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Sample records for acute dilated cardiomyopathy

  1. Dilated cardiomyopathy

    International Nuclear Information System (INIS)

    Salvatore, M.; Cuocolo, A.

    1988-01-01

    Radionuclide techniques are easily obtainable, noninvasive examinations that provide useful information in the evaluation, diagnosis and management of patients with dilated cardiomyopathy. The gated blood pool scan allows the assessment of ventricular size, configuration, and wall and septal thickness. These data allow the functional class of the cardiomyopathy (congestive, restrictive or hypertrophic) to be defined. Often THallium-201 myocardial perfusion imaging adds further information and is particularly useful in distinguishing congestive cardiomyopathy from severe coronary artery disease and in depicting septal abnormalities in hipertrophic cardiomyopathy. Useful as these techniques are, they are not substitutes for conventional approaches to diagnosis. Careful history taking and physical examination, as well as scrutiny of the electrocardiogram, chest X-ray and echocardiogram should be standard practice for the evaluation of patients with suspected cardiomyopathy. Judicious use of noninvasive techniques may obviate the need for cardiac catheterization in many patients

  2. Dilated cardiomyopathy

    Science.gov (United States)

    ... Valve replacement or repair For advanced cardiomyopathy: A heart transplant may be recommended if standard treatments have not ... accountability. A.D.A.M. is among the first to achieve this important distinction for online health ...

  3. Propionic acidemia in a previously healthy adolescent with acute onset of dilated cardiomyopathy.

    Science.gov (United States)

    Laemmle, Alexander; Balmer, Christian; Doell, Carsten; Sass, Jörn Oliver; Häberle, Johannes; Baumgartner, Matthias R

    2014-07-01

    Propionic acidemia (PA) is a rare autosomal recessive organic aciduria resulting from defects in propionyl-CoA-carboxylase (PCC), a key enzyme of intermediate energy metabolism. PA mostly manifests during the neonatal period, when affected newborns develop severe metabolic acidosis and hyperammonemia. We present a previously healthy teenager, who suffered from acute fatigue and breathlessness. The patient was tachycardic, displayed a precordial heave and a systolic murmur. Cardiac investigations revealed severe dilated cardiomyopathy (DCM). Biochemical work up led to the diagnosis of PA. Remarkably, this patient of consanguineous Hispanic origin was in a good general health condition before the acute onset of DCM. Diagnosis of PA was confirmed by enzymatic and molecular genetic analysis, the latter revealing a novel homozygous mutation in the PCCB gene (c.1229G > A; p.R410Q). Residual PCC enzyme activity of approximately 14 % of normal was detected in patient's lymphocytes and fibroblasts, thereby providing a possible explanation for the hitherto asymptomatic phenotype. Isolated DCM, although rare, can be the leading and/or sole symptom of late-onset PA. Therefore, patients with DCM should receive a comprehensive diagnostic evaluation including selective screening for inborn errors of metabolism.

  4. Dilated cardiomyopathy following use of xenadrine EFX

    Science.gov (United States)

    Riccioni, Graziano; Speziale, Giuseppe; Scotti, Luca; Bucciarelli, Valentina; Cappetti, Silvia; Nasso, Giuseppe; Gallina, Sabina; Bucciarelli, Tonino

    2015-01-01

    We describe a case of a 35-year-old man presented at the emergency room of our institution with acute onset of dyspnea and dizziness. He was a body builder and had been using Xenadrine EFX for weight loss reduction. The laboratory analyses were normal. A chest radiograph showed an enlarged cardiac silhouette with clear lung fields. Transtoracic two-dimensional color Doppler echocardiography revealed a diffuse hypokinesia with a marked decreased in systolic function and a high teledyastolic diameter. This case document the possible relation to use of Xenadrine EFX for weight loss and the recurrence of dilated cardiomyopathy. PMID:26680256

  5. Genetics Home Reference: familial dilated cardiomyopathy

    Science.gov (United States)

    ... familial dilated cardiomyopathy can include an irregular heartbeat (arrhythmia), shortness of breath (dyspnea), extreme tiredness (fatigue), fainting ... Celebrates Its 15th Anniversary National DNA Day 2018 Newborn Screening Saves Lives Act Turns 10 All Bulletins ...

  6. 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)....

  7. Mitochondrial Cardiomyopathy Presenting as Dilated Phase of Hypertrophic Cardiomyopathy Diagnosed with Histological and Genetic Analyses

    Directory of Open Access Journals (Sweden)

    Toshiki Kuno

    2017-01-01

    Full Text Available We report a case with 46-year-old man diagnosed with mitochondrial cardiomyopathy in the dilated phase of hypertrophic cardiomyopathy. Since cardiac magnetic resonance imaging, beta-methyl-p-I123-iodophenyl-pentadecanoic myocardial scintigraphy, and positron emission tomography/computed tomography revealed no remarkable findings, we performed electron microscopic examination, which aided in diagnosing mitochondrial cardiomyopathy. Muscle biopsy was also compatible with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes and DNA analysis also concluded it. Since muscle biopsy is less invasive for patients compared to endomyocardial biopsy, cardiologists need to consider it. The diagnosis of mitochondrial cardiomyopathy is helpful because it is a genetic condition and also for consideration of device therapy, as well as management for acute crisis.

  8. Dilated cardiomyopathy in cats - A case report

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    K. Jeyaraja

    2013-08-01

    Full Text Available Two cats were brought to Madras Veterinary College Teaching Hospital with the history and clinical signs suggestive of congestive heart failure ie, coughing, exercise intolerance, dyspnea, abdominal distension etc. There was history of feeding the cat with home made diet in one case and in other with commercial dog food. Based on electrocardiographic, radiographic and echocardiographic findings, the diagnosis of dilated cardiomyopathy was done in both the cases. The cases were managed with enalapril maleate, furosemide, dietary taurine supplementation and other supportive therapy. Among these two cases, one cat died on 2nd day of treatment and the other showed recovery after 8 days of treatment. [Vet World 2013; 6(4.000: 226-227

  9. A Case Report of Reversible Dilated Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Abhishek Singhai

    2014-01-01

    Full Text Available Dilated cardiomyopathy (DCM is mostly an idiopathic disease with a progressive and irreversible course. It carries poor prognosis and outcome. Rarely, a reversible metabolic etiology that is amenable to specific therapy is identified. Alteration in thyroid status can lead to changes in systolic and diastolic function of left ventricle. Heart is sensitive to thyroid hormone changes, and cardiac disorders are commonly associated with both hyper and hypothyroidism. Diastolic dysfunction is the most common abnormality reported in hypothyroidism. In systolic function, prolonged systolic time interval or normal cardiac function has been reported by most workers. DCM is a rare presentation of hypothyroidism. Here, we report a case of 40-year-old female diagnosed with DCM due to hypothyroidism

  10. Shared Genetic Predisposition in Peripartum and Dilated Cardiomyopathies.

    Science.gov (United States)

    Ware, James S; Li, Jian; Mazaika, Erica; Yasso, Christopher M; DeSouza, Tiffany; Cappola, Thomas P; Tsai, Emily J; Hilfiker-Kleiner, Denise; Kamiya, Chizuko A; Mazzarotto, Francesco; Cook, Stuart A; Halder, Indrani; Prasad, Sanjay K; Pisarcik, Jessica; Hanley-Yanez, Karen; Alharethi, Rami; Damp, Julie; Hsich, Eileen; Elkayam, Uri; Sheppard, Richard; Kealey, Angela; Alexis, Jeffrey; Ramani, Gautam; Safirstein, Jordan; Boehmer, John; Pauly, Daniel F; Wittstein, Ilan S; Thohan, Vinay; Zucker, Mark J; Liu, Peter; Gorcsan, John; McNamara, Dennis M; Seidman, Christine E; Seidman, Jonathan G; Arany, Zoltan

    2016-01-21

    Background Peripartum cardiomyopathy shares some clinical features with idiopathic dilated cardiomyopathy, a disorder caused by mutations in more than 40 genes, including TTN, which encodes the sarcomere protein titin. Methods In 172 women with peripartum cardiomyopathy, we sequenced 43 genes with variants that have been associated with dilated cardiomyopathy. We compared the prevalence of different variant types (nonsense, frameshift, and splicing) in these women with the prevalence of such variants in persons with dilated cardiomyopathy and with population controls. Results We identified 26 distinct, rare truncating variants in eight genes among women with peripartum cardiomyopathy. The prevalence of truncating variants (26 in 172 [15%]) was significantly higher than that in a reference population of 60,706 persons (4.7%, P=1.3×10(-7)) but was similar to that in a cohort of patients with dilated cardiomyopathy (55 of 332 patients [17%], P=0.81). Two thirds of identified truncating variants were in TTN, as seen in 10% of the patients and in 1.4% of the reference population (P=2.7×10(-10)); almost all TTN variants were located in the titin A-band. Seven of the TTN truncating variants were previously reported in patients with idiopathic dilated cardiomyopathy. In a clinically well-characterized cohort of 83 women with peripartum cardiomyopathy, the presence of TTN truncating variants was significantly correlated with a lower ejection fraction at 1-year follow-up (P=0.005). Conclusions The distribution of truncating variants in a large series of women with peripartum cardiomyopathy was remarkably similar to that found in patients with idiopathic dilated cardiomyopathy. TTN truncating variants were the most prevalent genetic predisposition in each disorder.

  11. Intraventricular vortex properties in nonischemic dilated cardiomyopathy

    Science.gov (United States)

    Benito, Yolanda; Alhama, Marta; Yotti, Raquel; Martínez-Legazpi, Pablo; del Villar, Candelas Pérez; Pérez-David, Esther; González-Mansilla, Ana; Santa-Marta, Cristina; Barrio, Alicia; Fernández-Avilés, Francisco; del Álamo, Juan C.

    2014-01-01

    Vortices may have a role in optimizing the mechanical efficiency and blood mixing of the left ventricle (LV). We aimed to characterize the size, position, circulation, and kinetic energy (KE) of LV main vortex cores in patients with nonischemic dilated cardiomyopathy (NIDCM) and analyze their physiological correlates. We used digital processing of color-Doppler images to study flow evolution in 61 patients with NIDCM and 61 age-matched control subjects. Vortex features showed a characteristic biphasic temporal course during diastole. Because late filling contributed significantly to flow entrainment, vortex KE reached its maximum at the time of the peak A wave, storing 26 ± 20% of total KE delivered by inflow (range: 1–74%). Patients with NIDCM showed larger and stronger vortices than control subjects (circulation: 0.008 ± 0.007 vs. 0.006 ± 0.005 m2/s, respectively, P = 0.02; KE: 7 ± 8 vs. 5 ± 5 mJ/m, P = 0.04), even when corrected for LV size. This helped confining the filling jet in the dilated ventricle. The vortex Reynolds number was also higher in the NIDCM group. By multivariate analysis, vortex KE was related to the KE generated by inflow and to chamber short-axis diameter. In 21 patients studied head to head, Doppler measurements of circulation and KE closely correlated with phase-contract magnetic resonance values (intraclass correlation coefficient = 0.82 and 0.76, respectively). Thus, the biphasic nature of filling determines normal vortex physiology. Vortex formation is exaggerated in patients with NIDCM due to chamber remodeling, and enlarged vortices are helpful for ameliorating convective pressure losses and facilitating transport. These findings can be accurately studied using ultrasound. PMID:24414062

  12. Characterization and Long-Term Prognosis of Postmyocarditic Dilated Cardiomyopathy Compared With Idiopathic Dilated Cardiomyopathy.

    Science.gov (United States)

    Merlo, Marco; Anzini, Marco; Bussani, Rossana; Artico, Jessica; Barbati, Giulia; Stolfo, Davide; Gigli, Marta; Muça, Matilda; Naso, Paola; Ramani, Federica; Di Lenarda, Andrea; Pinamonti, Bruno; Sinagra, Gianfranco

    2016-09-15

    Dilated cardiomyopathy (DC) is the final common pathway of different pathogenetic processes and presents a significant prognostic heterogeneity, possibly related to its etiologic variety. The characterization and long-term prognosis of postmyocarditic dilated cardiomyopathy (PM-DC) remain unknown. This study assesses the clinical-instrumental evolution and long-term prognosis of a large cohort of patients with PM-DC. We analyzed 175 patients affected with DC consecutively enrolled from 1993 to 2008 with endomyocardial biopsy (EMB) data available. PM-DC was defined in the presence of borderline myocarditis at EMB or persistent left ventricular dysfunction 1 year after diagnosis of active myocarditis at EMB. Other patients were defined as affected by idiopathic dilated cardiomyopathy (IDC). Analysis of follow-up evaluations was performed at 24, 60, and 120 months. We found 72 PM-DC of 175 enrolled patients (41%). Compared with IDC, patients with PM-DC were more frequently females and less frequently presented a familial history of DC. No other baseline significant differences were found. During the long-term follow-up (median 154, first to third interquartile range 78 to 220 months), patients with PM-DC showed a trend toward slower disease progression. Globally, 18 patients with PM-DC (25%) versus 49 with IDC (48%) experienced death/heart transplantation (p = 0.045). The prognostic advantage for patients with PM-DC became significant beyond 40 months of follow-up. At multivariable time-dependent Cox analysis, PM-DC was confirmed to have a global independent protective role (hazard ratio 0.53, 95% confidence interval 0.28 to 0.97, p = 0.04). In conclusion, PM-DC is characterized by better long-term prognosis compared with IDC. An exhaustive etiologic characterization appears relevant in the prognostic assessment of DC. Copyright © 2016 Elsevier Inc. All rights reserved.

  13. Molecular genetics of dilated cardiomyopathy in the Dobermann dog

    NARCIS (Netherlands)

    Stabej, Polona

    2005-01-01

    Canine dilated cardiomyopathy (DCM) is a disease of the myocardium associated with dilatation and impaired contraction of the ventricles. It primarily affects large and giant breed dogs with Dobermanns being one of the most frequently affected. The high prevalence of DCM in specific breeds suggests

  14. Spontaneous Dilated Cardiomyopathy and Right-Sided Heart Failure as a Differential Diagnosis for Hepatosis Dietetica in a Production Pig.

    Science.gov (United States)

    Collins, Dalis E; Eaton, Kathryn A; Hoenerhoff, Mark J

    2015-08-01

    An experimentally naïve 37.7-kg Yorkshire-crossbred gilt died unexpectedly 2 d after arrival. Necropsy revealed severe dilated cardiomyopathy characterized grossly by markedly dilated ventricles and thinned ventricular walls and interventricular septum. Histologically there was multifocal myofiber attenuation and patchy loss of myofiber cross striations. The liver contained submassive to massive, diffuse hepatic centrilobular hemorrhage and degeneration. These lesions supported a diagnosis of dilated cardiomyopathy with right heart failure and secondary hepatic degeneration due to marked acute passive congestion. To our knowledge, this case is the first report of spontaneous dilated cardiomyopathy in swine and represents a potential diagnostic challenge regarding the differentiation of the cardiac-associated liver lesion from hepatosis dietetica. The diagnosis of dilated cardiomyopathy and right-sided heart failure was supported by the character of the hepatic lesion, absence of typical gross or histologic lesions of mulberry heart disease, and normal selenium levels.

  15. Dobutamine Stress Echocardiography in Patients with Dilated Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Miloradovic Vladimir

    2016-06-01

    Full Text Available A clear distinction between two of the most common forms of dilated cardiomyopathy is very important due to their different prediction and therapeutic approaches. Dobutamine stress echocardiography appears to be a noninvasive selection method due to its clear differentiation potential. Major factors influence test interpretation, resulting in a wide interval of diagnostic accuracy for this test. Fraction flow reserve (FFR is a novel invasive method for estimating coronary artery stenosis responsible for myocardium ischaemia. Decisions about lesion significance in coronary blood vessels have thus far been based on angiographic estimations, but this approach is being replaced by FFR measurements, which serve as a new gold standard and involve a noninvasive test. The goal of this study was to clearly differentiate two forms of dilated cardiomyopathies through analysis of the segmented mobility of the left ventricular wall. Fifty patients were analysed: 20 with ischaemic dilated cardiomyopathy, which was confirmed not only through coronary angiography but also functionally through FFR measurement, and 30 patients with nonischaemic dilated cardiomyopathy, which was confirmed by coronary angiography. A standard dobutamine stress echocardiography protocol was implemented. A positive dobutamine stress echocardiography test was defined as the presence of emerging incidents in segment contractility or worsening of existing incidents in at least one segment. Statistically relevant diff erences in the movement dynamics of a number of differently characterised segments during the observed time intervals (ANOVA p=0.000 was noted in both groups of patients, as was variation in the index value of the summarized mobility of the left chamber wall. In patients with ischaemic cardiomyopathies, regional contractility worsened at the maximum dose of dobutamine; in contrast, this feature slightly improved in nonischaemic cardiomyopathy patients. The results indicate

  16. A Rare Occurance with Epidermolysis Bullosa Disease: Dilated Cardiomyopathy

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    Derya Cimen

    2014-02-01

    Full Text Available Epidermolysis bullosa is a congenital and herediter vesiculobullous disease. Dystrophic form of this disease is characterized by severe malnutrition, failure to thrive, adhesions at fingers, joint contractures related with the formation of scar tissues, carcinoma of the skin, anemia, hipoalbuminemia, wound enfections and sepsis. Rarely, mortal dilated cardiomyopathy may occur in patients. In this report we present a 13 year-old pediatric patient with dilated cardiomyopathy, clinically diagnosed with Epidermolysis bullosa as well as a review of recent related literature.

  17. Cardiac sarcoid: a chameleon masquerading as hypertrophic cardiomyopathy and dilated cardiomyopathy in the same patient.

    Science.gov (United States)

    Agarwal, Anushree; Sulemanjee, Nasir Z; Cheema, Omar; Downey, Francis X; Tajik, A Jamil

    2014-05-01

    Sarcoidosis is a multisystem, granulomatous disease of unknown etiology often seen in young adults, with cardiac involvement in more than one-quarter of sarcoid patients. The clinical presentation of cardiac sarcoid depends upon the location and extent of myocardium involved. Although cardiac sarcoid may produce asymmetrical septal hypertrophy, it is most commonly considered in the differential diagnosis of dilated cardiomyopathy. The hypertrophic stage of cardiac sarcoid is rarely seen. We describe a case of cardiac sarcoid in a young patient wherein a distinctive appearance of the cardiac sarcoid spectrum from "hypertrophic" stage to thinned/scarred stage, masquerading as hypertrophic cardiomyopathy followed by dilated cardiomyopathy, is demonstrated. © 2014, Wiley Periodicals, Inc.

  18. Thallium scintigraphy for the prognosis of idiopathic dilated cardiomyopathy

    International Nuclear Information System (INIS)

    Yabe, Toshikazu; Furuno, Takashi; Kitaoka, Hiroaki; Matsumura, Yoshihisa; Yamasaki, Naohito; Doi, Yoshinori

    2002-01-01

    This study evaluated the significance of perfusion defects demonstrated by thallium-201 and age in the prognosis of patients with idiopathic dilated cardiomyopathy. Seventy-four dilated cardiomyopathy patients underwent thallium scintigraphy as well as clinical and hemodynamic examination. Abnormal perfusion defects were present in 23 of 38 patients aged <60 years (61%) and in 26 of 36 elderly patients aged ≥60 years (72%; NS). Univariate analysis showed that such perfusion defects were a significant predictor of cardiac death only in patients aged <60 years (p=0.015). Stepwise discriminant analysis also revealed that perfusion defects were a significant predictor in patients aged <60 years (Wilks' lambda 0.499, chi-square test 20.2, p=0.003). Perfusion defects were not more important than the history of syncope or stroke in elderly dilated cardiomyopathy patients. Twenty-one patients died of disease-related causes during 58±43 months. The five-year survival rate was better in patients aged <60 years without than in those with perfusion defects (100% vs 58.4%, respectively), but not affected in patients aged ≥60 years (66.7% vs 62.2%). Thallium scintigraphy is valuable for the prognosis of patients with dilated cardiomyopathy aged <60 years who are usually candidates for heart transplantation. Absence of thallium perfusion defects may indicate good long-term prognosis. (author)

  19. Thallium scintigraphy for the prognosis of idiopathic dilated cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Yabe, Toshikazu; Furuno, Takashi; Kitaoka, Hiroaki; Matsumura, Yoshihisa; Yamasaki, Naohito; Doi, Yoshinori [Kochi Medical School, Nankoku (Japan)

    2002-11-01

    This study evaluated the significance of perfusion defects demonstrated by thallium-201 and age in the prognosis of patients with idiopathic dilated cardiomyopathy. Seventy-four dilated cardiomyopathy patients underwent thallium scintigraphy as well as clinical and hemodynamic examination. Abnormal perfusion defects were present in 23 of 38 patients aged <60 years (61%) and in 26 of 36 elderly patients aged {>=}60 years (72%; NS). Univariate analysis showed that such perfusion defects were a significant predictor of cardiac death only in patients aged <60 years (p=0.015). Stepwise discriminant analysis also revealed that perfusion defects were a significant predictor in patients aged <60 years (Wilks' lambda 0.499, chi-square test 20.2, p=0.003). Perfusion defects were not more important than the history of syncope or stroke in elderly dilated cardiomyopathy patients. Twenty-one patients died of disease-related causes during 58{+-}43 months. The five-year survival rate was better in patients aged <60 years without than in those with perfusion defects (100% vs 58.4%, respectively), but not affected in patients aged {>=}60 years (66.7% vs 62.2%). Thallium scintigraphy is valuable for the prognosis of patients with dilated cardiomyopathy aged <60 years who are usually candidates for heart transplantation. Absence of thallium perfusion defects may indicate good long-term prognosis. (author)

  20. Acute peritonitis as the first presentation of valvular cardiomyopathy.

    LENUS (Irish Health Repository)

    Higgins, Nikki

    2012-02-01

    Valvular cardiomyopathy can present a diagnostic challenge in the absence of overt cardiac symptoms. This report describes the case of a 46-year-old woman who presented with acute peritonitis associated with vomiting and abdominal distension. Subsequent abdominal computed tomography and ultrasound revealed bibasal pleural effusions, ascites, and normal ovaries. An echocardiogram revealed that all cardiac chambers were dilated with a global decrease in contractility and severe mitral, tricuspid, and aortic regurgitation. A diagnosis of cardiomyopathy with acute heart failure, secondary to valvular heart disease, was secured. Acute peritonitis as the presenting feature of valvular cardiomyopathy is a rare clinical entity.

  1. Acute peritonitis as the first presentation of valvular cardiomyopathy.

    Science.gov (United States)

    Higgins, Nikki; Burke, John P; McCreery, Charles J

    2012-01-01

    Valvular cardiomyopathy can present a diagnostic challenge in the absence of overt cardiac symptoms. This report describes the case of a 46-year-old woman who presented with acute peritonitis associated with vomiting and abdominal distension. Subsequent abdominal computed tomography and ultrasound revealed bibasal pleural effusions, ascites, and normal ovaries. An echocardiogram revealed that all cardiac chambers were dilated with a global decrease in contractility and severe mitral, tricuspid, and aortic regurgitation. A diagnosis of cardiomyopathy with acute heart failure, secondary to valvular heart disease, was secured. Acute peritonitis as the presenting feature of valvular cardiomyopathy is a rare clinical entity.

  2. Oxidative Stress in Dilated Cardiomyopathy Caused by MYBPC3 Mutation

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    Thomas L. Lynch

    2015-01-01

    Full Text Available Cardiomyopathies can result from mutations in genes encoding sarcomere proteins including MYBPC3, which encodes cardiac myosin binding protein-C (cMyBP-C. However, whether oxidative stress is augmented due to contractile dysfunction and cardiomyocyte damage in MYBPC3-mutated cardiomyopathies has not been elucidated. To determine whether oxidative stress markers were elevated in MYBPC3-mutated cardiomyopathies, a previously characterized 3-month-old mouse model of dilated cardiomyopathy (DCM expressing a homozygous MYBPC3 mutation (cMyBP-C(t/t was used, compared to wild-type (WT mice. Echocardiography confirmed decreased percentage of fractional shortening in DCM versus WT hearts. Histopathological analysis indicated a significant increase in myocardial disarray and fibrosis while the second harmonic generation imaging revealed disorganized sarcomeric structure and myocyte damage in DCM hearts when compared to WT hearts. Intriguingly, DCM mouse heart homogenates had decreased glutathione (GSH/GSSG ratio and increased protein carbonyl and lipid malondialdehyde content compared to WT heart homogenates, consistent with elevated oxidative stress. Importantly, a similar result was observed in human cardiomyopathy heart homogenate samples. These results were further supported by reduced signals for mitochondrial semiquinone radicals and Fe-S clusters in DCM mouse hearts measured using electron paramagnetic resonance spectroscopy. In conclusion, we demonstrate elevated oxidative stress in MYPBC3-mutated DCM mice, which may exacerbate the development of heart failure.

  3. Proposal for a revised definition of dilated cardiomyopathy, hypokinetic non-dilated cardiomyopathy, and its implications for clinical practice

    DEFF Research Database (Denmark)

    Pinto, Yigal M; Elliott, Perry M; Arbustini, Eloisa

    2016-01-01

    In this paper the Working Group on Myocardial and Pericardial Disease proposes a revised definition of dilated cardiomyopathy (DCM) in an attempt to bridge the gap between our recent understanding of the disease spectrum and its clinical presentation in relatives, which is key for early diagnosis...... and the institution of potential preventative measures. We also provide practical hints to identify subsets of the DCM syndrome where aetiology directed management has great clinical relevance....

  4. Subtle abnormalities in contractile function are an early manifestation of sarcomere mutations in dilated cardiomyopathy

    DEFF Research Database (Denmark)

    Lakdawala, Neal K; Thune, Jens J; Colan, Steven D

    2012-01-01

    Sarcomere mutations cause both dilated cardiomyopathy (DCM) and hypertrophic cardiomyopathy (HCM); however, the steps leading from mutation to disease are not well described. By studying mutation carriers before a clinical diagnosis develops, we characterize the early manifestations of sarcomere...

  5. A Case of Dilated Cardiomyopathy Associated with 3-Hydroxy-3-Methylglutaryl-Coenzyme A (HMG CoA Lyase Deficiency

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    Alexander A. C. Leung

    2009-01-01

    Full Text Available 3-hydroxy-3-methylglutaryl-coenzyme A (HMG CoA lyase deficiency is an inborn error of metabolism characterized by impairment of ketogenesis and leucine catabolism resulting in an organic acidopathy. In 1994, a case of dilated cardiomyopathy and fatal arrhythmia was reported in a 7-month-old infant. We report a case of dilated cardiomyopathy in association with HMG CoA lyase deficiency in a 23-year-old man with the acute presentation of heart failure. To our knowledge, this is the first case reported in an adult.

  6. Atlas of the clinical genetics of human dilated cardiomyopathy

    DEFF Research Database (Denmark)

    Haas, Jan; Frese, Karen S; Peil, Barbara

    2015-01-01

    AIM: Numerous genes are known to cause dilated cardiomyopathy (DCM). However, until now technological limitations have hindered elucidation of the contribution of all clinically relevant disease genes to DCM phenotypes in larger cohorts. We now utilized next-generation sequencing to overcome......, leading to 99.1% coverage of the target region with at least 50-fold and a mean read depth of 2415. In this well characterized cohort, we find the highest number of known cardiomyopathy mutations in plakophilin-2, myosin-binding protein C-3, and desmoplakin. When we include yet unknown but predicted......: This is to our knowledge, the first study that comprehensively investigated the genetics of DCM in a large-scale cohort and across a broad gene panel of the known DCM genes. Our results underline the high analytical quality and feasibility of Next-Generation Sequencing in clinical genetic diagnostics and provide...

  7. Diagnosis and assessment of dilated cardiomyopathy: a guideline protocol from the British Society of Echocardiography

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    Thomas Mathew

    2017-06-01

    Full Text Available Heart failure (HF is a debilitating and life-threatening condition, with 5-year survival rate lower than breast or prostate cancer. It is the leading cause of hospital admission in over 65s, and these admissions are projected to rise by more than 50% over the next 25 years. Transthoracic echocardiography (TTE is the first-line step in diagnosis in acute and chronic HF and provides immediate information on chamber volumes, ventricular systolic and diastolic function, wall thickness, valve function and the presence of pericardial effusion, while contributing to information on aetiology. Dilated cardiomyopathy (DCM is the third most common cause of HF and is the most common cardiomyopathy. It is defined by the presence of left ventricular dilatation and left ventricular systolic dysfunction in the absence of abnormal loading conditions (hypertension and valve disease or coronary artery disease sufficient to cause global systolic impairment. This document provides a practical approach to diagnosis and assessment of dilated cardiomyopathy that is aimed at the practising sonographer.

  8. Genotype-specific pathogenic effects in human dilated cardiomyopathy.

    Science.gov (United States)

    Bollen, Ilse A E; Schuldt, Maike; Harakalova, Magdalena; Vink, Aryan; Asselbergs, Folkert W; Pinto, Jose R; Krüger, Martina; Kuster, Diederik W D; van der Velden, Jolanda

    2017-07-15

    Mutations in genes encoding cardiac troponin I (TNNI3) and cardiac troponin T (TNNT2) caused altered troponin protein stoichiometry in patients with dilated cardiomyopathy. TNNI3 p.98trunc resulted in haploinsufficiency, increased Ca 2+ -sensitivity and reduced length-dependent activation. TNNT2 p.K217del caused increased passive tension. A mutation in the gene encoding Lamin A/C (LMNA p.R331Q ) led to reduced maximal force development through secondary disease remodelling in patients suffering from dilated cardiomyopathy. Our study shows that different gene mutations induce dilated cardiomyopathy via diverse cellular pathways. Dilated cardiomyopathy (DCM) can be caused by mutations in sarcomeric and non-sarcomeric genes. In this study we defined the pathogenic effects of three DCM-causing mutations: the sarcomeric mutations in genes encoding cardiac troponin I (TNNI3 p.98truncation ) and cardiac troponin T (TNNT2 p.K217deletion ; also known as the p.K210del) and the non-sarcomeric gene mutation encoding lamin A/C (LMNA p.R331Q ). We assessed sarcomeric protein expression and phosphorylation and contractile behaviour in single membrane-permeabilized cardiomyocytes in human left ventricular heart tissue. Exchange with recombinant troponin complex was used to establish the direct pathogenic effects of the mutations in TNNI3 and TNNT2. The TNNI3 p.98trunc and TNNT2 p.K217del mutation showed reduced expression of troponin I to 39% and 51%, troponin T to 64% and 53%, and troponin C to 73% and 97% of controls, respectively, and altered stoichiometry between the three cardiac troponin subunits. The TNNI3 p.98trunc showed pure haploinsufficiency, increased Ca 2+ -sensitivity and impaired length-dependent activation. The TNNT2 p.K217del mutation showed a significant increase in passive tension that was not due to changes in titin isoform composition or phosphorylation. Exchange with wild-type troponin complex corrected troponin protein levels to 83% of controls in the TNNI3

  9. Genotype‐specific pathogenic effects in human dilated cardiomyopathy

    Science.gov (United States)

    Schuldt, Maike; Harakalova, Magdalena; Vink, Aryan; Asselbergs, Folkert W.; Pinto, Jose R.; Krüger, Martina; Kuster, Diederik W. D.; van der Velden, Jolanda

    2017-01-01

    Key points Mutations in genes encoding cardiac troponin I (TNNI3) and cardiac troponin T (TNNT2) caused altered troponin protein stoichiometry in patients with dilated cardiomyopathy. TNNI3p.98trunc resulted in haploinsufficiency, increased Ca2+‐sensitivity and reduced length‐dependent activation. TNNT2p.K217del caused increased passive tension.A mutation in the gene encoding Lamin A/C (LMNA p.R331Q) led to reduced maximal force development through secondary disease remodelling in patients suffering from dilated cardiomyopathy.Our study shows that different gene mutations induce dilated cardiomyopathy via diverse cellular pathways. Abstract Dilated cardiomyopathy (DCM) can be caused by mutations in sarcomeric and non‐sarcomeric genes. In this study we defined the pathogenic effects of three DCM‐causing mutations: the sarcomeric mutations in genes encoding cardiac troponin I (TNNI3p.98truncation) and cardiac troponin T (TNNT2p.K217deletion; also known as the p.K210del) and the non‐sarcomeric gene mutation encoding lamin A/C (LMNAp.R331Q). We assessed sarcomeric protein expression and phosphorylation and contractile behaviour in single membrane‐permeabilized cardiomyocytes in human left ventricular heart tissue. Exchange with recombinant troponin complex was used to establish the direct pathogenic effects of the mutations in TNNI3 and TNNT2. The TNNI3p.98trunc and TNNT2p.K217del mutation showed reduced expression of troponin I to 39% and 51%, troponin T to 64% and 53%, and troponin C to 73% and 97% of controls, respectively, and altered stoichiometry between the three cardiac troponin subunits. The TNNI3p.98trunc showed pure haploinsufficiency, increased Ca2+‐sensitivity and impaired length‐dependent activation. The TNNT2p.K217del mutation showed a significant increase in passive tension that was not due to changes in titin isoform composition or phosphorylation. Exchange with wild‐type troponin complex corrected troponin protein levels to 83% of

  10. Data of methylome and transcriptome derived from human dilated cardiomyopathy

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    Bong-Seok Jo

    2016-12-01

    Full Text Available Alterations in DNA methylation and gene expression have been implicated in the development of human dilated cardiomyopathy (DCM. Differentially methylated probes (DMPs and differentially expressed genes (DEGs were identified between the left ventricle (LV, a pathological locus for DCM and the right ventricle (RV, a proxy for normal hearts. The data in this DiB are for supporting our report entitled “Methylome analysis reveals alterations in DNA methylation in the regulatory regions of left ventricle development genes in human dilated cardiomyopathy” (Bong-Seok Jo, In-Uk Koh, Jae-Bum Bae, Ho-Yeong Yu, Eun-Seok Jeon, Hae-Young Lee, Jae-Joong Kim, Murim Choi, Sun Shim Choi, 2016 [1].

  11. Multiple Genetic Associations with Irish Wolfhound Dilated Cardiomyopathy

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    Siobhan Simpson

    2016-01-01

    Full Text Available Cardiac disease is a leading cause of morbidity and mortality in dogs and humans, with dilated cardiomyopathy being a large contributor to this. The Irish Wolfhound (IWH is one of the most commonly affected breeds and one of the few breeds with genetic loci associated with the disease. Mutations in more than 50 genes are associated with human dilated cardiomyopathy (DCM, yet very few are also associated with canine DCM. Furthermore, none of the identified canine loci explain many cases of the disease and previous work has indicated that genotypes at multiple loci may act together to influence disease development. In this study, loci previously associated with DCM in IWH were tested for associations in a new cohort both individually and in combination. We have identified loci significantly associated with the disease individually, but no genotypes individually or in pairs conferred a significantly greater risk of developing DCM than the population risk. However combining three loci together did result in the identification of a genotype which conferred a greater risk of disease than the overall population risk. This study suggests multiple rather than individual genetic factors, cooperating to influence DCM risk in IWH.

  12. Frequency and echocardiographic study of dilated cardiomyopathy in children presenting with cardiac failure

    International Nuclear Information System (INIS)

    Khan, M.A.; Mohammad, J.; Hussain, M.

    2004-01-01

    Objective: To evaluate the role of echocardiography in diagnosis of dilated cardiomyopathy as a cause of cardiac failure in children. Design: This was descriptive study. Children presenting with cardiac failure from indoor patients were selected and echocardiography along with chest X- ray, ECG, cardiac enzymes and ASO titre was performed in all patients. Subject: Fifty hospitalized patients with congestive heart failure were selected consecutively from hospitalized patients. Main Outcome: Role of echocardiography in the diagnosis of dilated cardiomyopathy in children presenting with cardiac failure. Results: Out of fifty patients admitted with cardiac failure 27 (54%) cases were found to be dilated cardiomyopathy while congenital heart disease, myocarditis and rheumatic heart disease were found in 12 (24%), 8 (16%) and 3 (6%) cases respectively. Conclusion: Dilated cardiomyopathy is an important cause of cardiac failure in children and echocardiography is an important tool to diagnose and differentiate dilated cardiomyopathy from other causes of cardiac failure. (author)

  13. Magnetic resonance imaging of dilated cardiomyopathy; MRT bei dilatativen Kardiomyopathien

    Energy Technology Data Exchange (ETDEWEB)

    D' Anastasi, M. [Klinikum der Ludwig-Maximilians-Universitaet Muenchen, Campus Grosshadern, Institut fuer Klinische Radiologie, Muenchen (Germany); Greif, M. [Klinikum der Ludwig-Maximilians-Universitaet Muenchen, Campus Grosshadern, Medizinische Klinik und Poliklinik I, Muenchen (Germany); Reiser, M.F.; Theisen, D. [Klinikum der Ludwig-Maximilians-Universitaet Muenchen, Campus Grosshadern, Institut fuer Klinische Radiologie, Muenchen (Germany); Deutsches Zentrum fuer Herzkreislaufforschung (DZHK), Muenchen (Germany)

    2013-01-15

    Dilated cardiomyopathy (DCM) is the most common type of cardiomyopathy with a prevalence of 1 out of 2,500 in adults. Due to mild clinical symptoms in the early phase of the disease, the true prevalence is probably even much higher. Patients present with variable clinical symptoms ranging from mild systolic impairment of left ventricular function to congestive heart failure. Even sudden cardiac death may be the first clinical symptom of DCM. The severity of the disease is defined by the degree of impairment of global left ventricular function. Arrhythmias, such as ventricular or supraventricular tachycardia, atrioventricular (AV) block, ventricular extrasystole and atrial fibrillation are common cardiac manifestations of DCM. Magnetic resonance imaging (MRI) plays an important role in the exact quantification of functional impairment of both ventricles and in the evaluation of regional wall motion abnormalities. With its excellent ability for the assessment of myocardial structure, it is becoming increasingly more important for risk stratification and therapy guidance. (orig.) [German] Die dilatative Kardiomyopathie (DCM) ist die haeufigste Form der Kardiomyopathie mit einer Praevalenz von 1/2500 Erwachsenen. Aufgrund der zunaechst milden klinischen Symptomatik ist jedoch von einer relativ hohen Dunkelziffer auszugehen. Die klinische Praesentation ist variabel, die Schwere der Erkrankung wird vom Ausmass der systolischen Funktionseinschraenkung bestimmt. Herzrhythmusstoerungen, wie ventrikulaere oder supraventrikulaere Tachykardien, AV-Blockierungen, ventrikulaere Extrasystolen und Vorhofflimmern sind moegliche klinische Manifestationen. Bei manchen Patienten ist der ploetzliche Herztod die erste klinische Manifestation der Erkrankung. Die kardiale MRT spielt eine bedeutende Rolle fuer die Beurteilung des Ausmasses der ventrikulaeren Dilatation, Dysfunktion und fuer die Beurteilung regionaler Wandbewegungsstoerungen. Darueber hinaus kann sie zur Anwendung kommen

  14. Ethanol vapour induced dilated cardiomyopathy in chick embryos

    International Nuclear Information System (INIS)

    Kamran, K.; Khan, M.Y.; Minhas, L.A.

    2013-01-01

    Objective: To study the effects of ethanol vapour inhalation on the heart chambers of chick embryo. Methods: The case-control study was conducted at the College of Physicians and Surgeons Pakistan regional centre in Islamabad from January to October 2007. Both experimental and control groups were divided into three sub-groups each, based on the day of the sacrifice. Each group was dissected on day 7, day 10 and day 22 or hatching whichever was earlier. The experimental sub-groups sacrificed on day 7, day 10 and on hatching, were exposed to ethanol vapours till day 6, 9 and 9 of incubation respectively. The diameter of all 4 chambers was measured in experimental hearts and compared with age-matched controls. SPSS 10 was used for statistical analysis. Results: Ethanol vapour exposure caused widening of all heart chambers in the experimental chick embryos sacrificed on day 7 and day 10 compared to the controls. The chambers of newly hatched chick hearts showed dilatation in all the chambers except the left ventricle. Conclusion: Ethanol vapour exposure during development affects the heart, resulting in the widening of all heart chambers. The exposure is as dangerous as drinking alcohol. Alcohol vapour exposure during development leads to progressive dilatation in different heart chambers, producing dilated cardiomyopathy. (author)

  15. Chronic oral exposure to the aldehyde pollutant acrolein induces dilated cardiomyopathy

    Science.gov (United States)

    Ismahil, Mohamed Ameen; Hamid, Tariq; Haberzettl, Petra; Gu, Yan; Chandrasekar, Bysani; Srivastava, Sanjay; Bhatnagar, Aruni

    2011-01-01

    Environmental triggers of dilated cardiomyopathy are poorly understood. Acute exposure to acrolein, a ubiquitous aldehyde pollutant, impairs cardiac function and cardioprotective responses in mice. Here, we tested the hypothesis that chronic oral exposure to acrolein induces inflammation and cardiomyopathy. C57BL/6 mice were gavage-fed acrolein (1 mg/kg) or water (vehicle) daily for 48 days. The dose was chosen based on estimates of human daily unsaturated aldehyde consumption. Compared with vehicle-fed mice, acrolein-fed mice exhibited significant (P acrolein adduct formation indicative of physical translocation of ingested acrolein to the heart. Acrolein also induced myocyte hypertrophy (∼2.2-fold increased myocyte area, P acrolein-exposed hearts, along with upregulated gene expression of proinflammatory cytokines tumor necrosis factor-α and interleukin-1β. Long-term oral exposure to acrolein, at an amount within the range of human unsaturated aldehyde intake, induces a phenotype of dilated cardiomyopathy in the mouse. Human exposure to acrolein may have analogous effects and raise consideration of an environmental, aldehyde-mediated basis for heart failure. PMID:21908791

  16. Cardiovascular genetics: technological advancements and applicability for dilated cardiomyopathy.

    Science.gov (United States)

    Kummeling, G J M; Baas, A F; Harakalova, M; van der Smagt, J J; Asselbergs, F W

    2015-07-01

    Genetics plays an important role in the pathophysiology of cardiovascular diseases, and is increasingly being integrated into clinical practice. Since 2008, both capacity and cost-efficiency of mutation screening of DNA have been increased magnificently due to the technological advancement obtained by next-generation sequencing. Hence, the discovery rate of genetic defects in cardiovascular genetics has grown rapidly and the financial threshold for gene diagnostics has been lowered, making large-scale DNA sequencing broadly accessible. In this review, the genetic variants, mutations and inheritance models are briefly introduced, after which an overview is provided of current clinical and technological applications in gene diagnostics and research for cardiovascular disease and in particular, dilated cardiomyopathy. Finally, a reflection on the future perspectives in cardiogenetics is given.

  17. Pathomorphological Changes of the Myocardium in Canine Dilated Cardiomyopathy (DCM

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    Janus Izabela

    2015-04-01

    Full Text Available The study was conducted on ventricular and atrial wall preparations from 11 dogs with clinically diagnosed dilated cardiomyopathy. After fixation, the specimens were stained with haematoxylin and eosin and Masson-Goldner trichrome technique. Parenchymal changes (fibrosis and fatty infiltration, vascular changes (congestion and coronary vessel wall hypertrophy, degenerative changes (loss of striation, changes in cardiomycyte and nuclei structure, and presence of inflammatory infiltrates (mononuclear and polynuclear were estimated. Complex histological changes in both ventricular and atrial muscles were shown. It was not determined whether the processes occurring in the myocardium have a primary character, or are a consequence of developing heart failure. Such issues will be put under further and more detailed examination.

  18. Basal wall hypercontraction of Takotsubo cardiomyopathy in a patient who had been diagnosed with dilated cardiomyopathy: a case report.

    Science.gov (United States)

    Ichihara, Noboru; Fujita, Shuichi; Kanzaki, Yumiko; Fujisaka, Tomohiro; Ozeki, Michishige; Ishizaka, Nobukazu

    2017-12-12

    Takotsubo cardiomyopathy is characterized by the basal hypercontractility and apical ballooning of the left ventriculum and T-wave inversion in the electrocardiogram. It has been suggested that Takotsubo cardiomyopathy might underlie the pathogenesis of persistent cardiac dysfunction; however, few reports are present demonstrating the advent of Takotsubo cardiomyopathy in patients with idiopathic cardiomyopathy. A 64-year-old women was admitted due to dyspnea on effort and lower extremity edema. She had been diagnosed with idiopathic dilated cardiomyopathy 2.5 years before owing to the reduced left ventricular ejection fraction (24%), normal coronary artery, and interstitial fibrosis of the myocardial samples. On admission, her electrocardiogram showed giant negative T wave in II, III, aVF, and precordial leads. Echocardiography showed dyskinesis of the left ventricular apex and hypercontraction of the basal wall, which had not been observed in the previous examinations. Coronary angiography showed normal coronary arteries, and apical ballooning and basal hypercontractility was confirmed by left ventriculography. On day 15 of admission, contraction of apical wall was recovered, and basal hypercontraction was disappeared. The present case is the first report demonstrating appearance the transient basal wall hypercontraction along with the advent of Takotsubo cardiomyopathy in a patient diagnosed with dilated cardiomyopathy. Whether such findings are indicative of fair prognosis and have the utility of understanding the pathogenesis of dilated cardiomyopathy needs further investigation.

