal manifestations of wounds that occur following skin injuries in the form of local proliferation of fibroblasts and increased production of collagen. There are several ways to cure these scars; treatment must be selected based on the nature of the scars. In this clinical trial, two methods-cryotherapy and intense pulsed light (IPL)-are compared in the treatment of scars, and the results are presented in terms of improvement level, complications, and patient satisfaction. This clinical trial was conducted in southeastern Iran. The intervention group included scars that underwent the IPL method and the control group, which consisted of scars that were subjected to cryotherapy. In both methods, intralesional corticosteroid injection was administered. To select samples, the easy sampling method was used. To determine the expected outcomes, the criteria determined in the Vancouver scar scale were used. Data were analyzed using the Mix Model, chi-square test, and t test. In this study, 166 samples of keloid and hypertrophic scars were cured using two methods (Cryotherapy, 83; IPL, 83). The recovery rate was higher in the Cryotherapy group than in the IPL group ( p  > 0.05), and the incidence of complications was also higher in the Cryotherapy group (14.5% vs. 12%). Moreover, patients were more satisfied, although not significantly so, with the cryotherapy method ( p  = 0.09). Both methods were highly successful in curing scars; participants were totally satisfied with both methods.

  20. Complex phenotype linked to a mutation in exon 11 of the lamin A/C gene: Hypertrophic cardiomyopathy, atrioventricular block, severe dyslipidemia and diabetes.

    Science.gov (United States)

    Francisco, Ana Rita G; Santos Gonçalves, Inês; Veiga, Fátima; Mendes Pedro, Mónica; Pinto, Fausto J; Brito, Dulce

    2017-09-01

    The lamin A/C (LMNA) gene encodes lamins A and C, which have an important role in nuclear cohesion and chromatin organization. Mutations in this gene usually lead to the so-called laminopathies, the primary cardiac manifestations of which are dilated cardiomyopathy and intracardiac conduction defects. Some mutations, associated with lipodystrophy but not cardiomyopathy, have been linked to metabolic abnormalities such as diabetes and severe dyslipidemia. Herein we describe a new phenotype associated with a mutation in exon 11 of the LMNA gene: hypertrophic cardiomyopathy, atrioventricular block, severe dyslipidemia and diabetes. A 64-year-old woman with hypertrophic cardiomyopathy and a point mutation in exon 11 of the LMNA gene (c.1718C>T, Ser573Leu) presented with severe symptomatic ventricular hypertrophy and left ventricular outflow tract obstruction. She underwent septal alcohol ablation, followed by Morrow myectomy. The patient was also diagnosed with severe dyslipidemia, diabetes and obesity, and fulfilled diagnostic criteria for metabolic syndrome. No other characteristics of LMNA mutation-related phenotypes were identified. The development of type III atrioventricular block with no apparent cause, and mildly depressed systolic function, prompted referral for cardiac resynchronization therapy. In conclusion, the association between LMNA mutations and different phenotypes is complex and not fully understood, and can present with a broad spectrum of severity. Copyright © 2017 Sociedade Portuguesa de Cardiologia. Publicado por Elsevier España, S.L.U. All rights reserved.

  1. In vivo efficacy of the AKT inhibitor ARQ 092 in Noonan Syndrome with multiple lentigines-associated hypertrophic cardiomyopathy.

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    Jianxun Wang

    Full Text Available Noonan Syndrome with Multiple Lentigines (NSML, formerly LEOPARD syndrome is an autosomal dominant "RASopathy" disorder manifesting in congenital heart disease. Most cases of NSML are caused by catalytically inactivating mutations in the protein tyrosine phosphatase (PTP, non-receptor type 11 (PTPN11, encoding the SH2 domain-containing PTP-2 (SHP2 protein. We previously generated knock-in mice harboring the PTPN11 mutation Y279C, one of the most common NSML alleles; these now-termed SHP2Y279C/+ mice recapitulate the human disorder and develop hypertrophic cardiomyopathy (HCM by 12 weeks of age. Functionally, heart and/or cardiomyocyte lysates from SHP2Y279C/+ mice exhibit increased basal and agonist-induced AKT and mTOR activities. Here, we sought to determine whether we could reverse the hypertrophy in SHP2Y279C/+ mice using ARQ 092, an oral and selective allosteric AKT inhibitor currently in clinical trials for patients with PI3K/AKT-driven tumors or Proteus syndrome. We obtained echocardiographs of SHP2Y279C/+ and wildtype (SHP2+/+ littermates, either in the presence or absence of ARQ 092 at 12, 14, and 16 weeks of age. While SHP2Y279C/+ mice developed significant left ventricular hypertrophy by 12 weeks, as indicated by decreased chamber dimension and increased posterior wall thickness, treatment of SHP2Y279C/+ mice with ARQ 092 normalized the hypertrophy in as early as 2 weeks following treatment, with hearts comparable in size to those in wildtype (SHP2+/+ mice. In addition, we observed an increase in fractional shortening (FS% in SHP2Y279C/+ mice, an effect of increased compensatory hypertrophy, which was not apparent in SHP2Y279C/+ mice treated with ARQ 092, suggesting functional improvement of HCM upon treatment with the AKT inhibitor. Finally, we found that ARQ 092 specifically inhibited AKT activity, as well as its downstream effectors, PRAS and S6RP in NSML mice. Taken together, these data suggest ARQ 092 may be a promising novel

  2. Tissue Doppler Imaging Combined with Advanced 12-Lead ECG Analysis Might Improve Early Diagnosis of Hypertrophic Cardiomyopathy in Childhood

    Science.gov (United States)

    Femlund, E.; Schlegel, T.; Liuba, P.