  19. Dilated cardiomyopathy with Graves disease in a young child

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    Yu Jung Choi

    2016-06-01

    Full Text Available Graves disease (GD can lead to complications such as cardiac arrhythmia and heart failure. Although dilated cardiomyopathy (DCMP has been occasionally reported in adults with GD, it is rare in children. We present the case of a 32-month-old boy with DCMP due to GD. He presented with irritability, vomiting, and diarrhea. He also had a history of weight loss over the past few months. On physical examination, he had tachycardia without fever, a mild diffuse goiter, and hepatomegaly. The chest radiograph showed cardiomegaly with pulmonary edema, while the echocardiography revealed a dilated left ventricle with an ejection fraction (EF of 28%. The thyroid function test (TFT showed elevated serum T3 and decreased thyroid stimulating hormone (TSH levels. The TSH receptor autoantibody titer was elevated. He was diagnosed with DCMP with GD; treatment with methylprednisolone, diuretics, inotropics, and methimazole was initiated. The EF improved after the TFT normalized. At follow-up several months later, although the TFT results again showed evidence of hyperthyroidism, his EF had not deteriorated. His cardiac function continues to remain normal 1.5 months after treatment was started, although he still has elevated T3 and high TSH receptor antibody titer levels due to poor compliance with drug therapy. To summarize, we report a young child with GD-induced DCMP who recovered completely with medical therapy and, even though the hyperthyroidism recurred several months later, there was no relapse of the DCMP.

  20. Terapia celular na cardiomiopatia dilatada Cell therapy in dilated cardiomyopathy

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    Helena Furtado Martino

    2006-05-01

    Full Text Available Homem de 41 anos em insuficiência cardíaca sistólica, CF III NYHA, estágio clínico C, por cardiomiopatia dilatada, foi submetido ao transplante autólogo da fração mononuclear da medula óssea, via sistema arterial coronariano, através de cateterismo cardíaco. Dois meses após o procedimento, houve diminuição do BNP plasmático, diminuição da área cardíaca ao estudo radiológico do tórax e à ressonância nuclear magnética. O ecocardiograma demonstrou diminuição do fluxo regurgitante secundário a dilatação do anel mitral. Na ergoespirometria houve melhor desempenho, com aumento do consumo máximo de oxigênio, sendo possível redução da terapêutica medicamentosa. A ausência de eventos adversos caracterizados por: instabilidade clínica/hemodinâmica, alteração enzimática ou eletrocardiográfica apontam para segurança e exeqüibilidade deste procedimento realizado e descrito com pioneirismo na cardiomiopatia dilatada.A forty-one-year-old male with systolic heart failure, FC-III NYHA, clinical stage C due to dilated cardiomyopathy was submitted to an autologous transplant of the mononuclear fraction of bone marrow via coronary artery system through heart catheterism. Two months after the procedure, there was a decrease in plasma BNP and cardiac area reduction at the thorax X-ray and nuclear magnetic resonance. The echocardiogram showed decrease of the secondary regurgitation and mitral ring dilatation. There was a better performance at the ergospirometry, with increase of the maximum oxygen consumption and consequent reduction in drug therapy. The absence of adverse events characterized by clinical/hemodynamic instability, enzymatic alteration or electrocardiogram demonstrate the safety and feasibility of this procedure carried out and described with pioneering spirit in dilated cardiomyopathy.

  1. Cell therapy in dilated cardiomyopathy: from animal models to clinical trials

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    C. del Corsso

    2011-05-01

    Full Text Available Dilated cardiomyopathy can be the end-stage form and common denominator of several cardiac disorders of known cause, such as hypertensive, ischemic, diabetic and Chagasic diseases. However, some individuals have clinical findings, such as an increase in ventricular chamber size and impaired contractility (classical manifestations of dilated cardiomyopathy even in the absence of a diagnosed primary disease. In these patients, dilated cardiomyopathy is classified as idiopathic since its etiology is obscure. Nevertheless, regardless of all of the advances in medical, pharmacological and surgical procedures, the fate of patients with dilated cardiomyopathy (of idiopathic or of any other known cause is linked to arrhythmic episodes, severe congestive heart failure and an increased risk of sudden cardiac death. In this review, we will summarize present data on the use of cell therapies in animal models of dilated cardiomyopathies and will discuss the few clinical trials that have been published so far involving patients affected by this disease. The animal models discussed here include those in which the cardiomyopathy is produced by genetic manipulation and those in which disease is induced by chemical or infectious agents. The specific model used clearly creates restrictions to translation of the proposed cell therapy to clinical practice, insofar as most of the clinical trials performed to date with cell therapy have used autologous cells. Thus, translation of genetic models of dilated cardiomyopathy may have to wait until the use of allogeneic cells becomes more widespread in clinical trials of cell therapies for cardiac diseases.

  2. Progress with genetic cardiomyopathies: Screening, counseling, and testing in dilated, hypertrophic and arrhythmogenic right ventricular dysplasia/cardiomyopathy

    Science.gov (United States)

    Hershberger, Ray E.; Cowan, Jason; Morales, Ana; Siegfried, Jill D.

    2010-01-01

    Summary This review focuses on the genetic cardiomyopathies: principally dilated cardiomyopathy (DCM), with salient features of hypertrophic cardiomyopathy (HCM) and arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C), regarding genetic etiology, genetic testing, and genetic counseling. Enormous progress has recently been made in identifying genetic causes for each cardiomyopathy, and key phenotype and genotype information is reviewed. Clinical genetic testing is rapidly emerging with a principal rationale of identifying at-risk asymptomatic or disease-free relatives. Knowledge of a disease-causing mutation can guide clinical surveillance for disease onset, thereby enhancing preventive and treatment interventions. Genetic counseling is also indicated for patients and their family members regarding the symptoms of their cardiomyopathy, its inheritance pattern, family screening recommendations, and genetic testing options and possible results. PMID:19808347

  3. The value of myocardial perfusion imaging in differentiating between idiopathic dilated cardiomyopathy from the ischemic form

    International Nuclear Information System (INIS)

    Fad, A.; Emami, F.; Eftekhari, M.; Saghari, M.; Fallahi, B.; Beiki, D.; Tkavar, A.

    2004-01-01

    Introduction: differentiating between ischemic cardiomyopathy and idiopathic dilated cardiomyopathy is important as coronary revascularization can improve prognosis in the ischemic subgroup. Due to inherent problems of coronary angiography in patients with depressed ejection fraction introducing a noninvasive tool to diagnose those who will benefit from angiography seems to be rewarding. We examined usefulness of myocardial perfusion scan in this group of patients. Materials and methods: study was performed on 64 patients (62 male and 2 female) aged 57.1 ± 6.7 y (mean ± SD) all with dilation of the left ventricular cavity and ejection fraction less than 40 % by echocardiography. Myocardial perfusion scan was performed in stress and rest phases. All the patients had coronary angiography which was used as the gold standard test. On each set of images, heart was arbitrary divided into 17 segments and perfusion abnormality in each segment was scored by a 5 grade scoring system (0-4). Summed stress Score was used as the scan criteria to differentiate dilated ischemic from idiopathic cardiomyopathy. Scores more than 17 were considered ischemic, and less than that, idiopathic. Results were compared with angiography. Results: from total 40 cases of ischemic cardiomyopathy (proved by angiography) 39 were correctly diagnosed by scan and only one case was mis categorized as idiopathic dilated cardiomyopathy . All 24 cases of idiopathic dilated cardiomyopathy were correctly diagnosed by scintigraphy. Sensitivity, specificity, positive predictive value, and negative predictive value of myocardial perfusion imaging for discrimination between ischemic and idiopathic dilated cardiomyopathy were 97.5 %, 100 %, 100 %, and 96 % respectively. Conclusion: Considering excellent accuracy of myocardial perfusion scan with scoring system in discrimination of ischemic dilated cardiomyopathy from idiopathic cardiomyopathy, this noninvasive test could be considered the main diagnostic test

  4. Is hepatitis C virus a cause of idiopathic dilated cardiomyopathy? A systematic review of literature

    OpenAIRE

    Reis, Francisco José Farias Borges dos; Sousa, Tiago Almeida de; Oliveira, Manoela S.; Dantas, Ney; Silveira, Martha; Braghiroly, Maria Ignês Freitas Melro; Paraná, Raymundo

    2006-01-01

    p.199-202 Recent studies have suggested that some patients with idiopathic dilated cardiomyopathy (IDC) are also afflicted with insidious forms of viral myocarditis. Participation of hepatitis C virus (HCV) in this process has been postulated. The objective of this study was to evaluate a possible association between hepatitis C virus and idiopathic dilated cardiomyopathy. Systematic review of the literature using electronic databases (MEDLINE, EMBASES, LILACS and COCHRANE) for the period ...

  5. High prevalence of myocardial monoclonal antimyosin antibody uptake in patients with chronic idiopathic dilated cardiomyopathy

    International Nuclear Information System (INIS)

    Obrador, D.; Ballester, M.; Carrio, I.; Berna, L.; Pons-Llado, G.

    1989-01-01

    Monoclonal antimyosin antibody studies were undertaken to assess the presence of myocardial uptake in patients with chronic idiopathic dilated cardiomyopathy. Three groups were studied: 17 patients with chronic (greater than 12 months) idiopathic dilated cardiomyopathy, 12 patients with a large, poorly contracting left ventricle not due to dilated cardiomyopathy (control patients) and 8 normal individuals. The patients in the cardiomyopathy and control groups showed a similar degree of clinical and functional impairment. Imaging was undertaken 48 h after antimyosin injection. The heart/lung ratio of antimyosin uptake was used to assess the results. The mean ratio in the cardiomyopathy group was 1.83 +/- 0.36 (range 1.40 to 2.80), a value significantly higher than that obtained in the control patients without cardiomyopathy (mean 1.46 +/- 0.04, range 1.38 to 1.50) or normal subjects (mean 1.46 +/- 0.13, range 1.31 to 1.6) (p less than 0.01). No difference in the ratio was noted between the normal subjects and control patients. Abnormal antimyosin uptake was seen in 12 (70%) of the 17 patients with cardiomyopathy and in only 1 (8%) of the 12 control patients. Positive monoclonal antimyosin antibody studies are highly prevalent in chronic idiopathic dilated cardiomyopathy

  6. Becker Muscular Dystrophy (BMD) caused by duplication of exons 3-6 of the dystrophin gene presenting as dilated cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Tsai, A.C.; Allingham-Hawkins, D.J.; Becker, L. [Univ. of Toronto, Ontario (Canada)] [and others

    1994-09-01

    X-linked dilated cardiomyopathy (XLCM) is a progressive myocardial disease presenting with congestive heart failure in teenage males without clinical signs of skeletal myopathy. Tight linkage of XLCM to the DMD locus has been demonstrated; it has been suggested that, at least in some families, XLCM is a {open_quotes}dystrophinopathy.{close_quotes} We report a 14-year-old boy who presented with acute heart failure due to dilated cardiomyopathy. He had no history of muscle weakness, but physical examination revealed pseudohypertrophy of the calf muscles. He subsequently received a heart transplantation. Family history was negative. Serum CK level at the time of diagnosis was 10,416. Myocardial biopsy showed no evidence of carditis. Dystrophin staining of cardiac and skeletal muscle with anti-sera to COOH and NH{sub 2}termini showed a patchy distribution of positivity suggestive of Becker muscular dystrophy. Analysis of 18 of the 79 dystrophin exons detected a duplication that included exons 3-6. The proband`s mother has an elevated serum CK and was confirmed to be a carrier of the same duplication. A mutation in the muscle promotor region of the dystrophin gene has been implicated in the etiology of SLCM. However, Towbin et al. (1991) argued that other 5{prime} mutations in the dystrophin gene could cause selective cardiomyopathy. The findings in our patient support the latter hypothesis. This suggests that there are multiple regions in the dystrophin gene which, when disrupted, can cause isolated dilated cardiomyopathy.

  7. Myocarditis in Paediatric Patients: Unveiling the Progression to Dilated Cardiomyopathy and Heart Failure

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    Inês Teixeira Farinha

    2016-11-01

    Full Text Available Myocarditis is a challenging and potentially life-threatening disease associated with high morbidity in some paediatric patients, due to its ability to present as an acute and fulminant disease and to ultimately progress to dilated cardiomyopathy. It has been described as an inflammatory disease of the myocardium caused by diverse aetiologies. Viral infection is the most frequent cause of myocarditis in developed countries, but bacterial and protozoal infections or drug hypersensitivity may also be causative agents. The prompt diagnosis in paediatric patients is difficult, as the spectrum of clinical manifestation can range from no myocardial dysfunction to sudden cardiac death. Recent studies on myocarditis pathogenesis have revealed a triphasic nature of this disease, which influences the diagnostic and therapeutic strategies to adopt in each patient. Endomyocardial biopsy remains the gold standard for diagnosing myocarditis, and several non-invasive diagnostic tools can be used to support the diagnosis. Intravenous immunoglobulin has become part of routine practice in the treatment of myocarditis in paediatric patients at many centres, but its true effect on the cardiac function has been the target of many studies. The aim of this review is to approach the recently discovered facets of paediatric myocarditis regarding its progression to dilated cardiomyopathy.

  8. Clinical predictors of outcome in patients with inflammatory dilated cardiomyopathy.

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    Konstantinos Karatolios

    Full Text Available The study objectives were to identify predictors of outcome in patients with inflammatory dilated cardiomyopathy (DCMi.From 2004 to 2008, 55 patients with biopsy-proven DCMi were identified and followed up for 58.2±19.8 months. Predictors of outcome were identified in a multivariable analysis with a Cox proportional hazards analysis. The primary endpoint was a composite of death, heart transplantation and hospitalization for heart failure or ventricular arrhythmias.For the primary endpoint, a QTc interval >440msec (HR 2.84; 95% CI 1.03-7.87; p = 0.044, a glomerular filtration rate (GFR 440msec, a GFR<60ml/min/1.73m2 and worsening of NYHA classification during follow-up were univariate predictors of adverse prognosis. In contrast, NYHA classification at baseline, left ventricular ejection fraction, atrial fibrillation, treatment with digitalis or viral genome detection were not related to outcome. After multivariable analysis, a GFR <60ml/min/1.73m2 remained independently associated with adverse outcome.

  9. Right ventricular function in patients with dilated cardiomyopathy

    International Nuclear Information System (INIS)

    Kubota, Shuhei; Kubota, Sachio; Iwase, Takashi; Iizuka, Toshio; Imai, Susumu; Murata, Kazuhiko; Inoue, Tomio; Suzuki, Tadashi; Sasaki, Yasuhito.

    1993-01-01

    The characteristics and pathogenesis of right ventricular dysfunction in 14 patients with dilated cardiomyopathy (DCM) were investigated by equilibrium right ventricular blood pool scintigraphy using ultrashort-lifetime 81m Kr. Thirteen patients with severe left ventricular dysfunction due to old anterior myocardial infarction (OMI) and nine normal subjects were used as controls. The right ventricular end-diastolic pressure and volume index, mean pulmonary arterial pressure, and total pulmonary vascular resistance index were almost the same in the DCM and OMI patients. The right ventricular ejection fraction was 44.2±6.0% (mean±SD) in DCM patients and 47.1±7.9% in OMI patients, both significantly lower than those in the normal subjects (54.5±5.3%), but with no difference between the two case groups. The right ventricular peak filling rate was significantly reduced in both case groups as compared with the normal subjects (2.46±0.81 EDV/sec). The reduction was significantly greater (p 81m Kr blood pool scintigraphy is useful in the study of the right ventricular systolic and diastolic function. The diastolic parameters are more sensitive indicators for evaluation of right ventricular function in DCM than the systolic parameters. (author)

  10. Apical Hypertrophic Cardiomyopathy Mimicking Acute Coronary Syndrome

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    Lütfü Bekar

    2013-03-01

    Full Text Available Apical hypertrophic cardiomyopathy is a rare form of hypertrophic cardiomyopathy and it can be mistaken for coronary artery disease due to the symptoms and electrocardiography findings. In this report, we aimed to present a patient referred to our clinic with complaints of chest pain and electrocardiography findings who had been misdiagnosed as non-ST elevation acute coronary syndrome.

  11. Genetic Testing for Dilated Cardiomyopathy in Clinical Practice

    Science.gov (United States)

    Lakdawala, Neal K.; Funke, Birgit H.; Baxter, Samantha; Cirino, Allison L.; Roberts, Amy E.; Judge, Daniel P.; Johnson, Nicole; Mendelsohn, Nancy J.; Morel, Chantal; Care, Melanie; Chung, Wendy K.; Jones, Carolyn; Psychogios, Apostolos; Duffy, Elizabeth; Rehm, Heidi L.; White, Emily; Seidman, J.G.; Seidman, Christine E.; Ho, Carolyn Y.

    2013-01-01

    Background Familial involvement is common in dilated cardiomyopathy (DCM) and >40 genes have been implicated in causing disease. However, the role of genetic testing in clinical practice is not well defined. We examined the experience of clinical genetic testing in a diverse DCM population to characterize the prevalence and predictors of gene mutations. Methods and Results We studied 264 unrelated adult and pediatric DCM index patients referred to 1 reference lab for clinical genetic testing. Up to 10 genes were analyzed (MYH7, TNNT2, TNNI3, TPM1, MYBPC3, ACTC, LMNA, PLN, TAZ, and LDB3), and 70% of patients were tested for all genes. The mean age was 26.6 ± 21.3 years, and 52% had a family history of DCM. Rigorous criteria were used to classify DNA variants as clinically relevant (mutations), variants of unknown clinical significance (VUS), or presumed benign. Mutations were found in 17.4% of patients, commonly involving MYH7, LMNA, or TNNT2 (78%). An additional 10.6% of patients had VUS. Genetic testing was rarely positive in older patients without a family history of DCM. Conversely in pediatric patients, family history did not increase the sensitivity of genetic testing. Conclusions Using rigorous criteria for classifying DNA variants, mutations were identified in 17% of a diverse group of DCM index patients referred for clinical genetic testing. The low sensitivity of genetic testing in DCM reflects limitations in both current methodology and knowledge of DCM-associated genes. However, if mutations are identified, genetic testing can help guide family management. PMID:22464770

  12. Genetic Modifiers of Duchenne Muscular Dystrophy and Dilated Cardiomyopathy.

    Directory of Open Access Journals (Sweden)

    Andrea Barp

    Full Text Available Dilated cardiomyopathy (DCM is a major complication and leading cause of death in Duchenne muscular dystrophy (DMD. DCM onset is variable, suggesting modifier effects of genetic or environmental factors. We aimed to determine if polymorphisms previously associated with age at loss of independent ambulation (LoA in DMD (rs28357094 in the SPP1 promoter, rs10880 and the VTTT/IAAM haplotype in LTBP4 also modify DCM onset.A multicentric cohort of 178 DMD patients was genotyped by TaqMan assays. We performed a time-to-event analysis of DCM onset, with age as time variable, and finding of left ventricular ejection fraction 70 mL/m2 as event (confirmed by a previous normal exam < 12 months prior; DCM-free patients were censored at the age of last echocardiographic follow-up.Patients were followed up to an average age of 15.9 ± 6.7 years. Seventy-one/178 patients developed DCM, and median age at onset was 20.0 years. Glucocorticoid corticosteroid treatment (n = 88 untreated; n = 75 treated; n = 15 unknown did not have a significant independent effect on DCM onset. Cardiological medications were not administered before DCM onset in this population. We observed trends towards a protective effect of the dominant G allele at SPP1 rs28357094 and recessive T allele at LTBP4 rs10880, which was statistically significant in steroid-treated patients for LTBP4 rs10880 (< 50% T/T patients developing DCM during follow-up [n = 13]; median DCM onset 17.6 years for C/C-C/T, log-rank p = 0.027.We report a putative protective effect of DMD genetic modifiers on the development of cardiac complications, that might aid in risk stratification if confirmed in independent cohorts.

  13. Characteristics of syncope in patients with dilated cardiomyopathy.

    Science.gov (United States)

    Rami, Abdel-Hadi; Lucian, Muresan; Dana, Pop; Dumitru, Zdrenghea

    2016-04-01

    Syncope carries a poor prognosis among patients with dilated cardiomyopathy (DCM). To assess the prevalence, describe the underlying mechanisms and to identify risk factors for syncope in patients with DCM. One thousand six hundred and ten medical files of 897 patients with a diagnosis of DCM were reviewed. Patients with syncope were identified and their clinical and paraclinical profiles were compared to an equal number of age- and sex-matched patients with DCM without syncope. Thirty patients (27 males) with an average age of 62.5 years were identified, corresponding to a prevalence of syncope of 3.3%. A cardiac origin of syncope was identified in 56% of patients (n=17): ventricular arrhythmias in 33% (n=10), and conduction disorders in 23% (n=7). Other mechanisms of syncope were neurally mediated in 7% (n=2) and orthostatic hypotension in 7% (n=2). In 30% of cases (n=9), the etiology was unidentified. There were no significant differences regarding the etiology of DCM, ejection fraction (35.3% vs 35.3%, p=1.0), NYHA class (mild or advanced, p=0.79) and associated conditions (hypertension, p=0.36; diabetes, p=0.75; atrial fibrillation, p=0.43; and dyslipidemia, p=0.33) between the two groups. However, among patients with syncope, patients with a noncardiac cause were more likely to have hypertension (61.53% vs 23.52%, p=0.08) and diabetes (46.15% vs 5.88%, p=0.03). In patients with DCM, syncope is a relatively rare finding. Cardiac causes (arrhythmias and conduction disorders) are responsible for the majority of cases. Risk factors for syncope in these patients remain to be determined. Copyright © 2015 Cardiological Society of India. Published by Elsevier B.V. All rights reserved.

  14. Frequency of echocardiographic complications of dilated cardiomyopathy at a tertiary care hospital

    International Nuclear Information System (INIS)

    Rashid, A.; Ahmed, H.N.; Ahmed, N.

    2012-01-01

    Dilated cardiomyopathy can lead to a variety of complications recognisable on clinical, echocardiographic, electrocardiographic and radiographic assessment. Among this, transthoracic echocardiography has the dual advantage of being helpful in making the diagnosis of dilated cardiomyopathy as well as an effective tool in early recognition of certain complications for timely management to improve the quality of life of these patients. Methods: This descriptive (case series) study was undertaken at departments of medicine, cardiology, paediatrics and obs/gyn, Ayub Teaching Hospital, Abbottabad from July to December, 2008. fifty patients of dilated cardiomyopathy without age and gender discrimination were selected by convenience sampling. Those with hypertrophic and restrictive cardiomyopathies, valvular and congenital heart disease, hypertension and ischemic heart disease were excluded. Results: mean age was 47.12 +- 17.9 year with male predominance (males=34, females=16). Mean ejection fraction was 30.6 +- 6.9%. complications revealed on echocardiography were intracardiac thrombi (5, 10%), spontaneous echo contrast (5, 10%), pericardial effusion (6, 12%), mitral regurgitation (46, 92%), tricuspid (25, 50%), aortic (5, 10%), pulmonary (2, 4%) multi-valvular regurgitation (28, 56%), and left atrial dilatation (36, 72%). Conclusion: lv systolic dysfunction, cardiac thrombi, spontaneous echo contrast, mitral and tricuspid regurgitation and left atrial enlargement are important complications of dilated cardiomyopathy. echocardiography is important tool towards identification of these complications. (author)

  15. Application of radionuclide techniques in evaluation of dilated cardiomyopathy and ischemic cardiomyopathy

    International Nuclear Information System (INIS)

    Tian Yueqin; Liu Xiujie; Shi Rongfang

    2000-01-01

    Objective: To assess the clinical significance of radionuclide techniques in differentiating dilated cardiomyopathy (DCM) from ischemic cardiomyopathy (CAD-CM). Methods: 28 patients (pts) with DCM and 55 pts with CAD-CM were studied. All pts underwent 99 Tc m -MIBI myocardial perfusion SPECT and 18 F-FDG myocardial metabolic PET. 73 pts had 99 Tc m -RBC radionuclide ventriculography and 68 pts had coronary angiography. Results: 23 pts (82%) with DCM showed perfusion abnormalities with mild and not segmental distribution. 52 pts (95%) with CAD-CM showed perfusion abnormalities that distributed along the coronary vessel territories. Perfusion defects were found in 4 pts (14%) with DCM and 45 pts (82%) with CAD-CM (P<0.01). The average perfusion score was 4.5 +- 2.6 in DCM and 9.5 +- 2.9 in CAD-CM, the area of perfusion diminished uptake was significantly smaller in DCM than in CAD-CM (P < 0.001). 2 pts with DCM and 18 pts with CAD-CM had metabolic defect. The patterns of perfusion/metabolic imaging showed mismatch in most pts with CAD-CM but match in pts with DCM. The LVEF in pts with DCM and CAD-CM was decreased but no significant difference between DCM and CAD-CM was observed. The RVEF in pts with DCM was significantly lower than that in pts with CAD-CM (32.4% +- 13.9% vs 40.9% +- 15.4%, P < 0.05). Conclusions: The radionuclide techniques showed to be helpful for distinguishing DCM from CAD-CM. The discriminate analysis revealed that segmental perfusion abnormality and RVEF were the most important factors for differentiation of DCM from CAD-CM

  16. A negative screen for mutations in calstabin 1 and 2 genes in patients with dilated cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Biagi Diogo G

    2012-01-01

    Full Text Available Abstract Background Calstabins 1 and 2 bind to Ryanodine receptors regulating muscle excitation-contraction coupling. Mutations in Ryanodine receptors affecting their interaction with calstabins lead to different cardiac pathologies. Animal studies suggest the involvement of calstabins with dilated cardiomyopathy. Results We tested the hypothesis that calstabins mutations may cause dilated cardiomyopathy in humans screening 186 patients with idiopathic dilated cardiomyopathy for genetic alterations in calstabins 1 and 2 genes (FKBP12 and FKBP12.6. No missense variant was found. Five no-coding variations were found but not related to the disease. Conclusions These data corroborate other studies suggesting that mutations in FKBP12 and FKBP12.6 genes are not commonly related to cardiac diseases.

  17. Tafazzin gene mutations are uncommon causes of dilated cardiomyopathy in adults

    Directory of Open Access Journals (Sweden)

    Matthew Taylor

    2011-07-01

    Full Text Available Barth syndrome is an X-linked genetic condition featuring neutropenia, skeletal myopathy, and dilated cardiomyopathy in boys due to tafazzin (TAZ mutations. Pure dilated cardiomyopathy without other features of Barth syndrome may also result from TAZ mutations and survival into adulthood has been described. Although TAZ testing is routinely included in dilated cardiomyopathy panels in adults, the prevalence of TAZ mutations in the adult population, including women who may be at risk to develop later onset disease due to TAZ mutations, has not been measured. We screened 292 families with dilated cardiomyopathy (209 male and 83 female probands for TAZ mutations using denaturing high-performance liquid chromatography and sequence analysis. Putative mutations were evaluated based on standard criteria including screening available relatives and healthy controls and for effects on splicing efficiency in the case of one intronic variant. Two variants suspicious for being pathogenic were found in two unrelated families (c.387T>C, Phe128Ser and c.507C>T, Leu169Leu. The Phe128Ser variant had been previously reported as a pathogenic mutation; however we determined that this variant is instead a rare polymorphism restricted to African Americans. The Leu169Leu variant was detected in a male patient and altered RNA processing in our minigene assay supporting a pathogenic role. No mutations in female subjects were detected. Tafazzin mutations were rare in our population of adults with dilated cardiomyopathy and none were found in females. Our findings indicate that genetic testing for tafazzin should not be routinely performed in dilated cardiomyopathy as suggested by current guidelines. Furthermore, the Phe128Ser variant is not pathogenic, but likely represents a benign polymorphism in persons of African American ancestry.

  18. [Dimenhydrinate overdosage in a 3(1/2) year-old-girl with dilative cardiomyopathy].

    Science.gov (United States)

    Girisch, M; Hofbeck, M; Rauch, R; Apitz, C; Sieverding, L

    2009-01-01

    Dimenhydrinate overdosage in a 3(1/2) year-old-girl with dilative cardiomyopathy. Dimenhydrinate (Vomex(R)) is frequently used in the treatment of sickness and vomiting. The symptoms of overdosage present like an anticholinergic syndrome. We report on the clinical findings of an intoxication with dimenhydrinate in a 3(1/2) year-old-girl with functional dilative cardiomyopathy following a congenital left ventricular diverticle. Especially in small children, with the application of 40 mg suppositories once or twice per day the maximum dose of 3.75 mg/kgBW/d is achieved.

  19. Tc-99m MAA findings in dilated cardiomyopathy with partial anomalous venous connections.

    Science.gov (United States)

    Ishii, Shirou; Shishido, Fumio; Miyajima, Masayuki; Sakuma, Koutarou; Shigihara, Takeshi; Kikuchi, Ken

    2011-07-01

    Tc-99m MAA showed asymmetric uptake in the lung field in a 21-year-old man with dilated cardiomyopathy. CT revealed partial anomalous venous connections in the left upper lobe. Angiogram of the left pulmonary upper lobe showed all the contrast material drained into the left vertical vein. The possible cause of relative increase in the left upper lobe blood flow is that right pulmonary blood flow is slowed by the high pressure in the left atrium due to dilated cardiomyopathy, whereas the flow from the left upper lobe drains into the superior vena cava which has less pressure than left atrium.

  20. Trimetazidine, a metabolic modulator, has cardiac and extracardiac benefits in idiopathic dilated cardiomyopathy

    DEFF Research Database (Denmark)

    Tuunanen, H.; Engblom, E.; Naum, A.

    2008-01-01

    perfusion, efficiency of work, and FFA oxidation in idiopathic dilated cardiomyopathy. METHODS AND RESULTS: Nineteen nondiabetic patients with idiopathic dilated cardiomyopathy on standard medication were randomized to single-blind trimetazidine (n=12) or placebo (n=7) for 3 months. Myocardial perfusion...... ejection fraction from 30.9+/-8.5% to 34.8+/-12% (P=0.027 versus placebo). Myocardial FFA uptake was unchanged, and beta-oxidation rate constant decreased only 10%. Myocardial perfusion, oxidative metabolism, and work efficiency remained unchanged. Trimetazidine decreased insulin resistance (glucose: 5...

  1. Canine candidate genes for dilated cardiomyopathy: annotation of and polymorphic markers for 14 genes

    Directory of Open Access Journals (Sweden)

    van Oost Bernard A

    2007-10-01

    Full Text Available Abstract Background Dilated cardiomyopathy is a myocardial disease occurring in humans and domestic animals and is characterized by dilatation of the left ventricle, reduced systolic function and increased sphericity of the left ventricle. Dilated cardiomyopathy has been observed in several, mostly large and giant, dog breeds, such as the Dobermann and the Great Dane. A number of genes have been identified, which are associated with dilated cardiomyopathy in the human, mouse and hamster. These genes mainly encode structural proteins of the cardiac myocyte. Results We present the annotation of, and marker development for, 14 of these genes of the dog genome, i.e. α-cardiac actin, caveolin 1, cysteine-rich protein 3, desmin, lamin A/C, LIM-domain binding factor 3, myosin heavy polypeptide 7, phospholamban, sarcoglycan δ, titin cap, α-tropomyosin, troponin I, troponin T and vinculin. A total of 33 Single Nucleotide Polymorphisms were identified for these canine genes and 11 polymorphic microsatellite repeats were developed. Conclusion The presented polymorphisms provide a tool to investigate the role of the corresponding genes in canine Dilated Cardiomyopathy by linkage analysis or association studies.

  2. Difference in myocardial flow reserve between patients with dilated cardiomyopathy and those with dilated phase of hypertrophic cardiomyopathy. Evaluation by 15O-water PET

    International Nuclear Information System (INIS)

    Ohba, Muneo; Kambara, Naoshige; Hosokawa, Ryohei

    2007-01-01

    The clinical features of patients with the dilated phase of hypertrophic cardiomyopathy (DHCM) may resemble those of patients with dilated cardiomyopathy (DCM); that is, systolic dysfunction and left ventricular dilatation. Myocardial flow reserve (MFR) is impaired in patients with nonischemic cardiomyopathy, and the reduced MFR may be related to poor prognosis. Several studies report that the mortality rate for patients with DHCM is higher than for DCM, but the difference between these 2 cardiomyopathies is still unclear. The purpose of this study was to assess the MFR of these 2 cardiomyopathies, using 15 O-water positron emission tomography (PET) to elucidate their differences. In total 30 patients were investigated: 23 with DCM (Group A) and 7 with DHCM (Group B). All those who were in a stable condition underwent cardiac catheterization. Myocardial blood flow (MBF) at rest and under adenosine 5'-triphosphate (ATP) infusion was measured by 15 O-water PET, and the MFR was calculated. There were no significant differences in the hemodynamics of the 2 groups. The mean MFR in DHCM was significantly lower than that in DCM (1.49±0.31 vs 2.62±1.08; p=0.042), whereas MBF at rest did not differ (DCM vs DHCM: 0.66±0.20 vs 0.49±0.05 ml·min -1 ·g -1 ; no significance (NS)). The MFR in both Group A and B was significantly decreased compared with the normal controls (MFR in normal controls: 5.15±1.64, p=0.00015, 0.00013, respectively). These results suggest that impaired vasodilatation (ie, dysfunction of the microcirculation) is more severe in patients with DHCM than in patients with DCM, even though patients' characteristics and hemodynamics do not differ. (author)

  3. Genetics Home Reference: dilated cardiomyopathy with ataxia syndrome

    Science.gov (United States)

    ... usual to recharge between beats . The irregular heartbeats (arrhythmia) can lead to fainting (syncope) or cardiac arrest ... Topic: Cardiomyopathy Health Topic: Movement Disorders Health Topic: Newborn Screening Genetic and Rare Diseases Information Center (1 ...

  4. Genetic Analysis of Toxin-Induced Dilated Cardiomyopathy in the Turkey (Meleagris gallopavo)

    OpenAIRE

    Gyenai, Kwaku Barima

    2005-01-01

    Dilated cardiomyopathy (DCM) or round heart disease is a muscle disease of the heart which is characterized by ventricular dilatation and abnormal systolic and diastolic left ventricular function. In animals, including turkeys and humans, DCM is the major cause of morbidity and mortality which results from heart failure. In the turkey, DCM can be idiopathic or induced. Since idiopathic or spontaneous DCM occurs in about 1-4% of normal turkeys, it is of significant concern to the poultry indus...

  5. Drugs and dilated cardiomyopathies: a case/noncase study in the French PharmacoVigilance Database

    Science.gov (United States)

    Montastruc, Guillaume; Favreliere, Sylvie; Sommet, Agnès; Pathak, Atul; Lapeyre-Mestre, Maryse; Perault-Pochat, Marie-Christine; Montastruc, Jean-Louis

    2010-01-01

    AIMS To evaluate putative associations between drugs and dilated cardiomyopathy. METHODS We used the case/noncase method in the French PharmacoVigilance Database (FPVD). Cases were all the observations with dilated cardiomyopathy registered into the FPVD between 1 January 1990 and 30 June 2007. Noncases were all other reports other than those studied. Anthracyclines were used as positive controls. Data were expressed as reporting odds ratio (ROR) with their 95% confidence intervals. RESULTS Out of the 258 729 adverse drug reaction (ADR) reports recorded in the FPVD between 1 January 1990 and 30 June 2007, 47 (22 men, mean age 49 years) were defined as dilated cardiomyopathy. In these 47 patients, 67 drugs were ‘suspect’. A significant ROR was found with cytotoxic (epirubicin, mitoxantrone, cyclophosphamide, gemcitabine, fluorouracil) and antiretroviral (lamividune, zidovudine, abacavir) but also with isotretinoin, prednisone, appetite suppressant (clobenzorex) and psychotropic [antipsychotic (clozapine, olanzapine), lithium, antidepressant (clomipramine, amitriptyline, fluvoxamine)] drugs. CONCLUSIONS The present study describes an association between some drugs and reports of dilated cardiomyopathies. This relationship involves not only some already suspected drugs (anthracyclines, antiretrovirals), but also other drugs (antipsychotics, lithium, antidepressants, retinoids) less known to induce such an ADR. Despite the mandatory limits of this kind of study (underreporting, confounding factors …), these data represent a pharmacovigilance signal and could contribute to establish further prospective studies in order to confirm such signals. PMID:20233200

  6. CARDIAC TRANSPLANTATION IN YOUNG PATIENT WITH DILATED CARDIOMYOPATHY AND SECONDARY ANTIPHOSPHOLIPID SYNDROME

    Directory of Open Access Journals (Sweden)

    E. V. Shlyakhto

    2013-01-01

    Full Text Available Patients with congestive heart failure have an increased incidence of thromboembolic events. The choice of me- dical management in patients with antiphospholipid antibodies and generalized thrombosis due to hypercoagula- bility is complex issue. We report heart transplant outcome in 15 years old patient with dilated cardiomyopathy and secondary anti-phospholipid syndrome. 

  7. Evaluation of the Diagnostic Work-Up in Children with Myocarditis and Idiopathic Dilated Cardiomyopathy

    NARCIS (Netherlands)

    S.L. den Boer (Susanna); R.P.J. Meijer; G.G. van Iperen (Gabrielle); A.D.J. ten Harkel (Arend); G.J. Du Marchie Sarvaas (Gideon); B. Straver; L. Rammeloo (Lukas); R. Tanke (Ronald); J.J.A. van Kampen (Jeroen); M. Dalinghaus (Michiel)

    2015-01-01

    textabstractThe underlying etiology of dilated cardiomyopathy (DCM) in children varies, 14–22 % is secondary to myocarditis, and the majority remains idiopathic. Etiology has prognostic value; however, ‘a clinical diagnosis of myocarditis’ has been frequently used because the gold standard

  8. Evaluation of the diagnostic work-up in children with myocarditis and idiopathic dilated cardiomyopathy

    NARCIS (Netherlands)

    den Boer, S. L.; Meijer, R. P. J.; van Iperen, G. G.; ten Harkel, A. D. J.; du Marchie Sarvaas, G. J.; Straver, B.; Rammeloo, L. A. J.; Tanke, R. B.; van Kampen, J. J. A.; Dalinghaus, M.

    2015-01-01

    The underlying etiology of dilated cardiomyopathy (DCM) in children varies, 14-22 % is secondary to myocarditis, and the majority remains idiopathic. Etiology has prognostic value; however, 'a clinical diagnosis of myocarditis' has been frequently used because the gold standard [endomyocardial

  9. Evaluation of the Diagnostic Work-Up in Children with Myocarditis and Idiopathic Dilated Cardiomyopathy

    NARCIS (Netherlands)

    den Boer, S. L.; Meijer, R. P. J.; van Iperen, G. G.; ten Harkel, A. D. J.; Sarvaas, G. J. du Marchie; Straver, B.; Rammeloo, L. A. J.; Tanke, R. B.; van Kampen, J. J. A.; Dalinghaus, M.

    The underlying etiology of dilated cardiomyopathy (DCM) in children varies, 14-22 % is secondary to myocarditis, and the majority remains idiopathic. Etiology has prognostic value; however, 'a clinical diagnosis of myocarditis' has been frequently used because the gold standard [endomyocardial

  10. Evaluation of the diagnostic work-up in children with myocarditis and idiopathic dilated cardiomyopathy

    NARCIS (Netherlands)

    Boer, S.L. den; Meijer, R.P.; Iperen, G.G. van; Harkel, A.D. Ten; Sarvaas, G.J.; Straver, B.; Rammeloo, L.A.; Tanke, R.B.; Kampen, J.J. van; Dalinghaus, M.

    2015-01-01

    The underlying etiology of dilated cardiomyopathy (DCM) in children varies, 14-22% is secondary to myocarditis, and the majority remains idiopathic. Etiology has prognostic value; however, 'a clinical diagnosis of myocarditis' has been frequently used because the gold standard [endomyocardial biopsy

  11. Propionic acidemia as a cause of adult-onset dilated cardiomyopathy

    NARCIS (Netherlands)

    Riemersma, M.; Hazebroek, M.R.; Helderman-van den Enden, A.T.; Salomons, G.S.; Ferdinandusse, S.; Brouwers, M.; Ploeg, L. van der; Heymans, S.; Glatz, J.F.C.; Wijngaard, A. van den; Krapels, I.P.C.; Bierau, J.; Brunner, H.G.

    2017-01-01

    Dilated cardiomyopathy (DCM) is extremely heterogeneous with a large proportion due to dominantly inherited disease-causing variants in sarcomeric genes. Recessive metabolic diseases may cause DCM, usually with onset in childhood, and in the context of systemic disease. Whether metabolic defects can

  12. Ischemic patterns assessed by positron emission tomography predict adverse outcome in patients with idiopathic dilated cardiomyopathy

    NARCIS (Netherlands)

    de Jong, Richard M.; Tio, Rene A.; van der Harst, Pim; Voors, Adriaan A.; Koning, Paul M.; Zeebregts, Clark J. A. M.; van Veldhuisen, Dirk J.; Dierckx, Rudi A. J. O.; Slart, Riemer H. J. A.

    2009-01-01

    Although patients with idiopathic dilated cardiomyopathy (DCM) have no coronary artery disease, regional impairment of myocardial perfusion combined with preserved metabolism has been found using positron emission tomography (PET). Our aim was to assess the prognostic relevance of PET-mismatch

  13. Evaluation of pulsatile plasma concentrations of growth hormone in healthy dogs and dogs with dilated cardiomyopathy

    NARCIS (Netherlands)

    Beijerink, N.J.; Lee, W.M.; Stokhof, A.A.; Voorhout, G.; Mol, J.A.; Kooistra, H.S.

    2011-01-01

    Abstract OBJECTIVE: To evaluate plasma concentrations of growth hormone (GH) and insulin-like growth factor I (IGF-I) in healthy dogs and large-breed dogs with dilated cardiomyopathy (DCM). ANIMALS: 8 dogs with DCM and 8 healthy control dogs of comparable age and body weight. PROCEDURES: Blood

  14. A systematic analysis of genetic dilated cardiomyopathy reveals numerous ubiquitously expressed and muscle-specific genes

    NARCIS (Netherlands)

    Harakalova, Magdalena; Kummeling, Gijs; Sammani, Arjan; Linschoten, Marijke; Baas, Annette F.; van der Smagt, Jasper; Doevendans, Pieter A.; van Tintelen, J. Peter; Dooijes, Dennis; Mokry, Michal; Asselbergs, Folkert W.

    2015-01-01

    Despite considerable progress being made in genetic diagnostics for dilated cardiomyopathy (DCM) using panels of the most prevalent genes, the cause remains unsolved in a substantial percentage of patients. We hypothesize that several previously described DCM genes with low or unknown prevalence

  15. Is hepatitis C virus a cause of idiopathic dilated cardiomyopathy? A systematic review of literature

    Directory of Open Access Journals (Sweden)

    Francisco José Farias Borges dos Reis

    Full Text Available Recent studies have suggested that some patients with idiopathic dilated cardiomyopathy (IDC are also afflicted with insidious forms of viral myocarditis. Participation of hepatitis C virus (HCV in this process has been postulated. The objective of this study was to evaluate a possible association between hepatitis C virus and idiopathic dilated cardiomyopathy. Systematic review of the literature using electronic databases (MEDLINE, EMBASES, LILACS and COCHRANE for the period from 1995 to 2005, limited to papers published in English, Spanish and Portuguese. Sixty-two papers were found, of which six were in accordance with the proposed methodology. After selection, the articles were classified by quality of data and number of variables studied. Most of the patients were male adults from 31 and 75 years old, who had ischemic cardiopathy excluded as etiology of the dilated cardiomyopathy. A significant association between dilated cardiomyopathy and hepatitis C virus was found in only two papers, both from Japan and by the same author. Most of the papers received low classifications, as they did not fulfill the systematization criteria.

  16. Drugs and dilated cardiomyopathies: A case/noncase study in the French PharmacoVigilance Database.

    Science.gov (United States)

    Montastruc, Guillaume; Favreliere, Sylvie; Sommet, Agnès; Pathak, Atul; Lapeyre-Mestre, Maryse; Perault-Pochat, Marie-Christine; Montastruc, Jean-Louis

    2010-03-01

    To evaluate putative associations between drugs and dilated cardiomyopathy. We used the case/noncase method in the French PharmacoVigilance Database (FPVD). Cases were all the observations with dilated cardiomyopathy registered into the FPVD between 1 January 1990 and 30 June 2007. Noncases were all other reports other than those studied. Anthracyclines were used as positive controls. Data were expressed as reporting odds ratio (ROR) with their 95% confidence intervals. Out of the 258 729 adverse drug reaction (ADR) reports recorded in the FPVD between 1 January 1990 and 30 June 2007, 47 (22 men, mean age 49 years) were defined as dilated cardiomyopathy. In these 47 patients, 67 drugs were 'suspect'. A significant ROR was found with cytotoxic (epirubicin, mitoxantrone, cyclophosphamide, gemcitabine, fluorouracil) and antiretroviral (lamividune, zidovudine, abacavir) but also with isotretinoin, prednisone, appetite suppressant (clobenzorex) and psychotropic [antipsychotic (clozapine, olanzapine), lithium, antidepressant (clomipramine, amitriptyline, fluvoxamine)] drugs. The present study describes an association between some drugs and reports of dilated cardiomyopathies. This relationship involves not only some already suspected drugs (anthracyclines, antiretrovirals), but also other drugs (antipsychotics, lithium, antidepressants, retinoids) less known to induce such an ADR. Despite the mandatory limits of this kind of study (underreporting, confounding factors . . .), these data represent a pharmacovigilance signal and could contribute to establish further prospective studies in order to confirm such signals.