    2011-01-01

    Optimization of early diagnosis of childhood hypertrophic cardiomyopathy (HCM) is essential in lowering the risk of HCM complications. Standard echocardiography (ECHO) has shown to be less sensitive in this regard. In this study, we sought to assess whether spatial QRS-T angle deviation, which has shown to predict HCM in adults with high sensitivity, and myocardial Tissue Doppler Imaging (TDI) could be additional tools in early diagnosis of HCM in childhood. Methods: Children and adolescents with familial HCM (n=10, median age 16, range 5-27 years), and without obvious hypertrophy but with heredity for HCM (n=12, median age 16, range 4-25 years, HCM or sudden death with autopsy-verified HCM in greater than or equal to 1 first-degree relative, HCM-risk) were additionally investigated with TDI and advanced 12-lead ECG analysis using Cardiax(Registered trademark) (IMED Co Ltd, Budapest, Hungary and Houston). Spatial QRS-T angle (SA) was derived from Kors regression-related transformation. Healthy age-matched controls (n=21) were also studied. All participants underwent thorough clinical examination. Results: Spatial QRS-T angle (Figure/ Panel A) and septal E/Ea ratio (Figure/Panel B) were most increased in HCM group as compared to the HCM-risk and control groups (p less than 0.05). Of note, these 2 variables showed a trend toward higher levels in HCM-risk group than in control group (p=0.05 for E/Ea and 0.06 for QRS/T by ANOVA). In a logistic regression model, increased SA and septal E/Ea ratio appeared to significantly predict both the disease (Chi-square in HCM group: 9 and 5, respectively, p less than 0.05 for both) and the risk for HCM (Chi-square in HCM-risk group: 5 and 4 respectively, p less than 0.05 for both), with further increased predictability level when these 2 variables were combined (Chi-square 10 in HCM group, and 7 in HCM-risk group, p less than 0.01 for both). Conclusions: In this small material, Tissue Doppler Imaging and spatial mean QRS-T angle

  3. Midregional pro-atrial natriuretic peptide: a novel marker of myocardial fibrosis in patients with hypertrophic cardiomyopathy.

    Science.gov (United States)

    Elmas, Elif; Doesch, Christina; Fluechter, Stephan; Freundt, Miriam; Weiss, Christel; Lang, Siegfried; Kälsch, Thorsten; Haghi, Dariush; Papassotiriou, Jana; Kunde, Jan; Schoenberg, Stefan O; Borggrefe, Martin; Papavassiliu, Theano

    2011-04-01

    We aimed to determine the diagnostic performance of biomarkers in predicting myocardial fibrosis assessed by late gadolinium enhancement (LGE) cardiovascular magnetic resonance imaging (CMR) in patients with hypertrophic cardiomyopathy (HCM). LGE CMR was performed in 40 consecutive patients with HCM. Left and right ventricular parameters, as well as the extent of LGE were determined and correlated to the plasma levels of midregional pro-atrial natriuretic peptide (MR-proANP), midregional pro-adrenomedullin (MR-proADM), carboxy-terminal pro-endothelin-1 (CT-proET-1), carboxy-terminal pro-vasopressin (CT-proAVP), matrix metalloproteinase-9 (MMP-9), tissue inhibitor of metalloproteinase-1 (TIMP-1) and interleukin-8 (IL-8). Myocardial fibrosis was assumed positive, if CMR indicated LGE. LGE was present in 26 of 40 patients with HCM (65%) with variable extent (mean: 14%, range: 1.3-42%). The extent of LGE was positively associated with MR-proANP (r = 0.4; P = 0.01). No correlations were found between LGE and MR-proADM (r = 0.1; P = 0.5), CT-proET-1 (r = 0.07; P = 0.66), CT-proAVP (r = 0.16; P = 0.3), MMP-9 (r = 0.01; P = 0.9), TIMP-1 (r = 0.02; P = 0.85), and IL-8 (r = 0.02; P = 0.89). After adjustment for confounding factors, MR-proANP was the only independent predictor associated with the presence of LGE (P = 0.007) in multivariate analysis. The area under the ROC curve (AUC) indicated good predictive performance (AUC = 0.882) of MR-proANP with respect to LGE. The odds ratio was 1.268 (95% confidence interval 1.066-1.508). The sensitivity of MR-proANP at a cut-off value of 207 pmol/L was 69%, the specificity 94%, the positive predictive value 90% and the negative predictive value 80%. The results imply that MR-proANP serves as a novel marker of myocardial fibrosis assessed by LGE CMR in patients with HCM.

  4. Hypertrophic Cardiomyopathy in Adulthood Associated With Low Cardiovascular Mortality With Contemporary Management Strategies.

    Science.gov (United States)

    Maron, Barry J; Rowin, Ethan J; Casey, Susan A; Link, Mark S; Lesser, John R; Chan, Raymond H M; Garberich, Ross F; Udelson, James E; Maron, Martin S

    2015-05-12

    Hypertrophic cardiomyopathy (HCM) has been prominently associated with adverse disease complications, including sudden death or heart failure death and a generally adverse prognosis, with annual mortality rates of up to 6%. This study determined whether recent advances in management strategy, including implantable cardioverter-defibrillators (ICDs), heart transplantation, or other therapeutic measures have significantly improved survival and the clinical course of adult HCM patients. We addressed long-term outcomes in 1,000 consecutive adult HCM patients presenting at 30 to 59 years of age (mean 45±8 years) over 7.2±5.2 years of follow-up. Of 1,000 patients, 918 (92%) survived to 53±9.2 years of age (range 32 to 80 years) with 91% experiencing no or only mild symptoms at last evaluation. HCM-related death occurred in 40 patients (4% [0.53%/year]) at 50±10 years from the following events: progressive heart failure (n=17); arrhythmic sudden death (SD) (n=17); and embolic stroke (n=2). In contrast, 56 other high-risk patients (5.6%) survived life-threatening events, most commonly with ICD interventions for ventricular tachyarrhythmias (n=33) or heart transplantation for advanced heart failure (n=18 [0.79%/year]). SD occurred in patients who declined ICD recommendations, had evaluations before application of prophylactic ICDs to HCM, or were without conventional risk factors. The 5- and 10-year survival rates (confined to HCM deaths) were 98% and 94%, respectively, not different from the expected all-cause mortality in the general U.S. population (p=0.25). Multivariate independent predictors of adverse outcome were younger age at diagnosis, female sex, and increased left atrial dimension. In a large longitudinally assessed adult HCM cohort, we have demonstrated that contemporary management strategies and treatment interventions, including ICDs for SD prevention, have significantly altered the clinical course, now resulting in a low disease-related mortality rate of

  5. Keloids and Hypertrophic Scars

    Science.gov (United States)

    ... to the skin both skin cells and connective tissue cells (fibroblasts) begin multiplying to repair the damage. A scar is made up of 'connective tissue', gristle-like fibers deposited in the skin by ...

  6. Hypertrophic pyloric stenosis

    DEFF Research Database (Denmark)

    Lund Kofoed, P E; Høst, A; Elle, B

    1988-01-01

    of the pyloric muscle in the HPS group and in the controls stresses the need to assess the muscle length, the muscle diameter, and the muscle wall thickness in establishing the sonographic diagnosis of HPS. We found the following criteria useful: muscle length greater than or equal to 19 mm, muscle diameter...