  17. Anti-calreticulin antibodies and calreticulin in sera of patients diagnosed with dilated or hypertrophic cardiomyopathy

    Czech Academy of Sciences Publication Activity Database

    Sánchez, Daniel; Gregor, P.; Čurila, K.; Hoffmanová, I.; Hábová, Věra; Tučková, Ludmila; Tlaskalová-Hogenová, Helena

    2016-01-01

    Roč. 49, č. 8 (2016), s. 554-562 ISSN 0891-6934 R&D Projects: GA ČR GA13-14608S; GA TA ČR TA04010762 Institutional support: RVO:61388971 Keywords : Anti-calreticulin antibodies * calreticulin * dilated cardiomyopathy Subject RIV: EC - Immunology Impact factor: 2.629, year: 2016

  18. Congenital complete atrioventricular block and dilated cardiomyopathy: new light for an old disease.

    Science.gov (United States)

    Paiva, Mariana; Ribeiro, Vania; Garcia, Raquel; Amorim, Sandra; Campelo, Manuel; Martins, Elisabete; Moura, Brenda; Cardoso, Jose; Maciel, M Júlia

    2012-01-01

    We present a case of a patient with known complete congenital atrioventricular block (CAVB) since the age of 7 years old that developed dilated cardiomyopathy ten years after VVI-R pacemaker implantation. He presented severe biventricular dysfunction and was symptomatic despite optimal medical therapy. Cardiac resynchronization therapy was used, and he showed clinical and electrocardiographic improvement a month later.

  19. Congenital Complete Atrioventricular Block and Dilated Cardiomyopathy: New Light for an Old Disease

    Directory of Open Access Journals (Sweden)

    Mariana Paiva

    2012-01-01

    Full Text Available We present a case of a patient with known complete congenital atrioventricular block (CAVB since the age of 7 years old that developed dilated cardiomyopathy ten years after VVI-R pacemaker implantation. He presented severe biventricular dysfunction and was symptomatic despite optimal medical therapy. Cardiac resynchronization therapy was used, and he showed clinical and electrocardiographic improvement a month later.

  20. Congenital Complete Atrioventricular Block and Dilated Cardiomyopathy: New Light for an Old Disease

    OpenAIRE

    Paiva, Mariana; Ribeiro, Vania; Garcia, Raquel; Amorim, Sandra; Campelo, Manuel; Martins, Elisabete; Moura, Brenda; Cardoso, Jose; Maciel, M. Júlia

    2012-01-01

    We present a case of a patient with known complete congenital atrioventricular block (CAVB) since the age of 7 years old that developed dilated cardiomyopathy ten years after VVI-R pacemaker implantation. He presented severe biventricular dysfunction and was symptomatic despite optimal medical therapy. Cardiac resynchronization therapy was used, and he showed clinical and electrocardiographic improvement a month later.

  1. In vivo 31P-NMR spectroscopy in patients with dilating cardiomyopathies

    International Nuclear Information System (INIS)

    Kaiser, W.A.; Pauleit, D.; Traeber, F.; Koehler, U.; Reiser, M.

    1992-01-01

    In vivo 31 P-NMR spectra of heart muscle were obtained in 7 patients with dilating cardiomyopathies and in 10 normal subjects, using an ISIS technique with a 1.5 Tesla body scanner (Philips Gyroscan S 15) and the results of the two groups were compared. In patients with cardiomyopathy there was a significant (p 31 P-NMR spectrum. The patients with the largest reduction of the PCr/ATP index also showed the most marked changes in functional measurements. The PCr/ATP ratio (as also suggested by animal experiments) appears to be a suitable non-invasive indicator for the diagnosis and staging of myocardial changes in dilating cardiomyopathies. (orig./GDG) [de

  2. Oxidative Stress in Dilated Cardiomyopathy Caused by MYBPC3 Mutation

    NARCIS (Netherlands)

    T.L. Lynch (Thomas L.); M. Sivaguru (Mayandi); M. Velayutham (Murugesan); A.J. Cardounel (Arturo J.); M. Michels (Michelle); D. Barefield (David); S. Govindan (Suresh); C.D. Remedios (Cristobal Dos); J. van der Velden (Jolanda); S. Sadayappan (Sakthivel)

    2015-01-01

    textabstractCardiomyopathies can result from mutations in genes encoding sarcomere proteins including MYBPC3, which encodes cardiac myosin binding protein-C (cMyBP-C). However, whether oxidative stress is augmented due to contractile dysfunction and cardiomyocyte damage in MYBPC3-mutated

  3. Comparison between Brain Natriuretic Peptide and Calcitonin Gene Related Peptide in Children with Dilated Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Noor Mohammad Noori

    2017-09-01

    Full Text Available Background: Dilated cardiomyopathy (DCM is revealed with the left ventricular dilatation and systolic dysfunction. This study was performed to determine the level of Calcitonin Gene Related Peptide (CGRP and Brain Natriuretic Peptide (BNP in children with dilated cardiomyopathy and controls and comparison of these two biomarkers in patients. Materials and Methods: This case-control study was performed from April 2014 to March 2015 on patients with DCM. The levels of BNP and CGRP were measured by ELISA and final amounts of biomarkers were compared with echocardiographic finding.37 DCM patients compared with 30 healthy children selected randomly from those who referred to the hospital for routine checkup. Results: In this study the mean age was 10.567± 5.50 and 12.135 ± 4.626 years for controls and cases, respectively (P=0.321. The majority of echocardiography indices in the left and right heart had different means in case and controls (P

  4. No mutation but high mRNA expression of Coxsackie-Adenovirus Receptor was observed in both dilated and ischemic cardiomyopathy.

    Science.gov (United States)

    Tatrai, Eniko; Bedi, Katalin; Kovalszky, Ilona; Hartyanszky, Istvan; Laszik, Andras; Acsady, Gyorgy; Sotonyi, Peter; Hubay, Marta

    2011-10-10

    The most common causes of acute myocarditis and the possible consequence of dilated cardiomyopathy are virus infections. The receptor of the two most common viruses connected to these myocardial diseases was identified as Coxsackie-Adenovirus Receptor. The purpose of this study was to assess Coxsackie-Adenovirus Receptor mRNA expression in the myocardium and search for mutations in the Coxsackie-Adenovirus Receptor gene to compare dilated, inflammatory and ischemic cardiomyopathy with control group. All the myocardial samples were obtained from 35 explanted hearts during heart transplantation, than DNA and RNA were isolated from the muscle samples. cDNA was generated from RNA using reverse transcription, and real-time PCR was performed with relative quantification by β-actin gene as endogenous control. Using DNA extracted from the myocardial samples, we sequenced all the seven exons of the Coxsackie-Adenovirus Receptor gene. Coxsackie-Adenovirus Receptor mRNA expression was higher in both ischemic and dilated cardiomyopathy groups than in inflammatory cardiomyopathy and healthy control groups. Sequencing of CAR gene showed no sign of mutation. Therefore, the sequences result of CAR exons did not show any mutation or polymorphism, that explains a determinant role of CAR in dilated or ischemic CM. Our results suggest that high mRNA expression of Coxsackie-Adenovirus Receptor may support its role in regeneration of the damaged myocardium rather than having any role in viral mediated heart disease. Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.

  5. Early Predictors of Survival to and After Heart Transplantation in Children with Dilated Cardiomyopathy

    Science.gov (United States)

    Pietra, Biagio A.; Kantor, Paul F.; Bartlett, Heather L.; Chin, Clifford; Canter, Charles E.; Larsen, Ranae L.; Edens, R. Erik; Colan, Steven D.; Towbin, Jeffrey A.; Lipshultz, Steven E.; Kirklin, James K.; Naftel, David C.; Hsu, Daphne T.

    2012-01-01

    Background The importance of clinical presentation and pre-transplantation course on outcome in children with dilated cardiomyopathy (DCM) listed for heart transplantation is not well defined. Methods and Results The impact of age, duration of illness, gender, race, ventricular geometry and the diagnosis of myocarditis on outcome in 261 DCM children enrolled in the Pediatric Cardiomyopathy Registry and Pediatric Heart Transplant Study was studied. Endpoints included: 1) listing as UNOS Status 1, 2) death while waiting and 3) death post-transplantation. The median age at the time of diagnosis was 3.4 years, and mean time from diagnosis to listing was 0.62±1.3 years. Risk factors associated with death while waiting were ventilator use and older age at listing in patients not mechanically ventilated (p=0.0006 and p=0.03, respectively). Shorter duration of illness (p=0.04) was associated with listing as UNOS Status 1. Death post-transplantation was associated with myocarditis at presentation (p=0.009), non-white race (p<0.0001) and a lower left ventricular end-diastolic dimension z-score at presentation (p=0.04). In the myocarditis group, 17% (4/23) died of acute rejection post-transplantation. Conclusions Mechanical ventilator use and older age at listing predicted death while waiting, while non-white race, smaller left ventricular dimension and myocarditis were associated with death post-transplantation. Although 97% of children with clinically or biopsy diagnosed myocarditis at presentation survived to transplantation, they had significantly higher mortality post-transplantation compared with children without myocarditis, raising the possibility that pre-existing viral infection or inflammation adversely affects graft survival. PMID:22800850

  6. Studying Dynamic Myofiber Aggregate Reorientation in Dilated Cardiomyopathy Using In Vivo Magnetic Resonance Diffusion Tensor Imaging.

    Science.gov (United States)

    von Deuster, Constantin; Sammut, Eva; Asner, Liya; Nordsletten, David; Lamata, Pablo; Stoeck, Christian T; Kozerke, Sebastian; Razavi, Reza

    2016-10-01

    The objective of this study is to assess the dynamic alterations of myocardial microstructure and strain between diastole and systole in patients with dilated cardiomyopathy relative to healthy controls using the magnetic resonance diffusion tensor imaging, myocardial tagging, and biomechanical modeling. Dual heart-phase diffusion tensor imaging was successfully performed in 9 patients and 9 controls. Tagging data were acquired for the diffusion tensor strain correction and cardiac motion analysis. Mean diffusivity, fractional anisotropy, and myocyte aggregate orientations were compared between both cohorts. Cardiac function was assessed by left ventricular ejection fraction, torsion, and strain. Computational modeling was used to study the impact of cardiac shape on fiber reorientation and how fiber orientations affect strain. In patients with dilated cardiomyopathy, a more longitudinal orientation of diastolic myofiber aggregates was measured compared with controls. Although a significant steepening of helix angles (HAs) during contraction was found in the controls, consistent change in HAs during contraction was absent in patients. Left ventricular ejection fraction, cardiac torsion, and strain were significantly lower in the patients compared with controls. Computational modeling revealed that the dilated heart results in reduced HA changes compared with a normal heart. Reduced torsion was found to be exacerbated by steeper HAs. Diffusion tensor imaging revealed reduced reorientation of myofiber aggregates during cardiac contraction in patients with dilated cardiomyopathy relative to controls. Left ventricular remodeling seems to be an important factor in the changes to myocyte orientation. Steeper HAs are coupled with a worsening in strain and torsion. Overall, the findings provide new insights into the structural alterations in patients with dilated cardiomyopathy. © 2016 The Authors.

  7. Prevalence of cardiac dyssynchrony and correlation with atrio-ventricular block and QRS width in dilated cardiomyopathy

    DEFF Research Database (Denmark)

    Anzouan-Kacou, J B; Ncho-Mottoh, M P; Konin, C

    2012-01-01

    Cardiac dyssynchrony causes disorganised cardiac contraction, delayed wall contraction and reduced pumping efficiency. We aimed to assess the prevalence of different types of dyssynchrony in patients with dilated cardiomyopathy (DCM), and to establish the correlation between atrio-ventricular block...

  8. Targeted sequence capture and GS-FLX Titanium sequencing of 23 hypertrophic and dilated cardiomyopathy genes: implementation into diagnostics

    NARCIS (Netherlands)

    Mook, Olaf R. F.; Haagmans, Martin A.; Soucy, Jean-François; van de Meerakker, Judith B. A.; Baas, Frank; Jakobs, Marja E.; Hofman, Nynke; Christiaans, Imke; Lekanne Deprez, Ronald H.; Mannens, Marcel M. A. M.

    2013-01-01

    Genetic evaluation of cardiomyopathies poses a challenge. Multiple genes are involved but no clear genotype-phenotype correlations have been found so far. In the past, genetic evaluation for hypertrophic (HCM) and dilated (DCM) cardiomyopathies was performed by sequential screening of a very limited

  9. QT dispersion on ECG Holter monitoring and risk of ventricular arrhythmias in patients with dilated cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Polyxeni Garyfallidis

    2010-05-01

    Full Text Available Background. QT dispersion (QTd is increased in patients with dilated cardiomyopathy. Increased QTd has been associated with the risk of sudden death. We studied: a the relation between QTd on 12-lead ECG and QTd-ECG Holter; b the relation between QTd apex (QTda and QTd end (QTde on ECG Holter and the risk of ventricular arrhythmias in patients with dilated cardiomyopathy. Methods and Results. 65 patients with dilated cardiomyopathy (33 idiopathic and 32 post-ischemic etiology; NYHA II-III were studied. We divided the patients into: Group A -patients with not-sustained ventricular arrhythmias-; and Group B -patients without arrhythmias-. A significant direct correlation between QTd calculated from 12-lead ECG and from ECG Holter was found in all patients. QTda/24h was not significantly different in the two groups (Gr.A 59.9±7.8 msec vs Gr.B 53.6±8.4 msec p=ns while QTde/24h was significantly higher in Group A (Gr.A 81.9±5.9 msec vs Gr.B 44.5±6.8 msec; p<0.005. In post-ischemic etiology (32 pts; 17 with arrhythmias the correlation between QTde/24h and ventricular arrhythmias was confirmed (Gr.A 81.4±7.8 msec vs Gr.B 42.6±6.2 msec p<0.002. Conclusions. ECG Holter recordings can evaluate QTd as well as the QTd on 12-lead ECG. An increased QTde/24h seems to be correlated with the occurence of ventricular arrhythmias in patients with dilated cardiomyopathy and can then be a useful tool to select patients at high risk for sudden death.

  10. Neopterin and Beta-2 Microglobulin Relations to Immunity and Inflammatory Status in Nonischemic Dilated Cardiomyopathy Patients

    Directory of Open Access Journals (Sweden)

    Celina Wojciechowska

    2014-01-01

    Full Text Available Background. The aim of the study was to assess the relationships among serum neopterin (NPT, β2-microglobulin (β2-M levels, clinical status, and endomyocardial biopsy results of dilated cardiomyopathy patients (DCM. Methods. Serum NPT and β-2 M were determined in 172 nonischaemic DCM patients who underwent right ventricular endomyocardial biopsy and 30 healthy subjects (ELISA test. The cryostat biopsy specimens were assessed using histology, immunohistology, and immunochemistry methods (HLA ABC, HLA DR expression, CD3 + lymphocytes, and macrophages counts. Results. The strong increase of HLA ABC or HLA DR expression was detected in 27.2% patients—group A—being low in 72.8% patients—group B. Neopterin level was increased in patients in group A compared to healthy controls 8.11 (4.50–12.57 versus 4.99 (2.66–8.28 nmol/L (P<0.05. β-2 microglobulin level was higher in DCM groups A (2.60 (1.71–3.58 and B (2.52 (1.51–3.72 than in the control group 1.75 (1.28–1.96 mg/L, P<0.001. Neopterin correlated positively with the number of macrophages in biopsy specimens (P<0.05 acute phase proteins: C-reactive proteins (P<0.05; fibrinogen (P<0.01; and NYHA functional class (P<0.05 and negatively with left ventricular ejection fraction (P<0.05. Conclusions. Neopterin but not β-2 microglobulin concentration reflected immune response in biopsy specimens. Neopterin correlated with acute phase proteins and stage of heart failure and may indicate a general immune and inflammatory activation in heart failure.

  11. An Upgrade on the Rabbit Model of Anthracycline-Induced Cardiomyopathy: Shorter Protocol, Reduced Mortality, and Higher Incidence of Overt Dilated Cardiomyopathy

    Science.gov (United States)

    Talavera, Jesús; Fernández-Del-Palacio, María Josefa; García-Nicolás, Obdulio; Seva, Juan; Brooks, Gavin; Moraleda, Jose M.

    2015-01-01

    Current protocols of anthracycline-induced cardiomyopathy in rabbits present with high premature mortality and nephrotoxicity, thus rendering them unsuitable for studies requiring long-term functional evaluation of myocardial function (e.g., stem cell therapy). We compared two previously described protocols to an in-house developed protocol in three groups: Group DOX2 received doxorubicin 2 mg/kg/week (8 weeks); Group DAU3 received daunorubicin 3 mg/kg/week (10 weeks); and Group DAU4 received daunorubicin 4 mg/kg/week (6 weeks). A cohort of rabbits received saline (control). Results of blood tests, cardiac troponin I, echocardiography, and histopathology were analysed. Whilst DOX2 and DAU3 rabbits showed high premature mortality (50% and 33%, resp.), DAU4 rabbits showed 7.6% premature mortality. None of DOX2 rabbits developed overt dilated cardiomyopathy; 66% of DAU3 rabbits developed overt dilated cardiomyopathy and quickly progressed to severe congestive heart failure. Interestingly, 92% of DAU4 rabbits showed overt dilated cardiomyopathy and 67% developed congestive heart failure exhibiting stable disease. DOX2 and DAU3 rabbits showed alterations of renal function, with DAU3 also exhibiting hepatic function compromise. Thus, a shortened protocol of anthracycline-induced cardiomyopathy as in DAU4 group results in high incidence of overt dilated cardiomyopathy, which insidiously progressed to congestive heart failure, associated to reduced systemic compromise and very low premature mortality. PMID:26788502

  12. A novel alpha-tropomyosin mutation associates with dilated and non-compaction cardiomyopathy and diminishes actin binding

    NARCIS (Netherlands)

    van de Meerakker, Judith B. A.; Christiaans, Imke; Barnett, Phil; Lekanne Deprez, Ronald H.; Ilgun, Aho; Mook, Olaf R. F.; Mannens, Marcel M. A. M.; Lam, Jan; Wilde, Arthur A. M.; Moorman, Antoon F. M.; Postma, Alex V.

    2013-01-01

    Dilated cardiomyopathy (DCM) is characterized by idiopathic dilatation and systolic contractile dysfunction of the ventricle(s) leading to an impaired systolic function. The origin of DCM is heterogeneous, but genetic transmission of the disease accounts for up to 50% of the cases. Mutations in

  13. Dilated cardiomyopathy - approach made simplified with myocardial perfusion scintigraphy and cardiac viability studies

    International Nuclear Information System (INIS)

    Pawar, Shwetal; Maitra, R.; Tilve, G.H.

    2010-01-01

    Full text: To differentiate the ischemic vs nonischemic dilated cardiomyopathy and to assess myocardial viability in the ischemic cardiomyopathy. Materials and Methods: 34 patients (24 males and 10 females) with dilated cardiomyopathy diagnosed on echocardiography with reduced left ventricular ejection fraction and global hypokinesia were included between the period of Jan 2009 and July 2010. All the patients underwent rest myocardial perfusion scintigraphy first; 45 minutes after intravenous injection of 7 mCi of 99m Tc MIBI. The stress myocardial perfusion imaging (after physical stress or gm/kg/min; 6 min infusion) was pharmacological stress with adenosine; 140 performed in the patients with normal perfusion at rest. The 18 F FDG (5 mCi) cardiac viability studies were performed in patient with abnormal rest myocardial perfusion. The images were acquired on GE Infinia systems and processed on Emory toolbox (ECT) to study the ischemia and viability. Results: The mean left ventricular ejection fraction was found to be 27.38% at rest. The stress and rest perfusion scintigraphy was carried out in 20/34(58%) patients in whom 9(45%) patients underwent pharmacological stress with Inj adenosine and 11(55%) patients underwent physical stress. The stress induced ischemia was diagnosed in 12(60%) patients and infarct in 2(10%) patients with mixed ischemia and infarct pattern in 2(10%) patients. The nonischemic cause was diagnosed in 4 patients. The cardiac viability study was carried out in 14/34 (42%) patients with 6(42%) viable, 5(35%) nonviable and 3(21%) mixed viable and nonviable patterns were identified. Conclusion: The myocardial perfusion scintigraphy was the simplified approach for differentiating ischemic verses nonischemic dilated cardiomyopathy with addition of cardiac viability study ( 18 FDG) made it one stop shop for the complete work-up of patients for further management

  14. Reversible dilated cardiomyopathy: into the thaumaturgy of the heart - Part 1

    Directory of Open Access Journals (Sweden)

    Giovanni Quarta

    2016-10-01

    Full Text Available Dilated cardiomyopathy (DCM is a genetic or acquired heart muscle disorder characterized by dilation and impaired contraction of one or both ventricles. In the acquired forms of the disease, if the pathogenic agent is persistent, undiagnosed or untreated, permanent ultrastructural and morphological changes may lead to irreversible dysfunction. Conversely, when DCM is promptly recognized and treated, the heart may show an extraordinary ability to recover from left ventricular (LV systolic dysfunction. While much research in heart failure has focused on morbidity and mortality associated with persistent LV systolic dysfunction, relatively little attention has been devoted to this remarkable potential for recovery. In this two-part review we will focus on the most common types of reversible DCM. The first part will deal with Tako-Tsubo cardiomyopathy, tachycardiainduced cardiomyopathy, metabolic DCM and recovery after Left ventricular assist device implantation. Although diverse in etiopathogenesis, genetic background, therapeutic options and outcome, the forms of DCM characterized by reversible LV dysfunction share similar challenges in diagnosis and clinical management. The identification of pathways to recovery may show the way for novel therapeutic targets ultimately benefitting all cardiac patients.

  15. Five-week use of a monopivot centrifugal blood pump as a right ventricular assist device in severe dilated cardiomyopathy.

    Science.gov (United States)

    Inoue, Takamichi; Kitamura, Tadashi; Torii, Shinzo; Hanayama, Naoji; Oka, Norihiko; Itatani, Keiichi; Tomoyasu, Takahiro; Irisawa, Yusuke; Shibata, Miyuki; Hayashi, Hidenori; Ono, Minoru; Miyaji, Kagami

    2014-03-01

    Right heart failure is a critical complication in patients requiring mechanical ventricular support. However, it is often difficult to provide adequate right ventricular support in the acute phase. A 41-year-old woman diagnosed with dilated cardiomyopathy with severe right heart failure underwent implantation of a paracorporeal pulsatile left ventricular assist device (LVAD, Nipro Corporation, Tokyo, Japan) and a MERA monopivot centrifugal pump (Senko Medical Instrument Manufacturing Co., Ltd., Tokyo, Japan) as a right ventricular assist device (RVAD). The patient developed ischemic enteritis 3 weeks after surgery, necessitating fasting and reversal of anticoagulation therapy. A target international normalized ratio of 1.5 was selected, and aspirin administration was discontinued. Following recovery without thromboembolic events, the patient failed the RVAD discontinuation test. Five weeks after surgery, the monopivot centrifugal pump was exchanged for a pulsatile pump. No thrombus was evident on the centrifugal pump. The patient was undergoing cardiac rehabilitation at the time of this writing and awaiting heart transplantation.

  16. Oculomotor apraxia and dilated cardiomyopathy with ataxia syndrome: A case report.

    Science.gov (United States)

    Benson, Matthew D; Ferreira, Patrick; MacDonald, Ian M

    2017-01-01

    Dilated cardiomyopathy with ataxia syndrome (DCMA) is a rare mitochondrial condition associated with early onset cardiomyopathy and non-progressive ataxia. The cardiac manifestations may be progressive and often severe, resulting in significant morbidity and mortality. While optic nerve atrophy has been described in patients with DCMA, to our knowledge, there have been no reports of additional ocular phenotypes. We present two related Dariusleut Hutterite patients with documented DCMA syndrome and disorders of ocular motility: poor smooth pursuit and difficulty initiating saccadic eye movements and maintaining target fixation. We thus report the first cases of oculomotor apraxia in DCMA syndrome. By identifying these associated findings early in life, we hope to improve both the clinical diagnostic accuracy and timeliness of intervention in cases of DCMA.

  17. Mice deleted for heart-type cytochrome c oxidase subunit 7a1 develop dilated cardiomyopathy.

    Science.gov (United States)

    Hüttemann, Maik; Klewer, Scott; Lee, Icksoo; Pecinova, Alena; Pecina, Petr; Liu, Jenney; Lee, Michael; Doan, Jeffrey W; Larson, Douglas; Slack, Elise; Maghsoodi, Bita; Erickson, Robert P; Grossman, Lawrence I

    2012-03-01

    Subunit 7a of mouse cytochrome c oxidase (Cox) displays a contractile muscle-specific isoform, Cox7a1, that is the major cardiac form. To gain insight into the role of this isoform, we have produced a new knockout mouse line that lacks Cox7a1. We show that homozygous and heterozygous Cox7a1 knockout mice, although viable, have reduced Cox activity and develop a dilated cardiomyopathy at 6 weeks of age. Surprisingly, the cardiomyopathy improves and stabilizes by 6 months of age. Cox7a1 knockout mice incorporate more of the "liver-type" isoform Cox7a2 into the cardiac Cox holoenzyme and, also surprisingly, have higher tissue ATP levels. Copyright © 2011 Elsevier B.V. and Mitochondria Research Society. All rights reserved. All rights reserved.

  18. Clinical assessment of serum myosin light chain I in patients with dilated cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Tsuda, Takashi; Izumi, Tohru; Shibata, Akira (Niigata Univ. (Japan). School of Medicine)

    1992-08-01

    Serum cardiac myosin light chain I (LCI) levels were quantitated using a radioimmunoassay kit in patients suspected of dilated cardiomyopathy (DCM). In this study, 55 patients were evaluated between 1986 and 1991. They were composed of 40 males and 15 females, and their age was 27-75 years (51[+-]11 years). The patients with renal dysfunction were excluded due to their serum creatinine levels (>2.0 mg/dl). After cardiac catheterization, endomyocardial biopsy and echocardiography, 44 patients were diagnosed as DCM, 2 as ischemic heart disease, 2 as chronic myocarditis, 1 as restrictive cardiomyopathy, 1 as dilated hypertrophic cardiomyopathy, 1 as cardiac amyloidosis, 2 as myopathy, 1 as polymyositis and 1 as hypothyroidism. Only two patients with DCM had elevated LCI. Besides, two patients with myopathy or hypothyroidism had elevated LCI. In the follow-up, one patient died suddenly 6 months later and another showed normal value of LCI four years later. LCI elevation in DCM was not related to either the severity of heart failure or cardiac function and it showed no finding of [sup 201]Tl myocardial defect or elevated CPK. The mechanism for elevated LCI in myopathy is related to a crossreaction with myosin light chain in the skeletal muscle. In hypothyroidism, it may be related to decreased clearance of normal LCI concentration or increased myosin light chain from damaged skeletal muscle. In conclusion, it is evident that the measurement of LCI is not helpful in clinical assessment of patients with DCM, but may be useful in detection of secondary cardiomyopathy. (author).

  19. Hypoxia induces dilated cardiomyopathy in the chick embryo: mechanism, intervention, and long-term consequences.

    Directory of Open Access Journals (Sweden)

    Andrei Tintu

    Full Text Available Intrauterine growth restriction is associated with an increased future risk for developing cardiovascular diseases. Hypoxia in utero is a common clinical cause of fetal growth restriction. We have previously shown that chronic hypoxia alters cardiovascular development in chick embryos. The aim of this study was to further characterize cardiac disease in hypoxic chick embryos.Chick embryos were exposed to hypoxia and cardiac structure was examined by histological methods one day prior to hatching (E20 and at adulthood. Cardiac function was assessed in vivo by echocardiography and ex vivo by contractility measurements in isolated heart muscle bundles and isolated cardiomyocytes. Chick embryos were exposed to vascular endothelial growth factor (VEGF and its scavenger soluble VEGF receptor-1 (sFlt-1 to investigate the potential role of this hypoxia-regulated cytokine.Growth restricted hypoxic chick embryos showed cardiomyopathy as evidenced by left ventricular (LV dilatation, reduced ventricular wall mass and increased apoptosis. Hypoxic hearts displayed pump dysfunction with decreased LV ejection fractions, accompanied by signs of diastolic dysfunction. Cardiomyopathy caused by hypoxia persisted into adulthood. Hypoxic embryonic hearts showed increases in VEGF expression. Systemic administration of rhVEGF(165 to normoxic chick embryos resulted in LV dilatation and a dose-dependent loss of LV wall mass. Lowering VEGF levels in hypoxic embryonic chick hearts by systemic administration of sFlt-1 yielded an almost complete normalization of the phenotype.Our data show that hypoxia causes a decreased cardiac performance and cardiomyopathy in chick embryos, involving a significant VEGF-mediated component. This cardiomyopathy persists into adulthood.

  20. Acute gastric dilatation secondary to septicemia in newborn: A rare ...

    African Journals Online (AJOL)

    ... case of acute gastric dilatation that developed in a two-days old child who was born premature and was suffering from septicemia and respiratory distress. The causes and the differential diagnosis of the rare condition are discussed. Keywords: Acute, dilatation, gastric, newborn. African Journal of Paediatric Surgery Vol.

  1. Myocardial damage assessed by indium-111-antimyosin: correlation with persistent enteroviral ribonucleic acid in dilated cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Bengel, F.M. [Department of Nuclear Medicine, University of Erlangen (Germany)]|[Department of Nuclear Medicine, Technical University of Munich (Germany); Feistel, H.; Wolf, F. [Department of Nuclear Medicine, University of Erlangen (Germany); Moshage, W.; Bachmann, K. [Department of Cardiology, University of Erlangen (Germany)

    1997-09-01

    The persistence of enteroviral ribonucleic acid (RNA) in the myocardium has been implicated as a pathogenetic factor in idiopathic dilated cardiomyopathy. Enteroviral persistence may lead to myocardial cell membrane damage, resulting in increased uptake of antimyosin antibodies. To further evaluate this hypothesis, a direct comparison of myocardial antimyosin uptake with the presence of enteroviral RNA was performed in ten patients (one female, nine male; 53{+-}8 years) with chronic dilated cardiomyopathy. Planar antimyosin images were obtained 48 h after the injection of indium-111-labelled antimyosin Fab. Using a region of interest technique, the heart to lung uptake ratio (HLR) was calculated as a semiquantitative parameter of myocardial tracer uptake. Cardiac catheterization was performed to assess left ventricular function and to obtain myocardial biopsy samples. In the biopsy samples, gene amplification by polymerase chain reaction (PCR) was used to specifically detect enteroviral RNA. In the ten patients, the left ventricular ejection fraction was 39%{+-}11% and the end-diastolic volume 131{+-}46 ml/m{sup 2}. The HLR was 1.72{+-}0.21 and showed no correlation with functional parameters. In two patients with a positive PCR consistent with persisting enteroviral RNA, the HLR was not higher than that in eight patients with a negative PCR (1.46{+-}0.18 vs 1.78{+-}0.18, respectively). These results suggest that increased uptake of {sup 111}In-antimyosin in chronic idiopathic dilated cardiomyopathy cannot be explained by pure persistence of enteroviral RNA. Other pathogenetic factors such as myocardial autoantibodies or microvascular spasm may be responsible for myocyte membrane damage detected by antimyosin. (orig.). With 3 figs., 1 tab.

  2. Autologous transplantation of bone marrow adult stem cells for the treatment of idiopathic dilated cardiomyopathy.

    Science.gov (United States)

    Westphal, Ricardo João; Bueno, Ronaldo Rocha Loures; Galvão, Paulo Bezerra de Araújo; Zanis Neto, José; Souza, Juliano Mendes; Guérios, Ênio Eduardo; Senegaglia, Alexandra Cristina; Brofman, Paulo Roberto; Pasquini, Ricardo; Cunha, Claudio Leinig Pereira da

    2014-12-01

    Morbimortality in patients with dilated idiopathic cardiomyopathy is high, even under optimal medical treatment. Autologous infusion of bone marrow adult stem cells has shown promising preliminary results in these patients. Determine the effectiveness of autologous transplantation of bone marrow adult stem cells on systolic and diastolic left ventricular function, and on the degree of mitral regurgitation in patients with dilated idiopathic cardiomyopathy in functional classes NYHA II and III. We administered 4,54 x 10(8) ± 0,89 x 10(8) bone marrow adult stem cells into the coronary arteries of 24 patients with dilated idiopathic cardiomyopathy in functional classes NYHA II and III. Changes in functional class, systolic and diastolic left ventricular function and degree of mitral regurgitation were assessed after 3 months, 6 months and 1 year. During follow-up, six patients (25%) improved functional class and eight (33.3%) kept stable. Left ventricular ejection fraction improved 8.9%, 9.7% e 13.6%, after 3, 6 and 12 months (p = 0.024; 0.017 and 0.018), respectively. There were no significant changes neither in diastolic left ventricular function nor in mitral regurgitation degree. A combined cardiac resynchronization and implantable cardioversion defibrillation was implanted in two patients (8.3%). Four patients (16.6%) had sudden death and four patients died due to terminal cardiac failure. Average survival of these eight patients was 2.6 years. Intracoronary infusion of bone marrow adult stem cells was associated with an improvement or stabilization of functional class and an improvement in left ventricular ejection fraction, suggesting the efficacy of this intervention. There were no significant changes neither in left ventricular diastolic function nor in the degree of mitral regurgitation.

  3. Autologous Transplantation of Bone Marrow Adult Stem Cells for the Treatment of Idiopathic Dilated Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Ricardo João Westphal

    2014-12-01

    Full Text Available Background: Morbimortality in patients with dilated idiopathic cardiomyopathy is high, even under optimal medical treatment. Autologous infusion of bone marrow adult stem cells has shown promising preliminary results in these patients. Objective: Determine the effectiveness of autologous transplantation of bone marrow adult stem cells on systolic and diastolic left ventricular function, and on the degree of mitral regurgitation in patients with dilated idiopathic cardiomyopathy in functional classes NYHA II and III. Methods: We administered 4,54 x 108 ± 0,89 x 108 bone marrow adult stem cells into the coronary arteries of 24 patients with dilated idiopathic cardiomyopathy in functional classes NYHA II and III. Changes in functional class, systolic and diastolic left ventricular function and degree of mitral regurgitation were assessed after 3 months, 6 months and 1 year. Results: During follow-up, six patients (25% improved functional class and eight (33.3% kept stable. Left ventricular ejection fraction improved 8.9%, 9.7% e 13.6%, after 3, 6 and 12 months (p = 0.024; 0.017 and 0.018, respectively. There were no significant changes neither in diastolic left ventricular function nor in mitral regurgitation degree. A combined cardiac resynchronization and implantable cardioversion defibrillation was implanted in two patients (8.3%. Four patients (16.6% had sudden death and four patients died due to terminal cardiac failure. Average survival of these eight patients was 2.6 years. Conclusion: Intracoronary infusion of bone marrow adult stem cells was associated with an improvement or stabilization of functional class and an improvement in left ventricular ejection fraction, suggesting the efficacy of this intervention. There were no significant changes neither in left ventricular diastolic function nor in the degree of mitral regurgitation.

  4. Myofilament Remodeling and Function Is More Impaired in Peripartum Cardiomyopathy Compared with Dilated Cardiomyopathy and Ischemic Heart Disease.

    Science.gov (United States)

    Bollen, Ilse A E; Ehler, Elisabeth; Fleischanderl, Karin; Bouwman, Floor; Kempers, Lanette; Ricke-Hoch, Melanie; Hilfiker-Kleiner, Denise; Dos Remedios, Cristobal G; Krüger, Martina; Vink, Aryan; Asselbergs, Folkert W; van Spaendonck-Zwarts, Karin Y; Pinto, Yigal M; Kuster, Diederik W D; van der Velden, Jolanda

    2017-12-01

    Peripartum cardiomyopathy (PPCM) and dilated cardiomyopathy (DCM) show similarities in clinical presentation. However, although DCM patients do not recover and slowly deteriorate further, PPCM patients show either a fast cardiac deterioration or complete recovery. The aim of this study was to assess if underlying cellular changes can explain the clinical similarities and differences in the two diseases. We, therefore, assessed sarcomeric protein expression, modification, titin isoform shift, and contractile behavior of cardiomyocytes in heart tissue of PPCM and DCM patients and compared these with nonfailing controls. Heart samples from ischemic heart disease (ISHD) patients served as heart failure control samples. Passive force was only increased in PPCM samples compared with controls, whereas PPCM, DCM, and ISHD samples all showed increased myofilament Ca 2+ sensitivity. Length-dependent activation was significantly impaired in PPCM compared with controls, no impairment was observed in ISHD samples, and DCM samples showed an intermediate response. Contractile impairments were caused by impaired protein kinase A (PKA)-mediated phosphorylation because exogenous PKA restored all parameters to control levels. Although DCM samples showed reexpression of EH-myomesin, an isoform usually only expressed in the heart before birth, PPCM and ISHD did not. The lack of EH-myomesin, combined with low PKA-mediated phosphorylation of myofilament proteins and increased compliant titin isoform, may explain the increase in passive force and blunted length-dependent activation of myofilaments in PPCM samples. Copyright © 2017 American Society for Investigative Pathology. Published by Elsevier Inc. All rights reserved.

  5. 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

  6. Adaptive cardiac resynchronization therapy for dilated cardiomyopathy with functional mitral regurgitation

    Directory of Open Access Journals (Sweden)

    Yoshiki Nagata, MD, PhD

    2017-10-01

    Full Text Available We report the case of a man in his 60s who had dilated cardiomyopathy with severe functional mitral regurgitation. Four years after a cardiac resynchronization therapy (CRT device with an implantable cardioverter defibrillator was implanted, this device was replaced with an adaptive CRT device because of battery consumption. Seven months after replacement of this device, the left ventricular pacing to right ventricular activation and the atrioventricular delay from automatic adjustments contributed to less functional mitral regurgitation. The findings from our case suggest that optimal CRT, by measuring intracardiac conduction parameters, is effective for functional mitral regurgitation.

  7. New and emerging biomarkers in left ventricular systolic dysfunction--insight into dilated cardiomyopathy.

    Science.gov (United States)

    Gopal, Deepa M; Sam, Flora

    2013-08-01

    Dilated cardiomyopathy (DCM) is characterized by deteriorating cardiac performance, impaired contraction and dilation of the left ventricle (or both ventricles). Blood markers--known as "biomarkers"--allow insight into underlying pathophysiologic mechanisms and biologic pathways while predicting outcomes and guiding heart failure management and/or therapies. In this review, we provide an alternative approach to conceptualize heart failure biomarkers: the cardiomyocyte, its surrounding microenvironment, and the macroenvironment, integrating these entities which may impact cellular processes involved in the pathogenesis and/or propagation of DCM. Newer biomarkers of left ventricular systolic dysfunction can be categorized under: (a) myocyte stress and stretch, (b) myocyte apoptosis, (c) cardiac interstitium, (d) inflammation, (e) oxidative stress, (f) cardiac energetics, (g) neurohormones, and (h) renal biomarkers. Biomarkers provide insight into the pathogenesis of DCM while predicting and potentially providing prognostic information in these patients with heart failure.

  8. Autosomal recessive dilated cardiomyopathy due to DOLK mutations results from abnormal dystroglycan O-mannosylation.

    Directory of Open Access Journals (Sweden)

    Dirk J Lefeber

    2011-12-01

    Full Text Available Genetic causes for autosomal recessive forms of dilated cardiomyopathy (DCM are only rarely identified, although they are thought to contribute considerably to sudden cardiac death and heart failure, especially in young children. Here, we describe 11 young patients (5-13 years with a predominant presentation of dilated cardiomyopathy (DCM. Metabolic investigations showed deficient protein N-glycosylation, leading to a diagnosis of Congenital Disorders of Glycosylation (CDG. Homozygosity mapping in the consanguineous families showed a locus with two known genes in the N-glycosylation pathway. In all individuals, pathogenic mutations were identified in DOLK, encoding the dolichol kinase responsible for formation of dolichol-phosphate. Enzyme analysis in patients' fibroblasts confirmed a dolichol kinase deficiency in all families. In comparison with the generally multisystem presentation in CDG, the nonsyndromic DCM in several individuals was remarkable. Investigation of other dolichol-phosphate dependent glycosylation pathways in biopsied heart tissue indicated reduced O-mannosylation of alpha-dystroglycan with concomitant functional loss of its laminin-binding capacity, which has been linked to DCM. We thus identified a combined deficiency of protein N-glycosylation and alpha-dystroglycan O-mannosylation in patients with nonsyndromic DCM due to autosomal recessive DOLK mutations.

  9. Association of dilated cardiomyopathy with the striatin mutation genotype in boxer dogs.

    Science.gov (United States)

    Meurs, K M; Stern, J A; Sisson, D D; Kittleson, M D; Cunningham, S M; Ames, M K; Atkins, C E; DeFrancesco, T; Hodge, T E; Keene, B W; Reina Doreste, Y; Leuthy, M; Motsinger-Reif, A A; Tou, S P

    2013-01-01

    Myocardial disease in the Boxer dog is characterized by 1 of 2 clinical presentations, dilated cardiomyopathy (DCM) characterized by ventricular systolic dysfunction, dilatation and tachyarrhythmias, and arrhythmogenic right ventricular cardiomyopathy (ARVC) characterized by ventricular tachyarrhythmias, syncope, and sudden death. Boxer ARVC has been associated with a deletion in the striatin gene in some families. We hypothesized that both presentations represent a single disease, and the development of DCM in the Boxer is associated with the striatin deletion. Thirty-three adult Boxer dogs with DCM, 29 adult Boxer dogs with the striatin deletion and ARVC, and 16 Boxers without cardiac disease. DNA samples were evaluated for the striatin deletion. Association of the deletion with the DCM phenotype was tested by a Fisher's exact test. T-tests were used to evaluate potential differences between the positive heterozygous and positive homozygous groups with DCM with regard to age, LVIDD, LVIDS, and FS%. Thirty of 33 dogs with DCM were positive for the striatin deletion. The striatin mutation and the homozygous genotype were strongly associated with the DCM phenotype (P Boxer dog and the striatin mutation, particularly with the homozygous genotype. The observation that 3/33 dogs developed DCM and lacked the striatin mutation suggests that there is at least 1 other cause of DCM in the Boxer dog. Copyright © 2013 by the American College of Veterinary Internal Medicine.

  10. [Long-term results of intracoronary transplantation of autologous bone marrow cells in dilated cardiomyopathy].

    Science.gov (United States)

    Bartolucci, Jorge; Verdugo, Fernando J; Carrion, Flavio; Abarzúa, Ema; Goset, Carlos; Lamich, Rubén; Sanhueza, Patricio; Pedreros, Pablo; Nazzal, Carolina; Khoury, Maroun; Figueroa, Fernando E

    2015-04-01

    Intracoronary delivery of autologous bone marrow mononuclear cells is an interesting therapeutic promise for patients with heart failure of different etiologies. To evaluate the long-term safety and efficacy of this therapy in patients with dilated cardiomyopathy of different etiologies under optimal medical treatment. Prospective, open-label, controlled clinical trial. Of 23 consecutive patients, 12 were assigned to autologous bone marrow mononuclear cell intracoronary transplantation, receiving a mean dose of 8.19 ± 4.43 x 10(6) CD34+ cells. Mortality, cardiovascular readmissions and cancer incidence rate, changes in functional capacity, quality of life questionnaires and echocardiographic measures from baseline, were assessed at long-term follow-up (37.7 ± 9.7 months) in patients receiving or not the cells. No significant differences were observed in mortality, cardiovascular readmissions or cancer incidence rate amongst groups. An improvement in functional class and quality of life questionnaires in the transplanted group was observed (p transplantation of autologous bone marrow mononuclear cells is feasible and safe in patients with dilated cardiomyopathy of diverse etiologies. This therapy was associated to persistent improvements in functional class and quality of life. There was also a non-significant long-term improvement of left ventricular function.

  11. Oral Chinese Herbal Medicine for Treatment of Dilated Cardiomyopathy: A Systematic Review and Meta-Analysis

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    Yu-Shuo Zhu

    2016-01-01

    Full Text Available Dilated cardiomyopathy (DCM is one of the main causes of heart failure and could increase death, hospitalization, and rehospitalization rate. The effect of conventional medicine treatment (CMT is limited; meanwhile, the combination of CMT and Oral Chinese Herbal Medicine (OCHM represents exciting adjunctive therapies. In this study, we ascertained the therapeutic effect of OCHM in combination with CMT for dilated cardiomyopathy by using meta-analysis methods for controlled clinical trials. We searched studies from five databases and extracted data from these studies. We also assessed the methodological quality of the included studies. We evaluated the following outcome measures to estimate the prognosis in patients with DCM: left ventricular ejection fraction (LVEF, left ventricular end-diastolic dimension (LVEDD, stroke volume (SV, brain natriuretic peptide (BNP, 6-minute walk test (6MWT, and overall efficacy. The result showed that OCHM combined with CMT for the improvement of therapeutic effect in DCM patients. However, the evidence remains weak due to the small sample size, high clinical heterogeneity, and poor methodological quality of the included trials. Further, large sample size and well-designed trials are needed.

  12. Imaging and 1-day kinetics of intracoronary stem cell transplantation in patients with idiopathic dilated cardiomyopathy

    International Nuclear Information System (INIS)

    Lezaic, Luka; Socan, Aljaz; Peitl, Petra Kolenc; Poglajen, Gregor; Sever, Matjaz; Cukjati, Marko; Cernelc, Peter; Vrtovec, Bojan

    2016-01-01

    Background: Stem cell transplantation is an emerging method of treatment for patients with cardiovascular disease. There are few studies completed or ongoing on stem cell therapy in patients with idiopathic dilated cardiomyopathy (IDCM). Information on stem cell homing and distribution in the myocardium after transplantation might provide important insight into effectiveness of transplantation procedure. Aim: To assess early engraftment, retention and migration of intracoronarily transplanted stem cells in the myocardium of patients with advanced dilated cardiomyopathy of non-ischaemic origin using stem cell labeling with 99m Tc-exametazime (HMPAO). Materials, methods: Thirty-five patients with IDCM and advanced heart failure were included in the study. Autologous hematopoietic (CD34 +) stem cells were harvested by peripheral blood apheresis after bone marrow stimulation, labeled with 99m Tc-HMPAO, tested for viability and injected into coronary vessel supplying areas of myocardium selected by myocardial perfusion scintigraphy as dysfunctional yet viable. Imaging was performed 1 h and 18 h after transplantation. Results: Myocardial stem cell retention ranged from 0 to 1.44% on early and 0–0.97% on delayed imaging. Significant efflux of stem cells occurred from site of delivery in this time period (p < 0.001). Stem cell viability was not affected by labeling. Conclusion: Stem cell labeling with 99m Tc-HMPAO is a feasible method for stem cell tracking after transplantation in patients with IDCM.

  13. Lamin A/C truncation in dilated cardiomyopathy with conduction disease

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    Huber Jill M

    2003-07-01

    Full Text Available Abstract Background Mutations in the gene encoding the nuclear membrane protein lamin A/C have been associated with at least 7 distinct diseases including autosomal dominant dilated cardiomyopathy with conduction system disease, autosomal dominant and recessive Emery Dreifuss Muscular Dystrophy, limb girdle muscular dystrophy type 1B, autosomal recessive type 2 Charcot Marie Tooth, mandibuloacral dysplasia, familial partial lipodystrophy and Hutchinson-Gilford progeria. Methods We used mutation detection to evaluate the lamin A/C gene in a 45 year-old woman with familial dilated cardiomyopathy and conduction system disease whose family has been well characterized for this phenotype 1. Results DNA from the proband was analyzed, and a novel 2 base-pair deletion c.908_909delCT in LMNA was identified. Conclusions Mutations in the gene encoding lamin A/C can lead to significant cardiac conduction system disease that can be successfully treated with pacemakers and/or defibrillators. Genetic screening can help assess risk for arrhythmia and need for device implantation.

  14. Comparison of Immune Profiles in Fetal Hearts with Idiopathic Dilated Cardiomyopathy, Maternal Autoimmune-Associated Dilated Cardiomyopathy and the Normal Fetus.

    Science.gov (United States)

    Nield, Lynne E; von Both, Ingo; Popel, Najla; Strachan, Kate; Manlhiot, Cedric; Shannon, Patrick; McCrindle, Brian W; Atkinson, Adelle; Miner, Steven E S; Jaeggi, Edgar T; Taylor, Glenn P

    2016-02-01

    The etiology of idiopathic dilated cardiomyopathy (iDCM) remains unknown. Immune therapies have improved outcome in fetuses with DCM born to mothers with autoimmune disease (aDCM). The purpose of this retrospective study was to compare the myocardial B and T cell profiles in fetuses and neonates with idiopathic DCM (iDCM) versus autoimmune-mediated DCM (aDCM) and to describe the normal cell maturation within the human fetal myocardium. Of 60 fetal autopsy cases identified from institutional databases, 10 had aDCM (18-38 weeks), 12 iDCM (19-37 weeks) and 38 had normal hearts (11-40 weeks). Paraffin-embedded myocardium sections were stained for all lymphocyte (CD45), B cells (CD20, CD79a), T cells (CD3, CD4, CD7, CD8) and monocyte (CD68) surface markers. Two independent, blinded cell counts were performed. Normal hearts expressed all B and T cell markers in a bimodal fashion, with peaks at 22 and 37 weeks of gestation. The aDCM cohort was most distinct from normal hearts, with less overall T cell markers [EST -9.1 (2.6) cells/mm(2), p = 0.001], CD4 [EST -2.0 (0.6), p = 0.001], CD3 [EST -3.9 (1.0), p cell markers [EST -4.9 (1.8), p = 0.01] and CD79a counts [EST -2.3 (0.9), p = 0.01]. The iDCM group had less overall B cell markers [EST -4.0 (1.8), p = 0.03] and CD79a [EST -1.7 (0.9), p = 0.05], but no difference in T cell markers. Autoimmune-mediated DCM fetuses have less B and T cell markers, whereas iDCM fetuses have less B cell markers compared with normal fetal hearts. The fetal immune system may play a role in the normal development of the heart and evolution of dilated cardiomyopathy.

  15. Long-term outcomes of dilated cardiomyopathy diagnosed during childhood: results from a national population-based study of childhood cardiomyopathy.

    Science.gov (United States)

    Alexander, Peta M A; Daubeney, Piers E F; Nugent, Alan W; Lee, Katherine J; Turner, Christian; Colan, Steven D; Robertson, Terry; Davis, Andrew M; Ramsay, James; Justo, Robert; Sholler, Gary F; King, Ingrid; Weintraub, Robert G

    2013-10-29

    Existing studies of childhood dilated cardiomyopathy deal mainly with early survival. This population-based study examines long-term outcomes for children with dilated cardiomyopathy. The diagnosis of dilated cardiomyopathy was based on clinical, echocardiographic, and pathological findings. The primary study end point included time to the combined outcome of death or cardiac transplantation. There were 175 patients 0 to time of diagnosis. Survival free from death or transplantation was 74% (95% confidence interval, 67-80) 1 year after diagnosis, 62% (95% confidence interval, 55-69) at 10 years, and 56% (95% confidence interval, 46-65) at 20 years. In multivariable analysis, age at diagnosis 5 years, familial cardiomyopathy, and lower baseline left ventricular fractional shortening Z score were associated with increased risk of death or transplantation, as was lower left ventricular fractional shortening Z score during follow-up. At 15 years after diagnosis, echocardiographic normalization had occurred in 69% of surviving study subjects. Normalization was related to higher baseline left ventricular fractional shortening Z score, higher left ventricular fractional shortening Z score during follow-up, and greater improvement in left ventricular fractional shortening Z score. Children with lymphocytic myocarditis had better survival and a higher rate of echocardiographic normalization. At the latest follow-up, 100 of 104 of survivors (96%) were free of cardiac symptoms, and 83 (80%) were no longer receiving pharmacotherapy. Death or transplantation occurred in 26% of patients with childhood dilated cardiomyopathy within 1 year of diagnosis and ~1% per year thereafter. Risk factors for death or transplantation include age at diagnosis, familial cardiomyopathy, and severity of left ventricular dysfunction. The majority of surviving subjects are well and free of cardiac medication.

  16. Altered expression of genes for Kir ion channels in dilated cardiomyopathy.

    Science.gov (United States)

    Szuts, Viktoria; Ménesi, Dalma; Varga-Orvos, Zoltán; Zvara, Ágnes; Houshmand, Nazanin; Bitay, Miklós; Bogáts, Gábor; Virág, László; Baczkó, István; Szalontai, Balázs; Geramipoor, Amir; Cotella, Diego; Wettwer, Erich; Ravens, Ursula; Deák, Ferenc; Puskás, László G; Papp, Julius Gy; Kiss, Ibolya; Varró, András; Jost, Norbert

    2013-08-01

    Dilated cardiomyopathy (DCM) is a multifactorial disease characterized by left ventricular dilation that is associated with systolic dysfunction and increased action potential duration. The Kir2.x K⁺ channels (encoded by KCNJ genes) regulate the inward rectifier current (IK1) contributing to the final repolarization in cardiac muscle. Here, we describe the transitions in the gene expression profiles of 4 KCNJ genes from healthy or dilated cardiomyopathic human hearts. In the healthy adult ventricles, KCNJ2, KCNJ12, and KCNJ4 (Kir2.1-2.3, respectively) genes were expressed at high levels, while expression of the KCNJ14 (Kir2.4) gene was low. In DCM ventricles, the levels of Kir2.1 and Kir2.3 were upregulated, but those of Kir2.2 channels were downregulated. Additionally, the expression of the DLG1 gene coding for the synapse-associated protein 97 (SAP97) anchoring molecule exhibited a 2-fold decline with increasing age in normal hearts, and it was robustly downregulated in young DCM patients. These adaptations could offer a new aspect for the explanation of the generally observed physiological and molecular alterations found in DCM.

  17. Markers of immune activation and apoptosis in endomyocardial biopsies of individuals with recent-onset dilated cardiomyopathy

    Czech Academy of Sciences Publication Activity Database

    Kubánek, M.; Sramko, M.; Malušková, J.; Chudíčková, Milada; Holáň, Vladimír; Kautzner, J.

    2013-01-01

    Roč. 34, č. 1 (2013), s. 211-211 ISSN 0195-668X. [Congress of the European-Society-of-Cardiology (ESC). 31.08.2013-,04.09.2013, Amsterdam] Institutional support: RVO:68378041 Keywords : dilated cardiomyopathy * immune activation and apoptosis Subject RIV: EC - Immunology

  18. Severe reversible dilated cardiomyopathy associated with a large left ventricular thrombus in a young child with middle aortic syndrome

    OpenAIRE

    Ponniah, U; Overholt, E

    2014-01-01

    this article reports a case of a seven-year girl who presented with severe dilated cardiomyopathy (DCM) associated with a large thrombus in the left ventricle (LV). She had a long segment stenosis of the lower thoracic descending aorta, possibly due to non-specific aortitis and underwent successful stent angioplasty. The LV thrombus resolved after heparin without sequelae.

  19. Our Experience with Autologous Bone Marrow Stem Cell Application in Dilated Cardiomyopathy

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    Mukund K

    2009-01-01

    Full Text Available Background - Use of autologous bone marrow stem cell is a newly evolving treatment modality for end stage cardiac failure as reported in the literature. We report our experience with two patients with dilated cardiomyopathy who underwent this treatment after failure of maximal conventional therapy. Methods - A 29 year old Male patient with history of orthopnea and PND, with a diagnosis of dilated cardiomyopathy and echocardiographic evidence of severe LV dysfunction was referred for further treatment. His echo on admission showed EF of 17% and no other abnormal findings except elevated bilirubin levels. He was in NYHA functional class IV. He received intracoronary injection of autologous bone marrow stem cells in January 2009. 254X106 cells were injected with a CD34+ of 0.20%. His clinical condition stabilized and he was discharged home. He received a second injection of 22X106 in vitro expanded stem cells with a CD34+ of 0.72% in Aug 2009. He is now in NYHA class II-III with EF 24%. A 31year old Male patient with history of increasing shortness of breath, severe over the past 3-4 days was admitted for evaluation and treatment. His echo on admission showed EF of 20% and was in NYHA functional class IV. Coronary angiogram was normal and he was stabilized on maximal anti failure measures. He received intracoronary autologous bone marrow stem cell injection of 56X106 with a CD34+ of 0.53% in August 2009. His clinical condition stabilized over the next 10 days and he was discharged home. Conclusions - In our experience of two cases of dilated cardiomyopathy, safety of intracoronary injection of autologous bone marrow stem cells both isolated and in vitro expanded has been proven in both the cases with efficacy proven in one of the cases. Long term follow-up of these two cases and inclusion of more number of similar cases where all available conventional therapies have not resulted in significant improvement for such studies are planned.

  20. Effect of arotinolol on left ventricular function in patients with idiopathic dilated cardiomyopathy.

    Science.gov (United States)

    Fan, Chao-mei; Yang, Hong; Li, Yi-shi; Xu, Li; Dou, Ke-fei; Zhao, Jing-lin; Yuan, Xian-qi; Zhao, Yan-fen; Shi, Rong-fang; Du, Xiu-qing; Lu, Na-qiang

    2007-12-01

    To evaluate the efficacy and safety of long-term treatment with arotinolol in patients with idiopathic dilated cardiomyopathy (IDCM). Sixty-three patients with IDCM were evaluated at baseline and after 12-month therapy with arotinolol. The conventional therapy for congestive heart failure was continued throughout the study with arotinolol as the only beta-blocker. Left ventricular function was assessed with the New York Heart Association functional class and two-dimensional echocardiography. After 12-month arotinolol treatment, there was a significant improvement in left ventricular systolic function. Left ventricular end-systolic dimension significantly decreased from 59.52 +/- 8.83 mm to 50.89 +/- 8.17 mm (P arotinolol treatment has a favorable effect on left ventricular function in patients with IDCM, and it is safe and well tolerated.

  1. Clinical and pathologic findings of myocarditis in two families with dilated cardiomyopathy

    International Nuclear Information System (INIS)

    O'Connell, J.B.; Fowles, R.E.; Robinson, J.A.; Subramanian, R.; Henkin, R.E.; Gunnar, R.M.

    1984-01-01

    The use of endomyocardial biopsy and gallium-67 scans in patients with dilated cardiomyopathy (DCM) has demonstrated the presence of myocardial inflammation in a subset of patients. A family with DCM was studied with endomyocardial biopsy and gallium-67 scanning; both identified the presence of myocarditis in the proband. Evaluation of histologic sections from deceased family members revealed myocarditis as the principal pathologic finding. This patient identified during life demonstrated a defect in suppressor lymphocytic function and improved with immunosuppressive therapy. A second family with DCM was discovered when postmortem examination of the proband and his father's heart showed myocarditis. A living sibling was identified with asymptomatic myocardial dysfunction. Longitudinal follow-up of surviving members of both families are in progress. This study indicates that thorough diagnostic evaluation of all patients with familial DCM should be pursued to identify subgroups with potentially treatable inflammation

  2. MLP and CARP are linked to chronic PKCα signalling in dilated cardiomyopathy.

    Science.gov (United States)

    Lange, Stephan; Gehmlich, Katja; Lun, Alexander S; Blondelle, Jordan; Hooper, Charlotte; Dalton, Nancy D; Alvarez, Erika A; Zhang, Xiaoyu; Bang, Marie-Louise; Abassi, Yama A; Dos Remedios, Cristobal G; Peterson, Kirk L; Chen, Ju; Ehler, Elisabeth

    2016-06-29

    MLP (muscle LIM protein)-deficient mice count among the first mouse models for dilated cardiomyopathy (DCM), yet the exact role of MLP in cardiac signalling processes is still enigmatic. Elevated PKCα signalling activity is known to be an important contributor to heart failure. Here we show that MLP directly inhibits the activity of PKCα. In end-stage DCM, PKCα is concentrated at the intercalated disc of cardiomyocytes, where it is sequestered by the adaptor protein CARP in a multiprotein complex together with PLCβ1. In mice deficient for both MLP and CARP the chronic PKCα signalling chain at the intercalated disc is broken and they remain healthy. Our results suggest that the main role of MLP in heart lies in the direct inhibition of PKCα and that chronic uninhibited PKCα activity at the intercalated disc in the absence of functional MLP leads to heart failure.

  3. Epidemiological study of dilated cardiomyopathy from eastern India with special reference to left atrial size

    Directory of Open Access Journals (Sweden)

    Rudrajit Paul, Saumen Nandi, Pradip K Sinha

    2014-07-01

    Full Text Available Dilated cardiomyopathy (DCM is a common cause of emergency visit in our country. The disease is often misdiagnosed and mistreated. There are very few studies on DCM from India. We undertook a small study on DCM patients from Eastern India to find the demographic and echocardiographic characteristics. Patients and methods: We under took this study in a tertiary care Medical College of Eastern India. All patients coming to the emergency with dyspnea were evaluated for cardiac dysfunction. Emergency echocardiography was done to diagnose dilated cardiomyopathy. Patients with DCM were then evaluated as per protocol. After stabilization, echocardiography was repeated to note the study parameters like left atrial diameter. Standard statistical tests were used. Results: we had a total of 70 patients in our study with a male: female ratio of 43:27. Most patients were aged over 40 years. Patients with COPD, history of radiation, malignancy or drug abuse were excluded. Most patients (47% were on NYHA stage 3 at the time of presentation. In our patient cohort, 24% were alcoholic and 46% were smokers. Atrial fibrillation was present in 15.7% of the patients and right and left bundle branch block had been present in 8 and 15 patients respectively. In echocardiography, increased left atrial (LA size (>40 mm was found in 45 patients. Many patients had valvular regurgitation, mitral, aortic or tricuspid. LA size was positively correlated with left ventricular systolic diameter (r=0.403 and negatively correlated with ejection fraction (r= -0.23. Analysis and conclusion: different ECG abnormalities like bundle branch block and arrhythmias like atrial fibrillation are quite common in DCM. In echocardiography, left atrial size is an important prognostic marker and correlates with left ventricular function.

  4. 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.

  5. Exome-wide association study reveals novel susceptibility genes to sporadic dilated cardiomyopathy.

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    Ulrike Esslinger

    Full Text Available Dilated cardiomyopathy (DCM is an important cause of heart failure with a strong familial component. We performed an exome-wide array-based association study (EWAS to assess the contribution of missense variants to sporadic DCM.116,855 single nucleotide variants (SNVs were analyzed in 2796 DCM patients and 6877 control subjects from 6 populations of European ancestry. We confirmed two previously identified associations with SNVs in BAG3 and ZBTB17 and discovered six novel DCM-associated loci (Q-value<0.01. The lead-SNVs at novel loci are common and located in TTN, SLC39A8, MLIP, FLNC, ALPK3 and FHOD3. In silico fine mapping identified HSPB7 as the most likely candidate at the ZBTB17 locus. Rare variant analysis (MAF<0.01 demonstrated significant association for TTN variants only (P = 0.0085. All candidate genes but one (SLC39A8 exhibit preferential expression in striated muscle tissues and mutations in TTN, BAG3, FLNC and FHOD3 are known to cause familial cardiomyopathy. We also investigated a panel of 48 known cardiomyopathy genes. Collectively, rare (n = 228, P = 0.0033 or common (n = 36, P = 0.019 variants with elevated in silico severity scores were associated with DCM, indicating that the spectrum of genes contributing to sporadic DCM extends beyond those identified here.We identified eight loci independently associated with sporadic DCM. The functions of the best candidate genes at these loci suggest that proteostasis regulation might play a role in DCM pathophysiology.

  6. Right ventricular bifocal stimulation in the treatment of dilated cardiomyopathy with heart failure

    Directory of Open Access Journals (Sweden)

    José Carlos Pachón Mateos

    1999-12-01

    Full Text Available OBJECTIVE: To describe a new more efficient method of endocardial cardiac stimulation, which produces a narrower QRS without using the coronary sinus or cardiac veins. METHODS: We studied 5 patients with severe dilated cardiomyopathy, chronic atrial fibrillation and AV block, who underwent definitive endocardial pacemaker implantation, with 2 leads, in the RV, one in the apex and the other in the interventricular septum (sub pulmonary, connected, respectively, to ventricular and atrial bicameral pacemaker outputs. Using Doppler echocardiography, we compared, in the same patient, conventional (VVI, high septal ("AAI" and bifocal ("DDT" with AV interval ~ 0 stimulation. RESULTS: The RV bifocal stimulation had the best results with an increase in ejection fraction and cardiac output and reduction in QRS duration, mitral regurgitation and in the left atrium area (p <= 0.01. The conventional method of stimulation showed the worst result. CONCLUSION: These results suggest that, when left ventricular stimulation is not possible, right ventricular bifocal stimulation should be used in patients with severe cardiomyopathy where a pacemaker is indicated.

  7. A young man with hemoptysis: Rare association of idiopathic pulmonary hemosiderosis, celiac disease and dilated cardiomyopathy

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    Gopi C Khilnani

    2015-01-01

    Full Text Available Idiopathic pulmonary hemosiderosis (IPH is a rare cause of recurrent diffuse alveolar hemorrhage (DAH with no specific treatment. Herein, we discuss a case of hemoptysis, who had IPH and other rare associations. A 19-year-old man presented with recurrent hemoptysis, generalized weakness and progressive dyspnea for 3 years. Earlier, he was diagnosed with anemia and was treated with blood transfusions and hematinics. On examination he had pallor, tachycardia and was underweight. Investigations revealed low level of hemoglobin (7.8 g/dl and iron deficiency. An electrocardiography (ECG showed sinus tachycardia, interventricular conduction delay and T-wave inversion. Echocardiography revealed dilated cardiomyopathy with left ventricular dysfunction. Computed tomography of the chest demonstrated bilateral diffuse ground glass opacity suggestive of pulmonary hemorrhage. Pulmonary function tests showed restrictive pattern with increased carbon monoxide diffusion. Bronchoalveolar lavage and transbronchial lung biopsy showed hemosiderin-laden macrophages. Patient could recall recurrent episodes of diarrhea in childhood. Serum antitissue transglutamase antibodies were raised (291.66 IU/ml, normal <30 IU/ml. Duodenal biopsy showed subtotal villous atrophy consistent with celiac disease. He was started on gluten-free diet, beta blockers and diuretics. After two years of treatment, he has been showing consistent improvement. Screening for CD is important in patients with IPH. Cardiomyopathy forms rare third association. All three show improvement with gluten-free diet.

  8. LEUCINE-RICH REPEAT CONTAINING 10 (LRRC10 AND DILATED CARDIOMYOPATHY

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    Matthew J Brody

    2016-08-01

    Full Text Available Leucine-rich repeat containing protein 10 (LRRC10 is a cardiomyocyte-specific member of the Leucine-rich repeat containing (LRRC protein superfamily with critical roles in cardiac function and disease pathogenesis. Recent studies have identified LRRC10 mutations in human idiopathic dilated cardiomyopathy (DCM and Lrrc10 homozygous knockout mice develop DCM, strongly linking LRRC10 to the molecular etiology of DCM. LRRC10 localizes to the dyad region in cardiomyocytes where it can interact with actin and α-actinin at the Z-disc and associate with T-tubule components. Indeed, this region is becoming increasingly recognized as a signaling center in cardiomyocytes, not only for calcium cycling, excitation-contraction coupling, and calcium-sensitive hypertrophic signaling, but also as a nodal signaling hub where the myocyte can sense and respond to mechanical stress. Disruption of a wide range of critical structural and signaling molecules in cardiomyocytes confers susceptibility to cardiomyopathies in addition to the more classically studied mutations in sarcomeric proteins. However, the molecular mechanisms underlying DCM remain unclear. Here, we review what is known about the cardiomyocyte functions of LRRC10, lessons learned about LRRC10 and DCM from the Lrrc10 knockout mouse model, and discuss ongoing efforts to elucidate molecular mechanisms whereby mutation or absence of LRRC10 mediates cardiac disease.

  9. Clinical usefulness of Tc-99m hexakis 2-methoxybutyl isonitrile gated SPECT in patients with dilated cardiomyopathy: retrospective analysis

    International Nuclear Information System (INIS)

    Khan, Z.R.; Khan, A.Z.

    2010-01-01

    Background: In Dilated cardiomyopathy the heart is enlarged and ventricles are dilated. Gated myocardial perfusion single photon emission computed tomography is considered state of the art for myocardial perfusion imaging. A retrospective analysis was conducted to evaluate patients with dilated cardiomyopathy with Tc-99m sestamibi gated myocardial perfusion single photon emission computed tomography to evaluate its clinical utility. Methods: A 10 year retrospective medical record review was done from 1991 to 2001 at Wake Forest University, North Carolina, USA. Eligibility criteria included a diagnosis of dilated cardiomyopathy and availability of coronary angiography and Tc-99m sestamibi cardiac imaging results. 26 cases were selected for the final review and inclusion in the study. The study was done with standard protocols for cardiac sestamibi imaging. Results: A total of 26 cases were included in the final analysis. Cases were divided into two main groups. Group-A included 16 patients with no correlation between Tc-99m sestamibi and cardiac catheterisation reports. Group-B included 10 patients with good correlation between the above tests. There were no significant differences between the left ventricular ejection fraction, angina history, sex distribution and diabetic status between the two groups. We applied Wilcoxon Signed Rank Test and z-test to quantify the difference between the two groups. Data was tabulated and z-test was performed. The calculated p value was <0.0001. This is significantly less than the tabulated p-value at 5% level of significance, i.e., 1.96. Significant differences exist between Group-A and Group-B. Conclusion: Tc-99m sestamibi is an excellent agent for investigating myocardial perfusion in dilated cardiomyopathy. The reversible and fixed perfusion defects (small to medium sized) seen in dilated cardiomyopathy after performance of Tc-99m sestamibi gated single photon emission computed tomography imaging may not be due to coronary

  10. Clinical usefulness of Tc-99m hexakis 2-methoxyisobutyl isonitrile gated spect in patients with dilated cardiomyopathy: retrospective analysis.

    Science.gov (United States)

    Khan, Zahid Rahman; Watson, Nat E; Khan, Afshan Zahid

    2010-01-01

    In Dilated cardiomyopathy the heart is enlarged and ventricles are dilated. Gated myocardial perfusion single photon emission computed tomography is considered state of the art for myocardial perfusion imaging. A retrospective analysis was conducted to evaluate patients with dilated cardiomyopathy with Tc-99m sestamibi gated myocardial perfusion single photon emission computed tomography to evaluate its clinical utility. A 10 year retrospective medical record review was done from 1991 to 2001 at Wake Forest University, North Carolina, USA. Eligibility criteria included a diagnosis of dilated cardiomyopathy and availability of coronary angiography and Tc-99m sestamibi cardiac imaging results. 26 cases were selected for the final review and inclusion in the study. The study was done with standard protocols for cardiac sestamibi imaging. A total of 26 cases were included in the final analysis. Cases were divided into two main groups. Group-A included 16 patients with no correlation between Tc-99m sestamibi and cardiac catheterisation reports. Group-B included 10 patients with good correlation between the above tests. There were no significant differences between the left ventricular ejection fraction, angina history, sex distribution and diabetic status between the two groups. We applied Wilcoxon Signed Rank Test and z-test to quantify the difference between the two groups. Data was tabulated and z-test was performed. The calculated p-value was <0.0001. This is significantly less than the tabulated p-value at 5% level of significance, i.e., 1.96. Significant differences exist between Group-A and Group-B. Tc-99m sestamibi is an excellent agent for investigating myocardial perfusion in dilated cardiomyopathy. The reversible and fixed perfusion defects (small to medium sized) seen in dilated cardiomyopathy after performance of Tc-99m sestamibi gated single photon emission computed tomography imaging may not be due to coronary artery disease. Tc-99m sestamibi single

  11. Hypothyroidism - A cause for dilated cardiomyopathy in dogs; four year study (2008-2011

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    Satish Kumar Karlapudi

    Full Text Available Aim: The study was carried out to understand the thyroid dysfunction and its association with dilated cardiomyopathy in dogs. Materials and Methods: The study was done at Teaching Veterinary Hospital, Bhoiguda, College of Veterinary Science, Hyderabad for four years, i.e., from 2008 to 2011. A total of 256 dogs of various breed, age and sex were presented with typical skin and coat abnormalities. Few were also exhibiting signs of low metabolic rate. Skin sample analysis was done to rule out the causes of dermatitis. Thyroid profile was estimated to diagnose hypothyroidism. Selected cases were also subjected for echocardiography to study the association of cardiomyopathy. Based on thyroid profile, hypothyroid dogs were treated with levothyroxine @20mcg/kg wt, once daily, orally on empty stomach and dilated cardiomyopathy (DCM associated patients were additionally supplemented with enalapril @0.5 mg/kg, twice daily, orally for 6 months. The hemato biochemical and echocardiographic aspects are discussed. Results: The classical signs that were recorded in almost all the thyroid dysfunction dogs (231 were bilateral alopecia, rat tail and pigmentation and whereas, dyspnoea at rest, exercise intolerance, obesity, pale mucosae and corneal lipidosis were the significant low metabolic rate signs noticed in 42 dogs. However, syncope and seizures were also recorded in 31 of these hypothyroid dogs. Echocardiographic evaluation revealed significantly (P<0.01 increased LVEDd and LVEDs along with decreased IVS and LVPW both at systole and diastole among 33 dogs. Normocytic, normochromic and non-regenerative anemia and significantly (P<0.05 low T3, fT4, tT4 along with elevated serum cholesterol, triglycerides, TSH, CKMB, LDH and ALP were the hemato – biochemical findings among these dogs. After the initiation of therapy, improvement in clinical signs was noticed from day 7 and complete clinical recovery by the end of therapy. However, a non significant

  12. Canopy 2 attenuates the transition from compensatory hypertrophy to dilated heart failure in hypertrophic cardiomyopathy.

    Science.gov (United States)

    Guo, Jian; Mihic, Anton; Wu, Jun; Zhang, Yuemei; Singh, Kaustabh; Dhingra, Sanjiv; Weisel, Richard D; Li, Ren-Ke

    2015-10-01

    A mismatch between adequate angiogenesis and overgrowth of myocytes may be a critical mechanism controlling the transition from adaptive hypertrophy to heart failure. Canopy 2 (CNPY2) was recently identified as a secreted, HIF-1α-regulated angiogenic growth factor. As angiogenic factors play important roles in the development of myocardial hypertrophy, we investigated the role of CNPY2 in molecular and functional changes during development of chronic heart failure using cardiac-specific transgenic (TG) mice that overexpress human CNPY2. We generated TG mice that constitutively express CNPY2 in the myocardium. Cardiomyopathy was induced in TG and wild-type (WT) mice by transverse aortic constriction (TAC). WT mice developed significant ventricular hypertrophy at 4 weeks and severe dilatation and heart failure at 12 weeks after TAC. However, TG mice preserved much better cardiac structure and function, with less severe ventricular dilatation and markedly reduced cardiac apoptosis and fibrosis following TAC. Excess CNPY2 in TG mice prevented significant loss of vasculature up to 12 weeks after TAC injury, resulting in a better local myocardial environment that facilitated myocyte survival and prevented excessive matrix remodelling compared with WT mice. TG mice had less accumulation of endogenous tumor suppressor p53 after TAC, indicating intrinsic activation of the p53-mediated repression of HIF-1α, and Cnpy2 was diminished in TG mice compared with WT controls. Our study showed a correlation between downregulation of endogenous mouse Cnpy2 and p53-mediated HIF-1α inhibition during late-stage hypertrophic development. Additional CNPY2 attenuated the transition from compensatory hypertrophic response to maladaptive ventricular dilatation and heart failure. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2015. For permissions please email: journals.permissions@oup.com.

  13. Effects of candesartan on electrical remodeling in the hearts of inherited dilated cardiomyopathy model mice.

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    Fuminori Odagiri

    Full Text Available Inherited dilated cardiomyopathy (DCM is characterized by dilatation and dysfunction of the ventricles, and often results in sudden death or heart failure (HF. Although angiotensin receptor blockers (ARBs have been used for the treatment of HF, little is known about the effects on postulated electrical remodeling that occurs in inherited DCM. The aim of this study was to examine the effects of candesartan, one of the ARBs, on cardiac function and electrical remodeling in the hearts of inherited DCM model mice (TNNT2 ΔK210. DCM mice were treated with candesartan in drinking water for 2 months from 1 month of age. Control, non-treated DCM mice showed an enlargement of the heart with prolongation of QRS and QT intervals, and died at t1/2 of 70 days. Candesartan dramatically extended the lifespan of DCM mice, suppressed cardiac dilatation, and improved the functional parameters of the myocardium. It also greatly suppressed prolongation of QRS and QT intervals and action potential duration (APD in the left ventricular myocardium and occurrence of ventricular arrhythmia. Expression analysis revealed that down-regulation of Kv4.2 (Ito channel protein, KChIP2 (auxiliary subunit of Kv4.2, and Kv1.5 (IKur channel protein in DCM was partially reversed by candesartan administration. Interestingly, non-treated DCM heart had both normal-sized myocytes with moderately decreased Ito and IKur and enlarged cells with greatly reduced K+ currents (Ito, IKur IK1 and Iss. Treatment with candesartan completely abrogated the emergence of the enlarged cells but did not reverse the Ito, and IKur in normal-sized cells in DCM hearts. Our results indicate that candesartan treatment suppresses structural remodeling to prevent severe electrical remodeling in inherited DCM.

  14. Resultados da cardiomioplastia no tratamento da cardiomiopatia dilatada Cardiomyoplasty results in the treatment of dilated cardiomyopathy

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    Luiz Felipe P Moreira

    1991-08-01

    Full Text Available A cardiomioplastia tem sido proposta como uma alternativa ao transplante cardíaco no tratamento das cardiomiopatias isquémicas ou dilatadas. No período de maio de 1988 a outubro de 1990, 16 pacientes portadores de cardiomiopatia dilatada foram submetidos à cardiomioplastia no Instituto do Coração. Dez pacientes estavam em classe funcional III e seis em classe IV. Não houve óbitos no período de pós-operatório imediato. O tempo médio de seguimento foi de 16,9 ± 2,5 meses e a sobrevida atuarial foi 74% no 1º ano e 64,8% no 2º ano após a cardiomioplastia, sendo influenciada pela má evolução dos pacientes operados com diâmetro de ventrículo esquerdo maior do que 80 mm. Esses valores foram superiores, contudo, à sobrevida de um e dois anos de 39,5 e 29,6%, respectivamente, apresentada pelo grupo controle de 20 pacientes mantidos clinicamente (p = 0,06. Cinco dos 11 pacientes em seguimento após a cardiomioplastia, retornaram à classe funcional I e seis estão em classe II. Aos seis meses de pós-operatório, foi documentada a elevação da fração de ejeção do ventrículo esquerdo de 20,1 ± 3,8 para 26 ± 7,8% pelo estudo radioisotópico (p Dynamic cardiomyplasty has been proposed as an alternative surgical treatment for severe cardiomyopathies. From May 1988 to October 1990, dynamic cardiomyoplasty was performed in 16 patients with dilated or chagasic cardiomyopathy at the Heart Institute. Ten patients were in New York Heart Association (NYHA Class III and six in Class IV. There were no operative deaths. During a men follow-up of 16.9 ± 2.5 months, the actuarial survival was 74% at 1 year and 64.8% at 2 years of follow-up. This survival was influenced by the worse evolution of patients with left ventricular internal diameter more than 80 mm. These results were, however, better than the survival of 39.5 and 29.6%, presented at the same periods, respectively, by 20 patients maintained under medical therapy. Five of the 11

  15. CLINICAL AND INSTRUMENTAL DATA AND SURVIVAL IN DILATED CARDIOMYOPATHY: THREE-YEAR OBSERVATION RESULTS

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    L. A. Zotova

    2014-07-01

    Full Text Available Aim — to assess survival of patients with dilated cardiomyopathy (DCM in 3‑year follow-up based on clinical history, clinical symptoms, indicators of instrumental methods of examination and tactics of the patients.Materials and methods. 105 patients with diagnosed cardiomyopathy were included in prospective single-center study. Follow‑up period was 3 years or until achieving primary endpoint. Complaints were collected, general clinical research, 6-minute walk test, electrocardiography in 12‑lead were performed annually. Also scale of evaluation of clinical status in patients with chronic heart failure (CHF in the modification of V.Y. Mareev and assessment of adherence were used annualy.Results. The group of patients with DCM was represented primarily by man, severe heart failure (III–IV functional class was originally diagnosed in almost 80 % of patients. During the 3 years of follow-up progression of heart failure, worsening of hemodynamic indices were identified in survivors. When evaluating recommended therapy with found that it meets the current guidelines of treatment of heart failure,however, low adherence to treatment of patients was revealed with statistically significant difference between groups of survivors and deceased patients. The annual mortality rate was 20 % and had no significant fluctuations. The main cause of death was heart failure decompensation.Conclusion. Progression of CHF in patients with DCM was confirmed .The most reliable method of assessing the progressive course of heart failure is 6‑minute walk test. The annual high mortality (20 % indicates an extremely poor prognosis for this disease. The major causes of death were progression of heart failure and sudden cardiac death.

  16. Clinical Profile and Prognosis of Patients with Right Ventricular Dilated Cardiomyopathy: Results of a Prospective Study

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    Ya.R. Akhmatov

    2015-12-01

    Full Text Available The aim of our study was to investigate the clinical prevalence of dilated cardiomyopathy (DCM with predominantly failure of the right-side heart (right ventricular DCM, RV-DCM, and features of the clinical course and prognosis of the disease compared to DCM with biventricular heart failure (BV-HF. The study design suggests a prospective observation of 300 patients with idiopathic DCM between 2000 and 2012. Herewith, we followed the criteria of the WHO/ISFC Task Force (1995 on the Definationa and Classification of Cardiomyopathies. All patients underwent a comprehensive examination. Two groups were formed for further comparative analysis. Group 1 included 22 patients (mean age 42.9±14.3 years, male/female 5/17 with RV-DCM. Group 2 included 38 patients (mean age 43.6±13.8, male/female 29/9 with DCM and BV-HF. The groups were matched for age, sex, NYHA class II-III, and disease duration. According to our aim, we studied 5-year survival prognosis and analyzed the incidence and causes of deaths, as well as the occurrence of nonfatal complications of the disease. Medical therapy for DCM patients was performed according to the CHF therapy guidelines (ACC/AHA 2001, 2005. The results of our investigations during many years of research have shown that the clinical incidence of RV-DCM was 7.3% among all forms of DCM. The study of life prognosis in patients with 2 forms of DCM showed that 5-year mortality of patients was about 50%. Herewith, we detected the differences in causes of death depending on the type of heart damage, primarily development of fatal pulmonary embolism.

  17. Autoantibodies in dilated cardiomyopathy induce vascular endothelial growth factor expression in cardiomyocytes

    Energy Technology Data Exchange (ETDEWEB)

    Saygili, Erol, E-mail: erol.saygili@med.uni-duesseldorf.de [Division of Cardiology, Pulmonology, and Vascular Medicine, University Hospital Düsseldorf, Moorenstrasse 5, D-40225 Düsseldorf (Germany); Noor-Ebad, Fawad; Schröder, Jörg W.; Mischke, Karl [Department of Cardiology, University RWTH Aachen, Pauwelsstr. 30, D-52074 Aachen (Germany); Saygili, Esra [Clinic for Gastroenterology, Hepatology and Infectious Diseases, Heinrich-Heine-University, Moorenstrasse 5, D-40225 Düsseldorf (Germany); Rackauskas, Gediminas [Department of Cardiovascular Medicine, Vilnius University Hospital Santariskiu Klinikos, Vilnius University (Lithuania); Marx, Nikolaus [Department of Cardiology, University RWTH Aachen, Pauwelsstr. 30, D-52074 Aachen (Germany); Kelm, Malte; Rana, Obaida R. [Division of Cardiology, Pulmonology, and Vascular Medicine, University Hospital Düsseldorf, Moorenstrasse 5, D-40225 Düsseldorf (Germany)

    2015-09-11

    Background: Autoantibodies have been identified as major predisposing factors for dilated cardiomyopathy (DCM). Patients with DCM show elevated serum levels of vascular endothelial growth factor (VEGF) whose source is unknown. Besides its well-investigated effects on angiogenesis, evidence is present that VEGF signaling is additionally involved in fibroblast proliferation and cardiomyocyte hypertrophy, hence in cardiac remodeling. Whether autoimmune effects in DCM impact cardiac VEGF signaling needs to be elucidated. Methods: Five DCM patients were treated by the immunoadsorption (IA) therapy on five consecutive days. The eluents from the IA columns were collected and prepared for cell culture. Cardiomyocytes from neonatal rats (NRCM) were incubated with increasing DCM-immunoglobulin-G (IgG) concentrations for 48 h. Polyclonal IgG (Venimmun N), which was used to restore IgG plasma levels in DCM patients after the IA therapy was additionally used for control cell culture purposes. Results: Elevated serum levels of VEGF decreased significantly after IA (Serum VEGF (ng/ml); DCM pre-IA: 45 ± 9.1 vs. DCM post–IA: 29 ± 6.7; P < 0.05). In cell culture, pretreatment of NRCM by DCM-IgG induced VEGF expression in a time and dose dependent manner. Biologically active VEGF that was secreted by NRCM significantly increased BNP mRNA levels in control cardiomyocytes and induced cell-proliferation of cultured cardiac fibroblast (Fibroblast proliferation; NRCM medium/HC-IgG: 1 ± 0.0 vs. NRCM medium/DCM-IgG 100 ng/ml: 5.6 ± 0.9; P < 0.05). Conclusion: The present study extends the knowledge about the possible link between autoimmune signaling in DCM and VEGF induction. Whether this observation plays a considerable role in cardiac remodeling during DCM development needs to be further elucidated. - Highlights: • Mechanisms of remodeling in dilated cardiomyopathy (DCM) are not fully understood. • Autoantibodies have been identified as major predisposing factors

  18. From discrete dilated cardiomyopathy to successful cardiac transplantation in congenital disorders of glycosylation due to dolichol kinase deficiency (DK1-CDG)

    NARCIS (Netherlands)

    Kapusta, L.; Zucker, N.; Frenckel, G.; Medalion, B.; Gal, T.B.; Birk, E.; Mandel, H.; Nasser, N.; Morgenstern, S.; Zuckermann, A.; Lefeber, D.J.; Brouwer, A.P. de; Wevers, R.A.; Lorber, A.; Morava, E.

    2013-01-01

    Congenital disorders of glycosylation are a growing group of inborn errors of protein glycosylation. Cardiac involvement is frequently observed in the most common form, PMM2-CDG, especially hypertrophic cardiomyopathy. Dilated cardiomyopathy, however, has been only observed in a few CDG subtypes,

  19. Interaction between Hsp60 and Bax in normal human myocardium and in myocardium affected by dilated cardiomyopathy

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    Tykhonkova I. O.

    2009-04-01

    Full Text Available The main functional compartments of molecular chaperone Hsp60 are mitochondria and cytoplasm. Up to 30 % of Hsp60 are located in cytoplasm of cardiomyocytes. The interaction between molecular chaperone Hsp60 and proapoptotic Bax protein in the cytoplasmic fraction from normal human heart tissue has been revealed by co-immunoprecipitation in contrast to myocardium affected by dilated cardiomyopathy, where this interaction has not been observed

  20. Serum Uric Acid Correlation with Echocardiographic Indices in Children with Dilated Cardiomyopathy

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    Shahrokh Rajaei

    2009-12-01

    Full Text Available Background: Dilated cardiomyopathy (DCMP is a myocardial disease characterized by dilated left ventricle or both ventricles and reduced contractility of the myocardium. In patients suffering from DCMP, the serum level of uric acid may increase. This research was designed to evaluate the effect of the serum level of uric acid on systolic and diastolic functions in patients with DCMP.Methods: This case-control study was performed on 30 patients with DCMP aged between 1 month and 12 years who were consistent with a control group in terms of age and gender. Patients suffering from congenital and acquired cardiac, renal, metabolic, endocrine, musculoskeletal, neurologic, vascular, and hematologic diseases were excluded. After physical examination, chest X-ray, and electrocardiography, systolic and diastolic parameters were measured via echocardiography, and fasting serum uric acid level was measured. The data were analyzed using the t-test and Pearson correlation coefficient.Results: The average age of the patients in the case and control groups was 7.28 and 7.13 years, respectively. There were 15 boys, and the rest were girls. The serum uric acid level in the case and control groups was 6.22 and 3.31 mg/dl, respectively; the difference was statistically significant (P value <0.01. There was a significant correlation between serum uric acid level and left ventricular isovolumic contraction, interventricular septal diameter, left ventricular septal diameter in diastole, and fractional shortening (P value <0.05.Conclusion: In children with DCMP, the serum level of uric acid increases significantly and this increase is significantly correlated with some of left heart echocardiographic parameters. This test is of predictive value for disease progression.

  1. A longitudinal study on BIO14.6 hamsters with dilated cardiomyopathy: micro-echocardiographic evaluation

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    Belfiore Maria

    2011-12-01

    Full Text Available Abstract Background In recent years, several new technologies for small-animal imaging have been developed. In particular, the use of ultrasound in animal imaging has focused on the investigation of accessible biological structures such as the heart, of which it provides a morphological and functional assessment. The purpose of this study was to investigate the role of micro-ultrasonography (μ-US in a longitudinal study on BIO14.6 cardiomyopathic hamsters treated with gene therapy. Methods Thirty hamsters were divided into three groups (n = 10: Group I, untreated BIO 14.6 hamsters; Group II, BIO 14.6 hamsters treated with gene therapy; Group III, untreated wild type (WT hamsters. All hamsters underwent serial μ-US sessions and were sacrificed at predetermined time points. Results μ-US revealed: in Group I, progressive dilation of the left ventricle with a change in heart morphology from an elliptical to a more spherical shape, altered configuration of the mitral valve and subvalvular apparatus, and severe reduction in ejection fraction; in Group II, mild decrease in contractile function and ejection fraction; in Group III, normal cardiac chamber morphology and function. There was a negative correlation between the percentage of fibrosis observed at histology and the ejection fraction obtained on μ-echocardiography (Spearman r: -0.839; p Conclusions Although histological examination remains indispensable for a conclusive diagnosis, high-frequency μ-echocardiography, thanks to the high spatial and contrast resolution, can be considered sufficient for monitoring therapeutic efficacy and/or the progression of dilated cardiomyopathy, providing an alternative tool for repeatable and noninvasive evaluation.