  7. High prevalence of Arginine to Glutamine Substitution at 98, 141 and 162 positions in Troponin I (TNNI3 associated with hypertrophic cardiomyopathy among Indians

    Directory of Open Access Journals (Sweden)

    Rani Deepa

    2012-08-01

    Full Text Available Abstract Background Troponin I (TNNI3 is the inhibitory subunit of the thin filament regulatory complex Troponin, which confers calcium-sensitivity to striated muscle actomyosin ATPase activity. Mutations (2-7% in this gene had been reported in hypertrophic cardiomyopathy patients (HCM. However, the frequencies of mutations and associated clinical presentation have not been established in cardiomyopathy patients of Indian origin, hence we have undertaken this study. Methods We have sequenced all the exons, including the exon-intron boundaries of TNNI3 gene in 101 hypertrophic cardiomyopathy patients (HCM, along with 160 healthy controls, inhabited in the same geographical region of southern India. Results Our study revealed a total of 16 mutations. Interestingly, we have observed Arginine to Glutamine (R to Q mutation at 3 positions 98, 141 and 162, exclusively in HCM patients with family history of sudden cardiac death. The novel R98Q was observed in a severe hypertrophic obstructive cardiomyopathy patient (HOCM. The R141Q mutation was observed in two familial cases of severe asymmetric septal hypertrophy (ASH++. The R162Q mutation was observed in a ASH++ patient with mean septal thickness of 29 mm, and have also consists of allelic heterogeneity by means of having one more synonymous (E179E mutation at g.4797: G → A: in the same exon 7, which replaces a very frequent codon (GAG: 85% with a rare codon (GAA: 14%. Screening for R162Q mutation in all the available family members revealed its presence in 9 individuals, including 7 with allelic heterogeneity (R162Q and E179E of which 4 were severely affected. We also found 2 novel SNPs, (g.2653; G → A and g.4003 C → T exclusively in HCM, and in silico analysis of these SNPs have predicted to cause defect in recognition/binding sites for proteins responsible for proper splicing. Conclusion Our study has provided valuable information regarding the prevalence of TNNI3 mutations in

  8. R-spondin 2 facilitates differentiation of proliferating chondrocytes into hypertrophic chondrocytes by enhancing Wnt/β-catenin signaling in endochondral ossification

    International Nuclear Information System (INIS)

    Takegami, Yasuhiko; Ohkawara, Bisei; Ito, Mikako; Masuda, Akio; Nakashima, Hiroaki; Ishiguro, Naoki; Ohno, Kinji

    2016-01-01

    Endochondral ossification is a crucial process for longitudinal growth of bones. Differentiating chondrocytes in growth cartilage form four sequential zones of proliferation, alignment into column, hypertrophy, and substitution of chondrocytes with osteoblasts. Wnt/β-catenin signaling is essential for differentiation of proliferating chondrocytes into hypertrophic chondrocytes in growth cartilage. R-spondin 2 (Rspo2), a member of R-spondin family, is an agonist for Wnt signaling, but its role in chondrocyte differentiation remains unknown. Here we report that growth cartilage of Rspo2-knockout mice shows a decreased amount of β-catenin and increased amounts collagen type II (CII) and Sox9 in the abnormally extended proliferating zone. In contrast, expression of collagen type X (CX) in the hypertrophic zone remains unchanged. Differentiating chondrogenic ATDC5 cells, mimicking proliferating chondrocytes, upregulate Rspo2 and its putative receptor, Lgr5, in parallel. Addition of recombinant human Rspo2 to differentiating ATDC5 cells decreases expressions of Col2a1, Sox9, and Acan, as well as production of proteoglycans. In contrast, lentivirus-mediated knockdown of Rspo2 has the opposite effect. The effect of Rspo2 on chondrogenic differentiation is mediated by Wnt/β-catenin signaling, and not by Wnt/PCP or Wnt/Ca 2+ signaling. We propose that Rspo2 activates Wnt/β-catenin signaling to reduce Col2a1 and Sox9 and to facilitate differentiation of proliferating chondrocytes into hypertrophic chondrocytes in growth cartilage. - Highlights: • Rspo2 is a secreted activator of Wnt, and its knockout shows extended proliferating chondrocytes in endochondral ossification. • In proliferating chondrocytes of Rspo2-knockout mice, Sox9 and collagen type 2 are increased and β-catenin is decreased. • Rspo2 and its receptor Lgr5, as well as Sox9 and collagen type 2, are expressed in differentiating ATDC5 chondrogenic cells. • In ATDC5 cells, Rspo2 decreases expressions

  9. R-spondin 2 facilitates differentiation of proliferating chondrocytes into hypertrophic chondrocytes by enhancing Wnt/β-catenin signaling in endochondral ossification

    Energy Technology Data Exchange (ETDEWEB)

    Takegami, Yasuhiko [Division of Neurogenetics, Center of Neurological Disease and Cancer, Nagoya University Graduate School of Medicine, Nagoya (Japan); Department of Orthopaedic Surgery, Nagoya University School of Medicine, Nagoya (Japan); Ohkawara, Bisei; Ito, Mikako; Masuda, Akio [Division of Neurogenetics, Center of Neurological Disease and Cancer, Nagoya University Graduate School of Medicine, Nagoya (Japan); Nakashima, Hiroaki; Ishiguro, Naoki [Department of Orthopaedic Surgery, Nagoya University School of Medicine, Nagoya (Japan); Ohno, Kinji, E-mail: ohnok@med.nagoya-u.ac.jp [Division of Neurogenetics, Center of Neurological Disease and Cancer, Nagoya University Graduate School of Medicine, Nagoya (Japan)

    2016-04-22

    Endochondral ossification is a crucial process for longitudinal growth of bones. Differentiating chondrocytes in growth cartilage form four sequential zones of proliferation, alignment into column, hypertrophy, and substitution of chondrocytes with osteoblasts. Wnt/β-catenin signaling is essential for differentiation of proliferating chondrocytes into hypertrophic chondrocytes in growth cartilage. R-spondin 2 (Rspo2), a member of R-spondin family, is an agonist for Wnt signaling, but its role in chondrocyte differentiation remains unknown. Here we report that growth cartilage of Rspo2-knockout mice shows a decreased amount of β-catenin and increased amounts collagen type II (CII) and Sox9 in the abnormally extended proliferating zone. In contrast, expression of collagen type X (CX) in the hypertrophic zone remains unchanged. Differentiating chondrogenic ATDC5 cells, mimicking proliferating chondrocytes, upregulate Rspo2 and its putative receptor, Lgr5, in parallel. Addition of recombinant human Rspo2 to differentiating ATDC5 cells decreases expressions of Col2a1, Sox9, and Acan, as well as production of proteoglycans. In contrast, lentivirus-mediated knockdown of Rspo2 has the opposite effect. The effect of Rspo2 on chondrogenic differentiation is mediated by Wnt/β-catenin signaling, and not by Wnt/PCP or Wnt/Ca{sup 2+} signaling. We propose that Rspo2 activates Wnt/β-catenin signaling to reduce Col2a1 and Sox9 and to facilitate differentiation of proliferating chondrocytes into hypertrophic chondrocytes in growth cartilage. - Highlights: • Rspo2 is a secreted activator of Wnt, and its knockout shows extended proliferating chondrocytes in endochondral ossification. • In proliferating chondrocytes of Rspo2-knockout mice, Sox9 and collagen type 2 are increased and β-catenin is decreased. • Rspo2 and its receptor Lgr5, as well as Sox9 and collagen type 2, are expressed in differentiating ATDC5 chondrogenic cells. • In ATDC5 cells, Rspo2 decreases

  10. Radiation-induced nerve root degeneration and hypertrophic neuropathy in the lumbosacral spinal cord of rats: The relation with changes in aging rats

    International Nuclear Information System (INIS)

    Kogel, A.J. van der

    1977-01-01

    Three-month-old WAG Rij rats were irradiated with 300 kV X-rays on the lumbar region of the spinal column with doses below the level for causing paralysis due to radiation radiculomyelopathy. 8-9 months after irradiation. degeneration of predominantly the ventral nerve roots of the cauda equina was observed. Three stages were distinguishable: I) Demyelination and proliferation of Schwann cells: II) Local swelling of ventral nerve roots, with concentric layers of Schwann cells resembling hypertrophic neuropathy: III) Malignant Schwannoma, invading roots and spinal cord. It is concluded that the degenerative and proliferative lesions represent a continuous series of stages of slowly progressive lesions. The ventral nerve root degeneration (Ist stage) is similar to that observed in aging, unirradiated rats, normally developing at the age of 18-20 months. (orig.) [de

  11. [Usefullness of Beta-blocker for Hemodynamic Changes Induced by Uterotonic Drug in a Patient with Hypertrophic Obstructive Cardiomyopathy Undergoing Elective Cesarean Section].

    Science.gov (United States)

    Tsukano, Yuri; Sugita, Michiko; Ikuta, Yoshihiro; Yamamoto, Tatsuo

    2015-06-01

    Combined spinal-epidural anesthesia (CSEA) was given to a 27-year-old woman with hypertrophic obstructive cardiomyopathy (HOCM) for a selective cesarean section. After the injection of uterotonic drug via uterine muscle and a vein after delivery, the patient developed dyspnea, tachycardia, ST-change on elecrocardiogram and hypotension. It is important in HOCM patients to control heart rate and left ventricular contractile force. We started to infuse beta-blocker (landiolol, 10 μg x kg(-1) x min(-1)) and improved these symptoms of the patient. This case demonstrates that CSEA is safe for HOCM patients and beta-blocker is effective to improve hemodynamic changes induced by uterotonic drug in these patients.

  12. Three-dimensional speckle tracking echocardiography allows detailed evaluation of left atrial function in hypertrophic cardiomyopathy--insights from the MAGYAR-Path Study.

    Science.gov (United States)

    Domsik, Péter; Kalapos, Anita; Chadaide, Számi; Sepp, Róbert; Hausinger, Péter; Forster, Tamás; Nemes, Attila

    2014-11-01

    Hypertrophic cardiomyopathy (HCM) represents a generalized myopathic process affecting both ventricular and atrial myocardium. Reduced left atrial (LA) function was demonstrated in HCM by different methods. Three-dimensional (3D) speckle tracking echocardiography (STE) has just been introduced for the evaluation of LA. This study was designed to compare 3DSTE-derived LA volumetric and strain parameters in HCM with healthy controls. The study comprised 23 consecutive HCM patients (mean age: 48.5 ± 15.1 years, 14 men). Their results were compared to 23 age- and gender-matched healthy controls. Complete two-dimensional Doppler echocardiography and 3DSTE have been performed in all cases. Calculated LA maximum (66.4 ± 20.4 mL vs. 36.0 ± 6.1 mL, P echocardiography allows detailed evaluation of LA (dys) function in HCM by volumetric and strain measurements. © 2014, Wiley Periodicals, Inc.

  13. Part two

    DEFF Research Database (Denmark)

    Nielsen, Mads Pagh; Kær, Søren Knudsen; Korsgaard, Anders

    2008-01-01

    A novel micro combined heat and power system and a dynamic model thereof were presented in part one of the publication. In the following, the control system and dynamic performance of the system are presented. The model is subjected to a measured consumption pattern of 25 Danish single family...... houses with measurements of heat, power and hot water consumption every 15th minute during one year. Three scenarios are analyzed ranging from heat following only (grid compensation for electricity) to heat and power following with net export of electricity during high and peak load hours. Average...

  14. Hypertensive heart disease versus hypertrophic cardiomyopathy: multi-parametric cardiovascular magnetic resonance discriminators when end-diastolic wall thickness ≥ 15 mm

    Energy Technology Data Exchange (ETDEWEB)

    Rodrigues, Jonathan C.L. [University Hospitals Bristol NHS Foundation Trust, NIHR Bristol Cardiovascular Biomedical Research Unit, Cardiac Magnetic Resonance Department, Bristol Heart Institute (United Kingdom); University of Bristol, School of Physiology, Pharmacology and Neuroscience, Faculty of Biomedical Sciences (United Kingdom); Rohan, Stephen [University of Bristol, Medical School, Faculty of Medicine and Dentistry (United Kingdom); Ghosh Dastidar, Amardeep; Harries, Iwan; Lawton, Christopher B. [University Hospitals Bristol NHS Foundation Trust, NIHR Bristol Cardiovascular Biomedical Research Unit, Cardiac Magnetic Resonance Department, Bristol Heart Institute (United Kingdom); Ratcliffe, Laura E.; Burchell, Amy E.; Nightingale, Angus K. [University Hospitals Bristol NHS Foundation Trust, CardioNomics Research Group, Clinical Research and Imaging Centre, Bristol Heart Institute (United Kingdom); Hart, Emma C.; Paton, Julian F.R. [University of Bristol, School of Physiology, Pharmacology and Neuroscience, Faculty of Biomedical Sciences (United Kingdom); University Hospitals Bristol NHS Foundation Trust, CardioNomics Research Group, Clinical Research and Imaging Centre, Bristol Heart Institute (United Kingdom); Hamilton, Mark C.K. [University Hospitals Bristol NHS Foundation Trust, Department of Radiology, Bristol Royal Infirmary (United Kingdom); Manghat, Nathan E. [University Hospitals Bristol NHS Foundation Trust, NIHR Bristol Cardiovascular Biomedical Research Unit, Cardiac Magnetic Resonance Department, Bristol Heart Institute (United Kingdom); University Hospitals Bristol NHS Foundation Trust, Department of Radiology, Bristol Royal Infirmary (United Kingdom)