  2. Usefulness of running wheel for detection of congestive heart failure in dilated cardiomyopathy mouse model.

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    Masami Sugihara

    Full Text Available BACKGROUND: Inherited dilated cardiomyopathy (DCM is a progressive disease that often results in death from congestive heart failure (CHF or sudden cardiac death (SCD. Mouse models with human DCM mutation are useful to investigate the developmental mechanisms of CHF and SCD, but knowledge of the severity of CHF in live mice is necessary. We aimed to diagnose CHF in live DCM model mice by measuring voluntary exercise using a running wheel and to determine causes of death in these mice. METHODOLOGY/PRINCIPAL FINDINGS: A knock-in mouse with a mutation in cardiac troponin T (ΔK210 (DCM mouse, which results in frequent death with a t(1/2 of 70 to 90 days, was used as a DCM model. Until 2 months of age, average wheel-running activity was similar between wild-type and DCM mice (approximately 7 km/day. At approximately 3 months, some DCM mice demonstrated low running activity (LO: 5 km/day. In the LO group, the lung weight/body weight ratio was much higher than that in the other groups, and the lungs were infiltrated with hemosiderin-loaded alveolar macrophages. Furthermore, echocardiography showed more severe ventricular dilation and a lower ejection fraction, whereas Electrocardiography (ECG revealed QRS widening. There were two patterns in the time courses of running activity before death in DCM mice: deaths with maintained activity and deaths with decreased activity. CONCLUSIONS/SIGNIFICANCE: Our results indicate that DCM mice with low running activity developed severe CHF and that running wheels are useful for detection of CHF in mouse models. We found that approximately half of ΔK210 DCM mice die suddenly before onset of CHF, whereas others develop CHF, deteriorate within 10 to 20 days, and die.

  3. Pitavastatin-attenuated cardiac dysfunction in mice with dilated cardiomyopathy via regulation of myocardial calcium handling proteins

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    Hu Wei

    2014-03-01

    Full Text Available C57BL/6 mice with dilated cardiomyopathy (DCM were randomly divided to receive placebo or pitavastatin at a dose of 1 or 3 mg kg-1d-1. After 8 weeks treatment, mice with dilated cardiomyopathy developed serious cardiac dysfunction characterized by significantly enhanced left ventricular end-diastolic diameter (LVIDd, decreased left ventricular ejection fraction (LVEF as well as left ventricular short axis fractional shortening (LVFS, accompanied with enlarged cardiomyocytes, and increased plasma levels of N-terminal pro-B type natriuretic peptide (NT-proBNP and plasma angiotensin II (AngII concentration. Moreover, myocardium sarcoplasmic reticulum Ca2+ pump (SERCA-2 activity was decreased. The ratio of phosphorylated phospholamban (PLB to total PLB decreased significantly with the down-regulation of SERCA- -2a and ryanodine receptor (RyR2 expression. Pitavastatin was found to ameliorate the cardiac dysfunction in mice with dilated cardiomyopathy by reversing the changes in the ratios of phosphorylated PLB to total PLB, SERCA-2a and RyR2 via reducing the plasma AngII concentration and the expressions of myocardium angiotensin II type 1 receptor (AT1R and protein kinase C (PKCb2. The possible underlying mechanism might be the regulation of myocardial AT1R-PKCb2-Ca2+ handling proteins.

  4. Relationship between thallium-201 myocardial SPECT and findings of endomyocardial biopsy specimens in dilated cardiomyopathy

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    Watanabe, Motohiro; Gotoh, Kohshi; Nagashima, Kenshi [Gifu Univ. (Japan). School of Medicine] (and others)

    2001-02-01

    The purpose of this study was to clarify which myocardial histological findings associated with dilated cardiomyopathy (DCM) are reflected in quantitative {sup 201}Tl myocardial SPECT. We obtained studied SPECT images from 21 patients with DCM 10 minutes and 2 hours after they received an injection of 111 MBq {sup 201}Tl at rest. We calculated the percent coefficient of variation of myocardial {sup 201}Tl counts [%CV(Tl)], the washout rate (WR), standard deviation of WR [SD(WR)], extent score (ES) and severity score (SS). We used image analysis to measure % fibrosis, % myocytes, the ratio of fibrous tissue to myocyte tissue (F/My), myocyte size and standard deviation of myocyte size [SD(My)] in left ventricular endomyocardial biopsy specimens. The %CV(Tl) was correlated with % fibrosis and F/My. The ES and SS also correlated with F/My. The correlation between SD(WR) and SD(My) was significant. The present findings suggest that %CV(Tl), ES and SS of rest {sup 201}Tl SPECT reflect myocardial fibrosis and that the standard deviation of washout reflects the distribution of myocyte size. (author)

  5. Protein aggregates and novel presenilin gene variants in idiopathic dilated cardiomyopathy.

    Science.gov (United States)

    Gianni, Davide; Li, Airong; Tesco, Giuseppina; McKay, Kenneth M; Moore, John; Raygor, Kunal; Rota, Marcello; Gwathmey, Judith K; Dec, G William; Aretz, Thomas; Leri, Annarosa; Semigran, Marc J; Anversa, Piero; Macgillivray, Thomas E; Tanzi, Rudolph E; del Monte, Federica

    2010-03-16

    Heart failure is a debilitating condition resulting in severe disability and death. In a subset of cases, clustered as idiopathic dilated cardiomyopathy (iDCM), the origin of heart failure is unknown. In the brain of patients with dementia, proteinaceous aggregates and abnormal oligomeric assemblies of beta-amyloid impair cell function and lead to cell death. We have similarly characterized fibrillar and oligomeric assemblies in the hearts of iDCM patients, pointing to abnormal protein aggregation as a determinant of iDCM. We also showed that oligomers alter myocyte Ca(2+) homeostasis. Additionally, we have identified 2 new sequence variants in the presenilin-1 (PSEN1) gene promoter leading to reduced gene and protein expression. We also show that presenilin-1 coimmunoprecipitates with SERCA2a. On the basis of these findings, we propose that 2 mechanisms may link protein aggregation and cardiac function: oligomer-induced changes on Ca(2+) handling and a direct effect of PSEN1 sequence variants on excitation-contraction coupling protein function.

  6. Scintigraphic evaluation of regional myocardial sympathetic activity in patients with dilated cardiomyopathy

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    Eno, Shin; Takeo, Eiichiro; Katsumoto, Masayuki; Sasaki, Satoshi; Fujii, Hideaki; Kanazawa, Ikuo [Chugoku Rosai General Hospital, Kure, Hiroshima (Japan)

    1998-09-01

    Qualitative and quantitative analyses of sympathetic activity of heart and nervous sympathetic dysfunction in myocardium were investigated by using {sup 123}I-MIBG myocardial scintigraphy. Twelve normal persons (4 males, 8 females, mean age 61.3{+-}7.4) and 13 cases of dilated cardiomyopathy (11 males, 2 females, mean age 62.5{+-}14.2) were selected. Decrease of uptake rate to myocardium of I-MIBG (delayed image) and increase of washout rate were observed. Myocardial sympathetic hyperactivity was regarded as a main reason of these phenomena. Low uptake rate to myocardium of I-MIBG (delayed image) and high washout rate were observed in the whole left ventricle, and no difference of sympathetic activity according to locus of left ventricle was found. Correlation coefficient of left ventricular end-diastolic dimension with the washout rate of I-MIBG was 0.52 (p<0.05). It was suggested that washout rate may increase with progress of disease stage. (K.H.)

  7. Viscoelastic behavior of human lamin A proteins in the context of dilated cardiomyopathy.

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    Avinanda Banerjee

    Full Text Available Lamins are intermediate filament proteins of type V constituting a nuclear lamina or filamentous meshwork which lines the nucleoplasmic side of the inner nuclear membrane. This protein mesh provides a supporting scaffold for the nuclear envelope and tethers interphase chromosome to the nuclear periphery. Mutations of mainly A-type lamins are found to be causative for at least 11 human diseases collectively termed as laminopathies majority of which are characterised by aberrant nuclei with altered structural rigidity, deformability and poor mechanotransduction behaviour. But the investigation of viscoelastic behavior of lamin A continues to elude the field. In order to address this problem, we hereby present the very first report on viscoelastic properties of wild type human lamin A and some of its mutants linked with Dilated cardiomyopathy (DCM using quantitative rheological measurements. We observed a dramatic strain-softening effect on lamin A network as an outcome of the strain amplitude sweep measurements which could arise from the large compliance of the quasi-cross-links in the network or that of the lamin A rods. In addition, the drastic stiffening of the differential elastic moduli on superposition of rotational and oscillatory shear stress reflect the increase in the stiffness of the laterally associated lamin A rods. These findings present a preliminary insight into distinct biomechanical properties of wild type lamin A protein and its mutants which in turn revealed interesting differences.

  8. Correlation between 67-gallium imaging and endo myocardial biopsy in children with severe dilated cardiomyopathy

    International Nuclear Information System (INIS)

    Camargo, P.R.; Mazzieri, R.; Snitcowsky, R.; Meneguetti, C.; Soares Junior, J.; Higuchi, M.L.; Ebaid, M.; Pileggi, F.

    1990-01-01

    A comparative study between 67 Ga imaging and endo myocardial biopsy (EB) in children with severe dilated cardiomyopathy (DC) is performed. The results in a group of patients with active myocarditis submitted to immunosuppressive therapy are evaluated and repeated after six months. In 32 patients (72.7%) the EB revealed presence of inflammatory process; 21 (65.6%) of these had a positive 67 Ga uptake and 11 (34.4%) negative. Twelve patients with no evidence of inflammatory process in the EB, nine (75%) presented negative 67 Ga uptake. However, when the intensity of myocardial inflammatory was analysed (mild, moderate and severe) and correlated with 67 Ga imaging, was observed that the majority of patients with negative 67 Ga uptake (11 patients) had inflammatory infiltration (nine patients). In this way the 67 Ga uptake demonstrated a good correlation in the diagnosis of moderate and severe inflammatory process in children with DC. This is important because the use of immunosuppressive drugs is indicated only in these group. (author)

  9. Comparative numerical study on left ventricular fluid dynamics after dilated cardiomyopathy.

    Science.gov (United States)

    Mangual, Jan O; Kraigher-Krainer, Elisabeth; De Luca, Alessio; Toncelli, Loira; Shah, Amil; Solomon, Scott; Galanti, Giorgio; Domenichini, Federico; Pedrizzetti, Gianni

    2013-06-21

    The role of flow on the progression of left ventricular (LV) remodeling has been presumed, although measurements are still limited and the intraventricular flow pattern in remodeling hearts has not been evaluated in a clinical setting. Comparative evaluation of intraventricular fluid dynamics is performed here between healthy subjects and dilated cardiomyopathy (DCM) patients. LV fluid dynamics is evaluated in 20 healthy young men and 8 DCM patients by combination of 3D echocardiography with direct numerical simulations of the equation governing blood motion. Results are analyzed in terms of quantitative global indicators of flow energetics and blood transit properties that are representative of the qualitative fluid dynamics behaviors. The flow in DCM exhibited qualitative differences due to the weakness of the formed vortices in the large LV chamber. DCM and healthy subjects show significant volumetric differences; these also reflect inflow properties like the vortex formation time, energy dissipation, and sub-volumes describing flow transit. Proper normalization permitted to define purely fluid dynamics indicators that are not influenced by volumetric measures. Cardiac fluid mechanics can be evaluated by a combination of imaging and numerical simulation. This pilot study on pathological changes in LV blood motion identified intraventricular flow indicators based on pure fluid mechanics that could potentially be integrated with existing indicators of cardiac mechanics in the evaluation of disease progression. Copyright © 2013 Elsevier Ltd. All rights reserved.

  10. Intracoronary allogeneic cardiosphere-derived stem cells are safe for use in dogs with dilated cardiomyopathy.

    Science.gov (United States)

    Hensley, Michael Taylor; Tang, Junnan; Woodruff, Kathleen; Defrancesco, Teresa; Tou, Sandra; Williams, Christina M; Breen, Mathew; Meurs, Kathryn; Keene, Bruce; Cheng, Ke

    2017-08-01

    Cardiosphere-derived cells (CDCs) have been shown to reduce scar size and increase viable myocardium in human patients with mild/moderate myocardial infarction. Studies in rodent models suggest that CDC therapy may confer therapeutic benefits in patients with non-ischaemic dilated cardiomyopathy (DCM). We sought to determine the safety and efficacy of allogeneic CDC in a large animal (canine) model of spontaneous DCM. Canine CDCs (cCDCs) were grown from a donor dog heart. Similar to human CDCs, cCDCs express CD105 and are slightly positive for c-kit and CD90. Thirty million of allogeneic cCDCs was infused into the coronary vessels of Doberman pinscher dogs with spontaneous DCM. Adverse events were closely monitored, and cardiac functions were measured by echocardiography. No adverse events occurred during and after cell infusion. Histology on dog hearts (after natural death) revealed no sign of immune rejection from the transplanted cells. © 2017 The Authors. Journal of Cellular and Molecular Medicine published by John Wiley & Sons Ltd and Foundation for Cellular and Molecular Medicine.

  11. PRELIMINARY CHARACTERIZATION OF DILATED CARDIOMYOPATHY IN A CAPTIVE POPULATION OF BANDED MONGOOSES (MUNGOS MUNGO).

    Science.gov (United States)

    López, Javier; Dukes-McEwan, Joanna; Drake, Gabby; Chantrey, Julian

    2017-09-01

    Between 2006 and 2015, a high incidence of dilated cardiomyopathy (DCM) was diagnosed in a captive population of banded mongooses (Mungos mungo) at Chester Zoo, United Kingdom. The aim of this study was to characterize DCM in these mongooses in order to raise awareness of this condition and help inform management and clinical decisions. Prospective clinical assessments, including echocardiography, radiography, and cardiac biomarkers, were carried out in four mongooses remaining in the collection. Radiographs from 15 mature mongooses were reviewed and cardiac size and metrics assessed. Ten postmortem reports and the histologic sections from nine of these cases were reviewed for cardiac lesions. Echocardiographic findings were consistent with a diagnosis of preclinical DCM in one out of the four cases assessed, and it was considered equivocal in a second case. Taurine levels were within normal limits for domestic carnivores. Radiographs in seven mongooses showed right-sided or generalized cardiomegaly. The width of the heart in intercostal spaces and vertebral-tracheal angle on the lateral view were the most-discriminatory radiographic variables for diagnosis of cardiac disease. At necropsy, there was gross pathological evidence consistent with DCM in seven out of 10 mongooses examined. Histopathologically, mild multifocal fibrosis and rare intermyofiber edema were observed. This study provides preliminary evidence that DCM occurs in captive banded mongoose, but etiology and wider prevalence need to be determined.

  12. Cardiomiopatia dilatada em cão: relato de caso Canine dilated cardiomyopathy: case report

    Directory of Open Access Journals (Sweden)

    Ruthnéa Aparecida Lázaro Muzzi

    2000-04-01

    Full Text Available Um canino macho, sem raça definida, com três anos, pesando 36kg, foi atendido no Hospital Veterinário da Universidade Federal de Minas Gerais, apresentando tosse noturna, intolerância ao exercício, cansaço freqüente, emagrecimento e episódios de síncope. O exame clínico revelou mucosas discretamente cianóticas, tempo de perfusão capilar aumentado, presença de sopro sistólico no ápice cardíaco esquerdo e sinais de insuficiência cardíaca congestiva. Foram realizados exames radiográficos, eletrocardiográficos e ecocardiográficos, sendo diagnosticada cardiomiopatia dilatada canina.A three year-old male mixed breed dog, weighing 36kg was presented to Universidade Federal de Minas Gerais Veterinary Hospital, Brazil with history of nocturnal coughing, exercise intolerance, weakness, weight loss and syncopal episodes. Physical examination revealed cyanotic mucous membranes, prolonged capillary refill time, systolic regurgitant murmur at left apex and signs of congestive heart failure. Diagnostic techniques for heart disease were performed such as radiography, eletrocardiography and ecocardiography. These tests showed canine dilated cardiomyopathy.

  13. Sensitivity analysis of left ventricle with dilated cardiomyopathy in fluid structure simulation.

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    Bee Ting Chan

    Full Text Available Dilated cardiomyopathy (DCM is the most common myocardial disease. It not only leads to systolic dysfunction but also diastolic deficiency. We sought to investigate the effect of idiopathic and ischemic DCM on the intraventricular fluid dynamics and myocardial wall mechanics using a 2D axisymmetrical fluid structure interaction model. In addition, we also studied the individual effect of parameters related to DCM, i.e. peak E-wave velocity, end systolic volume, wall compliance and sphericity index on several important fluid dynamics and myocardial wall mechanics variables during ventricular filling. Intraventricular fluid dynamics and myocardial wall deformation are significantly impaired under DCM conditions, being demonstrated by low vortex intensity, low flow propagation velocity, low intraventricular pressure difference (IVPD and strain rates, and high-end diastolic pressure and wall stress. Our sensitivity analysis results showed that flow propagation velocity substantially decreases with an increase in wall stiffness, and is relatively independent of preload at low-peak E-wave velocity. Early IVPD is mainly affected by the rate of change of the early filling velocity and end systolic volume which changes the ventriculo:annular ratio. Regional strain rate, on the other hand, is significantly correlated with regional stiffness, and therefore forms a useful indicator for myocardial regional ischemia. The sensitivity analysis results enhance our understanding of the mechanisms leading to clinically observable changes in patients with DCM.

  14. Sensitivity Analysis of Left Ventricle with Dilated Cardiomyopathy in Fluid Structure Simulation

    Science.gov (United States)

    Chan, Bee Ting; Abu Osman, Noor Azuan; Lim, Einly; Chee, Kok Han; Abdul Aziz, Yang Faridah; Abed, Amr Al; Lovell, Nigel H.; Dokos, Socrates

    2013-01-01

    Dilated cardiomyopathy (DCM) is the most common myocardial disease. It not only leads to systolic dysfunction but also diastolic deficiency. We sought to investigate the effect of idiopathic and ischemic DCM on the intraventricular fluid dynamics and myocardial wall mechanics using a 2D axisymmetrical fluid structure interaction model. In addition, we also studied the individual effect of parameters related to DCM, i.e. peak E-wave velocity, end systolic volume, wall compliance and sphericity index on several important fluid dynamics and myocardial wall mechanics variables during ventricular filling. Intraventricular fluid dynamics and myocardial wall deformation are significantly impaired under DCM conditions, being demonstrated by low vortex intensity, low flow propagation velocity, low intraventricular pressure difference (IVPD) and strain rates, and high-end diastolic pressure and wall stress. Our sensitivity analysis results showed that flow propagation velocity substantially decreases with an increase in wall stiffness, and is relatively independent of preload at low-peak E-wave velocity. Early IVPD is mainly affected by the rate of change of the early filling velocity and end systolic volume which changes the ventriculo:annular ratio. Regional strain rate, on the other hand, is significantly correlated with regional stiffness, and therefore forms a useful indicator for myocardial regional ischemia. The sensitivity analysis results enhance our understanding of the mechanisms leading to clinically observable changes in patients with DCM. PMID:23825628

  15. Young MLP deficient mice show diastolic dysfunction before the onset of dilated cardiomyopathy.

    Science.gov (United States)

    Lorenzen-Schmidt, Ilka; Stuyvers, Bruno D; ter Keurs, Henk E D J; Date, Moto-o; Hoshijima, Masahiko; Chien, Kenneth R; McCulloch, Andrew D; Omens, Jeffrey H

    2005-08-01

    Targeted deletion of cytoskeletal muscle LIM protein (MLP) in mice consistently leads to dilated cardiomyopathy (DCM) after one or more months. However, next to nothing is known at present about the mechanisms of this process. We investigated whether diastolic performance including passive mechanics and systolic behavior are altered in 2-week-old MLP knockout (MLPKO) mice, in which heart size, fractional shortening and ejection fraction are still normal. Right ventricular trabeculae were isolated from 2-week-old MLPKO and wildtype mice and placed in an apparatus that allowed force measurements and sarcomere length measurements using laser diffraction. During a twitch from the unloaded state at 1 Hz, MLPKO muscles relengthened to slack length more slowly than controls, although the corresponding force relaxation time was unchanged. Active developed stress at a diastolic sarcomere length of 2.00 microm was preserved in MLPKO trabeculae over a wide range of pacing frequencies. Force relaxation under the same conditions was consistently prolonged compared with wildtype controls, whereas time to peak and maximum rate of force generation were not significantly altered. Ca2+ content of the sarcoplasmic reticulum (SR) and the quantities of Ca2+ handling proteins were similar in both genotypes. In summary, young MLPKO mice revealed substantial alterations in passive myocardial properties and relaxation time, but not in most systolic characteristics. These results indicate that the progression to heart failure in the MLPKO model may be driven by diastolic myocardial dysfunction and abnormal passive properties rather than systolic dysfunction.

  16. Dilated cardiomyopathy and amebic gastritis in a giant anteater (Myrmecophaga tridactyla).

    Science.gov (United States)

    Coke, Rob L; Carpenter, James W; Aboellail, Tawfk; Armbrust, Laura; Isaza, Ramiro

    2002-09-01

    An approximately 11-mo-old female giant anteater (Myrmecophaga tridactyla) exhibited anorexia, lethargy, hypothermia, depression, and minimal response to external stimuli. Radiography and ultrasonography revealed an enlarged heart, with free gas and fluid in the abdomen. Abdominocentesis produced a clear brown fluid with an acute to subacute septic suppurative exudate. Cardiac ultrasonography revealed a dilated, thin-walled left ventricle with a comparatively low fractional shortening. Despite intensive supportive care, the anteater died. Postmortem findings included gastric ulceration with perforation near the pylorus. Entameba spp. and Acanthamoeba spp. were both identified in large numbers at the site of the gastric ulceration and perforation.

  17. Acute myocardial infarction and stress cardiomyopathy following the Christchurch earthquakes.

    Science.gov (United States)

    Chan, Christina; Elliott, John; Troughton, Richard; Frampton, Christopher; Smyth, David; Crozier, Ian; Bridgman, Paul

    2013-01-01

    Christchurch, New Zealand, was struck by 2 major earthquakes at 4:36 am on 4 September 2010, magnitude 7.1 and at 12:51 pm on 22 February 2011, magnitude 6.3. Both events caused widespread destruction. Christchurch Hospital was the region's only acute care hospital. It remained functional following both earthquakes. We were able to examine the effects of the 2 earthquakes on acute cardiac presentations. Patients admitted under Cardiology in Christchurch Hospital 3 week prior to and 5 weeks following both earthquakes were analysed, with corresponding control periods in September 2009 and February 2010. Patients were categorised based on diagnosis: ST elevation myocardial infarction, Non ST elevation myocardial infarction, stress cardiomyopathy, unstable angina, stable angina, non cardiac chest pain, arrhythmia and others. There was a significant increase in overall admissions (pearthquake. This pattern was not seen after the early afternoon February earthquake. Instead, there was a very large number of stress cardiomyopathy admissions with 21 cases (95% CI 2.6-6.4) in 4 days. There had been 6 stress cardiomyopathy cases after the first earthquake (95% CI 0.44-2.62). Statistical analysis showed this to be a significant difference between the earthquakes (pearthquake triggered a large increase in ST elevation myocardial infarction and a few stress cardiomyopathy cases. The early afternoon February earthquake caused significantly more stress cardiomyopathy. Two major earthquakes occurring at different times of day differed in their effect on acute cardiac events.

  18. Histological and morphometric analysis of dilated cardiomyopathy with special reference to collagen IV expression

    Directory of Open Access Journals (Sweden)

    Parul Jain

    2017-01-01

    Full Text Available Introduction: Collagen distribution alterations are well known in dilated cardiomyopathy. There are also changes in microvasculature along with other histomorphorphological features. Aims and Objectives: To study the histomorphological features of DCM along with their quantitative correlation with LVEF. Alterations in collagen IV distribution pattern and microvasculature in DCM were also evaluated. Materials and Methods: The present study includes 34 right ventricular endomyocardial biopsies, 7 explanted native hearts and 41 autopsy control hearts. Sections were taken from lower half of right interventricular septum and stained for H and E, Masson trichrome and immunohistochemistry for CD34, SMA and Collagen IV to study the histological features, pattern of fibrosis, capillary and arteriolar distribution and collagen IV expression respectively. Morphometric analysis was carried out in all cases and controls using Image analysis software Image pro plus 7 and correlated with left ventricular ejection fraction. Results: The histomorphological changes of DCM include myocyte hypertrophy, nucleomegaly, and interstitial fibrosis. Interfiber fibrosis was the commonest. There was evidence of myocarditis, ischemic change and vessel wall alterations. Considerable alteration in Collagen IV distribution was observed with reduction in intensity and proportion of staining around myocytes quantified using Allred scoring against uniform pericellular staining in controls. Morphometric analysis revealed significant increase in nuclear area, myocyte width, percentage of fibrosis and reduction in capillary myocyte ratio in cases as compared to controls. There was no significant difference in arteriolar density. No significant association was observed between morphometric parameters and LVEF. Conclusion: Histomorphological changes in DCM are non-specific. Quantitation of histological parameters cannot be used to predict the disease progression as there was no

  19. Autophagic vacuoles in cardiomyocytes of dilated cardiomyopathy with initially decompensated heart failure predict improved prognosis.

    Science.gov (United States)

    Saito, Tsunenori; Asai, Kuniya; Sato, Shigeru; Hayashi, Meiso; Adachi, Akiko; Sasaki, Yoshihiro; Takano, Hitoshi; Mizuno, Kyoichi; Shimizu, Wataru

    2016-01-01

    Autophagy is a process of bulk protein degradation and organelle turnover, and is a current therapeutic target in several diseases. The present study aimed to clarify the significance of myocardial autophagy of patients with dilated cardiomyopathy (DCM). Left ventricular endomyocardial biopsy was performed in 250 consecutive patients with DCM (54.9±13.9 years; male, 79%), initially presenting with decompensated heart failure (HF). The association of these findings with HF mortality or recurrence was examined. Myofilament changes, which are apparent in the degenerated cardiomyocytes of DCM, were recognized in 164 patients (66%), and autophagic vacuoles in cardiomyocytes were identified in or near the area of myofilament changes in 86 patients (34%). Morphometrically, fibrosis (odds ratio [OR], 0.96; 95% confidence interval [CI], 0.93 to 0.99) and mitochondrial abnormality (OR, 2.24; 95% CI, 1.23 to 4.08) were independently related with autophagic vacuoles. During the follow-up period of 4.9±3.9 y, 24 patients (10%) died, including 10 (4%) who died of HF, and 67 (27%) were readmitted for HF recurrence. Multivariate analysis identified a family history of DCM (hazard ratio [HR], 2.117; 95% CI, 1.199 to 3.738), hemoglobin level (HR, 0.845; 95% CI, 0.749 to 0.953), myofilament changes (HR, 13.525; 95% CI, 5.340 to 34.255), and autophagic vacuoles (HR, 0.214; 95% CI, 0.114 to 0.400) as independent predictors of death or readmission due to HF recurrence. In conclusion, autophagic vacuoles in cardiomyocytes are associated with a better HF prognosis in patients with DCM, suggesting autophagy may play a role in the prevention of myocardial degeneration.

  20. Prognostic value of sympathetic innervation and cardiac asynchrony in dilated cardiomyopathy

    International Nuclear Information System (INIS)

    Manrique, Alain; Hitzel, Anne; Vera, Pierre; Bernard, Mathieu; Bauer, Fabrice; Menard, Jean-Francois; Sabatier, Remi; Jacobson, Arnold; Agostini, Denis

    2008-01-01

    The purpose of the study is to examine prognostic values of cardiac I-123 metaiodobenzylguanidine (MIBG) uptake and cardiac dyssynchrony in patients with dilated cardiomyopathy (DCM). Ninety-four patients with non-ischemic DCM underwent I-123 MIBG imaging for assessing cardiac sympathetic innervation and equilibrium radionuclide angiography. Mean phase angles and SD of the phase histogram were computed for both right ventricular (RV) and left ventricular (LV). Phase measures of interventricular (RV-LV) and intraventricular (SD-RV and SD-LV) asynchrony were computed. Most patients were receiving beta-blockers (89%) and angiotensin-converting enzyme inhibitors (88%). One patient (1%) was lost to follow-up, six had cardiac death (6.4%), eight had heart transplantation (8.6%), and seven had unplanned hospitalization for heart failure (7.5%; mean follow-up: 37 ± 16 months). Patients with poor clinical outcome were older, had higher The New York Heart Association functional class, impaired right ventricular ejection fraction and left ventricular ejection fraction, and impaired cardiac I-123 MIBG uptake. On multivariate analysis, I-123 MIBG heart-to-mediastinum (H/M) uptake ratio <1.6 was the only predictor of both primary (cardiac death or heart transplantation, RR = 7.02, p < 0.01) and secondary (cardiac death, heart transplantation, or recurrent heart failure, RR = 8.10, p = 0.0008) end points. In patients receiving modern medical therapy involving beta-blockers, I-123 MIBG uptake, but not intra-LV asynchrony, was predictive of clinical outcome. The impact of beta-blockers on the prognostic value of ventricular asynchrony remains to be clarified. (orig.)

  1. Cholesterol metabolism as a prognostic marker in patients with mildly symptomatic nonischemic dilated cardiomyopathy.

    Science.gov (United States)

    Sawamura, Akinori; Okumura, Takahiro; Hiraiwa, Hiroaki; Aoki, Soichiro; Kondo, Toru; Ichii, Takeo; Furusawa, Kenji; Watanabe, Naoki; Kano, Naoaki; Fukaya, Kenji; Morimoto, Ryota; Bando, Yasuko K; Murohara, Toyoaki

    2017-06-01

    Little is known about whether the alteration of cholesterol metabolism reflects abdominal organ impairments due to heart failure. Therefore, we investigated the prognostic value of cholesterol metabolism by evaluating serum campesterol and lathosterol levels in patients with early-stage nonischemic dilated cardiomyopathy (NIDCM). We enrolled 64 patients with NIDCM (median age 57.5 years, 31% female) with New York Heart Association functional class I/II. Serum campesterol and lathosterol levels were measured in all patients. The patients were then divided into four subsets based on the median non-cholesterol sterol levels (campesterol 3.6μg/mL, lathosterol 1.4μg/mL): reference (R-subset), high-campesterol/high-lathosterol; absorption-reduced (A-subset), low-campesterol/high-lathosterol; synthesis-reduced (S-subset), high-campesterol/low-lathosterol; double-reduced (D-subset), low-campesterol/low-lathosterol. Endpoint was a composite of cardiac events, including cardiac-related death, hospitalization for worsening heart failure, and lethal arrhythmia. Median brain natriuretic peptide (BNP) level was 114pg/mL. Mean left ventricular ejection fraction was 31.4%. D-subset had the lowest total cholesterol level and cardiac index and the highest BNP level and pulmonary capillary wedge pressure. D-subset also had the highest cardiac event rate during the mean 3.8 years of follow-up (log-rank p=0.001). Multivariate regression analysis showed that D-subset was an independent determinant of cardiac events. The receiver operating characteristic curve analysis revealed that total cholesterol cholesterol absorption and liver synthesis predicts future cardiac events in patients with mildly symptomatic NIDCM. Copyright © 2016 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.

  2. Outcome of Patients With Cardiac Sarcoidosis Who Received Cardiac Resynchronization Therapy: Comparison With Dilated Cardiomyopathy Patients.

    Science.gov (United States)

    Yufu, Kunio; Kondo, Hidekazu; Shinohara, Tetsuji; Kawano, Kyoko; Ishii, Yumi; Miyoshi, Miho; Imamura, Takaaki; Saito, Shotaro; Okada, Norihiro; Akioka, Hidefumi; Teshima, Yasushi; Nakagawa, Mikiko; Takahashi, Naohiko

    2017-02-01

    Cardiac resynchronization therapy (CRT) has been shown to be effective for patients with chronic heart failure; however, the efficacy of CRT in patients with cardiac sarcoidosis (CS) has not been established. We compared the outcomes of patients with CS who received CRT to patients with dilated cardiomyopathy (DCM). The incidence of major adverse cerebral and cardiovascular events (MACCE) in 11 consecutive CS patients (8 females; mean age, 66 ± 8.0 years) who received CRT were compared with 29 DCM patients (9 females; mean age, 70 ± 8.9 years). Females and patients with previous right ventricular pacing were largely included in the comparison of CS and DCM patients (P < 0.05 and P < 0.0001, respectively). During the mean follow-up period (465 ± 383 days for CS and 729 ± 393 days for DCM), MACCE were evident in 9 patients (23%); specifically, 5 CS and 4 DCM patients developed MACCE (45% vs. 14%, P < 0.05), respectively. Kaplan-Meier survival analysis demonstrated that CS patients had a higher prevalence of MACCE than DCM patients (log rank = 6.306, P = 0.0120; and Wilcoxon = 7.1333, P = 0.0076). Based on univariate analysis, the etiology of CS was associated with MACCE. Our results suggest that the long-term outcome of CRT in patients with CS was very poor compared with DCM patients. Thus, caution should be exercised regarding the indication of CRT in patients with CS. © 2016 Wiley Periodicals, Inc.

  3. A safety and feasibility study of cell therapy in dilated cardiomyopathy

    Directory of Open Access Journals (Sweden)

    H.F. Martino

    2010-10-01

    Full Text Available The aim of this study was to determine if bone marrow mononuclear cell (BMMC transplantation is safe for moderate to severe idiopathic dilated cardiomyopathy (IDC. Clinical trials have shown that this procedure is safe and effective for ischemic patients, but little information is available regarding non-ischemic patients. Twenty-four patients with IDC, optimized therapy, age 46 ± 11.6 years, 17 males, NYHA classes II-IV, and left ventricular ejection fraction <35% were enrolled in the study. Clinical evaluation at baseline and 6 months after stem cell therapy to assess heart function included echocardiogram, magnetic resonance imaging, cardiopulmonary test, Minnesota Quality of Life Questionnaire, and NYHA classification. After cell transplantation 1 patient showed a transient increase in enzyme levels and 2 patients presented arrhythmias that were reversed within 72 h. Four patients died during follow-up, between 6 and 12 weeks after therapy. Clinical evaluation showed improvement in most patients as reflected by statistically significant decreases in Minnesota Quality of Life Questionnaire (63 ± 17.9 baseline vs 28.8 ± 16.75 at 6 months and in class III-IV NYHA patients (18/24 baseline vs 2/20 at 6 months. Cardiopulmonary exercise tests demonstrated increased peak oxygen consumption (12.2 ± 2.4 at baseline vs 15.8 ± 7.1 mL·kg-1·min-1 at 6 months and walked distance (377.2 ± 85.4 vs 444.1 ± 77.9 m at 6 months in the 6-min walk test, which was not accompanied by increased left ventricular ejection fraction. Our findings indicate that BMMC therapy in IDC patients with severe ventricular dysfunction is feasible and that larger, randomized and placebo-controlled trials are warranted.

  4. Relationship between HLA-DQA1 polymorphism and genetic susceptibility to idiopathic dilated cardiomyopathy.

    Science.gov (United States)

    Liu, Wei; Li, Wei-min; Sun, Ning-ling

    2004-10-01

    Autoimmune mechanisms are likely to participate in the pathogenesis of subgroup of idiopathic dilated cardiomyopathy (IDC), and components of the major histocompatibility complex may serve as markers for the propensity to develop immune-mediated myocardial damage. Human leukocyte antigen (HLA) class II genes, especially highly polymorphic HLA-DQ genes, play an important role in the activation of immune responses, and thus control the predisposition for or protect from IDC. This study was conducted to investigate the HLA-DQA1 allele polymorphisms in IDC patients and to explore the underlying immunological mechanism and the hereditary susceptibility to IDC. The polymerase chain reaction sequence-specific primers (PCR-SSP) technique was used to analyze the polymorphisms in the second exon of DQA1 in three groups: 72 IDC patients diagnosed according to the criteria of World Health Organization (IDC group); 100 end-stage heart failure patients suffering from a disease of known etiology (HF group); and 100 healthy subjects enrolled for the study during a routine health survey (control group). Patients in the IDC group were stratified according to ejection fraction (EF). Those with EF values were higher than 35% were placed into subgroup 1; subgroup 2 included patients with an EF value of 15% - 35%; and subgroup 3 consisted of those whose EF values less than 15%. The frequency of HLA-DQA1 *0501 alleles was significantly higher in the IDC group (0.39) than that in the HF group (0.12) and the control group (0.09) (both P HLA-DQA1 *0501 allele confers susceptibility to IDC, while the DQA1 *0201 allele confers protection against it, which indicates that genetic background involved in IDC and heart failure is different. HLA-DQA1 genes are involved in the regulation of specific immune responses by auto- or foreign anti-myocardium antibody.

  5. Propionic acidemia as a cause of adult-onset dilated cardiomyopathy.

    Science.gov (United States)

    Riemersma, Moniek; Hazebroek, Mark R; Helderman-van den Enden, Appolonia T J M; Salomons, Gajja S; Ferdinandusse, Sacha; Brouwers, Martijn C G J; van der Ploeg, Liesbeth; Heymans, Stephane; Glatz, Jan F C; van den Wijngaard, Arthur; Krapels, Ingrid P C; Bierau, Jörgen; Brunner, Han G

    2017-11-01

    Dilated cardiomyopathy (DCM) is extremely heterogeneous with a large proportion due to dominantly inherited disease-causing variants in sarcomeric genes. Recessive metabolic diseases may cause DCM, usually with onset in childhood, and in the context of systemic disease. Whether metabolic defects can also cause adult-onset DCM is currently unknown. Therefore, we performed an extensive metabolic screening in 36 consecutive adult-onset DCM patients. Diagnoses were confirmed by Sanger sequencing and multiplex ligation-dependent probe amplification (MLPA). Measurement of propionyl-CoA carboxylase (PCC) activity was done in fibroblasts. Whole exome sequencing (WES) data of 157 additional DCM patients were analyzed for genetic defects. We found a metabolic profile characteristic for propionic acidemia in a patient with severe DCM from 55 years of age. Genetic analysis demonstrated compound heterozygous variants in PCCA. Enzymatic activity of PCC in fibroblasts was markedly reduced. A targeted analysis of the PCCA and PCCB genes using available WES data from 157 further DCM patients subsequently identified another patient with propionic acidemia. This patient had compound heterozygous variants in PCCB, and developed severe DCM from 42 years of age. Adult-onset DCM can be caused by propionic acidemia, an autosomal recessive inheritable metabolic disorder usually presenting as neonatal or childhood disease. Current guidelines advise a low-protein diet to ameliorate or prevent detrimental aspects of the disease. Long-term follow-up of a larger group of patients may show whether this diet would also ameliorate DCM. Our results suggest that diagnostic metabolic screening to identify propionic acidemia and related disorders in DCM patients is justified.

  6. Impact of myocardial perfusion abnormality on prognosis in patients with non-ischemic dilated cardiomyopathy

    International Nuclear Information System (INIS)

    Sobajima, Mitsuo; Nozawa, Takashi; Suzuki, Takayuki; Ohori, Takashi; Shida, Takuya; Matsuki, Akira; Inoue, Hiroshi

    2010-01-01

    Myocardial perfusion imaging shows various patterns in patients with non-ischemic dilated cardiomyopathy (DCM). However, influences of regional abnormalities of myocardial perfusion or ventricular wall motion on prognosis in DCM patients remains to be clarified. Accordingly, we investigated a relation between myocardial perfusion patterns and long-term prognosis in DCM patients. Sixty-two patients were divided into 2 groups according to patterns of 99m Tc-Tetrofosmin scintigraphy, id est (i.e.) large focal defects (focal) and minimally impaired perfusion or multiple small defects (non-focal). There were no differences between the 2 groups in left ventricular (LV) end-diastolic dimensions (63.4±9.1 and 63.8.4± 7.5 mm, respectively) and LV ejection fraction (30.3±9.2 and 27.9±7.8%, respectively), indicating LV systolic dysfunction was comparable between the groups. The focal group had a higher prevalence of brain natriuretic peptide ≥200 ng/dl and plasma norepinephrine ≥500 pg/ml than the non-focal group (p<0.05), and had longer QRS durations (p<0.05). The focal group had non-sustained ventricular tachycardia (VT) (p<0.05) on 24-h electrocardiogram recording and a history of VT/ventricular fibrillation more frequently (p<0.05), and had higher New York Heart Association functional class than the non-focal group (p<0.05). The mortality was significantly higher in the focal group (56.0%) than in the non-focal group (28.6%) and the survival curves revealed worse prognosis in the focal group during a follow-up period of 5.3±2.8 years. Non-ischemic DCM patients with focal defects are accompanied by more advanced heart failure and poor prognosis compared to those with minimally impaired perfusion or multiple small defects, despite comparable LV systolic dysfunction. (author)

  7. Prognostic significance of large perfusion defects on thallium-201 myocardial scintigraphy in dilated cardiomyopathy

    International Nuclear Information System (INIS)

    Takata, Jun; Doi, Yoshinori; Chikamori, Taishiro; Yonezawa, Yoshihiro; Hamashige, Naohisa; Kuzume, Osamu; Ozawa, Toshio

    1989-01-01

    To evaluate the prognostic significance of perfusion abnormalities, particularly large defects, in dilated cardiomyopathy (DCM), we performed thallium-201 myocardial scintigraphy and 24-hour ambulatory ECG monitoring in 27 patients. The abnormal scintigraphic patterns and the presence of ventricular tachycardia (VT) were correlated with causes of death during a follow-up period of 30.0±19.4 months. Eight patients had large defects (LD), 11 had multiple small defects (MSD), and eight had no defects (NL). The patients with LD had extensive ventricular akinesis in the region of the perfusion defect, significantly elevated LVEDP (LD 20.6±7.4 mmHg, MSD 15.5±7.6 mmHg, NL 10.3±2.3 mmHg: LD vs NL; p<0.01, MSD vs NL; p<0.05), and reduced ejection fraction (LD 23.9±9.1%, MSD 32.7±7.2%, NL 40.3±7.7%: LD vs MSD; p<0.05, MSD vs NL; p<0.01). VT was detected in 11 patients; among whom three had LD, six had MSD, and two had no defects. Among seven patients who died during follow-up (five of heart failure, one sudden death, and one non-cardiac death), five had LD and two had MSD. There were no deaths among patients without defects. Among 11 patients with VT, only one died suddenly. In conclusion, large scintigraphic defects correlated well with severe LV dysfunction, and this is an important variable in predicting outcomes in DCM. (author)

  8. Indium-111 antimyosin antibody imaging and thallium-201 imaging. A comparative myocardial scintigraphic study using single-photon emission computed tomography in patients with myocarditis and dilated cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Yamada, Takehiko; Matsumori, Akira; Nohara, Ryuji; Konishi, Junji; Sasayama, Shigetake [Kyoto Univ. (Japan). Faculty of Medicine; Tamaki, Nagara

    1997-10-01

    Indium-111 antimyosin antibody imaging (a tracer of myocardial necrosis) and thallium-201 imaging (a tracer of myocardial perfusion) were compared in patients with myocarditis and dilated cardiomyopathy. The distribution of each tracer and antimyosin/thallium-201 overlapping were evaluated with single-photon emission computed tomography (SPECT). Scintigraphic data were classified into 5 patterns according to the distribution of both images and were compared with histologic findings of endomyocardial biopsy: AM-D, intense and diffuse antimyosin uptake and no perfusion abnormality (active myocarditis); AM-L, localized antimyosin uptake and no perfusion abnormality (active myocarditis); HM, no antimyosin uptake with or without perfusion abnormality (healed myocarditis); DCM-NH, diffuse antimyosin uptake and inhomogeneous thallium-201 uptake (dilated cardiomyopathy); DCM-PD, diffuse or localized antimyosin uptake and myocardial perfusion defect(s) (dilated cardiomyopathy). Patients with dilated-phase hypertrophic cardiomyopathy were frequently found in the DCM-PD group. Taken together, comparative antimyosin/thallium-201 SPECT images are useful for evaluating the activity of myocarditis and ongoing myocardial damage even in areas with no perfusion in patients with dilated cardiomyopathy. (author)

  9. Comparison of Benefits from Cardiac Resynchronization Therapy between Patients with Ischemic Cardiomyopathy and Patients with Idiopathic Dilated Cardiomyopathy

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    Talia Alenabi

    2009-06-01

    Full Text Available Background: Cardiac resynchronization therapy (CRT is an effective treatment for patients with moderate to severe heart failure. However, 20-30% of patients remain non-responders to CRT. We sought to identify which patients benefit the most from CRT in regard to the etiology of heart failure. Methods: Eighty-three consecutive patients (62 men who had a biventricular pacemaker inserted at Tehran Heart Center between May 2004 and March 2007 were evaluated retrospectively. The inclusion criteria were comprised of New York Heart Association (NYHA class III or IV, left ventricular ejection fraction120ms. After 6 months, response was defined as being alive, no hospitalization for cardiac decompensation, and an improvement in NYHA class>1 grade. Results: After 6 months, 60 patients out of the 83 patients were responders. Amongst the 83 patients, 48 had ischemic cardiomyopathy and 35 had non-ischemic cardiomyopathy. A cross-tabulation of response versus etiology showed no significant difference between ischemic versus non-ischemic cardiomyopathy with regard to response to CRT (P=0.322. Conclusion: According to our study, there was no difference in response to CRT between ischemic versus non-ischemic cardiomyopathy at six months’ follow-up.