    2017-03-15

    European guidelines state left ventricular (LV) end-diastolic wall thickness (EDWT) ≥15 mm suggests hypertrophic cardiomyopathy (HCM), but distinguishing from hypertensive heart disease (HHD) is challenging. We identify cardiovascular magnetic resonance (CMR) predictors of HHD over HCM when EDWT ≥15 mm. 2481 consecutive clinical CMRs between 2014 and 2015 were reviewed. 464 segments from 29 HCM subjects with EDWT ≥15 mm but without other cardiac abnormality, hypertension or renal impairment were analyzed. 432 segments from 27 HHD subjects with EDWT ≥15 mm but without concomitant cardiac pathology were analyzed. Magnitude and location of maximal EDWT, presence of late gadolinium enhancement (LGE), LV asymmetry (>1.5-fold opposing segment) and systolic anterior motion of the mitral valve (SAM) were measured. Multivariate logistic regression was performed. Significance was defined as p<0.05. HHD and HCM cohorts were age-/gender-matched. HHD had significantly increased indexed LV mass (110±27 g/m{sup 2} vs. 91±31 g/m{sup 2}, p=0.016) but no difference in site or magnitude of maximal EDWT. Mid-wall LGE was significantly more prevalent in HCM. Elevated indexed LVM, mid-wall LGE and absence of SAM were significant multivariate predictors of HHD, but LV asymmetry was not. Increased indexed LV mass, absence of mid-wall LGE and absence of SAM are better CMR discriminators of HHD from HCM than EDWT ≥15 mm. circle Hypertrophic cardiomyopathy (HCM) is often diagnosed with end-diastolic wall thickness ≥15 mm. (orig.)

  15. Hypertensive heart disease versus hypertrophic cardiomyopathy: multi-parametric cardiovascular magnetic resonance discriminators when end-diastolic wall thickness ≥ 15 mm

    International Nuclear Information System (INIS)

    Rodrigues, Jonathan C.L.; Rohan, Stephen; Ghosh Dastidar, Amardeep; Harries, Iwan; Lawton, Christopher B.; Ratcliffe, Laura E.; Burchell, Amy E.; Nightingale, Angus K.; Hart, Emma C.; Paton, Julian F.R.; Hamilton, Mark C.K.; Manghat, Nathan E.

    2017-01-01

    European guidelines state left ventricular (LV) end-diastolic wall thickness (EDWT) ≥15 mm suggests hypertrophic cardiomyopathy (HCM), but distinguishing from hypertensive heart disease (HHD) is challenging. We identify cardiovascular magnetic resonance (CMR) predictors of HHD over HCM when EDWT ≥15 mm. 2481 consecutive clinical CMRs between 2014 and 2015 were reviewed. 464 segments from 29 HCM subjects with EDWT ≥15 mm but without other cardiac abnormality, hypertension or renal impairment were analyzed. 432 segments from 27 HHD subjects with EDWT ≥15 mm but without concomitant cardiac pathology were analyzed. Magnitude and location of maximal EDWT, presence of late gadolinium enhancement (LGE), LV asymmetry (>1.5-fold opposing segment) and systolic anterior motion of the mitral valve (SAM) were measured. Multivariate logistic regression was performed. Significance was defined as p<0.05. HHD and HCM cohorts were age-/gender-matched. HHD had significantly increased indexed LV mass (110±27 g/m"2 vs. 91±31 g/m"2, p=0.016) but no difference in site or magnitude of maximal EDWT. Mid-wall LGE was significantly more prevalent in HCM. Elevated indexed LVM, mid-wall LGE and absence of SAM were significant multivariate predictors of HHD, but LV asymmetry was not. Increased indexed LV mass, absence of mid-wall LGE and absence of SAM are better CMR discriminators of HHD from HCM than EDWT ≥15 mm. circle Hypertrophic cardiomyopathy (HCM) is often diagnosed with end-diastolic wall thickness ≥15 mm. (orig.)

  16. Regulation of inward rectifier potassium current ionic channel remodeling by AT1 -Calcineurin-NFAT signaling pathway in stretch-induced hypertrophic atrial myocytes.

    Science.gov (United States)

    He, Jionghong; Xu, Yanan; Yang, Long; Xia, Guiling; Deng, Na; Yang, Yongyao; Tian, Ye; Fu, Zenan; Huang, Yongqi

    2018-05-02

    Previous studies have shown that the activation of angiotensin II receptor type I (AT 1 ) is attributed to cardiac remodeling stimulated by increased heart load, and that it is followed by the activation of the calcineurin-nuclear factor of activated T-cells (NFAT) signaling pathway. Additionally, AT 1 has been found to be a regulator of cardiocyte ionic channel remodeling, and calcineurin-NFAT signals participate in the regulation of cardiocyte ionic channel expression. A hypothesis therefore follows that stretch stimulation may regulate cardiocyte ionic channel remodeling by activating the AT 1 -calcineurin-NFAT pathway. Here, we investigated the role of the AT 1 -calcineurin-NFAT pathway in the remodeling of inward rectifier potassium (I k1 ) channel, in addition to its role in changing action potential, in stretch-induced hypertrophic atrial myocytes of neonatal rats. Our results showed that increased stretch significantly led to atrial myocytes hypertrophy; it also increased the activity of calcineurin enzymatic activity, which was subsequently attenuated by telmisartan or cyclosporine-A. The level of NFAT 3 protein in nuclear extracts, the mRNA and protein expression of Kir2.1 in whole cell extracts, and the density of I k1 were noticeably increased in stretched samples. Stretch stimulation significantly shortened the action potential duration (APD) of repolarization at the 50% and 90% level. Telmisartan, cyclosporine-A, and 11R-VIVIT attenuated stretch-induced alterations in the levels of NFAT 3 , mRNA and protein expression of Kir2.1, the density of I k1 , and the APD. Our findings suggest that the AT 1 -calcineurin-NFAT signaling pathway played an important role in regulating I k1 channel remodeling and APD change in stretch-induced hypertrophic atrial myocytes of neonatal rats. This article is protected by copyright. All rights reserved.