  10. Dilated cardiomyopathy secondary to vitamin D deficiency and hypocalcaemia in the Irish paediatric population. A case report.

    LENUS (Irish Health Repository)

    Glackin, S

    2017-03-01

    We identified three infants with dilated cardiomyopathy (DCM) secondary to severe vitamin D deficieny and hypocalcaemia. All infants were exclusively breast fed, from dark skinned ethnic backgrounds, born and living in Ireland. None of these pregnant mothers or infants received the recommended vitamin D supplementation. Each infant presented in heart failure and required inotropic support as well as calcium and vitamin D replacement. Cardiac function subsequently improved. This highlights the public health issue that many high risk pregnant mothers and infants are not receiving the recommended vitamin D supplementation.

  11. Nicotinamide Riboside Preserves Cardiac Function in a Mouse Model of Dilated Cardiomyopathy.

    Science.gov (United States)

    Diguet, Nicolas; Trammell, Samuel A J; Tannous, Cynthia; Deloux, Robin; Piquereau, Jérôme; Mougenot, Nathalie; Gouge, Anne; Gressette, Mélanie; Manoury, Boris; Blanc, Jocelyne; Breton, Marie; Decaux, Jean-François; Lavery, Gareth; Baczkó, István; Zoll, Joffrey; Garnier, Anne; Li, Zhenlin; Brenner, Charles; Mericskay, Mathias

    2017-12-07

    Background -Myocardial metabolic impairment is a major feature in chronic heart failure (HF). As the major coenzyme in fuel oxidation and oxidative phosphorylation and a substrate for enzymes signaling energy stress and oxidative stress response, NAD + is emerging as a metabolic target in a number of diseases including HF. Little is known on mechanisms regulating homeostasis of NAD + in the failing heart. Methods -To explore possible alterations of NAD + homeostasis in the failing heart, we quantified expression of NAD + biosynthetic enzymes in human failing heart and in the heart of a mouse model of dilated cardiomyopathy (DCM) triggered by SRF transcription factor depletion in the heart (SRF HKO ) or of cardiac hypertrophy triggered by transverse aorta constriction (TAC). We studied the impact of NAD + precursor supplementation on cardiac function in both mouse models. Results -We observed a 30% loss in levels of NAD + in the murine failing heart of both DCM and TAC mice that was accompanied by a decrease in expression of the NAMPT enzyme that recycles the nicotinamide (NAM) precursor whereas the nicotinamide riboside kinase 2 (NMRK2) that phosphorylates the nicotinamide riboside (NR) precursor is increased, to a higher level in the DCM (40 fold) than in TAC (4 fold). This shift was also observed in human failing heart biopsies compared to non-failing controls. We show that the Nmrk2 gene is an AMPK and PPARalpha responsive gene that is activated by energy stress and NAD + depletion in isolated rat cardiomyocytes. NR efficiently rescues NAD + synthesis in response to FK866-mediated inhibition of NAMPT and stimulates glycolysis in cardiomyocytes. Accordingly, we show that NR supplementation in food attenuates the development of HF in mice, more robustly in DCM, and partially after TAC, by stabilizing myocardial NAD + levels in the failing heart. NR treatment also robustly increases the myocardial levels of three metabolites, nicotinic acid adenine dinucleotide

  12. Genetic association study identifies HSPB7 as a risk gene for idiopathic dilated cardiomyopathy.

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    Klaus Stark

    2010-10-01

    Full Text Available Dilated cardiomyopathy (DCM is a structural heart disease with strong genetic background. Monogenic forms of DCM are observed in families with mutations located mostly in genes encoding structural and sarcomeric proteins. However, strong evidence suggests that genetic factors also affect the susceptibility to idiopathic DCM. To identify risk alleles for non-familial forms of DCM, we carried out a case-control association study, genotyping 664 DCM cases and 1,874 population-based healthy controls from Germany using a 50K human cardiovascular disease bead chip covering more than 2,000 genes pre-selected for cardiovascular relevance. After quality control, 30,920 single nucleotide polymorphisms (SNP were tested for association with the disease by logistic regression adjusted for gender, and results were genomic-control corrected. The analysis revealed a significant association between a SNP in HSPB7 gene (rs1739843, minor allele frequency 39% and idiopathic DCM (p = 1.06 × 10⁻⁶, OR  = 0.67 [95% CI 0.57-0.79] for the minor allele T. Three more SNPs showed p < 2.21 × 10⁻⁵. De novo genotyping of these four SNPs was done in three independent case-control studies of idiopathic DCM. Association between SNP rs1739843 and DCM was significant in all replication samples: Germany (n =564, n = 981 controls, p = 2.07 × 10⁻³, OR = 0.79 [95% CI 0.67-0.92], France 1 (n = 433 cases, n = 395 controls, p =3.73 × 10⁻³, OR  = 0.74 [95% CI 0.60-0.91], and France 2 (n = 249 cases, n = 380 controls, p = 2.26 × 10⁻⁴, OR  = 0.63 [95% CI 0.50-0.81]. The combined analysis of all four studies including a total of n = 1,910 cases and n = 3,630 controls showed highly significant evidence for association between rs1739843 and idiopathic DCM (p = 5.28 × 10⁻¹³, OR= 0.72 [95% CI 0.65-0.78]. None of the other three SNPs showed significant results in the replication stage.This finding of the HSPB7 gene from a genetic search for idiopathic DCM using

  13. Genetic Association Study Identifies HSPB7 as a Risk Gene for Idiopathic Dilated Cardiomyopathy

    Science.gov (United States)

    Stark, Klaus; Esslinger, Ulrike B.; Reinhard, Wibke; Petrov, George; Winkler, Thomas; Komajda, Michel; Isnard, Richard; Charron, Philippe; Villard, Eric; Cambien, François; Tiret, Laurence; Aumont, Marie-Claude; Dubourg, Olivier; Trochu, Jean-Noël; Fauchier, Laurent; DeGroote, Pascal; Richter, Anette; Maisch, Bernhard; Wichter, Thomas; Zollbrecht, Christa; Grassl, Martina; Schunkert, Heribert; Linsel-Nitschke, Patrick; Erdmann, Jeanette; Baumert, Jens; Illig, Thomas; Klopp, Norman; Wichmann, H.-Erich; Meisinger, Christa; Koenig, Wolfgang; Lichtner, Peter; Meitinger, Thomas; Schillert, Arne; König, Inke R.; Hetzer, Roland; Heid, Iris M.; Regitz-Zagrosek, Vera; Hengstenberg, Christian

    2010-01-01

    Dilated cardiomyopathy (DCM) is a structural heart disease with strong genetic background. Monogenic forms of DCM are observed in families with mutations located mostly in genes encoding structural and sarcomeric proteins. However, strong evidence suggests that genetic factors also affect the susceptibility to idiopathic DCM. To identify risk alleles for non-familial forms of DCM, we carried out a case-control association study, genotyping 664 DCM cases and 1,874 population-based healthy controls from Germany using a 50K human cardiovascular disease bead chip covering more than 2,000 genes pre-selected for cardiovascular relevance. After quality control, 30,920 single nucleotide polymorphisms (SNP) were tested for association with the disease by logistic regression adjusted for gender, and results were genomic-control corrected. The analysis revealed a significant association between a SNP in HSPB7 gene (rs1739843, minor allele frequency 39%) and idiopathic DCM (p = 1.06×10−6, OR = 0.67 [95% CI 0.57–0.79] for the minor allele T). Three more SNPs showed p < 2.21×10−5. De novo genotyping of these four SNPs was done in three independent case-control studies of idiopathic DCM. Association between SNP rs1739843 and DCM was significant in all replication samples: Germany (n = 564, n = 981 controls, p = 2.07×10−3, OR = 0.79 [95% CI 0.67–0.92]), France 1 (n = 433 cases, n = 395 controls, p = 3.73×10−3, OR = 0.74 [95% CI 0.60–0.91]), and France 2 (n = 249 cases, n = 380 controls, p = 2.26×10−4, OR = 0.63 [95% CI 0.50–0.81]). The combined analysis of all four studies including a total of n = 1,910 cases and n = 3,630 controls showed highly significant evidence for association between rs1739843 and idiopathic DCM (p = 5.28×10−13, OR = 0.72 [95% CI 0.65–0.78]). None of the other three SNPs showed significant results in the replication stage. This finding of the HSPB7 gene from a

  14. Analysis of left ventricular fluid dynamics in dilated cardiomyopathy by echocardiographic particle image velocimetry.

    Science.gov (United States)

    Tang, Chouchou; Zhu, Yizhong; Zhang, Jing; Niu, Chengcheng; Liu, Dan; Liao, Yacong; Zhu, Lijun; Peng, Qinghai

    2018-01-01

    The aim was to analyze left ventricular (LV) fluid dynamics in dilated cardiomyopathy (DCM) by using echocardiographic particle image velocimetry (E-PIV). Twenty patients with DCM and twenty healthy volunteers were examined. LV ultrasound contrast was administered by intravenous bolus injection. At least three dynamic contrast-enhanced echocardiographic images of cardiac cycles from apical three-chamber view and four-chamber view were obtained. The acquired echocardiographic image loops were processed off line by HyperFlow. In healthy hearts, the filling flow in the left ventricle finally comes to be a single large clockwise vortex, which smoothly redirects the blood to the outflow tract. Meanwhile, aberrant flow patterns are observed in the patients with DCM. In the DCM group, the vortex area (0.237 ± 0.063 vs 0.196 ± 0.129, P = .029), vortex depth (0.396 ± 0.134 vs 0.293 ± 0.143, P = .025), and vortex length (0.534 ± 0.089 vs 0.435 ± 0.176, P = .004) are significantly higher. The flow force angle (29.979 ± 8.208 vs 35.896 ± 6.044, P = .013) is significantly lower, and energy dissipation (0.975 ± 0.552 vs 0.578 ± 0.295, P = .006) is significantly higher. A negative linear relation is indicated between the following pairs of parameters: vortex depth and LV ejection fraction (EF) (r = -.350, P = .027); vortex length and LV EF (r = -.321, P = .044); energy dissipation and LV EF (r = -.523, P = .001). A positive linear relation is indicated between flow force angle and LV EF (r = .365, P = .021). E-PIV can effectively and quantitatively evaluate LV fluid dynamics in patients with DCM. LV fluid dynamics and LV systolic function interact with and affect each other. © 2017 Wiley Periodicals, Inc.

  15. Determinants of Atrial Electromechanical Delay in Patients with Functional Mitral Regurgitation and Non-ischemic Dilated Cardiomyopathy

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    Bengi Bakal Ruken

    2014-12-01

    Full Text Available Introduction: Atrial conduction time has important hemodynamic effects on ventricular filling and is accepted as a predictor of atrial fibrillation. In this study we assessed atrial conduction time in patients with non ischemic dilated cardiomyopathy (NIDCMP and functional mitral regurgitation (MR and aimed to determine factors predicting atrial conduction time prolongation. Methods: Sixty five patients with non ischemic dilated cardiomyopathy who have moderate to severe MR and 60 control subjects were included in the study. In addition to conventional echocardiographic measures used to asses left ventricle and MR, atrial electromechanical coupling (time interval from the onset of P wave on surface electrocardiogram [ECG] to the beginning of A wave interval with tissue Doppler echocardiography [PA], intra- and interatrial electromechanical delay (intra and inter AEMD were measured. Results: The correlations between inter AEMD and left atrial (LA size, MR volume, isovolumetric relaxation time (IVRT, deceleration time (DT, systolic pulmonary artery pressure (PAPs, E/A ratio and E/e’ were very poor. Similarly, intra AEMD was not correlated to LA size , MR volume, IVRT, DT, PAPs, E/A ratio and E/e’. However, both inter AEMD and intra AEMD had good correlation with left ventricular mass index, tenting area (TA, tenting distance (TD, coaptation septal distance (CSD, sphericity index (SI. Conclusion: Prolongation of inter and intra AEMDs were found to be well correlated with parameters reflecting left ventricular and mitral annular remodeling.

  16. Left ventricular assist for pediatric patients with dilated cardiomyopathy using the Medos VAD cannula and a centrifugal pump.

    Science.gov (United States)

    Huang, Shu-Chien; Chi, Nai-Hsin; Chen, Chun-An; Chen, Yih-Sharng; Chou, Nai-Kuan; Ko, Wen-Je; Wang, Shoei-Shen

    2009-11-01

    Ventricular assist devices for small pediatric patients are expensive and commercially unavailable in Taiwan. We used the Medos ventricular assist device cannula (Medos, Aachen, Germany) and a centrifugal pump to support pediatric patients with dilated cardiomyopathy and decompensated heart failure. From January 2007 to December 2008, three pediatric patients with dilated cardiomyopathy were supported using a centrifugal pump as the left ventricular assist device. The Medos arterial cannula was sutured to the ascending aorta, and the Apex cannula was fixed into the left ventricular apex. When the patient was weaned off of cardiopulmonary bypass, the left ventricular assist device pump was started. The pump flow was gradually titrated according to the filling status of the left ventricle. All the left ventricular assist devices were successfully implanted and functioned well. Two patients on extracorporeal membrane oxygenation had severe lung edema before left ventricular assist device implantation. Both patients required extracorporeal membrane oxygenation for the postoperative period until the pulmonary edema was resolved. Among the three patients, two successfully bridged to heart transplantation after support for 6 and 11 days, respectively. The first patient (10 kg) expired due to systemic emboli 30 days after left ventricular assist device support. In summary, these results suggest that the Medos ventricular assist device cannula and a centrifugal pump is an option for temporary left ventricular assist device support in patients with intractable heart failure and as a bridge to heart transplantation.

  17. Cardiomyocyte-Specific Ablation of Med1 Subunit of the Mediator Complex Causes Lethal Dilated Cardiomyopathy in Mice.

    Science.gov (United States)

    Jia, Yuzhi; Chang, Hsiang-Chun; Schipma, Matthew J; Liu, Jing; Shete, Varsha; Liu, Ning; Sato, Tatsuya; Thorp, Edward B; Barger, Philip M; Zhu, Yi-Jun; Viswakarma, Navin; Kanwar, Yashpal S; Ardehali, Hossein; Thimmapaya, Bayar; Reddy, Janardan K

    2016-01-01

    Mediator, an evolutionarily conserved multi-protein complex consisting of about 30 subunits, is a key component of the polymerase II mediated gene transcription. Germline deletion of the Mediator subunit 1 (Med1) of the Mediator in mice results in mid-gestational embryonic lethality with developmental impairment of multiple organs including heart. Here we show that cardiomyocyte-specific deletion of Med1 in mice (csMed1-/-) during late gestational and early postnatal development by intercrossing Med1fl/fl mice to α-MyHC-Cre transgenic mice results in lethality within 10 days after weaning due to dilated cardiomyopathy-related ventricular dilation and heart failure. The csMed1-/- mouse heart manifests mitochondrial damage, increased apoptosis and interstitial fibrosis. Global gene expression analysis revealed that loss of Med1 in heart down-regulates more than 200 genes including Acadm, Cacna1s, Atp2a2, Ryr2, Pde1c, Pln, PGC1α, and PGC1β that are critical for calcium signaling, cardiac muscle contraction, arrhythmogenic right ventricular cardiomyopathy, dilated cardiomyopathy and peroxisome proliferator-activated receptor regulated energy metabolism. Many genes essential for oxidative phosphorylation and proper mitochondrial function such as genes coding for the succinate dehydrogenase subunits of the mitochondrial complex II are also down-regulated in csMed1-/- heart contributing to myocardial injury. Data also showed up-regulation of about 180 genes including Tgfb2, Ace, Atf3, Ctgf, Angpt14, Col9a2, Wisp2, Nppa, Nppb, and Actn1 that are linked to cardiac muscle contraction, cardiac hypertrophy, cardiac fibrosis and myocardial injury. Furthermore, we demonstrate that cardiac specific deletion of Med1 in adult mice using tamoxifen-inducible Cre approach (TmcsMed1-/-), results in rapid development of cardiomyopathy and death within 4 weeks. We found that the key findings of the csMed1-/- studies described above are highly reproducible in TmcsMed1-/- mouse heart

  18. Treatment of inflammatory dilated cardiomyopathy and (peri)myocarditis with immunosuppression and i.v. immunoglobulins.

    Science.gov (United States)

    Maisch, Bernhard; Hufnagel, Günther; Kölsch, Susanne; Funck, Rainer; Richter, Annette; Rupp, Heinz; Herzum, Matthias; Pankuweit, Sabine

    2004-09-01

    Treatment objectives in inflammatory dilated cardiomyopathy (DCMi), myocarditis (M) and peri(myo)carditis are 1) the elimination of inflammatory cells from the myocardium and pericardium, 2) the elimination or (second best) mitigation of B-cell products such as antibodies and immuncomplexes directed against cardiac epitopes such as sarcolemmal, fibrillary and mitochondrial epitopes, and 3) the eradication of the causative viral or microbial agent, if present. A "non-specific" anti-inflammatory treatment in peri(myo)carditis can be carried out with antiphlogistics (NSAIDs preferably colchicine 1-3 mg/d) independent from the presence of the infective agent. In larger virus and bacteria negative effusions we recommend intrapericardial instillation of cristalloid triamcinolon (Volon A) at a dose of 500 mg/m(2), which should be left in place to have a sustained effect over at least 4 weeks. This will effectively prevent recurrences particularly when colchicine is added over a period of at least 3-6 months. Taking into account the 2004 ESC task force recommendations on the management of pericardial diseases the treatment recommendation for NSAIDs and colchicine can be classified as level of evidence A, indication class I, for intrapericardial triamcinolon instillation as level of evidence B, indication class IIa. In (immuno)histologically validated autoreactive (virus negative) myocarditis and DCMi double-blind randomized trials are lacking to demonstrate the superiority of immunosuppression over conventional heart failure management. The only published randomized and double-blind immunosuppression treatment trial (American Myocarditis Treatment Trial) was underpowered and did not distinguish viral from non-viral disease. It showed neither benefit nor harm of a combination of cyclosporin and prednisone. A number of retrospective analyses of immunosuppression in myocarditis showed some benefit of surrogate parameters (ejection fraction, exercise tolerance) but improvement

  19. Human actin mutations associated with hypertrophic and dilated cardiomyopathies demonstrate distinct thin filament regulatory properties in vitro.

    Science.gov (United States)

    Debold, Edward P; Saber, Walid; Cheema, Yaser; Bookwalter, Carol S; Trybus, Kathleen M; Warshaw, David M; Vanburen, Peter

    2010-02-01

    Two cardiomyopathic mutations were expressed in human cardiac actin, using a Baculovirus/insect cell system; E99K is associated with hypertrophic cardiomyopathy whereas R312H is associated with dilated cardiomyopathy. The hypothesis that the divergent phenotypes of these two cardiomyopathies are associated with fundamental differences in the molecular mechanics and thin filament regulation of the underlying actin mutation was tested using the in vitro motility and laser trap assays. In the presence of troponin (Tn) and tropomyosin (Tm), beta-cardiac myosin moved both E99K and R312H thin filaments at significantly (pATP concentration revealed similar ATP binding rates but slowed ADP release rates for the two actin mutants compared to WT. Single molecule laser trap experiments performed using both unregulated (i.e. actin) and regulated thin filaments in the absence of Ca(++) revealed that neither actin mutation significantly affected the myosin's unitary step size (d) or duration of strong actin binding (t(on)) at 20 microM ATP. However, the frequency of individual strong-binding events in the presence of Tn and Tm, was significantly lower for E99K than WT at comparable myosin surface concentrations. The cooperativity of a second myosin head binding to the thin filament was also impaired by E99K. In conclusion, E99K inhibits the activation of the thin filament by myosin strong-binding whereas R312H demonstrates enhanced calcium activation. Copyright 2009 Elsevier Ltd. All rights reserved.

  20. A novel mutation and first report of dilated cardiomyopathy in ALG6-CDG (CDG-Ic: a case report

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    Zagal Ahmad

    2010-04-01

    Full Text Available Abstract Congenital disorders of glycosylation (CDG are an expanding group of inherited metabolic diseases with multisystem involvement. ALG6-CDG (CDGIc is an endoplasmatic reticulum defect in N-glycan assembly. It is usually milder than PMM2-CDG (CDG-Ia and so is its natural course. It is characterized by psychomotor retardation, seizures, ataxia, and hypotonia. In contrast to PMM2-CDG (CDGIa, there is no cerebellar hypoplasia. Cardiomyopathy has been reported in a few CDG types and in a number of patients with unexplained CDG. We report an 11 year old Saudi boy with severe psychomotor retardation, seizures, strabismus, inverted nipples, dilated cardiomyopathy, and a type 1 pattern of serum transferrin isoelectrofocusing. Phosphomannomutase and phosphomannose isomerase activities were normal in fibroblasts. Full gene sequencing of the ALG6 gene revealed a novel mutation namely c.482A>G (p.Y161C and heterozygosity in the parents. This report highlights the importance to consider CDG in the differential diagnosis of unexplained cardiomyopathy.

  1. Effects of Omega-3 Polyunsaturated Fatty Acids on Heart Function and Oxidative Stress Biomarkers in Pediatric Patients with Dilated Cardiomyopathy

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    Omidreza Firuzi

    2013-06-01

    Full Text Available Objectives: Dilated cardiomyopathy is the most prevalent type of cardiomyopathy in children, which results in congestive heart failure and causes significant morbidity and mortality. This study, aims to investigate the effect of supplementation with omega-3 polyunsaturated fatty acids (n-3 PUFA on heart function and oxidative stress biomarkers in these patients. Methods: The present research was a case-control study on pediatric patients with dilated cardiomyopathy, who received n-3 PUFA and anti-failure therapy for 6 months (group 1, n = 6, or anti-failure therapy alone for 6 months (group 2, n = 6, as well as age matched normal individuals (group 3, n = 6, and evaluated the cardiac function and biomarkers of oxidative stress. Results: Echocardiographic parameters, such as left ventricular ejection fraction, shortening fraction, tissue Doppler Ea and Aa waves of lateral annulus of tricuspid valve, and Ea and S wave of septum, were significantly improved in group 1 after n-3 PUFA compared to pre- treatment status, while they were not changed after treatment in group 2. Antioxidant enzymes, including catalase and glutathione peroxidase activities in erythrocytes were slightly decreased, while plasma 8-iso-prostaglandin F2α concentrations were somewhat increased in group 1 compared to groups 2 and 3, however these changes were not statistically significant. Total antioxidant capacity of plasma was similar in all 3 groups. Conclusions: The results indicate that some echocardiographic parameters were significantly improved in patients receiving omega-3 fish oil. However, omega-3 had no significant effect on oxidative stress biomarkers.

  2. Effect of hypertension at presentation on prognosis in patients with dilated cardiomyopathy presenting with normal renal angiogram

    Directory of Open Access Journals (Sweden)

    Swetha Balije

    2016-01-01

    Full Text Available Background & objectives: Dilated cardiomyopathy (DCM is a progressive disease of heart with systolic and diastolic dysfunction carrying a poor long-term prognosis. The prognostic index and predictors of mortality are considered to be useful in guiding the treatment. This study was undertaken to evaluate the effects of hypertension at presentation on prognosis in patients with DCM presenting with normal renal and coronary angiogram. Methods: An observational, analytical, non-interventional and a combination of retrospective and prospective study was conducted in patients between 15 and 75 yr of age with DCM having on and off symptoms while receiving treatment in a cardiology outpatient department for more than a year. Sixty patients who fulfilled the inclusion criteria were enrolled in the study. Left ventricular systolic and diastolic functions were assessed by echocardiography along with New York Heart Association (NYHA functional class prospectively and at baseline retrospectively. Patients were grouped into two categories: DCM with hypertension at presentation (HTNAP, Category 1 and DCM without hypertension at presentation (NHTNAP, Category 2. The primary end-points were the number and dose of parenteral drugs at hospitalization, duration of hospital stay and change in the left ventricular (LV systolic function expressed as LV ejection fraction, and the secondary end-points included overall mortality, change in LV and right ventricular systolic and diastolic functions and change in the NYHA functional class between baseline and three month follow up in patients. Results: Thirty five and 25 patients presented with HTNAP and NHTNAP, respectively (total 60. The overall mortality was 10 per cent (6/60. The number of hospitalizations was less in HTNAP category and of days of hospital stay was 6.3 in HTNAP and 9.8 in NHTNAP, the difference being significant (P < 0.001. The HTNAP category required less parenteral diuretics and inotropes compared

  3. Breast Cancer Clinical Trial of Chemotherapy and Trastuzumab: Potential Tool to Identify Cardiac Modifying Variants of Dilated Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Daniel J. Serie

    2017-05-01

    Full Text Available Doxorubicin and the ERBB2 targeted therapy, trastuzumab, are routinely used in the treatment of HER2+ breast cancer. In mouse models, doxorubicin is known to cause cardiomyopathy and conditional cardiac knock out of Erbb2 results in dilated cardiomyopathy and increased sensitivity to doxorubicin-induced cell death. In humans, these drugs also result in cardiac phenotypes, but severity and reversibility is highly variable. We examined the association of decline in left ventricular ejection fraction (LVEF at 15,204 single nucleotide polymorphisms (SNPs spanning 72 cardiomyopathy genes, in 800 breast cancer patients who received doxorubicin and trastuzumab. For 7033 common SNPs (minor allele frequency (MAF > 0.01 we performed single marker linear regression. For all SNPs, we performed gene-based testing with SNP-set (Sequence Kernel Association Tests: SKAT, SKAT-O and SKAT-common/rare under rare variant non-burden; rare variant optimized burden and non-burden tests; and a combination of rare and common variants respectively. Single marker analyses identified seven missense variants in OBSCN (p = 0.0045–0.0009, MAF = 0.18–0.50 and two in TTN (both p = 0.04, MAF = 0.22. Gene-based rare variant analyses, SKAT and SKAT-O, performed very similarly (ILK, TCAP, DSC2, VCL, FXN, DSP and KCNQ1, p = 0.042–0.006. Gene-based tests of rare/common variants were significant at the nominal 5% level for OBSCN as well as TCAP, DSC2, VCL, NEXN, KCNJ2 and DMD (p = 0.044–0.008. Our results suggest that rare and common variants in OBSCN, as well as in other genes, could have modifying effects in cardiomyopathy.

  4. Modeling and study of the mechanism of dilated cardiomyopathy using induced pluripotent stem cells derived from individuals with Duchenne muscular dystrophy

    Directory of Open Access Journals (Sweden)

    Bo Lin

    2015-05-01

    Full Text Available Duchenne muscular dystrophy (DMD is caused by mutations in the dystrophin gene (DMD, and is characterized by progressive weakness in skeletal and cardiac muscles. Currently, dilated cardiomyopathy due to cardiac muscle loss is one of the major causes of lethality in late-stage DMD patients. To study the molecular mechanisms underlying dilated cardiomyopathy in DMD heart, we generated cardiomyocytes (CMs from DMD and healthy control induced pluripotent stem cells (iPSCs. DMD iPSC-derived CMs (iPSC-CMs displayed dystrophin deficiency, as well as the elevated levels of resting Ca2+, mitochondrial damage and cell apoptosis. Additionally, we found an activated mitochondria-mediated signaling network underlying the enhanced apoptosis in DMD iPSC-CMs. Furthermore, when we treated DMD iPSC-CMs with the membrane sealant Poloxamer 188, it significantly decreased the resting cytosolic Ca2+ level, repressed caspase-3 (CASP3 activation and consequently suppressed apoptosis in DMD iPSC-CMs. Taken together, using DMD patient-derived iPSC-CMs, we established an in vitro model that manifests the major phenotypes of dilated cardiomyopathy in DMD patients, and uncovered a potential new disease mechanism. Our model could be used for the mechanistic study of human muscular dystrophy, as well as future preclinical testing of novel therapeutic compounds for dilated cardiomyopathy in DMD patients.

  5. Modeling and study of the mechanism of dilated cardiomyopathy using induced pluripotent stem cells derived from individuals with Duchenne muscular dystrophy.

    Science.gov (United States)

    Lin, Bo; Li, Yang; Han, Lu; Kaplan, Aaron D; Ao, Ying; Kalra, Spandan; Bett, Glenna C L; Rasmusson, Randall L; Denning, Chris; Yang, Lei

    2015-05-01

    Duchenne muscular dystrophy (DMD) is caused by mutations in the dystrophin gene (DMD), and is characterized by progressive weakness in skeletal and cardiac muscles. Currently, dilated cardiomyopathy due to cardiac muscle loss is one of the major causes of lethality in late-stage DMD patients. To study the molecular mechanisms underlying dilated cardiomyopathy in DMD heart, we generated cardiomyocytes (CMs) from DMD and healthy control induced pluripotent stem cells (iPSCs). DMD iPSC-derived CMs (iPSC-CMs) displayed dystrophin deficiency, as well as the elevated levels of resting Ca(2+), mitochondrial damage and cell apoptosis. Additionally, we found an activated mitochondria-mediated signaling network underlying the enhanced apoptosis in DMD iPSC-CMs. Furthermore, when we treated DMD iPSC-CMs with the membrane sealant Poloxamer 188, it significantly decreased the resting cytosolic Ca(2+) level, repressed caspase-3 (CASP3) activation and consequently suppressed apoptosis in DMD iPSC-CMs. Taken together, using DMD patient-derived iPSC-CMs, we established an in vitro model that manifests the major phenotypes of dilated cardiomyopathy in DMD patients, and uncovered a potential new disease mechanism. Our model could be used for the mechanistic study of human muscular dystrophy, as well as future preclinical testing of novel therapeutic compounds for dilated cardiomyopathy in DMD patients. © 2015. Published by The Company of Biologists Ltd.

  6. STAT3 activity is necessary and sufficient for the development of immune-mediated myocarditis in mice and promotes progression to dilated cardiomyopathy

    Science.gov (United States)

    Camporeale, Annalisa; Marino, Francesca; Papageorgiou, Anna; Carai, Paolo; Fornero, Sara; Fletcher, Steven; Page, Brent D G; Gunning, Patrick; Forni, Marco; Chiarle, Roberto; Morello, Mara; Jensen, Ole; Levi, Renzo; Heymans, Stephane; Poli, Valeria

    2013-01-01

    Myocarditis, often triggered by viral infection, may lead to heart auto-immunity and dilated cardiomyopathy. What determines the switch between disease resolution and progression is however incompletely understood. We show that pharmacological inhibition of STAT3, the main mediator of IL-6 signalling and of Th17-cell differentiation, protects mice from the development of Experimental Auto-immune Myocarditis reducing liver production of the complement component C3, and can act therapeutically when administered at disease peak. Further, we demonstrate that STAT3 is sufficient when constitutively active for triggering the onset of immune-mediated myocarditis, involving enhanced complement C3 production and IL-6 signalling amplification in the liver. Disease development can be prevented by C3 depletion and IL-6 receptor neutralization. This appears to be relevant to disease pathogenesis in humans, since acute myocarditis patients display significantly elevated circulating IL-6 and C3 levels and activated heart STAT3. Thus, aberrant IL-6/STAT3-mediated induction of liver acute phase response genes including C3, which occurs as a consequence of pre-existing inflammatory conditions, might represent an important factor determining the degree of myocarditis and its clinical outcome. PMID:23460527

  7. Correlation between changes in diastolic dysfunction and health-related quality of life after cardiac rehabilitation program in dilated cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Sherin H.M. Mehani

    2013-03-01

    Full Text Available Chronic heart failure (CHF is a complex syndrome characterized by progressive decline in left ventricular function, low exercise tolerance and raised mortality and morbidity. Left ventricular diastolic dysfunction plays a major role in CHF and progression of most cardiac diseases. The current recommended goals can theoretically be accomplished via exercise and pharmacological therapy so the aim of the present study was to evaluate the impact of cardiac rehabilitation program on diastolic dysfunction and health related quality of life and to determine the correlation between changes in left ventricular diastolic dysfunction and domains of health-related quality of life (HRQoL. Forty patients with chronic heart failure were diagnosed as having dilated cardiomyopathy (DCM with systolic and diastolic dysfunction. The patients were equally and randomly divided into training and control groups. Only 30 of them completed the study duration. The training group participated in rehabilitation program in the form of circuit-interval aerobic training adjusted according to 55–80% of heart rate reserve for a period of 7 months. Circuit training improved both diastolic and systolic dysfunction in the training group. On the other hand, only a significant correlation was found between improvement in diastolic dysfunction and health related quality of life measured by Kansas City Cardiomyopathy Questionnaire. It was concluded that improvement in diastolic dysfunction as a result of rehabilitation program is one of the important underlying mechanisms responsible for improvement in health-related quality of life in DCM patients.

  8. [Competitive sports and dilated cardiomyopathy: the case of a 32-year-old soccer player with ventricular tachycardia].

    Science.gov (United States)

    Scharhag, J; Meyer, T; Görge, G; Kindermann, W

    2003-01-24

    A 32-year-old competitive soccer player presented with palpitations he had felt for 4 weeks during maximal activity (soccer training and match). The physical examination and an exercise electrocardiogram were carried out by his general practitioner up to 19 s at 350 W and a heart rate of 147/min without showing any abnormalities. All blood parameters revealed no signs of illness. During treadmill exercise at a heart rate of 181/min, a non-sustained ventricular tachycardia was induced. Echocardiography showed a dilated left ventricle with an enddiastolic diameter of 70 mm and low fractional shortening (28 %). Cardiac catheterization demonstrated a diminished left ventricular ejection fraction (38 %) and an enlarged enddiastolic volume (199 ml) without signs of coronary artery disease. Electrophysiologic testing induced a non-sustained ventricular tachycardia. The echocardiographic and angiographic results indicated a dilated cardiomyopathy. Competitive sports activities were stopped and treatment with a beta-blocker (metoprolol) and an ACE-antagonist (ramipril) was started. In young male and female athletes, the possibility of severe cardiac abnormalities have to be considered even in the presence of good physical fitness and performance. To reach a high sensitivity for diagnostic ergometry, the work-load must reach the maximal capacity of the cardio-pulmonary system. Differences in the exercise performance of athletes and untrained subjects have to be considered.

  9. Sinus Balloon Dilation as Treatment for Acute Sphenoid Sinusitis with Impaired Vision for a Child

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    Yin Zhao

    2016-01-01

    Full Text Available This paper is about sinus balloon dilatation in treatment of acute left sphenoid sinusitis with left impaired vision in a child. Balloon catheter dilatation (BCD of the sinus ostia is a new technique. It has been shown to be a minimally invasive technique to manage chronic sinusitis. However, this method is rarely used in the treatment of acute sinusitis. So far, we know of no reported cases of sinus balloon dilatation in treatment of this case, especially for children.

  10. Cardiomyopathy

    Science.gov (United States)

    ... often in middle-aged people and is more likely to affect men. The most common cause is coronary artery disease or heart attack. Hypertrophic cardiomyopathy. This type involves abnormal thickening of ...

  11. The absence of dystrophin brain isoform expression in healthy human heart ventricles explains the pathogenesis of 5' X-linked dilated cardiomyopathy

    OpenAIRE

    Neri, Marcella; Valli, Emanuele; Alfano, Giovanna; Bovolenta, Matteo; Spitali, Pietro; Rapezzi, Claudio; Muntoni, Francesco; Banfi, Sandro; Perini, Giovanni; Gualandi, Francesca; Ferlini, Alessandra

    2012-01-01

    Abstract Background In X-linked dilated cardiomyopathy due to dystrophin mutations which abolish the expression of the M isoform (5'-XLDC), the skeletal muscle is spared through the up-regulation of the Brain (B) isoform, a compensatory mechanism that does not appear to occur in the heart of affected individuals. Methods We quantitatively studied the expression topography of both B and M isoforms in various human heart regions through in-situ RNA hybridization, Reverse-Transcriptase and Real-...

  12. First description of a novel mitochondrial mutation in the MT-TI gene associated with multiple mitochondrial DNA deletion and depletion in family with severe dilated mitochondrial cardiomyopathy.

    Science.gov (United States)

    Alila-Fersi, Olfa; Tabebi, Mouna; Maalej, Marwa; Belguith, Neila; Keskes, Leila; Mkaouar-Rebai, Emna; Fakhfakh, Faiza

    2018-03-18

    Mitochondria are essential for early cardiac development and impaired mitochondrial function was described associated with heart diseases such as hypertrophic or dilated mitochondrial cardiomyopathy. In this study, we report a family including two individuals with severe dilated mitochondrial cardiomyopathy. The whole mitochondrial genome screening showed the presence of several variations and a novel homoplasmic mutation m.4318-4322delC in the MT-TI gene shared by the two patients and their mother and leading to a disruption of the tRNA Ile secondary structure. In addition, a mitochondrial depletion was present in blood leucocyte of the two affected brother whereas a de novo heteroplasmic multiple deletion in the major arc of mtDNA was present in blood leucocyte and mucosa of only one of them. These deletions in the major arc of the mtDNA resulted to the loss of several protein-encoding genes and also some tRNA genes. The mtDNA deletion and depletion could result to an impairment of the oxidative phosphorylation and energy metabolism in the respiratory chain in the studied patients. Our report is the first description of a family with severe lethal dilated mitochondrial cardiomyopathy and presenting several mtDNA abnormalities including punctual mutation, deletion and depletion. Copyright © 2018 Elsevier Inc. All rights reserved.

  13. [Successful endoscopic dilatation of a stenosis in relation to an ileorectal anastomosis by acute ileus].

    Science.gov (United States)

    Kjærgaard, Jane Christensen; Hendel, Jakob; Gügenur, Ismail

    2014-02-17

    Endoscopic dilatation is a treatment option for patients with Crohns disease suffering from stenosis in relation to an ileorectal anastomosis. We present a case of a patient with Crohns disease who was admitted with acute obstructive symptoms due to a stensosis of the ileorectal anastomosis. The patient was septic. We performed a successful endoscopic dilatation in the acute phase and the patient was discharged few days after an uneventful recovery. Endoscopic dilatation of an ileorectal anastomsis in patients with Crohns disease is a treatment option in the acute setting when performed by experienced endoscopists.

  14. Evaluation of sympathetic activity by 123I-metaiodobenzylguanidine myocardial scintigraphy in dilated cardiomyopathy patients with sleep breathing disorder

    International Nuclear Information System (INIS)

    Nanjo, Shuji; Fujimoto, Shinichiro; Yamashiro, Yoshihiro

    2009-01-01

    Because increased sympathetic nervous activity (SNA) in patients with dilated cardiomyopathy (DCM) associated with sleep breathing disorder (SBD) is known to deteriorate the prognosis of cardiac failure, 123 I-metaiodobenzylguanidine (MIBG) myocardial scintigraphy was used as the investigative tool in the present study. The study group comprised 53 patients (47 men, 6 women; mean age 56±3 years) with chronic stable DCM. Patients were divided into SBD(+) or SBD(-) group according to 24-h pulse oximetry results. SBD(+) was defined when the 3% oxygen desaturation index was more than 15/h during sleep. In total, 32 patients were SBD(-) and 21 were SBD(+). In both groups, pulse oximetry were performed during sleep and awakening pulse rate, and measurement of the blood levels of catecholamines and B-type natriuretic peptide was performed. MIBG myocardial scintigraphy and echocardiography were performed at the same time. No significant difference was found between the 2 groups in catecholamine levels or left ventricular ejection fraction. However, MIBG had a significantly increased washout rate and a significantly decreased delayed heart to mediastinum ratio in the SBD(+) group compared with the SBD(-) group. SNA is increased in DCM patients when associated with SBD. MIBG myocardial scintigraphy may be a sensitive method of detecting increased SNA. (author)

  15. Evaluation of acceleration and deceleration cardiac processes using phase-rectified signal averaging in healthy and idiopathic dilated cardiomyopathy subjects.

    Science.gov (United States)

    Bas, Rosana; Vallverdú, Montserrat; Valencia, Jose F; Voss, Andreas; de Luna, Antonio Bayés; Caminal, Pere

    2015-02-01

    The aim of the present study was to investigate the suitability of the Phase-Rectified Signal Averaging (PRSA) method for improved risk prediction in cardiac patients. Moreover, this technique, which separately evaluates acceleration and deceleration processes of cardiac rhythm, allows the effect of sympathetic and vagal modulations of beat-to-beat intervals to be characterized. Holter recordings of idiopathic dilated cardiomyopathy (IDC) patients were analyzed: high-risk (HR), who suffered sudden cardiac death (SCD) during the follow-up; and low-risk (LR), without any kind of cardiac-related death. Moreover, a control group of healthy subjects was analyzed. PRSA indexes were analyzed, for different time scales T and wavelet scales s, from RR series of 24 h-ECG recordings, awake periods and sleep periods. Also, the behavior of these indexes from simulated data was analyzed and compared with real data results. Outcomes demonstrated the PRSA capacity to significantly discriminate healthy subjects from IDC patients and HR from LR patients on a higher level than traditional temporal and spectral measures. The behavior of PRSA indexes agrees with experimental evidences related to cardiac autonomic modulations. Also, these parameters reflect more regularity of the autonomic nervous system (ANS) in HR patients. Copyright © 2015 IPEM. Published by Elsevier Ltd. All rights reserved.

  16. Serum carbohydrate antigen 125 levels in nonischemic dilated cardiomyopathy: a useful biomarker for prognosis and functional mitral regurgitation.

    Science.gov (United States)

    Karaca, Oguz; Guler, Gamze B; Guler, Ekrem; Gunes, H Murat; Alizade, Elnur; Agus, Hicaz Z; Gol, Gokhan; Kahveci, Gokhan; Esen, Ozlem; Esen, A Metin; Turkmen, Muhsin

    2012-01-01

    The authors investigated the prognostic relevance of serum carbohydrate antigen 125 (CA125) levels in nonischemic dilated cardiomyopathy (NICMP) and assessed whether increased levels relate to the degree of functional mitral regurgitation (FMR). Seventy-seven patients with NICMP were enrolled and followed-up for 10 ± 2 months in this prospective study. Receiver-operating characteristic analysis established a cutoff CA125 value of 25 U/mL for predicting mortality. Patients were divided into two groups according to their CA125 levels (CA125 <25 U/mL [n=58] and CA125 ≥ 25 U/mL [n=19]). Patients with high CA125 values had statistically worse functional status, higher B-type natriuretic peptide (BNP) levels, higher left ventricular volumes, lower ejection fraction, higher E/Em ratio, higher pulmonary artery systolic pressure, and more severe FMR. On the multivariate analysis, serum CA125 (P=.002) and severe FMR (P=.04) were identified as the independent predictors of mortality. Serum CA125 levels also correlated with BNP levels and FMR severity (P<.001). Serum CA125 is a powerful prognostic biomarker that is associated with the severity of heart failure, serum BNP levels and several echocardiographic parameters including left ventricular volumes, systolic and diastolic functions, pulmonary artery pressure, and the degree of FMR. Serum CA125 was also shown as an independent predictor of mortality during 10 ± 2 months of follow-up. © 2011 Wiley Periodicals, Inc.

  17. Noninvasive measurements of cardiac high-energy phosphate metabolites in dilated cardiomyopathy by using 31P spectroscopic chemical shift imaging

    International Nuclear Information System (INIS)

    Hansch, A.; Rzanny, R.; Heyne, J.-P.; Reichenbach, J.R.; Kaiser, W.A.; Leder, U.

    2005-01-01

    Dilated cardiomyopathy (DCM) is accompanied by an impaired cardiac energy metabolism. The aim of this study was to investigate metabolic ratios in patients with DCM compared to controls by using spectroscopic two-dimensional chemical shift imaging (2D-CSI). Twenty volunteers and 15 patients with severe symptoms (left ventricular ejection fraction, LVEF 30%) of DCM were investigated. Cardiac 31 P MR 2D-CSI measurements (voxel size: 40 x 40 x 100 mm 3 ) were performed with a 1.5 T whole-body scanner. Measurement time ranged from 15 min to 30 min. Peak areas and ratios of different metabolites were evaluated, including high-energy phosphates (PCr, ATP), 2,3-diphosphoglycerate (2,3-DPG) and phosphodiesters (PDE). In addition, we evaluated how PCr/ATP ratios correlate with LVEF as an established prognostic factor of heart failure. The PCr/γ-ATP ratio was significantly decreased in patients with moderate and severe DCM and showed a linear correlation with reduced LVEFs. PDE/ATP ratios were significantly increased only in patients with severe DCM as compared to volunteers. Applying 31 P MRS with commonly-available 2D-CSI sequences is a valuable technique to evaluate DCM by determining PCr/ATP ratios noninvasively. In addition to reduced PCr/ATP ratios observed in patients suffering from DCM, significantly-increased PDE/ATP ratios were found in patients with severe DCM. (orig.)

  18. The Role of Serum Adiponectin for Outcome Prediction in Patients with Dilated Cardiomyopathy and Advanced Heart Failure

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    Vaida Baltrūnienė

    2017-01-01

    Full Text Available Clinical interpretation of patients’ plasma adiponectin (APN remains challenging; its value as biomarker in dilated cardiomyopathy (DCM is equivocal. We evaluated whether circulating APN level is an independent predictor of composite outcome: death, left ventricle assist device (LVAD implantation, and heart transplantation (HT in patients with nonischemic DCM. 57 patients with nonischemic DCM (average LV diastolic diameter 6.85 cm, LV ejection fraction 26.63%, and pulmonary capillary wedge pressure 22.06  mmHg were enrolled. Patients underwent echocardiography, right heart catheterization, and endomyocardial biopsy. During a mean follow-up of 33.42 months, 15 (26% patients died, 12 (21% patients underwent HT, and 8 (14% patients were implanted with LVAD. APN level was significantly higher in patients who experienced study endpoints (23.4 versus 10.9 ug/ml, p=0.01. APN was associated with worse outcome in univariate Cox proportional hazards model (HR 1.04, CI 1.02–1.07, p=0.001 but lost significance adjusting for other covariates. Average global strain (AGS is an independent outcome predictor (HR 1.42, CI 1.081–1.866, p=0.012. Increased circulating APN level was associated with higher mortality and may be an additive prognostic marker in DCM with advanced HF. Combination of serum (APN, BNP, TNF-α and echocardiographic (AGS markers may increase the HF predicting power for the nonischemic DCM patients.

  19. Reversible Stress Cardiomyopathy Presenting as Acute Coronary Syndrome with Elevated Troponin in the Absence of Regional Wall Motion Abnormalities: A Forme Fruste of Stress Cardiomyopathy?

    Directory of Open Access Journals (Sweden)

    Mahesh Anantha Narayanan

    2014-01-01

    Full Text Available We present a case of reversible stress cardiomyopathy in a surgical patient, described here as a forme fruste due to its atypical features. It is important to recognize such unusual presentation of stress cardiomyopathy that mimics acute coronary syndrome. Stress cardiomyopathy commonly presents as acute coronary syndrome and is characterized by typical or atypical variants of regional wall motion abnormalities. We report a 60-year-old Caucasian male with reversible stress cardiomyopathy following a sternal fracture fixation. Although the patient had several typical features of stress cardiomyopathy including physical stress, ST-segment elevation, elevated cardiac biomarkers and normal epicardial coronaries, there were few features that were atypical, including unusual age, gender, absence of regional wall motion abnormalities, high lateral ST elevation, and high troponin-ejection fraction product. In conclusion, this could represent a forme fruste of stress cardiomyopathy.

  20. An Uncommon Association of Familial Partial Lipodystrophy, Dilated Cardiomyopathy, and Conduction System Disease

    Directory of Open Access Journals (Sweden)

    Ragesh Panikkath MD

    2016-07-01

    Full Text Available A 46-year-old African American woman presented with severe respiratory distress requiring intubation and was diagnosed with nonischemic cardiomyopathy. She had the typical phenotype of familial partial lipodystrophy 2 (FPLD2. Sequence analysis of LMNA gene showed a heterozygous missense mutation at exon 8 (c.1444C>T causing amino acid change, p.R482W. She later developed severe coronary artery disease requiring multiple percutaneous coronary interventions and coronary artery bypass surgery. She was later diagnosed with diabetes, primary hyperparathyroidism, and euthyroid multinodular goiter. She had sinus nodal and atrioventricular nodal disease and had an implantable cardioverter defibrillator implantation due to persistent left ventricular dysfunction. The device eroded through the skin few months after implantation and needed a re-implant on the contralateral side. She had atrial flutter requiring ablation. This patient with FPLD2 had most of the reported cardiac complications of FPLD2. This case is presented to improve the awareness of the presentation of this disease among cardiologists and internists.

  1. Comparação do desfecho entre a cardiopatia chagásica e a miocardiopatia dilatada idiopática Comparison of outcome between Chagas cardiomyopathy and idiopathic dilated cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Amanda Pires Barbosa

    2011-12-01

    Full Text Available FUNDAMENTO: Pouco se sabe sobre o desfecho dos pacientes com cardiopatia chagásica, em comparação aos pacientes com miocardiopatia dilatada idiopática na era contemporânea. OBJETIVO: Comparar o desfecho dos pacientes chagásicos com insuficiência cardíaca sistólica crônica decorrente da cardiopatia chagásica ao observado em pacientes com MDI na era contemporânea. MÉTODOS: Foi incluído um total de 352 pacientes (246 com cardiomiopatia chagásica e 106 com miocardiopatia dilatada idiopática, seguidos prospectivamente em nossa Instituição, de janeiro de 2000 a janeiro de 2008. Todos os pacientes receberam tratamento clínico contemporâneo padrão. RESULTADOS: Na análise multivariada com o modelo de risco proporcional de Cox, o uso da digoxina (relação de risco = 3,17; intervalo de confiança de 95%, de 1,62 a 6,18; p = 0,001 necessitou de suporte inotrópico (relação de risco = 2,08; intervalo de confiança de 95%, de 1,43 a 3,02; p BACKGROUND: Little is known about the outcome of patients with Chagas cardiomyopathy in comparison to that of patients with Idiopathic Dilated Cardiomyopathy in the contemporary era. OBJECTIVE: To compare the outcome of chagasic patients with chronic systolic heart failure secondary to Chagas cardiomyopathy with that observed in patients with IDC in the contemporary era. METHODS: A total of 352 patients (246 with Chagas cardiomyopathy, 106 with Idiopathic Dilated Cardiomyopathy prospectively followed at our Institution from January, 2000 to January, 2008 were included. All patients received standard contemporary medical therapy. RESULTS: In Cox proportional hazards model multivariate analysis, digoxin use (Hazard Ratio=3.17; 95% Confidence Interval 1.62 to 6.18; p=0.001, need of inotropic support (Hazard Ratio=2.08; 95% Confidence Interval 1.43 to 3.02; p<0.005, left ventricular ejection fraction (Hazard Ratio=0.97; 95% Confidence Interval 0.95 to 0.99; p<0.005, and Chagas cardiomyopathy etiology

  2. Impact of shocks on mortality in patients with ischemic or dilated cardiomyopathy and defibrillators implanted for primary prevention.

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    Florian Streitner

    Full Text Available BACKGROUND: Emerging interest is seen in the paradox of defibrillator shocks for ventricular tachyarrhythmia and increased mortality risk. Particularly in patients with dilated cardiomyopathy (DCM, the prognostic importance of shocks is unclear. The purpose of this study was to compare the outcome after shocks in patients with ischemic cardiomyopathy (ICM or DCM and defibrillators (ICD implanted for primary prevention. METHODS AND RESULTS: Data of 561 patients were analyzed (mean age 68.6±10.6 years, mean left ventricular ejection fraction 28.6±7.3%. During a median follow-up of 49.3 months, occurrence of device therapies and all-cause mortality were recorded. 74 out of 561 patients (13.2% experienced ≥1 appropriate and 51 out of 561 patients (9.1% ≥1 inappropriate shock. All-cause mortality was 24.2% (136 out of 561 subjects. Appropriate shock was associated with a trend to higher mortality in the overall patient population (HR 1.48, 95% CI 0.96-2.28, log rank p = 0.072. The effect was significant in ICM patients (HR 1.61, 95% CI 1.00-2.59, log rank p = 0.049 but not in DCM patients (HR 1.03, 95% CI 0.36-2.96, log rank p = 0.96. Appropriate shocks occurring before the median follow-up revealed a much stronger impact on mortality (HR for the overall patient population 2.12, 95% CI 1.24-3.63, p = 0.005. The effect was driven by ICM patients (HR 2.48, 95% CI 1.41-4.37, p = 0.001, as appropriate shocks again did not influence survival of DCM patients (HR 0.63, 95% CI 0.083-4.75, p = 0.65. Appropriate shocks occurring after the median follow-up and inappropriate shocks occurring at any time revealed no impact on survival in any of the groups (p = ns. CONCLUSION: Appropriate shocks are associated with reduced survival in patients with ICM but not in patients with DCM and ICDs implanted for primary prevention. Furthermore, the negative effect of appropriate shocks on survival in ICM patients is only evident within the

  3. Early sensitization of myofilaments to Ca2+ prevents genetically linked dilated cardiomyopathy in mice.

    Science.gov (United States)

    Alves, Marco L; Warren, Chad M; Simon, Jillian N; Gaffin, Robert D; Montminy, Eric M; Wieczorek, David F; Solaro, R John; Wolska, Beata M

    2017-07-01

    Dilated cardiomoypathies (DCM) are a heterogeneous group of inherited and acquired diseases characterized by decreased contractility and enlargement of cardiac chambers and a major cause of morbidity and mortality. Mice with Glu54Lys mutation in α-tropomyosin (Tm54) demonstrate typical DCM phenotype with reduced myofilament Ca2+ sensitivity. We tested the hypothesis that early sensitization of the myofilaments to Ca2+ in DCM can prevent the DCM phenotype. To sensitize Tm54 myofilaments, we used a genetic approach and crossbred Tm54 mice with mice expressing slow skeletal troponin I (ssTnI) that sensitizes myofilaments to Ca2+. Four groups of mice were used: non-transgenic (NTG), Tm54, ssTnI and Tm54/ssTnI (DTG). Systolic function was significantly reduced in the Tm54 mice compared to NTG, but restored in DTG mice. Tm54 mice also showed increased diastolic LV dimensions and HW/BW ratios, when compared to NTG, which were improved in the DTG group. β-myosin heavy chain expression was increased in the Tm54 animals compared to NTG and was partially restored in DTG group. Analysis by 2D-DIGE indicated a significant decrease in two phosphorylated spots of cardiac troponin I (cTnI) in the DTG animals compared to NTG and Tm54. Analysis by 2D-DIGE also indicated no significant changes in troponin T, regulatory light chain, myosin binding protein C and tropomyosin phosphorylation. Our data indicate that decreased myofilament Ca2+ sensitivity is an essential element in the pathophysiology of thin filament linked DCM. Sensitization of myofilaments to Ca2+ in the early stage of DCM may be a useful therapeutic strategy in thin filament linked DCM. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2017. For permissions please email: journals.permissions@oup.com.

  4. HLA-DQA1, -DQB1 polymorphism and genetic susceptibility to idiopathic dilated cardiomyopathy in Hans of northern China.

    Science.gov (United States)

    Liu, Wei; Li, Wei Min; Sun, Ning Ling

    2005-07-01

    Autoimmune mechanisms are likely to participate in the pathogenesis of at least a subgroup of idiopathic dilated cardiomyopathy (IDC), and components of the major histocompatibility complex (MHC) may serve as markers for the propensity to develop immune-mediated myocardial damage. Human leukocyte antigen (HLA) class II genes, especially HLA-DQ genes, which are highly polymorphic, play an important role in the activation of immune responses and thus control the predisposition to, or protection from, IDC. This study was conducted to investigate the association of HLA-DQA1, -DQB1 allele polymorphisms with an autoantibody against the myocardial mitochondria ADP/ATP carrier, and to explore susceptibility to idiopathic dilated cardiomyopathy (IDC) among the Han ethnic group in northern China and the immunological mechanisms and hereditary susceptibility to IDC. Polymerase chain reaction sequence-specific primer (PCR-SSP) techniques were used to analyze polymorphisms of the second exon of HLA-DQA1 and -DQB1 alleles among 68 unrelated IDC patients, 4 probands of IDC pedigrees, and 100 healthy controls, all of Han nationality and having lived in northern China for a long time. Following echocardiography examination the IDC subjects were stratified according to ejection fraction (EF) values. Those with EF values higher than 50% were placed in subgroup 1, subgroup 2 included the patients with an EF value of 15-35%, and subgroup 3 consisted of those whose EF values were less than 15%. An autoantibody against the myocardial mitochondria ADP/ATP carrier was examined using immunoblot analysis. The frequencies of HLA-DQA1*0501 and HLA-DQB1*0303 were 0.3889 and 0.1806 in the IDC group, significantly higher than those of the healthy controls (0.0900 and 0.0364 respectively, both P HLA-DQA1*0501 among patients whose EF was less than 15% than those whose EF values were > or =15%. Conversely, the frequencies of HLA-DQA1*0201 and -DQB1*0502, *0504 were significantly lower in the IDC

  5. Epicardial adipose tissue assessed by cardiac magnetic resonance imaging in patients with heart failure due to dilated cardiomyopathy.

    Science.gov (United States)

    Doesch, Christina; Streitner, Florian; Bellm, Steven; Suselbeck, Tim; Haghi, Dariusch; Heggemann, Felix; Schoenberg, Stefan O; Michaely, Henrik; Borggrefe, Martin; Papavassiliu, Theano

    2013-03-01

    We sought to investigate the association of the EAT with CMR parameters of ventricular remodelling and left ventricular (LV) dysfunction in patients with non-ischemic dilated cardiomyopathy (DCM). One hundred and fifty subjects (112 consecutive patients with DCM and 48 healthy controls) underwent CMR examination. Function, volumes, dimensions, the LV remodelling index (LVRI), the presence of late gadolinium enhancement (LGE) and the amount of EAT were assessed. Compared to healthy controls, patients with DCM revealed a significantly reduced indexed EAT mass (31.7 ± 5.6 g/m(2) vs 24.0 ± 7.5 g/m(2) , panalysis in DCM patients showed that with increasing LV end-diastolic mass index (LV-EDMI) (r = 0.417, P < 0.0001), increasing LV end-diastolic volume index (r = 0.251, P = 0.01) and increasing LV end-diastolic diameter (r = 0.220, P = 0.02), there was also a significantly increased amount of EAT mass. However, there was no correlation between the EAT and the LV ejection fraction (r = 0.0085, P = 0.37), right ventricular ejection fraction (r = 0.049, P = 0.6), LVRI (r = 0.116, P = 0.2) and the extent of LGE % (r = 0.189, P = 0.1). Among the healthy controls, the amount of EAT only correlated with increasing age (r = 0.461, P = 0.001), BMI (r = 0.426, P = 0.003) and LV-EDMI (r = 0.346, P = 0.02). In patients with DCM the amount of EAT is decreased compared to healthy controls irrespective of LV function impairment. However, an increase in LV mass and volumes is associated with a significantly increase in EAT in patients with DCM. Copyright © 2012 The Obesity Society.

  6. Dilated cardiomyopathy mutations in δ-sarcoglycan exert a dominant-negative effect on cardiac myocyte mechanical stability.

    Science.gov (United States)

    Campbell, Matthew D; Witcher, Marc; Gopal, Anoop; Michele, Daniel E

    2016-05-01

    Delta-sarcoglycan is a component of the sarcoglycan subcomplex within the dystrophin-glycoprotein complex located at the plasma membrane of muscle cells. While recessive mutations in δ-sarcoglycan cause limb girdle muscular dystrophy 2F, dominant mutations in δ-sarcoglycan have been linked to inherited dilated cardiomyopathy (DCM). The purpose of this study was to investigate functional cellular defects present in adult cardiac myocytes expressing mutant δ-sarcoglycans harboring the dominant inherited DCM mutations R71T or R97Q. This study demonstrates that DCM mutant δ-sarcoglycans can be stably expressed in adult rat cardiac myocytes and traffic similarly to wild-type δ-sarcoglycan to the plasma membrane, without perturbing assembly of the dystrophin-glycoprotein complex. However, expression of DCM mutant δ-sarcoglycan in adult rat cardiac myocytes is sufficient to alter cardiac myocyte plasma membrane stability in the presence of mechanical strain. Upon cyclical cell stretching, cardiac myocytes expressing mutant δ-sarcoglycan R97Q or R71T have increased cell-impermeant dye uptake and undergo contractures at greater frequencies than myocytes expressing normal δ-sarcoglycan. Additionally, the R71T mutation creates an ectopic N-linked glycosylation site that results in aberrant glycosylation of the extracellular domain of δ-sarcoglycan. Therefore, appropriate glycosylation of δ-sarcoglycan may also be necessary for proper δ-sarcoglycan function and overall dystrophin-glycoprotein complex function. These studies demonstrate that DCM mutations in δ-sarcoglycan can exert a dominant negative effect on dystrophin-glycoprotein complex function leading to myocardial mechanical instability that may underlie the pathogenesis of δ-sarcoglycan-associated DCM. Copyright © 2016 the American Physiological Society.

  7. Homozygous EEF1A2 mutation causes dilated cardiomyopathy, failure to thrive, global developmental delay, epilepsy and early death.

    Science.gov (United States)

    Cao, Siqi; Smith, Laura L; Padilla-Lopez, Sergio R; Guida, Brandon S; Blume, Elizabeth; Shi, Jiahai; Morton, Sarah U; Brownstein, Catherine A; Beggs, Alan H; Kruer, Michael C; Agrawal, Pankaj B

    2017-09-15

    Eukaryotic elongation factor 1A (EEF1A), is encoded by two distinct isoforms, EEF1A1 and EEF1A2; whereas EEF1A1 is expressed almost ubiquitously, EEF1A2 expression is limited such that it is only detectable in skeletal muscle, heart, brain and spinal cord. Currently, the role of EEF1A2 in normal cardiac development and function is unclear. There have been several reports linking de novo dominant EEF1A2 mutations to neurological issues in humans. We report a pair of siblings carrying a homozygous missense mutation p.P333L in EEF1A2 who exhibited global developmental delay, failure to thrive, dilated cardiomyopathy and epilepsy, ultimately leading to death in early childhood. A third sibling also died of a similar presentation, but DNA was unavailable to confirm the mutation. Functional genomic analysis was performed in S. cerevisiae and zebrafish. In S. cerevisiae, there was no evidence for a dominant-negative effect. Previously identified putative de novo mutations failed to complement yeast strains lacking the EEF1A ortholog showing a major growth defect. In contrast, the introduction of the mutation seen in our family led to a milder growth defect. To evaluate its function in zebrafish, we knocked down eef1a2 expression using translation blocking and splice-site interfering morpholinos. EEF1A2-deficient zebrafish had skeletal muscle weakness, cardiac failure and small heads. Human EEF1A2 wild-type mRNA successfully rescued the morphant phenotype, but mutant RNA did not. Overall, EEF1A2 appears to be critical for normal heart function in humans, and its deficiency results in clinical abnormalities in neurologic function as well as in skeletal and cardiac muscle defects. © The Author 2017. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  8. Oxidative Stress Markers and C-Reactive Protein Are Related to Severity of Heart Failure in Patients with Dilated Cardiomyopathy

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    Celina Wojciechowska

    2014-01-01

    Full Text Available Background. The aim of study was to determine relationships between functional capacity (NYHA class, left ventricle ejection fraction (LVEF, hemodynamic parameters, and biomarkers of redox state and inflammation in patients with dilated cardiomyopathy (DCM. Methods. DCM patients (n=109, aged 45.97±10.82 years, NYHA class IIV, and LVEF 2.94±7.1% were studied. Controls comprised age-matched healthy volunteers (n=28. Echocardiography and right heart catheterization were performed. Serum activities of superoxide dismutase isoenzymes (MnSOD and CuZnSOD, concentrations of uric acid (UA, malondialdehyde (MDA, and C-reactive protein (hs-CRP were measured. Results. MnSOD, UA, hs-CRP, and MDA were significantly higher in DCM patients compared to controls. Except MDA concentration, above parameters were higher in patients in III-IV NYHA class or with lower LVEF. hsCRP correlated with of MnSOD (P<0.05 and CuZnSOD activity (P<0.01. Both isoenzymes positively correlated with mPAP and pulmonary capillary wedge pressure (MnSOD, resp., P<0.01 and P<0.05 and CuZnSOD P<0.05; P<0.05. UA positively correlated with MnSOD (P<0.05, mPAP (P<0.05, and PVRI (P<0.05. The negative correlation between LVEF and UA (P<0.01 was detected. Conclusion. There are relationships among the severity of symptoms of heart failure, echocardiographic hemodynamic parameters, oxidative stress, and inflammatory activation. Increased MnSOD activity indicates the mitochondrial source of ROS in patients with advanced heart failure.

  9. Extracellular vesicles do not contribute to higher circulating levels of soluble LRP1 in idiopathic dilated cardiomyopathy.

    Science.gov (United States)

    Roura, Santiago; Gálvez-Montón, Carolina; de Gonzalo-Calvo, David; Valero, Ana Gámez; Gastelurrutia, Paloma; Revuelta-López, Elena; Prat-Vidal, Cristina; Soler-Botija, Carolina; Llucià-Valldeperas, Aida; Perea-Gil, Isaac; Iborra-Egea, Oriol; Borràs, Francesc E; Lupón, Josep; Llorente-Cortés, Vicenta; Bayes-Genis, Antoni

    2017-11-01

    Idiopathic dilated cardiomyopathy (IDCM) is a frequent cause of heart transplantation. Potentially valuable blood markers are being sought, and low-density lipoprotein receptor-related protein 1 (LRP1) has been linked to the underlying molecular basis of the disease. This study compared circulating levels of soluble LRP1 (sLRP1) in IDCM patients and healthy controls and elucidated whether sLRP1 is exported out of the myocardium through extracellular vesicles (EVs) to gain a better understanding of the pathogenesis of the disease. LRP1 α chain expression was analysed in samples collected from the left ventricles of explanted hearts using immunohistochemistry. sLRP1 concentrations were determined in platelet-free plasma by enzyme-linked immunosorbent assay. Plasma-derived EVs were extracted by size-exclusion chromatography (SEC) and characterized by nanoparticle tracking analysis and cryo-transmission electron microscopy. The distributions of vesicular (CD9, CD81) and myocardial (caveolin-3) proteins and LRP1 α chain were assessed in SEC fractions by flow cytometry. LRP1 α chain was preferably localized to blood vessels in IDCM compared to control myocardium. Circulating sLRP1 was increased in IDCM patients. CD9- and CD81-positive fractions enriched with membrane vesicles with the expected size and morphology were isolated from both groups. The LRP1 α chain was not present in these SEC fractions, which were also positive for caveolin-3. The increase in circulating sLRP1 in IDCM patients may be clinically valuable. Although EVs do not contribute to higher sLRP1 levels in IDCM, a comprehensive analysis of EV content would provide further insights into the search for novel blood markers. © 2017 The Authors. Journal of Cellular and Molecular Medicine published by John Wiley & Sons Ltd and Foundation for Cellular and Molecular Medicine.

  10. Role of coxsackievirus and adenovirus receptor (CAR) expression and viral load of adenovirus and enterovirus in patients with dilated cardiomyopathy.

    Science.gov (United States)

    Sharma, Mirnalini; Mishra, Baijayantimala; Saikia, Uma Nahar; Bahl, Ajay; Ratho, Radha Kanta; Talwar, Kewal Kishan

    2016-01-01

    Enteroviruses (EVs) and adenoviruses (AdVs) are two important etiological agents of viral myocarditis and dilated cardiomyopathy (DCM). Both these viruses share a common receptor, the coxsackievirus and adenovirus receptor (CAR), for their infection. However, the role of viral load and CAR expression in disease severity has not yet been completely elucidated. The present study aimed to determine viral load of EV and AdV in DCM patients and correlate them with the level of CAR expression in these patients. Sixty-three DCM cases and 30 controls, each of whom died of heart disease other than DCM and non-cardiac disease respectively, were included. Viral load was determined by TaqMan real-time PCR using primers and probes specific for the AdV hexon gene and the 5'UTR region of EV. The CAR mRNA level was semi-quantitated by RT-PCR, and antigen expression was studied by immunohistochemistry. A significantly high AdV load (p < 0.05) and CAR expression (p < 0.05) were observed in DCM cases versus controls, whereas the EV load showed no significant difference. The data suggests a clinical threshold of 128 AdV copies/500 ng of DNA for DCM, with 66.7 % sensitivity and 65 % specificity. A positive correlation between AdV load and CAR expression (p < 0.001) was also observed in DCM cases. The high adenoviral load and increased CAR expression in DCM and their association with adverse disease outcome indicates role of both virus and receptor in disease pathogenesis. Thus, the need for targeting both the virus and the receptor for treatment of viral myocarditis and early DCM requires further confirmation with larger studies.

  11. Postoperative Takotsubo cardiomyopathy triggered by intraoperative fluid overload and acute hypertensive crisis.

    Science.gov (United States)

    Varutti, Rosanna; Setti, Tommaso; Ezri, Tiberiu; Nicolosi, Gianluigi; Rellini, Gianluigi; Cassin, Matteo; Leykin, Yigal

    2015-04-01

    The Takotsubo cardiomyopathy is a rare haemodynamic dysfunction, only recently reported perioperatively. While the diagnostic criteria have been established and the outcome is known as favorable, the pathophysiological mechanisms are not entirely understood. Here we present the case of a patient scheduled for laparoscopic hysterectomy and adnexectomy, who early postoperatively developed a Takotsubo cardiomyopathy supposedly triggered by an acute hypertensive crisis due to intraoperative fluid overload.

  12. Ventricular resynchronization through biventricular cardiac pacing for the treatment of refractory heart failure in dilated cardiomyopathy

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    Silas S. Galvão Fº

    2002-01-01

    Full Text Available OBJECTIVE: The biventricular pacing (BVP approach has good results in the treatment of congestive heart failure (CHF in patients (pts with disorders of intraventricular conduction. METHODS: We have applied BVP to 28 pts, with left ventricular pacing using minitoracotomy in 3 pts and the transvenous aproach via coronary sinus in 25 pts. The mean duration of the QRS complexes was 187 ms, in the presence of the left branch block in 22 pts, and right branch block + divisional hemiblock in 6 pts. All pts had been considerated candidates to cardiac transplantation, and were under optimized drug therapy. Sixteen pts were in Functional Class (NYHA IV, and 12 in class III. The ejection fraction varied from 22 to 46% (average = 34%. The pacing mode employed was biventricular triple-chamber in 22 pts, and bi-ventricular dual-chamber in 6 pts (one with ICD. RESULTS: The pts were followed up for a period that ranged from 10 days to 14 months (mean 5 months. All pts presented clinical improvement after implant, chaging the NYHA Functional Class at the end of follow-up to Class I (9pts, Class II (10 pts and Class III (6 pts. The initial mean ejection fraction have-raised to 37%. Two pts died suddenly. One patient died due to a pulmonary fungal infection. CONCLUSION: Ventricular resynchronization through BVP, improved significantly the Functional Class and, therefore, the quality of life. Assessments of myocardial function acutely performed do not reflect the clinical improvement observed.

  13. Acute Effects of Nasal CPAP in Patients With Hypertrophic Cardiomyopathy.

    Science.gov (United States)

    Nerbass, Flávia B; Salemi, Vera M C; Pedrosa, Rodrigo P; Portilho, Natanael de P; Ferreira-Filho, Julio C A; Moriya, Henrique T; Antunes, Murillo O; Arteaga-Fernández, Edmundo; Drager, Luciano F; Lorenzi-Filho, Geraldo

    2016-11-01

    Hypertrophic cardiomyopathy (HCM) is a common genetic disease that may cause left ventricular outflow tract (LVOT) obstruction, heart failure, and sudden death. Recent studies have shown a high prevalence of OSA among patients with HCM. Because the hemodynamics in patients with LVOT obstruction are unstable and depend on the loading conditions of the heart, we evaluated the acute effects of CPAP on hemodynamics and cardiac performance in patients with HCM. We studied 26 stable patients with HCM divided into nonobstructive HCM (n = 12) and obstructive HCM (n = 14) groups (LVOT gradient pressure lower or higher than 30 mm Hg, respectively). Patients in the supine position while awake were continuously monitored with beat-to-beat BP measurements and electrocardiography. Two-dimensional echocardiography was performed at rest (baseline) and after 20 min of nasal CPAP at 1.5 cm H 2 O and 10 cm H 2 O, which was applied in a random order interposed by 10 min without CPAP. BP, cardiac output, stroke volume, heart rate, left ventricular ejection fraction, and LVOT gradient did not change during the study period in either group. CPAP at 10 cm H 2 O decreased right atrial size and right ventricular relaxation in all patients. It also decreased left atrial volume significantly and decreased right ventricular outflow acceleration time, suggesting an increase in pulmonary artery pressure in patients with obstructive HCM. The acute application of CPAP is apparently safe in patients with HCM, because CPAP does not lead to hemodynamic compromise. Long-term studies in patients with HCM and sleep apnea and nocturnal CPAP are warranted. ClinicalTrials.gov; No. NCT01631006; URL: www.clinicaltrials.gov. Copyright © 2016 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.

  14. Effects of carvedilol in heart failure due to dilated cardiomyopathy. Results of a double-blind randomized placebo-controlled study (CARIBE study)

    OpenAIRE

    Chizzola,Paulo Roberto; Freitas,Humberto Felício Gonçalves; Caldas,Márcia Azevedo; Costa,Joicely Melo da; Meneghetti,Cláudio; Marinho,Norma Vasconcelos Saldanha; Mansur,Alfredo José; Ramires,José Antonio Franchini; Bocchi,Edimar Alcides

    2000-01-01

    OBJECTIVE: To assess the effects of carvedilol in patients with idiopathic dilated cardiomyopathy. METHODS: In a double-blind randomized placebo-controlled study, 30 patients (7 women) with functional class II and III heart failure were assessed. Their ages ranged from 28 to 66 years (mean of 43±9 years), and their left ventricular ejection fraction varied from 8% to 35%. Carvedilol was added to the usual therapy of 20 patients; placebo was added to the usual therapy of 10 patients. The initi...

  15. Therapeutic effects of coenzyme Q10 on dilated cardiomyopathy. Assessment by {sup 123}I-BMIPP myocardial single photon emission computed tomography (SPECT). A multicenter trial in Osaka University Medical School Group

    Energy Technology Data Exchange (ETDEWEB)

    Nishimura, Tsunehiko; Hori, Masatsugu [Osaka Univ. (Japan). Faculty of Medicine

    1996-01-01

    To evaluate therapeutic effects of Coenzyme Q10 (CoQ10), 15 patients with dilated cardiomyopathy were investigated by {sup 123}I-BMIPP myocardial single photon emission computed tomography (SPECT). The BMIPP defect score was determined semiquantitatively by using representative short and long axial SPECT images. Mean BMIPP defect score with CoQ10 treatment was significantly low, 7.7{+-}6.1 compared to 12.7{+-}7.4 without CoQ10 treatment. On the other hand, in 8 patients of dilated cardiomyopathy, % fractional shortening using echocardiography was not different before and after CoQ10 treatment. In conclusion, {sup 123}I-BMIPP myocardial SPECT was proved to be sensitive to evaluate the therapeutic effects of CoQ10, which improve myocardial mitochondrial function, in the cases of dilated cardiomyopathy. (author).

  16. Th17-related cytokines in systemic lupus erythematosus patients with dilated cardiomyopathies: a possible linkage to parvovirus B19 infection.

    Directory of Open Access Journals (Sweden)

    Der-Yuan Chen

    Full Text Available Dilated cardiomyopathies (DCM are a major cause of mortality in patients with systemic lupus erythematosus (SLE. Immune responses induced by human parvovirus B19 (B19 are considered an important pathogenic mechanism in myocarditis or DCM. However, little is known about Th17-related cytokines in SLE patients with DCM about the linkage with B19 infection. IgM and IgG against B19 viral protein, and serum levels of Th17-related cytokines were determined using ELISA in eight SLE patients with DCM and six patients with valvular heart disease (VHD. Humoral responses of anti-B19-VP1u and anti-B19-NS1 antibody were assessed using Western blot and B19 DNA was detected by nested Polymerase Chain Reaction (PCR. Levels of interleukin (IL-17, IL-6, IL-1β, and tumor necrosis factor (TNF-α were significantly higher in SLE patients with DCM (mean ± SEM, 390.99±125.48 pg/ml, 370.24±114.09 pg/ml, 36.01±16.90 pg/ml, and 183.84±82.94 pg/ml, respectively compared to healthy controls (51.32±3.04 pg/ml, p<0.001; 36.88±6.64 pg/ml, p<0.001; 5.39±0.62 pg/ml, p<0.005; and 82.13±2.42 pg/ml, p<0.005, respectively. Levels of IL-17 and IL-6 were higher in SLE patients with DCM versus those with VHD (both p<0.01. Five (62.5% of DCM patients had detectable anti-B19-NS1 IgG and four (50.0% of them had anti-B19-VP1u IgG, whereas only one (16.7% of VHD patients had detectable anti-B19-NS1 IgG and anti-B19-VP1u IgG. Serum levels of IL-17, IL-6 and IL-1β were markedly higher in SLE patients with anti-B19-VP1u IgG and anti-B19-NS1 IgG compared to those without anti-B19-VP1u IgG or anti-B19-NS1 IgG, respectively. These suggest a potential association of B19 with DCM and Th17-related cytokines implicated in the pathogenesis of DCM in SLE patients.

  17. Association of Nicotinamide Phosphoribosyltransferase (NAMPT Gene Polymorphisms and of Serum NAMPT Levels with Dilated Cardiomyopathy in a Chinese Population

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    Qingyu Dou

    2015-09-01

    Full Text Available Nicotinamide phosphoribosyltransferase (NAMPT has crucial roles for myocardial development, cardiomyocyte energy metabolism and cell death/survival by regulating NAD+-dependent sirtuin-1 (SIRT1 deacetylase. This study aimed to determine if the single nucleotide polymorphisms (SNPs of the NAMPT gene may affect the susceptibility and prognosis for patients with dilated cardiomyopathy (DCM and to describe the association of serum NAMPT levels with clinical features of DCM. Three SNPs (rs61330082, rs2505568, and rs9034 were analyzed by the polymerase chain reaction-restriction fragment length polymorphism method in a case-control study of 394 DCM patients and 395 controls from China. Serum NAMPT levels were measured by enzyme-linked immunosorbent assay kits. The homozygote for the minor allele at rs2505568 and rs9034 could not be detected in this study. Rs9034 T allele and CT genotype were associated with increased DCM risk (OR: 1.63, 95% CI = 1.16–2.27, p = 0.005 and OR: 1.72, 95% CI = 1.20–2.50, p = 0.0027, respectively. Nominally significant decreased DCM risk was found to be associated with the A allele and AT genotype of rs2505568 (OR: 0.48, 95% CI = 0.35–0.67, p < 0.0001 and OR: 0.44, 95% CI = 0.31–0.62, p < 0.0001, respectively, but it should be interpreted with caution because of Hardy-Weinberg disequilibrium in the control group. Of five haplotypes constructed, TAC (rs61330082-rs2505568-rs9034 was a protective haplotype to DCM (OR: 0.22, 95% CI = 0.13–0.39, p = 1.84 × 10−8. The Cox multivariate survival analysis indicated that the rs9034 CT genotype (hazard ratio (HR: 0.59, 95% CI = 0.37–0.96, p = 0.03 was an independently multivariate predictor for longer overall survival in DCM patients. Serum NAMPT levels were significantly higher in the DCM group than controls (p < 0.0001 and gradually increased with the increase of New York Heart Association grade in DCM patients. However, there was a lack of association of the three

  18. Prevalence of cardiac dyssynchrony and correlation with atrio-ventricular block and QRS width in dilated cardiomyopathy: an echocardiographic study.

    Science.gov (United States)

    Anzouan-Kacou, J B; Ncho-Mottoh, M P; Konin, C; N'Guetta, A R; Ekou, K A; Koffi, B J; Soya, K E; Tango, M E; Abouo-N'Dori, R

    2012-08-01

    Cardiac dyssynchrony causes disorganised cardiac contraction, delayed wall contraction and reduced pumping efficiency. We aimed to assess the prevalence of different types of dyssynchrony in patients with dilated cardiomyopathy (DCM), and to establish the correlation between atrio-ventricular block and atrio-ventricular dyssynchrony (AVD), and between impaired intra-ventricular conduction and the existence of inter-ventricular dyssynchrony (inter-VD) and intra-left ventricular dyssynchrony (intra-LVD). We included 40 patients in New York Heart Association stage III or IV, admitted consecutively with DCM with severe left ventricular dysfunction (left ventricular end-diastolic diameter ≥ 60 mm and/or ≥ 30 mm/m(2)) and left ventricular ejection fraction atrio-ventricular dyssynchrony (AVD), inter-VD and intra-LVD was respectively 40, 47.5 and 70%. Two patients (5%) did not exhibit dyssynchrony. AVD was present with a similar frequency in the two groups (37.5% in group 1 vs 40.6% in group 2, p = 0.8). There was no correlation of the magnitude of AVD with the duration of the PR interval (from the beginning of the P wave to the beginning of the QRS complex) (r(2) = 0.02, p = 0.37) or the QRS width (r(2) = 0.01, p = 0.38). A greater proportion of patients with inter-VD was observed in group 1 (87.5 vs 60%, p = 0.03). There was a trend towards a more important inter-ventricular mechanical delay according to QRS width (r(2) = 0.009, p = 0.06). The proportion of intra-LVD was similar in all groups, with a high prevalence (87.5% in group 1 and 65.6% in group 2, p = 0.39). The assessment of cardiac dyssynchrony is possible in our country. Intra-ventricular mechanical dyssynchrony had a high prevalence in patients with DCM, irrespective of the QRS width. These data emphasise the usefulness of echocardiography in the screening of patients.

  19. Objective criteria for septal fibrosis in non-ischemic dilated cardiomyopathy: validation for the prediction of future cardiovascular events.

    Science.gov (United States)

    Mikami, Yoko; Cornhill, Aidan; Heydari, Bobak; Joncas, Sebastien X; Almehmadi, Fahad; Zahrani, Mohammed; Bokhari, Mahmoud; Stirrat, John; Yee, Raymond; Merchant, Naeem; Lydell, Carmen P; Howarth, Andrew G; White, James A

    2016-11-14

    Expert subjective reporting of mid-wall septal fibrosis on late gadolinium enhancement (LGE) images has been shown to predict major cardiovascular outcomes in patients with non-ischemic dilated cardiomyopathy (NIDCM). This study aims to establish objective criteria for non-experts to report clinically relevant septal fibrosis and compare its performance by such readers versus experts for the prediction of cardiovascular events. LGE cardiovascular magnetic resonance (CMR) was performed in 118 consecutive patients with NIDCM (mean age 57 ± 14, 42 % female) and the presence of septal fibrosis scored by expert readers. CMR-naive readers performed signal threshold-based LGE quantification by referencing mean values of remote tissue and applying these to a pre-defined anatomic region to measure septal fibrosis. All patients were followed for the primary composite outcome of cardiac mortality or appropriate implantable cardioverter-defibrillator (ICD) therapy. The mean LVEF was 32 ± 12 %. At a median follow-up of 1.9 years, 20 patients (17 %) experienced a primary composite outcome. Expert visual scoring identified 55 patients with septal fibrosis. Non-expert septal fibrosis quantification was highly reproducible and identified mean septal fibrosis burden for three measured thresholds as follows; 5SD: 2.9 ± 3.6 %, 3SD: 6.9 ± 6.3 %, and 2SD: 11.1 ± 7.5 % of the left ventricular (LV) mass, respectively. By ROC analysis, optimal thresholds for prediction of the primary outcome were; 5SD: 2.74 % (HR 8.7, p 5SD threshold) was the strongest independent predictor of the primary outcome (HR 8.7) and provided improved risk reclassification beyond LVEF alone (NRI 0.54, 95 % CI 0.16-0.92, p = 0.005). Novice readers were able to achieve superior risk prediction for future cardiovascular events versus experts using objective criteria for septal fibrosis in patients with NIDCM. Patients with a septal fibrosis burden >2.74 % of the LV mass (>5SD

  20. Significance of thymosin β4 and implication of PINCH-1-ILK-α-parvin (PIP complex in human dilated cardiomyopathy.

    Directory of Open Access Journals (Sweden)

    Nikolai Sopko

    Full Text Available Myocardial remodeling is a major contributor in the development of heart failure (HF after myocardial infarction (MI. Integrin-linked kinase (ILK, LIM-only adaptor PINCH-1, and α-parvin are essential components of focal adhesions (FAs, which are highly expressed in the heart. ILK binds tightly to PINCH-1 and α-parvin, which regulates FA assembly and promotes cell survival via the activation of the kinase Akt. Mice lacking ILK, PINCH or α-parvin have been shown to develop severe defects in the heart, suggesting that these proteins play a critical role in heart function. Utilizing failing human heart tissues (dilated cardiomyopathy, DCM, we found a 2.27-fold (p<0.001 enhanced expression of PINCH, 4 fold for α-parvin, and 10.5 fold (p<0.001 for ILK as compared to non-failing (NF counterparts. No significant enhancements were found for the PINCH isoform PINCH-2 and parvin isoform β-parvin. Using a co-immunoprecipitation method, we also found that the PINCH-1-ILK-α-parvin (PIP complex and Akt activation were significantly up-regulated. These observations were further corroborated with the mouse myocardial infarction (MI and transaortic constriction (TAC model. Thymosin beta4 (Tβ4, an effective cell penetrating peptide for treating MI, was found to further enhance the level of PIP components and Akt activation, while substantially suppressing NF-κB activation and collagen expression--the hallmarks of cardiac fibrosis. In the presence of an Akt inhibitor, wortmannin, we show that Tβ4 had a decreased effect in protecting the heart from MI. These data suggest that the PIP complex and activation of Akt play critical roles in HF development. Tβ4 treatment likely improves cardiac function by enhancing PIP mediated Akt activation and suppressing NF-κB activation and collagen-mediated fibrosis. These data provide significant insight into the role of the PIP-Akt pathway and its regulation by Tβ4 treatment in post-MI.

  1. The effect of β-blocker on hamster model BIO 53.58 with dilated cardiomyopathy determined using 123I-MIBG myocardial scintigraphy

    International Nuclear Information System (INIS)

    Inoue, Aritomo; Yamashina, Shohei; Yamazaki, Junichi

    2003-01-01

    123 I-metaiodobenzylguanidine (MIBG) myocardial scintigraphy is currently used to evaluate cardiac sympathetic nerve function, but MIBG also has the capacity to evaluate dilated cardiomyopathy (DCM) severity and therapeutic effectiveness. In this study, we administered β-blockers to a DCM hamster model and evaluated the effect of therapy using MIBG. We also pathologically compared the effects of myocardial fibrosis suppression. BIO 53.58 hamsters were divided into the following five groups based on β-blocker administration: vehicle (COT), 2 mg/kg/day carvedilol (CLT), 20 mg/kg/day (CHT) carvedilol, 4 mg/kg/day (MLT) metoprolol, 40 mg/kg/day (MHT) metoprolol. F1B hamsters were administered a vehicle (COF). Plasma catecholamine, noradrenaline (p-NADR), adrenaline (p-ADR), and dopamine (p-DOPA) were assayed, and MIBG was performed. The count ratio of the heart to the mediastinum (H/M) and left ventricle myocardial washout ratio (WR) were calculated. We then performed an autopsy and calculated the percent change in fibrotic area from myocardial sections. H/M of the initial image in the COT group was significantly lower at 2.4±0.2 than the 2.9±0.7 in the COF group (p<0.05). The CLT and CHT groups had higher H/M values compared to the COT group (3.1±0.6, 3.0±0.6 versus 2.4±0.2: p<0.05). Significant correlations were evident between the H/M of the delayed image and p-NADR and p-DOPA (p<0.05, p<0.01, respectively) as well as between WR and p-NADR and p-DOPA (p<0.05). Percent change in fibrotic area was significantly lower in the β-blocker groups than in the COT group (p<0.05). Significant negative correlations were seen between the H/M of the delayed image and the percent change in fibrosis area. The delayed image H/M and WR acutely reflected cardiac disorder and sympathetic nerve function disorder in BIO 53.58 hamsters. In the carvedilol-administered groups, there was improvement compared to the initial H/M image, indicating the efficacy of the β-blocker in DCM

  2. Cardiomyopathy in neurological disorders.

    Science.gov (United States)

    Finsterer, Josef; Stöllberger, Claudia; Wahbi, Karim

    2013-01-01

    According to the American Heart Association, cardiomyopathies are classified as primary (solely or predominantly confined to heart muscle), secondary (those showing pathological myocardial involvement as part of a neuromuscular disorder) and those in which cardiomyopathy is the first/predominant manifestation of a neuromuscular disorder. Cardiomyopathies may be further classified as hypertrophic cardiomyopathy, dilated cardiomyopathy, restrictive cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, or unclassified cardiomyopathy (noncompaction, Takotsubo-cardiomyopathy). This review focuses on secondary cardiomyopathies and those in which cardiomyopathy is the predominant manifestation of a myopathy. Any of them may cause neurological disease, and any of them may be a manifestation of a neurological disorder. Neurological disease most frequently caused by cardiomyopathies is ischemic stroke, followed by transitory ischemic attack, syncope, or vertigo. Neurological disease, which most frequently manifests with cardiomyopathies are the neuromuscular disorders. Most commonly associated with cardiomyopathies are muscular dystrophies, myofibrillar myopathies, congenital myopathies and metabolic myopathies. Management of neurological disease caused by cardiomyopathies is not at variance from the same neurological disorders due to other causes. Management of secondary cardiomyopathies is not different from that of cardiomyopathies due to other causes either. Patients with neuromuscular disorders require early cardiologic investigations and close follow-ups, patients with cardiomyopathies require neurological investigation and avoidance of muscle toxic medication if a neuromuscular disorder is diagnosed. Which patients with cardiomyopathy profit most from primary stroke prevention is unsolved and requires further investigations. Copyright © 2013 Elsevier Inc. All rights reserved.

  3. Association of myocardial inotropic reserve and adrenergic nerve alterations in idiopathic dilated cardiomyopathy. A dobutamine stress echocardiographic and 123-I-MIBG scintigraphic study

    International Nuclear Information System (INIS)

    Prassopoulos, V.P.; Koukouraki, S.; Velidaki, A.; Karkavitsas, N.; Parthenakis, F.; Patrianakos, A.; Kochiadakis, G.; Papadimitriou, E.; Vardas, P.