  17. The N-terminal cleavage of chondromodulin-I in growth-plate cartilage at the hypertrophic and calcified zones during bone development.

    Directory of Open Access Journals (Sweden)

    Shigenori Miura

    Full Text Available Chondromodulin-I (ChM-I is a 20-25 kDa anti-angiogenic glycoprotein in cartilage matrix. In the present study, we identified a novel 14-kDa species of ChM-I by immunoblotting, and purified it by immunoprecipitation with a newly raised monoclonal antibody against ChM-I. The N-terminal amino acid sequencing indicated that it was an N-terminal truncated form of ChM-I generated by the proteolytic cleavage at Asp37-Asp38. This 14-kDa ChM-I was shown by the modified Boyden chamber assay to have very little inhibitory activity on the VEGF-A-induced migration of vascular endothelial cells in contrast to the intact 20-25 kDa form of ChM-I (ID50 = 8 nM. Immunohistochemistry suggested that 20-25 kDa ChM-I was exclusively localized in the avascular zones, i.e. the resting, proliferating, and prehypertrophic zones, of the cartilaginous molds of developing long bone, whereas the 14-kDa form of ChM-I was found in hypertrophic and calcified zones. Immunoblotting demonstrated that mature growth-plate chondrocytes isolated from rat costal cartilage actively secrete ChM-I almost exclusively as the intact 20-25 kDa form into the medium in primary culture. Taken together, our results suggest that intact 20-25 kDa ChM-I is stored as a component of extracellular matrix in the avascular cartilage zones, but it is inactivated by a single N-terminal proteolytic cleavage in the hypertrophic zone of growth-plate cartilage.

  18. Controlling cyanobacterial blooms in hypertrophic Lake Taihu, China: will nitrogen reductions cause replacement of non-N2 fixing by N2 fixing taxa?

    Directory of Open Access Journals (Sweden)

    Hans W Paerl

    Full Text Available Excessive anthropogenic nitrogen (N and phosphorus (P inputs have caused an alarming increase in harmful cyanobacterial blooms, threatening sustainability of lakes and reservoirs worldwide. Hypertrophic Lake Taihu, China's third largest freshwater lake, typifies this predicament, with toxic blooms of the non-N2 fixing cyanobacteria Microcystis spp. dominating from spring through fall. Previous studies indicate N and P reductions are needed to reduce bloom magnitude and duration. However, N reductions may encourage replacement of non-N2 fixing with N2 fixing cyanobacteria. This potentially counterproductive scenario was evaluated using replicate, large (1000 L, in-lake mesocosms during summer bloom periods. N+P additions led to maximum phytoplankton production. Phosphorus enrichment, which promoted N limitation, resulted in increases in N2 fixing taxa (Anabaena spp., but it did not lead to significant replacement of non-N2 fixing with N2 fixing cyanobacteria, and N2 fixation rates remained ecologically insignificant. Furthermore, P enrichment failed to increase phytoplankton production relative to controls, indicating that N was the most limiting nutrient throughout this period. We propose that Microcystis spp. and other non-N2 fixing genera can maintain dominance in this shallow, highly turbid, nutrient-enriched lake by outcompeting N2 fixing taxa for existing sources of N and P stored and cycled in the lake. To bring Taihu and other hypertrophic systems below the bloom threshold, both N and P reductions will be needed until the legacy of high N and P loading and sediment nutrient storage in these systems is depleted. At that point, a more exclusive focus on P reductions may be feasible.

  19. Musculoskeletal disorders associated with HIV infection and AIDS. Part II: Non-infectious musculoskeletal conditions

    International Nuclear Information System (INIS)

    Tehranzadeh, Jamshid; Ter-Oganesyan, Ramon R.; Steinbach, Lynne S.

    2004-01-01

    This section of a two-part series on musculoskeletal disorders associated with HIV infection and AIDS reviews the non-infectious musculoskeletal conditions. In the first part, the infectious conditions were reviewed. The non-infectious conditions include polymyositis, drug-induced myopathy, myositis ossificans, adhesive capsulitis, avascular necrosis, bone marrow abnormalities, and hypertrophic osteoarthropathy. Inflammatory and reactive arthropathies are more prevalent in HIV-positive individuals, and a separate section is dedicated to these conditions, including Reiter's syndrome, psoriatic arthritis, HIV-associated arthritis, painful articular syndrome, and acute symmetric polyarthritis. Lastly, we include a discussion of HIV-related neoplastic processes that affect the musculoskeletal system, namely Kaposi's sarcoma and non-Hodgkin's lymphoma. (orig.)

  20. Musculoskeletal disorders associated with HIV infection and AIDS. Part II: Non-infectious musculoskeletal conditions

    Energy Technology Data Exchange (ETDEWEB)

    Tehranzadeh, Jamshid [Department of Radiological Sciences, University of California, Irvine, CA (United States); Department of Radiological Sciences, Orange, CA (United States); Ter-Oganesyan, Ramon R. [College of Medicine, University of California, Irvine, CA (United States); Steinbach, Lynne S. [Department of Radiological Sciences, University of California, San Francisco (United States)

    2004-06-01

    This section of a two-part series on musculoskeletal disorders associated with HIV infection and AIDS reviews the non-infectious musculoskeletal conditions. In the first part, the infectious conditions were reviewed. The non-infectious conditions include polymyositis, drug-induced myopathy, myositis ossificans, adhesive capsulitis, avascular necrosis, bone marrow abnormalities, and hypertrophic osteoarthropathy. Inflammatory and reactive arthropathies are more prevalent in HIV-positive individuals, and a separate section is dedicated to these conditions, including Reiter's syndrome, psoriatic arthritis, HIV-associated arthritis, painful articular syndrome, and acute symmetric polyarthritis. Lastly, we include a discussion of HIV-related neoplastic processes that affect the musculoskeletal system, namely Kaposi's sarcoma and non-Hodgkin's lymphoma. (orig.)

  1. (segunda parte

    Directory of Open Access Journals (Sweden)

    José María Oliva-Martínez

    2004-01-01

    Full Text Available Este trabajo es la continuación de otro anterior (Oliva, 2004, ambos dedicados al estudio de la importancia del pensamiento analógico en la construcción histórica de la noción de fuerza gravitatoria y del modelo de Sistema Solar. En aquella ocasión analizamos dicho papel durante el período comprendido desde las antiguas civilizaciones hasta llegar a la revolución copernicana con científicos como Copérnico, Gilbert, Kepler o el propio Galileo. En esta segunda parte, se continúa con algunos de los razonamientos analógicos proporcionados desde la vertiente mecanicista, capitaneada por Descartes y desde la tradición subsiguiente que se desarrolló en línea con la utilización del método de la analogía como criterio argumentativo (Huyghens, Hooke, Bernoulli, etc.. Dedicamos asimismo un capítulo aparte a la figura de Newton, quien continúa con dicha tradición en su intento de explicar la naturaleza de la gravitación. Finalmente se procede, a modo de síntesis, a realizar una clasificación de distintos tipos de razonamientos analógicos aportados en el desarrollo histórico en torno a estos temas, estudiando el papel científico y divulgativo de cada uno

  2. The L‐type Ca2+ channel facilitates abnormal metabolic activity in the cTnI‐G203S mouse model of hypertrophic cardiomyopathy

    Science.gov (United States)

    Viola, Helena; Johnstone, Victoria; Cserne Szappanos, Henrietta; Richman, Tara; Tsoutsman, Tatiana; Filipovska, Aleksandra; Semsarian, Christopher

    2016-01-01

    Key points Genetic mutations in cardiac troponin I (cTnI) are associated with development of hypertrophic cardiomyopathy characterized by myocyte remodelling, disorganization of cytoskeletal proteins and altered energy metabolism.The L‐type Ca2+ channel is the main route for calcium influx and is crucial to cardiac excitation and contraction. The channel also regulates mitochondrial function in the heart by a functional communication between the channel and mitochondria via the cytoskeletal network.We find that L‐type Ca2+ channel kinetics are altered in cTnI‐G203S cardiac myocytes and that activation of the channel causes a significantly greater increase in mitochondrial membrane potential and metabolic activity in cTnI‐G203S cardiac myocytes.These responses occur as a result of impaired communication between the L‐type Ca2+ channel and cytoskeletal protein F‐actin, involving decreased movement of actin–myosin and block of the mitochondrial voltage‐dependent anion channel, resulting in a ‘hypermetabolic’ mitochondrial state.We propose that L‐type Ca2+ channel antagonists, such as diltiazem, might be effective in reducing the cardiomyopathy by normalizing mitochondrial metabolic activity. Abstract Genetic mutations in cardiac troponin I (cTnI) account for 5% of families with hypertrophic cardiomyopathy. Hypertrophic cardiomyopathy is associated with disorganization of cytoskeletal proteins and altered energy metabolism. The L‐type Ca2+ channel (ICa‐L) plays an important role in regulating mitochondrial function. This involves a functional communication between the channel and mitochondria via the cytoskeletal network. We investigate the role of ICa‐L in regulating mitochondrial function in 25‐ to 30‐week‐old cardiomyopathic mice expressing the human disease‐causing mutation Gly203Ser in cTnI (cTnI‐G203S). The inactivation rate of ICa‐L is significantly faster in cTnI‐G203S myocytes [cTnI‐G203S: τ1 = 40.68 ± 3.22, n

  3. Efficacy of IPL device combined with intralesional corticosteroid injection for the treatment of keloids and hypertrophic scars with regards to the recovery of skin barrier function: A pilot study.

    Science.gov (United States)

    Kim, Dong Young; Park, Hyun Sun; Yoon, Hyun-Sun; Cho, Soyun

    2015-10-01

    Keloids and hypertrophic scars are prevalent and psychologically distressful dermatologic conditions. Various treatment modalities have been tried but without complete success by any one method. We evaluated the efficacy of a combination of intense pulsed light (IPL) device and intralesional corticosteroid injection for the treatment of keloids and hypertrophic scars with respect to the recovery of skin barrier function. Totally 52 Korean patients were treated by the combined treatment at 4-8-week intervals. Using digital photographs, changes in scar appearance were assessed with modified Vancouver Scar Scale (MVSS), physicians' global assessment (PGA) and patient's satisfaction score. In 12 patients, the stratum corneum (SC) barrier function was assessed by measuring transepidermal water loss (TEWL) and SC capacitance. Most scars demonstrated significant clinical improvement in MVSS, PGA and patient's satisfaction score after the combined therapy. A significant decrease of TEWL and elevation of SC capacitance were also documented after the treatment. The combination therapy (IPL + corticosteroid injection) not only improves the appearance of keloids and hypertrophic scars but also increases the recovery level of skin hydration status in terms of the skin barrier function.

  4. Effects of verapamil on left ventricular systolic and diastolic function in patients with hypertrophic cardiomyopathy: pressure-volume analysis with a nonimaging scintillation probe.

    Science.gov (United States)

    Bonow, R O; Ostrow, H G; Rosing, D R; Cannon, R O; Lipson, L C; Maron, B J; Kent, K M; Bacharach, S L; Green, M V

    1983-11-01

    To investigate the effects of verapamil on left ventricular systolic and diastolic function in patients with hypertrophic cardiomyopathy, we studied 14 patients at catheterization with a nonimaging scintillation probe before and after serial intravenous infusions of low-, medium-, and high-dose verapamil (total dose 0.17 to 0.72 mg/kg). Percent change in radionuclide stroke counts after verapamil correlated well with percent change in thermodilution stroke volume (r = .87), and changes in diastolic and systolic counts were used to assess relative changes in left ventricular volumes after verapamil. Verapamil produced dose-related increases in end-diastolic counts (19 +/- 9% increase; p less than .001), end-systolic counts (91 +/- 54% increase; p less than .001), and stroke counts (7 +/- 10% increase; p less than .02). This was associated with a decrease in ejection fraction (83 +/- 8% control, 73 +/- 10% verapamil; p less than .001) and, in the 10 patients with left ventricular outflow tract gradients, a reduction in gradient (62 +/- 27 mm Hg control, 32 +/- 35 mm Hg verapamil; p less than .01). The end-systolic pressure-volume relation was shifted downward and rightward in all patients, suggesting a negative inotropic effect. In 10 patients, left ventricular pressure-volume loops were constructed with simultaneous micromanometer pressure recordings and the radionuclide time-activity curve. In five patients, verapamil shifted the diastolic pressure-volume curve downward and rightward, demonstrating improved pressure-volume relations despite the negative inotropic effect, and also increased the peak rate of rapid diastolic filling. In the other five patients, the diastolic pressure-volume relation was unaltered by verapamil, and increased end-diastolic volumes occurred at higher end-diastolic pressures; in these patients, the peak rate of left ventricular diastolic filling was not changed by verapamil. The negative inotropic effects of intravenous verapamil are

  5. [Role and related mechanism of S1P/S1P1 signal pathway during post conditioning of hypertrophic cardiomyocytes].

    Science.gov (United States)

    Bao, X H; Li, H X; Tao, J; Li, X M; Yang, Y N; Ma, Y T; Chen, B D

    2016-05-24

    To study the role and mechanism of sphingosine-1-phosphate (S1P)/ sphingosine-1-phosphate receptor 1(S1P1) signal pathway during post conditioning of hypertrophic cardiomyocytes. Neonatal rat cardiomyocytes were isolated and cultured, then stimulated by norepinephrine (NE) to induce cardiomyocytes hypertrophy. Using tri-gas incubator to create hypoxia and reoxygenation enviroment to mimic ischemia-reperfusion and postconditioning. Hypertrophic cardiomyoctyes were divided into five groups according to the presence or absence of various drugs and postconditiong and relevant signal pathways changes were detected: (1) IPost group (hypoxia+ postconditioning); (2) IPost+ S1P group (cells were pretreated with S1P (1 μmol/L) for 2 h before IPost); (3) IPost+ W-146+ S1P group (cells in IPost+ W-146+ S1P group were pretreated with S1P1 inhibitor W-146 (0.4 μmol/L) for 20 min); (4) IPost+ PD98059+ S1P group (cells in IPost+ S1P group were pretreated with MAPK antagonist PD98059 (125 μmol/L) for 20 min); (5) IPost+ LY-294002+ S1P group (cells in IPost+ S1P group were pretreated with PI3K antagonist LY294002 (0.1 μmol/L) for 20 min). Apoptosis was detected by flow cytometry and protein expression of relevant signal pathways were detected by Western blot. (1)Apoptosis rate was significantly increased in hypoxia/reoxygenation (27.90±4.49)% group compared with normal control group (7.97±2.18)%, which could be significantly reduced in IPost group (15.90±1.77)% (all PS1P and IPost+ S1P+ LY-294002 groups than in IPost and IPost+ S1P+ W-146 and IPost+ S1P+ PD98059 group (all PS1P and IPost+ S1P+ LY-294002 group than in IPost and IPost+ S1P+ W-146 group and IPost+ S1P+ PD98059 group (all PS1P+ W-146 and IPost+ S1P+ PD98059 groups. p-ERK1/2 and p-Akt levels in IPost+ S1P+ W-146 group and IPost+ S1P+ PD98059 were similar as in IPost group. S1P can play protective role on NE induced cardiomyocytes hypertrophy during post conditioning through downregulating caspase-3 expression and

  6. Temporal changes in carbon and nitrogen stable isotope ratios of macrozoobenthos on an artificial tidal flat facing a hypertrophic canal, inner Tokyo Bay

    International Nuclear Information System (INIS)

    Kanaya, Gen; Nakamura, Yasuo; Koizumi, Tomoyoshi; Yamada, Katsumasa; Koshikawa, Hiroshi; Kohzu, Ayato; Maki, Hideaki

    2013-01-01

    Highlights: • Temporal changes in food web structure were analyzed in a tidal flat in a hypertrophic coastal bay. • Microphytobenthos mainly supported the benthic food web throughout seasons. • Phytoplankton and terrestrial detritus were utilized after red tides and urban runoffs. • Seasonal changes in consumer-δ 15 N was much larger in inner Tokyo Bay than in other estuaries. • This study showed specific characteristics of benthic food web in highly urbanized estuaries. -- Abstract: Temporal changes in benthic food web structure were analyzed in an artificial tidal flat in inner Tokyo Bay, Japan, using carbon and nitrogen stable isotope ratios (δ 13 C and δ 15 N). Microphytobenthos were the most important food sources of macrozoobenthos, due to high microphytobenthic biomass on the tidal flat, while phytoplankton in canal water (canal POM PP ), terrestrial materials from urban surface runoff (canal POM TM ), and marsh plants were less important. Dietary contribution of microphytobenthos was highest in April to June, while decreased towards December owing to the supply of canal POM PP and canal POM TM following red tides and heavy rainfall events in summer to fall. Temporal changes in δ 15 N (Δδ 15 N) of consumer corresponded well to the 15 N-enrichment in canal POM PP in summer. A meta-analysis showed that the consumer-Δδ 15 N was considerably larger in inner Tokyo Bay than those in other estuaries, which may be a specific characteristic of benthic food web in highly urbanized estuaries

  7. Hemodynamic changes in systolic and diastolic function during isoproterenol challenge predicts symptomatic response to myectomy in hypertrophic cardiomyopathy with labile obstruction.

    Science.gov (United States)

    Prasad, Megha; Geske, Jeffrey B; Sorajja, Paul; Ommen, Steve R; Schaff, Hartzell V; Gersh, Bernard J; Nishimura, Rick A

    2016-11-15

    We aimed to assess the utility of changes in systolic and diastolic function by isoproterenol challenge in predicting symptom resolution post-myectomy in selected patients with hypertrophic cardiomyopathy (HCM) and labile obstruction. In a subset of symptomatic HCM patients without resting/provocable obstruction on noninvasive assessment, isoproterenol challenge during hemodynamic catheterization may elicit labile left ventricular outflow tract (LVOT) obstruction, and demonstrate the effect of obstruction on diastolic function. These changes may determine whether patients achieve complete symptom resolution post-myectomy. Between February 2003 and April 2009, 18 symptomatic HCM patients without LVOT obstruction on noninvasive testing underwent isoproterenol provocation and septal myectomy due to presence of provocable gradient and were followed for 4 (IQR 3-7) years. Thirteen (72.2%) had complete symptom resolution, while 5 (27.8%) had improved, but persistent symptoms. Those with provoked gradient >100 mm Hg or increase in left atrial pressure (LAP) with isoproterenol had symptom resolution. Symptomatic HCM patients without LVOT gradient on noninvasive testing may demonstrate labile obstruction with isoproterenol. With isoproterenol, patients with high LVOT gradient or increa