    2002-01-01

    Aim: Evaluation of contractile reserve is important in congestive hear failure. The aim of this study was to examine the relationship between the myocardial response to dobutamine by stress echocardiography and the sympathetic nerve alterations by 123-I-Metaiodobenzylguanidine (MIBG)scintigraphy and how both contribute to predict exercise capacity in patients with idiopathic dilated cardiomyopathy. Materials-Methods: We studied 20 patients with idiopathic dilated cardiomyopathy (IDC) and ejection fraction < 45% (M/F 13/7, age 56±11 years) while 15 healthy individuals served as controls. Echocardiography and myocardial scintigraphic study with 123-I- MIBG, provided quantitative assessment of left ventricular (LV) wall motion and heart to mediastinum uptake(H/M) ratio and washout. All patients underwent a cardiopulmonary exercise test with a modified Naughton protocol and gas exchange data were analyzed. According to LV response to dobutamine, patients were divided into two groups: those in whom contractility improved in ≥ five segments (Group I: 11 patients) and those in whom contractility improved in < 5 segments (Group II : 9 patients). Results: MIBG uptake was significantly lower in patients than in controls (p < 0.001). MIBG uptake and washout was higher in Group I compared to Group II (P<0,01 and p<0,05). Late MIBG H/M was correlated with resting ejection fraction (r=0,70), wall motion score index (WMSI) (r=-0,50), end systolic wall stress (r=-0,61), washout (r=-0,57), and oxygen consumption at peak exercise (r=0,64) and at anaerobic threshold (r=0,67). LV ejection fraction increased in both groups at Dobutamine, with a higher increase in Group I (p=0,008). WMSI changes at dobutamine correlated significantly with resting ejection fraction (r=0,46) early (r=0,53) and late (r=0,54) MIBG. Multivariate analysis revealed that only the late MIBG uptake was independently associated with the improvement in WMSI. Conclusions: The present data indicate that in

  4. Is it possible to differentiate between Takotsubo cardiomyopathy and acute anterior ST-elevation myocardial infarction?

    NARCIS (Netherlands)

    Vervaat, Fabienne E.; Christensen, Thomas E.; Smeijers, Loes; Holmvang, Lene; Hasbak, Philip; Szabo, Balazs M.; Widdershoven, J.W. M. G.; Wagner, Galen S.; Bang, Lia E.; Gorgels, Anton P. M.

    2015-01-01

    Introduction Several studies have investigated the ability of the twelve-lead electrocardiogram (ECG) to reliably distinguish Takotsubo cardiomyopathy (TC) from an acute anterior ST-segment elevation myocardial infarction (STEMI). In these studies, only ECG changes were required – ST-segment

  5. Acute gastric dilatation complicating the use of mydriatics in a preterm newborn

    Energy Technology Data Exchange (ETDEWEB)

    Sarici, S.Ue.; Yurdakoek, M.; Uenal, S. [Div. of Neonatology, Hacettepe University School of Medicine, Ankara (Turkey)

    2001-08-01

    A 2-month-old girl who had been born at 27-weeks' gestation was admitted for her screening examination for retinopathy of prematurity and given two drops each of cyclopentolate 0.5 % and phenylephrine 2.5 %. Approximately 2 h after completion of the examination, the infant had episodes of apnoea and vomiting. She was noted to be distended, and an abdominal radiograph demonstrated acute gastric dilatation. Apnoea, vomiting and distension resolved after 18 h and a repeat abdominal radiograph demonstrated resolution of the gastric dilatation. (orig.)

  6. Acute gastric dilatation complicating the use of mydriatics in a preterm newborn

    International Nuclear Information System (INIS)

    Sarici, S.Ue.; Yurdakoek, M.; Uenal, S.

    2001-01-01

    A 2-month-old girl who had been born at 27-weeks' gestation was admitted for her screening examination for retinopathy of prematurity and given two drops each of cyclopentolate 0.5 % and phenylephrine 2.5 %. Approximately 2 h after completion of the examination, the infant had episodes of apnoea and vomiting. She was noted to be distended, and an abdominal radiograph demonstrated acute gastric dilatation. Apnoea, vomiting and distension resolved after 18 h and a repeat abdominal radiograph demonstrated resolution of the gastric dilatation. (orig.)

  7. 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

    in the sudden cardiac death (SCD) of the proband at the age of 21 years. Further, a DCM causing novel mutation p.Gly377Ser was identified which resulted in the milder phenotype. The present study shows that there is genetic and phenotypic heterogeneity of cardiomyopathies in Indian population. Further...

  8. Abdominal aortic occlusion and vascular compromise secondary to acute gastric dilatation in a patient with bulimia.

    Science.gov (United States)

    Elsharif, M; Doulias, T; Aljundi, W; Balchandra, S

    2014-11-01

    Acute gastric dilation is a rare but recognised complication in patients with bulimia and anorexia following binge episodes owing to decreased bowel motility. We present a rare case of acute gastric dilation secondary to bulimia in an otherwise healthy 18-year-old female patient that resulted in compression and complete occlusion of the abdominal aorta, leading to acute mesenteric and bilateral lower limb ischaemia. This resolved immediately following a laparotomy and gastric decompression. Management of these patients is very challenging owing to the lack of a successful precedent. To our knowledge, such a catastrophic complication has only ever been reported once in the literature and the outcome was fatal. Our case is of additional importance as it offers a successful management strategy for these patients.

  9. Myocardial adrenergic status in patients with idiopatic dilated cardiomyopathies and with chagas' disease studied by the myocardic scintillography with I-123-MIBG

    International Nuclear Information System (INIS)

    Freire, R.; Marinho, N.; Freitas, H.; Chizzola, P.; Buchpiguel, C.; Mansur, A.; Meneghetti, C.; Bocchi, E.

    1997-01-01

    Full text: Metaiodobenzylguanidine labeled with I-123 MIBG is a radiopharmaceutical used to study the cardiac adrenergic activity. The objective of this study was to test the hypothesis that congestive cardiac failure (CCF) patients with Chagas' disease etiology would have a scintillographic pattern of uptake of MIBG similar to that presented by patients with idiopatic dilated cardiomyopathies (IDCM). We studied 24 patients, 10 with Chagas' disease and 14 with IDMC.The results show that the presented alterations in the cardiac uptake of 15m and 4 h, in the turn over (wash out) and in the distribution (ratio heart/mediastinum), presented in the patients with Chagas' disease are similar (p=ns) to that found in the IDCM patients. Although the IDCM and Chagas' disease etiology are different, both courses with a decrease in the number of adrenergic receptors available at myocardial cell level, demonstrated by similarity in the presented scintillographic pattern

  10. Prediction of cardiac sympathetic nerve activity and cardiac functional outcome after treatment in patients with dilated cardiomyopathy. Examination using dobutamine gated blood pool scintigraphy

    Energy Technology Data Exchange (ETDEWEB)

    Kasama, Shu; Toyama, Takuji; Iwasaki, Tsutomu; Suzuki, Tadashi [Gunma Univ., Maebashi (Japan). School of Medicine; Hoshizaki, Hiroshi; Oshima, Shigeru; Taniguchi, Koichi; Nagai, Ryozo

    2000-07-01

    This study evaluated whether dobutamine gated blood pool scintigraphy can predict improvement of cardiac sympathetic nerve activity and cardiac function. Sixteen patients (10 men and 6 women, mean age 59{+-}13 years) with dilated cardiomyopathy underwent dobutamine gated blood pool scintigraphy to measure left ventricular ejection fraction (LVEF) using tracer at 0, 5, 10 and 15 {mu}g/kg/min before treatment. Patients were divided into good responders (LVEF increase {>=}15%) 8 patients (GR Group) and poor responders (LVEF increase <15%) 8 patients (PR Group) after treatment with {beta}-blocker or amiodarone with a background treatment of digitalis, diuretics and angiotensin converting enzyme inhibitor. I-123 metaiodobenzylguanidine (MIBG) imaging to evaluate cardiac sympathetic nerve activity and echocardiography were performed before and at one year after treatment. MIBG imaging was obtained 4 hours after tracer injection, and the heart/mediastinum count ratio (H/M ratio) calculated from the anterior planar image and the total defect score (TDS) from the single photon emission computed tomography image. LVEF and left ventricular endo-diastolic dimension (LVDd) were measured by echocardiography and New York Heart Association (NYHA) functional class was evaluated. The GR Group showed TDS decreased from 28{+-}6 to 17{+-}12 (p<0.05), H/M ratio increased from 1.79{+-}0.26 to 2.07{+-}0.32 (p<0.05), LVEF increased from 29{+-}8% to 48{+-}10% (p<0.01), and LVDd decreased from 65{+-}4 mm to 58{+-}5 mm (p<0.05). In contrast, the PR group showed no significant changes in TDS. H/M ratio, LVEF and LVDd. NYHA functional class improved in both groups. The improvement was better in the GR Group than in the PR group. Dobutamine gated blood pool scintigraphy is useful to predict the improvement of the cardiac sympathetic nerve activity and cardiac function, and symptoms after treatment in patients with dilated cardiomyopathy. (author)

  11. Parent reports of health-related quality of life and heart failure severity score independently predict outcome in children with dilated cardiomyopathy.

    Science.gov (United States)

    den Boer, Susanna L; Baart, Sara J; van der Meulen, Marijke H; van Iperen, Gabriëlle G; Backx, Ad P; Ten Harkel, Arend D; Rammeloo, Lukas A; du Marchie Sarvaas, Gideon J; Tanke, Ronald B; Helbing, Willem A; Utens, Elisabeth M; Dalinghaus, Michiel

    2017-08-01

    Dilated cardiomyopathy in children causes heart failure and has a poor prognosis. Health-related quality of life in this patient group is unknown. Moreover, results may provide detailed information of parents' sense of their child's functioning. We hypothesised that health-related quality of life, as rated by parents, and the paediatric heart failure score, as assessed by physicians, have both predictive value on outcome. Methods and results In this prospective study, health-related quality of life was assessed by parent reports: the Infant Toddler Quality of Life questionnaire (0-4 years) or Child Health Questionnaire-Parent Form 50 (4-18 years) at 3-6-month intervals. We included 90 children (median age 3.8 years, interquartile range (IQR) 0.9-12.3) whose parents completed 515 questionnaires. At the same visit, physicians completed the New York University Pediatric Heart Failure Index. Compared with Dutch normative data, quality of life was severely impaired at diagnosis (0-4 years: 7/10 subscales and 4-18 years: 8/11 subscales) and ⩾1 year after diagnosis (3/10 and 6/11 subscales). Older children were more impaired (pFailure Index were independently predictive of the risk of death and heart transplantation (hazard ratio 1.24 per 10% decrease of predicted, 95% confidence interval (CI) 1.06-1.47 and hazard ratio 1.38 per unit, 95% CI 1.19-1.61, respectively). Physical impairment rated by parents and heart failure severity assessed by physicians independently predicted the risk of death or heart transplantation in children with dilated cardiomyopathy.

  12. Allogeneic cardiospheres delivered via percutaneous transendocardial injection increase viable myocardium, decrease scar size, and attenuate cardiac dilatation in porcine ischemic cardiomyopathy.

    Directory of Open Access Journals (Sweden)

    Kristine Yee

    Full Text Available Epicardial injection of heart-derived cell products is safe and effective post-myocardial infarction (MI, but clinically-translatable transendocardial injection has never been evaluated. We sought to assess the feasibility, safety and efficacy of percutaneous transendocardial injection of heart-derived cells in porcine chronic ischemic cardiomyopathy.We studied a total of 89 minipigs; 63 completed the specified protocols. After NOGA-guided transendocardial injection, we quantified engraftment of escalating doses of allogeneic cardiospheres or cardiosphere-derived cells in minipigs (n = 22 post-MI. Next, a dose-ranging, blinded, randomized, placebo-controlled ("dose optimization" study of transendocardial injection of the better-engrafting product was performed in infarcted minipigs (n = 16. Finally, the superior product and dose (150 million cardiospheres were tested in a blinded, randomized, placebo-controlled ("pivotal" study (n = 22. Contrast-enhanced cardiac MRI revealed that all cardiosphere doses preserved systolic function and attenuated remodeling. The maximum feasible dose (150 million cells was most effective in reducing scar size, increasing viable myocardium and improving ejection fraction. In the pivotal study, eight weeks post-injection, histopathology demonstrated no excess inflammation, and no myocyte hypertrophy, in treated minipigs versus controls. No alloreactive donor-specific antibodies developed over time. MRI showed reduced scar size, increased viable mass, and attenuation of cardiac dilatation with no effect on ejection fraction in the treated group compared to placebo.Dose-optimized injection of allogeneic cardiospheres is safe, decreases scar size, increases viable myocardium, and attenuates cardiac dilatation in porcine chronic ischemic cardiomyopathy. The decreases in scar size, mirrored by increases in viable myocardium, are consistent with therapeutic regeneration.

  13. Cardiomyopathies and anaesthesia

    Directory of Open Access Journals (Sweden)

    Rajiv Juneja

    2017-01-01

    Full Text Available Cardiomyopathy is considered as a heart muscle disease of multiple aetiologies, unlike other cardiac diseases related to a definitive pathophysiology. With more and more research and with the advent of genetic analysis pin pointing the disease causing mutations, causative factors have been defined and classifications and definitions have changed over time. Patients with these conditions present to anaesthesiologists in elective and emergency situations, placement of automated internal cardioverter defibrillator (AICD devices or biventricular pacing but may also be diagnosed at anaesthetic pre-assessment. We describe cardiomyopathies such as dilated cardiomyopathy, hypertrophic cardiomyopathy, post-partum cardiomyopathy and Takotsubo cardiomyopathy in brief and their anaesthetic management.

  14. Acute gastric dilatation in Duchenne muscular dystrophy: a case report and review of the literature.

    Science.gov (United States)

    Bensen, E S; Jaffe, K M; Tarr, P I

    1996-05-01

    Duchenne muscular dystrophy (DMD) is the most common neuromuscular disorder of childhood. Its clinical characteristics that derive from skeletal muscle involvement have been well described. Less well known is that visceral smooth muscle is affected in DMD. We report a case of a 19-year-old man with DMD who presented with severe nonradiating epigastric pain. He was initially sent home from the emergency department with a diagnosis of costochondritis. Acute gastric dilation was not considered in the differential diagnosis despite supportive history, physical examination findings, and radiographs. The case illustrates the lack of familiarity by clinicians of the gastrointestinal manifestations of DMD, including gastric dilatation and intestinal pseudoobstruction. Following a case discussion, the literature relevant to acute gastric atony is reviewed.

  15. MRI of the cardiomyopathies

    Energy Technology Data Exchange (ETDEWEB)

    Di Cesare, Ernesto E-mail: ernesto.dicesare@cc.univaq.it

    2001-06-01

    We examined the potentialities of Magnetic resonance imaging (MRI) in the evaluation of the main cardiomyopathies: hypertrophic, dilated, restrictive and arrhythmogenic right ventricular. The hypertrophic cardiomyopathy is generally adequately investigated by echocardiography, that well defines the myocardial thickening and the obstruction of the left ventricular output. However, by echocardiography we still have difficulties in the evaluation of the apex of the left ventricle and the right ventricle involvement. MRI provides a complete evaluation of the heart with a clear evidence also of the echocardiographic dark zones by means of a clear evidence of the apex of the right ventricle. The dilated form is also well investigated by MRI that provides a clear evaluation of the volumes, mass and ejection fraction by means of the 3D analysis including conditions of the ventricular remodelling. Moreover, this technique helps in the differential diagnosis of acute myocarditis. In the acute phase of myocarditis (first 2 weeks), in fact, the myocardium produces high signal intensity on the T2 weighted sequences due to the presence of oedema. The third form of cardiomyopathy is the restrictive one, characterised by reduced diastolic filling and diastolic volume, normality of the systolic function and parietal thickness, interstitial fibrosis and enlargement of both atria. The mean potentiality of MRI is related to the differential diagnosis with constrictive pericarditis. Only in the former, the pericardium appears irregularly thickened with areas exceeding 4 mm of pericardial thickness. Finally, the right ventricular arrhythmogenic cardiomyopathy represents the main indication to MRI evaluation. With this imaging modality we are can obtain a clear morpho-functional evaluation of the right ventricle and distinguish the intramyocardial adipose substitution characterised by areas of high signal in the myocardium.

  16. MRI of the cardiomyopathies

    International Nuclear Information System (INIS)

    Di Cesare, Ernesto

    2001-01-01

    We examined the potentialities of Magnetic resonance imaging (MRI) in the evaluation of the main cardiomyopathies: hypertrophic, dilated, restrictive and arrhythmogenic right ventricular. The hypertrophic cardiomyopathy is generally adequately investigated by echocardiography, that well defines the myocardial thickening and the obstruction of the left ventricular output. However, by echocardiography we still have difficulties in the evaluation of the apex of the left ventricle and the right ventricle involvement. MRI provides a complete evaluation of the heart with a clear evidence also of the echocardiographic dark zones by means of a clear evidence of the apex of the right ventricle. The dilated form is also well investigated by MRI that provides a clear evaluation of the volumes, mass and ejection fraction by means of the 3D analysis including conditions of the ventricular remodelling. Moreover, this technique helps in the differential diagnosis of acute myocarditis. In the acute phase of myocarditis (first 2 weeks), in fact, the myocardium produces high signal intensity on the T2 weighted sequences due to the presence of oedema. The third form of cardiomyopathy is the restrictive one, characterised by reduced diastolic filling and diastolic volume, normality of the systolic function and parietal thickness, interstitial fibrosis and enlargement of both atria. The mean potentiality of MRI is related to the differential diagnosis with constrictive pericarditis. Only in the former, the pericardium appears irregularly thickened with areas exceeding 4 mm of pericardial thickness. Finally, the right ventricular arrhythmogenic cardiomyopathy represents the main indication to MRI evaluation. With this imaging modality we are can obtain a clear morpho-functional evaluation of the right ventricle and distinguish the intramyocardial adipose substitution characterised by areas of high signal in the myocardium

  17. 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.

  18. Acute pulmonary edema due to stress cardiomyopathy in a patient with aortic stenosis: a case report

    OpenAIRE

    Bayer, Monika F

    2009-01-01

    Introduction Stress cardiomyopathy is a condition of chest pain, breathlessness, abnormal heart rhythms and sometimes congestive heart failure or shock precipitated by intense mental or physical stress. Case presentation A 64-year-old male with a known diagnosis of moderate-to-severe aortic stenosis and advised that valve replacement was not urgent, presented with acute pulmonary edema following extraordinary mental distress. The patient was misdiagnosed as having a "massive heart attack" and...

  19. Locally expressed IGF1 propeptide improves mouse heart function in induced dilated cardiomyopathy by blocking myocardial fibrosis and SRF-dependent CTGF induction

    Directory of Open Access Journals (Sweden)

    Melissa Touvron

    2012-07-01

    Cardiac fibrosis is critically involved in the adverse remodeling accompanying dilated cardiomyopathies (DCMs, which leads to cardiac dysfunction and heart failure (HF. Connective tissue growth factor (CTGF, a profibrotic cytokine, plays a key role in this deleterious process. Some beneficial effects of IGF1 on cardiomyopathy have been described, but its potential role in improving DCM is less well characterized. We investigated the consequences of expressing a cardiac-specific transgene encoding locally acting IGF1 propeptide (muscle-produced IGF1; mIGF1 on disease progression in a mouse model of DCM [cardiac-specific and inducible serum response factor (SRF gene disruption] that mimics some forms of human DCM. Cardiac-specific mIGF1 expression substantially extended the lifespan of SRF mutant mice, markedly improved cardiac functions, and delayed both DCM and HF. These protective effects were accompanied by an overall improvement in cardiomyocyte architecture and a massive reduction of myocardial fibrosis with a concomitant amelioration of inflammation. At least some of the beneficial effects of mIGF1 transgene expression were due to mIGF1 counteracting the strong increase in CTGF expression within cardiomyocytes caused by SRF deficiency, resulting in the blockade of fibroblast proliferation and related myocardial fibrosis. These findings demonstrate that SRF plays a key role in the modulation of cardiac fibrosis through repression of cardiomyocyte CTGF expression in a paracrine fashion. They also explain how impaired SRF function observed in human HF promotes fibrosis and adverse cardiac remodeling. Locally acting mIGF1 efficiently protects the myocardium from these adverse processes, and might thus represent a therapeutic avenue to counter DCM.

  20. CLINICAL AND MORPHOLOGICAL APPROACH TO DIAGNOSIS OF "IDIOPATHIC" ARRHYTHMIAS AND DILATED CARDIOMYOPATHY SYNDROME AS A BASIS FOR DIFFERENTIATED THERAPY. Part II (Treatment

    Directory of Open Access Journals (Sweden)

    O. V. Blagova

    2014-01-01

    Full Text Available Part I (Diagnostics, was published in the journal “Rational Pharmacotherapy in Cardiology” 2014;10(1:62-72Aim. To develop a comprehensive clinical and morphological approach to the nosological diagnosis and treatment of "idiopathic" arrhythmias (IA and the syndrome of dilated cardiomyopathy (DCM.Material and methods. Patients (n=320 with IA (n=190; 117 women, age 45.3±14.8 years and DCM (n=130, 41 women, age 46.9±12.5 years were included in the main group. 51 people (patients with ischemic heart disease; heart valve disease, hypertrophic cardiomyopathy, who underwent open-heart surgery; healthy volunteers were included in the control group. Along with the standard tests evaluation of the level of anti-heart antibodies (185 patients with IA and 122 with DCM, viral serology (166 and 122, multispiral computed tomography (42 and 88, cardiac magnetic resonance imaging (41 and 22, coronary angiography (19 and 54, myocardial biopsy/autopsy (19/0 and 33/9 were performed.Results. According to morphological study infectious-immune myocarditis was found in 78.9% patients with IA and 66.7% patients with DCM, arrhythmogenic right ventricular dysplasia in 5.3% and 4.8% of patients, respectively. Other genetic cardiomyopathies, including combination with myocarditis were revealed in other patients. The frequency of the viral genome detection in the myocardium in IA, DCM and the control group was 17.6%, 66.7% and 77.1%, respectively. However in the control group the incidence of myocarditis and anti-heart antibodies titers were significantly lower than in the main group, where a strong correlation between myocarditis and anti-heart antibodies titers was found. The algorithm of noninvasive nosological diagnostics was developed; it allowed to verify diagnosis in 95% of IA patients and 89% DCM patientsThe basic therapy (antiviral drugs, corticosteroids, hydroxychloroquine, azathioprine was performed in some patients with myocarditis. Improving of the

  1. CLINICAL AND MORPHOLOGICAL APPROACH TO DIAGNOSIS OF "IDIOPATHIC" ARRHYTHMIAS AND DILATED CARDIOMYOPATHY SYNDROME AS A BASIS FOR DIFFERENTIATED THERAPY. Part II (Treatment

    Directory of Open Access Journals (Sweden)

    O. V. Blagova

    2015-09-01

    Full Text Available Part I (Diagnostics, was published in the journal “Rational Pharmacotherapy in Cardiology” 2014;10(1:62-72Aim. To develop a comprehensive clinical and morphological approach to the nosological diagnosis and treatment of "idiopathic" arrhythmias (IA and the syndrome of dilated cardiomyopathy (DCM.Material and methods. Patients (n=320 with IA (n=190; 117 women, age 45.3±14.8 years and DCM (n=130, 41 women, age 46.9±12.5 years were included in the main group. 51 people (patients with ischemic heart disease; heart valve disease, hypertrophic cardiomyopathy, who underwent open-heart surgery; healthy volunteers were included in the control group. Along with the standard tests evaluation of the level of anti-heart antibodies (185 patients with IA and 122 with DCM, viral serology (166 and 122, multispiral computed tomography (42 and 88, cardiac magnetic resonance imaging (41 and 22, coronary angiography (19 and 54, myocardial biopsy/autopsy (19/0 and 33/9 were performed.Results. According to morphological study infectious-immune myocarditis was found in 78.9% patients with IA and 66.7% patients with DCM, arrhythmogenic right ventricular dysplasia in 5.3% and 4.8% of patients, respectively. Other genetic cardiomyopathies, including combination with myocarditis were revealed in other patients. The frequency of the viral genome detection in the myocardium in IA, DCM and the control group was 17.6%, 66.7% and 77.1%, respectively. However in the control group the incidence of myocarditis and anti-heart antibodies titers were significantly lower than in the main group, where a strong correlation between myocarditis and anti-heart antibodies titers was found. The algorithm of noninvasive nosological diagnostics was developed; it allowed to verify diagnosis in 95% of IA patients and 89% DCM patientsThe basic therapy (antiviral drugs, corticosteroids, hydroxychloroquine, azathioprine was performed in some patients with myocarditis. Improving of the

  2. Use of iodine-123 metaiodobenzylguanidine myocardial imaging to predict the effectiveness of {beta}-blocker therapy in patients with dilated cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Fukuoka, Shuji [Department of Radiology, National Cardiovascular Center, Osaka (Japan); Hayashida, Kohei [Department of Radiology, National Cardiovascular Center, Osaka (Japan); Hirose, Yoshiaki [Department of Radiology, National Cardiovascular Center, Osaka (Japan); Shimotsu, Yoriko [Department of Radiology, National Cardiovascular Center, Osaka (Japan); Ishida, Yoshio [Department of Radiology, National Cardiovascular Center, Osaka (Japan); Kakuchi, Hiroyuki [Department of Internal Medicine, National Cardiovascular Center, Osaka (Japan); Eto, Tanenao [First Department of Internal Medicine, Miyazaki Medical College, Miyazaki (Japan)

    1997-05-01

    We studied 13 patients with dilated cardiomyopathy (DCM) and seven normal subjects. We obtained myocardial SPET images 15 min and 4 h after administration of {sup 123}I-MIBG (111 MBq). Studies were performed in the patients with DCM before and 1 and 3 months after the administration of metoprolol and in the normal subjects. We calculated the regional {sup 123}I-MIBG washout rate (r-WR) in the SPET image, and the global {sup 123}I-MIBG washout rate (g-WR) and heart-mediastinum activity ratio (H/M) using the anterior planar image. We classified patients into those showing a {>=}5% increase in LV ejection fraction (LVEF) at 3 months compared with LVEF values before the treatment (group I, n=7) and those showing a <5% increase in LVEF (group II, n=6). In normal subjects, the r-WR values in each of the anterior, lateral, septal and inferior segments were significantly lower than those in groups I and II. These values were 18%{+-}9%, 18%{+-}15%, 20%{+-}12% and 21%{+-}15%, respectively. This study demonstrated that with regional assessment {sup 123}I-MIBG SPET imaging can be used to predict the functional improvement of LVEF at 1 month of {beta}-blocker therapy in patients with DCM. (orig./VHE). With 4 figs., 1 tab.

  3. Oxaliplatin-induced acquired long QT syndrome with torsades de pointes and myocardial injury in a patient with dilated cardiomyopathy and rectal cancer

    Directory of Open Access Journals (Sweden)

    Rei-Yeuh Chang

    2013-08-01

    Full Text Available A 67-year-old woman presented with a history of dilated cardiomyopathy with congestive heart failure since 2003, who subsequently developed lower rectal cancer (adenocarcinoma with liver, bone, and lymph node metastasis. Abdominoperineal resection and hepatectomy were performed. The patient received two rounds of intravenous chemotherapy, including 12 and six courses of FOLFOX4 (5-fluorouracil, leucovorin, and oxaliplatin; 85 mg/m2 per cycle. She underwent a third round of intravenous FOLFOX4 because of tumor progression. During the 21st course of FOLFOX4 regimen, the patient developed ST segment depression in lead II and prolongation of QT interval with polymorphic ventricular tachycardia, torsades de pointes right after the start of oxaliplatin infusion. Immediate defibrillation and cardiopulmonary resuscitation were administered, and the patient regained spontaneous circulation and consciousness. Twelve-lead electrocardiogram showed ST segment elevation in III, aVF, and ST segment depression in V4–6 after resuscitation. To our knowledge, prolongation of QT interval with torsades de pointes and coronary spasm with myocardial injury that were stabilized in one patient following oxaliplatin infusion has not been reported. We present a patient with these rare complications.

  4. The Prognostic Value of Late Gadolinium-Enhanced Cardiac Magnetic Resonance Imaging in Nonischemic Dilated Cardiomyopathy: A Review and Meta-Analysis.

    Science.gov (United States)

    Becker, Marthe A J; Cornel, Jan H; van de Ven, Peter M; van Rossum, Albert C; Allaart, Cornelis P; Germans, Tjeerd

    2018-04-13

    This review and meta-analysis reviews the prognostic value of cardiac magnetic resonance (CMR) in nonischemic dilated cardiomyopathy (DCM). Late gadolinium-enhanced (LGE) CMR is a noninvasive method to determine the underlying cause of DCM and previous studies reported the prognostic value of the presence of LGE to identify patients at risk of major adverse cardiovascular events. PubMed was searched for studies describing the prognostic implication of LGE in patients with DCM for the specified endpoints cardiovascular mortality, major ventricular arrhythmic events including appropriate implantable cardioverter-defibrillator therapy, rehospitalization for heart failure, and left ventricular reverse remodeling. Data from 34 studies were included, with a total of 4,554 patients. Contrast enhancement was present in 44.8% of DCM patients. Patients with LGE had increased cardiovascular mortality (odds ratio [OR]: 3.40; 95% confidence interval [CI]: 2.04 to 5.67), ventricular arrhythmic events (OR: 4.52; 95% CI: 3.41 to 5.99), and rehospitalization for heart failure (OR: 2.66; 95% CI: 1.67 to 4.24) compared with those without LGE. Moreover, the absence of LGE predicted left ventricular reverse remodeling (OR: 0.15; 95% CI: 0.06 to 0.36). The presence of LGE on CMR substantially worsens prognosis for adverse cardiovascular events in DCM patients, and the absence indicates left ventricular reverse remodeling. Copyright © 2018 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

  5. The prognostic value of right ventricular long axis strain in non-ischaemic dilated cardiomyopathies using standard cardiac magnetic resonance imaging

    Energy Technology Data Exchange (ETDEWEB)

    Arenja, Nisha [University of Heidelberg, Department of Cardiology, Angiology and Pneumology, Heidelberg (Germany); Kantonsspital Olten, Department of Cardiology, Solothurner Spitaeler AG, Olten (Switzerland); Riffel, Johannes H.; Halder, Manuel; Djiokou, Charly N.; Fritz, Thomas; Andre, Florian; Siepen, Fabian aus dem; Zelniker, Thomas; Meder, Benjamin; Kayvanpour, Elham; Korosoglou, Grigorios [University of Heidelberg, Department of Cardiology, Angiology and Pneumology, Heidelberg (Germany); Katus, Hugo A. [University of Heidelberg, Department of Cardiology, Angiology and Pneumology, Heidelberg (Germany); DZHK (German Centre for Cardiovascular Research), partner site Heidelberg, Heidelberg (Germany); Buss, Sebastian J. [University of Heidelberg, Department of Cardiology, Angiology and Pneumology, Heidelberg (Germany); Das Radiologische Zentrum - Radiology Center, Sinsheim-Eberbach-Erbach-Walldorf-Heidelberg (Germany)

    2017-09-15

    To investigate the association of right ventricular long axis strain (RV-LAS), a parameter of longitudinal function, with outcome in patients with non-ischaemic dilated cardiomyopathy (NIDCM). In 441 patients with NIDCM, RV-LAS was analysed retrospectively by measuring the length between the epicardial border of the left ventricular apex and the middle of a line connecting the origins of the tricuspidal valve leaflets in end-diastole and end-systole on non-contrast standard cine sequences. The primary endpoint (cardiac death or heart transplantation) occurred in 41 patients, whereas 95 reached the combined endpoint (including cardiac decompensation and sustained ventricular arrhythmias) during a median follow-up of 4.2 years. Kaplan-Meier survival curves showed a poor outcome in patients with RV-LAS values below -10% (log-rank, p < 0.0001). In a risk stratification model RV-LAS improved prediction of outcome in addition to RV ejection fraction (RVEF) and presence of late gadolinium enhancement. Assessment of RV-LAS offered incremental information compared to clinical symptoms, biomarkers and RVEF. Even in the subgroup with normal RVEF (>45%, n = 213) reduced RV-LAS was still associated with poor outcome. Assessment of RV-LAS is an independent indicator of outcome in patients with NIDCM and offers incremental information beyond clinical and cardiac MR parameters. (orig.)

  6. Usefulness of microvolt T-wave alternans for prediction of ventricular tachyarrhythmic events in patients with dilated cardiomyopathy: results from a prospective observational study

    Science.gov (United States)

    Hohnloser, Stefan H.; Klingenheben, Thomas; Bloomfield, Daniel; Dabbous, Omar; Cohen, Richard J.

    2003-01-01

    OBJECTIVES: This study was designed to evaluate the ability of microvolt-level T-wave alternans (MTWA) to identify prospectively patients with idiopathic dilated cardiomyopathy (DCM) at risk of ventricular tachyarrhythmic events and to compare its predictive accuracy with that of conventional risk stratifiers. BACKGROUND: Patients with DCM are at increased risk of sudden death from ventricular tachyarrhythmias. At present, there are no established methods of assessing this risk. METHODS: A total of 137 patients with DCM underwent risk stratification through assessment of MTWA, left ventricular ejection fraction, baroreflex sensitivity (BRS), heart rate variability, presence of nonsustained ventricular tachycardia (VT), signal-averaged electrocardiogram, and presence of intraventricular conduction defect. The study end point was either sudden death, resuscitated ventricular fibrillation, or documented hemodynamically unstable VT. RESULTS: During an average follow-up of 14 +/- 6 months, MTWA and BRS were significant univariate predictors of ventricular tachyarrhythmic events (p wave alternans is a powerful independent predictor of ventricular tachyarrhythmic events in patients with DCM.

  7. Effects of carvedilol in heart failure due to dilated cardiomyopathy. Results of a double-blind randomized placebo-controlled study (CARIBE study

    Directory of Open Access Journals (Sweden)

    Paulo Roberto Chizzola

    2000-03-01

    Full Text Available OBJECTIVE: To assess the effects of carvedilol in patients with idiopathic dilated cardiomyopathy. METHODS: In a double-blind randomized placebo-controlled study, 30 patients (7 women with functional class II and III heart failure were assessed. Their ages ranged from 28 to 66 years (mean of 43±9 years, and their left ventricular ejection fraction varied from 8% to 35%. Carvedilol was added to the usual therapy of 20 patients; placebo was added to the usual therapy of 10 patients. The initial dose of carvedilol was 12.5 mg, which was increased weekly until it reached 75 mg/day, according to the patient's tolerance. Clinical assessment, electrocardiogram, echocardiogram, and radionuclide ventriculography were performed in the pretreatment phase, being repeated after 2 and 6 months of medication use. RESULTS: A reduction in heart rate (p=0.016 as well as an increase in left ventricular shortening fraction (p=0.02 and in left ventricular ejection fraction (p=0.017 occurred in the group using carvedilol as compared with that using placebo. CONCLUSION: Carvedilol added to the usual therapy for heart failure resulted in better heart function.

  8. Contrast-enhanced T1 mapping-based extracellular volume fraction independently predicts clinical outcome in patients with non-ischemic dilated cardiomyopathy: a prospective cohort study

    Energy Technology Data Exchange (ETDEWEB)

    Youn, Jong-Chan [Hallym University College of Medicine, Division of Cardiology, Dongtan Sacred Heart Hospital, Hwaseong (Korea, Republic of); Yonsei University College of Medicine, Division of Cardiology, Severance Cardiovascular Hospital, Seoul (Korea, Republic of); Hong, Yoo Jin; Lee, Hye-Jeong; Han, Kyunghwa; Suh, Young Joo; Hur, Jin; Kim, Young Jin; Choi, Byoung Wook [Yonsei University College of Medicine, Department of Radiology, Research Institute of Radiological Science, Severance Hospital, Seoul (Korea, Republic of); Shim, Chi Young; Hong, Geu-Ru; Kang, Seok-Min [Yonsei University College of Medicine, Division of Cardiology, Severance Cardiovascular Hospital, Seoul (Korea, Republic of)

    2017-09-15

    We aimed to evaluate the prognostic role of cardiac magnetic resonance imaging (CMR)-based extracellular volume fraction (ECV) in patients with non-ischemic dilated cardiomyopathy (NIDCM) and compare it with late gadolinium enhancement (LGE) parameters. This was a single-center, prospective, cohort study of 117 NIDCM patients (71 men, 51.9 ± 16.7 years) who underwent clinical 3.0-T CMR. Myocardial ECV and LGE were quantified on the left ventricular myocardium. The presence of midwall LGE was also detected. Nineteen healthy subjects served as controls. The primary end points were cardiovascular (CV) events defined by CV death, rehospitalization due to heart failure, and heart transplantation. During the follow-up period (median duration, 11.2 months; 25{sup th}-75{sup th} percentile, 7.8-21.9 months), the primary end points occurred in 19 patients (16.2%). The ECV (per 3% and 1% increase) was associated with a hazard ratio of 1.80 and 1.22 (95% confidence interval [CI], 1.48-2.20 and 1.14-1.30, respectively; p < 0.001) for the CV events. Multivariable analysis also indicated that ECV was an independent prognostic factor and had a higher prognostic value (Harrell's c statistic, 0.88) than LGE quantification values (0.77) or midwall LGE (0.80). CMR-based ECV independently predicts the clinical outcome in NIDCM patients. (orig.)

  9. The Efficacy of Endoscopic Papillary Balloon Dilation for Patients with Acute Biliary Pancreatitis

    Directory of Open Access Journals (Sweden)

    Wei-Chih Sun

    2015-01-01

    Full Text Available Background. No study investigated the efficacy and safety of endoscopic papillary balloon dilation (EPBD for the treatment of acute biliary pancreatitis (ABP. Method. We retrospectively reviewed the effects of EPBD on patients with ABP from February 2003 to December 2012. The general data, findings of image studies, details of the procedure, and outcomes after EPBD were analyzed. Result. Total 183 patients (male/female: 110/73 were enrolled. The mean age was 65.9 years. Among them, 155 patients had mild pancreatitis. The meantime from admission to EPBD was 3.3 days. Cholangiogram revealed filling defects inside the common bile duct (CBD in 149 patients. The mean dilating balloon size was 10.5 mm and mean duration of the dilating procedure was 4.3 minutes. Overall, 124 patients had gross stones retrieved from CBD. Four (2.2% adverse events and 2 (1.1% intraprocedure bleeding incidents but no procedure-related mortality were noted. Bilirubin and amylase levels significantly decreased after EPBD. On average, patients resumed oral intake within 1.4 days. The clinical parameters and outcomes were similar in patients with different severity of pancreatitis. Conclusion. EPBD can be effective and safe for the treatment of ABP, even in patients presenting with severe disease.

  10. Sex differences in cardiomyopathies

    NARCIS (Netherlands)

    Meyer, Sven; van der Meer, Peter; van Tintelen, J. Peter; van den Berg, Maarten P.

    Cardiomyopathies are a heterogeneous group of heart muscle diseases with a variety of specific phenotypes. According to the contemporary European Society of Cardiology classification, they are classified into hypertrophic (HCM), dilated (DCM), arrhythmogenic right ventricular (ARVC), restrictive

  11. Unexpected Death Due to Acute Gastric Dilatation and Gastric Necrosis in an 11-Year-Old Boy.

    Science.gov (United States)

    Kocaoglu, Canan; Akillioglu, Ishak; Gunduz, Metin; Sekmenli, Tamer

    2017-11-01

    This report is related to the unusual case of an 11-year-old boy presenting with acute gastric dilatation after the intake of excessive food and carbonated beverages after a 12-hour fasting who died of complications of acute reperfusion syndrome after decompression of the stomach with gastrotomy. An enormously distended stomach was encountered without volvulus and obstruction in the operation. Autopsy and histological findings revealed a severely distended stomach, the walls of which were notably thin and displayed transmural necrosis. The reported case demonstrated that enormous food and beverage intake may cause acute gastric dilatation and gastric necrosis, and subsequently, sudden death may develop in children. The subject reported here is the youngest child in literature dying from acute gastric dilatation and necrosis without any underlying disorders and exposed to autopsy.

  12. Systemic vascular function, measured with forearm flow mediated dilatation, in acute and stable cerebrovascular disease: a case-control study

    Directory of Open Access Journals (Sweden)

    Blacker David

    2010-10-01

    Full Text Available Abstract Background Acute ischaemic stroke is associated with alteration in systemic markers of vascular function. We measured forearm vascular function (using forearm flow mediated dilatation to clarify whether recent acute ischaemic stroke/TIA is associated with impaired systemic vascular function. Methods Prospective case control study enrolling 17 patients with recent acute ischaemic stroke/TIA and 17 sex matched controls with stroke more than two years previously. Forearm vascular function was measured using flow medicated dilatation (FMD. Results Flow mediated dilatation was 6.0 ± 1.1% in acute stroke/TIA patients and 4.7 ± 1.0% among control subjects (p = 0.18. The mean paired difference in FMD between subjects with recent acute stroke and controls was 1.25% (95% CI -0.65, 3.14; p = 0.18. Endothelium independent dilatation was measured in six pairs of participants and was similar in acute stroke/TIA patients (22.6 ± 4.3% and control subjects (19.1 ± 2.6%; p = 0.43. Conclusions Despite the small size of this study, these data indicate that recent acute stroke is not necessarily associated with a clinically important reduction in FMD.

  13. The LMNA mutation p.Arg321Ter associated with dilated cardiomyopathy leads to reduced expression and a skewed ratio of lamin A and lamin C proteins

    International Nuclear Information System (INIS)

    Al-Saaidi, Rasha; Rasmussen, Torsten B.; Palmfeldt, Johan; Nissen, Peter H.; Beqqali, Abdelaziz; Hansen, Jakob; Pinto, Yigal M.; Boesen, Thomas; Mogensen, Jens; Bross, Peter

    2013-01-01

    Dilated cardiomyopathy (DCM) is a disease of the heart muscle characterized by cardiac chamber enlargement and reduced systolic function of the left ventricle. Mutations in the LMNA gene represent the most frequent known genetic cause of DCM associated with disease of the conduction systems. The LMNA gene generates two major transcripts encoding the nuclear lamina major components lamin A and lamin C by alternative splicing. Both haploinsuffiency and dominant negative effects have been proposed as disease mechanism for premature termination codon (PTC) mutations in LMNA. These mechanisms however are still not clearly established. In this study, we used a representative LMNA nonsense mutation, p.Arg321Ter, to shed light on the molecular disease mechanisms. Cultured fibroblasts from three DCM patients carrying this mutation were analyzed. Quantitative reverse transcriptase PCR and sequencing of these PCR products indicated that transcripts from the mutant allele were degraded by the nonsense-mediated mRNA decay (NMD) mechanism. The fact that no truncated mutant protein was detectable in western blot (WB) analysis strengthens the notion that the mutant transcript is efficiently degraded. Furthermore, WB analysis showed that the expression of lamin C protein was reduced by the expected approximately 50%. Clearly decreased lamin A and lamin C levels were also observed by immunofluorescence microscopy analysis. However, results from both WB and nano-liquid chromatography/mass spectrometry demonstrated that the levels of lamin A protein were more reduced suggesting an effect on expression of lamin A from the wild type allele. PCR analysis of the ratio of lamin A to lamin C transcripts showed unchanged relative amounts of lamin A transcript suggesting that the effect on the wild type allele was operative at the protein level. Immunofluorescence microscopy analysis showed no abnormal nuclear morphology of patient fibroblast cells. Based on these data, we propose that

  14. The LMNA mutation p.Arg321Ter associated with dilated cardiomyopathy leads to reduced expression and a skewed ratio of lamin A and lamin C proteins

    Energy Technology Data Exchange (ETDEWEB)

    Al-Saaidi, Rasha [Research Unit for Molecular Medicine, Aarhus University and Aarhus University Hospital, Aarhus (Denmark); Rasmussen, Torsten B. [Department of Cardiology, Aarhus University Hospital, Aarhus (Denmark); Palmfeldt, Johan [Research Unit for Molecular Medicine, Aarhus University and Aarhus University Hospital, Aarhus (Denmark); Nissen, Peter H. [Department of Clinical Biochemistry, Aarhus University Hospital, Aarhus (Denmark); Beqqali, Abdelaziz [Heart Failure Research Center, Academic Medical Center, Amsterdam (Netherlands); Hansen, Jakob [Department of Forensic Medicine, Bioanalytical Unit, University of Aarhus (Denmark); Pinto, Yigal M. [Heart Failure Research Center, Academic Medical Center, Amsterdam (Netherlands); Boesen, Thomas [Department of Molecular Biology and Genetics, University of Aarhus (Denmark); Mogensen, Jens [Department of Cardiology, Odense University Hospital, Odense (Denmark); Bross, Peter, E-mail: peter.bross@ki.au.dk [Research Unit for Molecular Medicine, Aarhus University and Aarhus University Hospital, Aarhus (Denmark)

    2013-11-15

    Dilated cardiomyopathy (DCM) is a disease of the heart muscle characterized by cardiac chamber enlargement and reduced systolic function of the left ventricle. Mutations in the LMNA gene represent the most frequent known genetic cause of DCM associated with disease of the conduction systems. The LMNA gene generates two major transcripts encoding the nuclear lamina major components lamin A and lamin C by alternative splicing. Both haploinsuffiency and dominant negative effects have been proposed as disease mechanism for premature termination codon (PTC) mutations in LMNA. These mechanisms however are still not clearly established. In this study, we used a representative LMNA nonsense mutation, p.Arg321Ter, to shed light on the molecular disease mechanisms. Cultured fibroblasts from three DCM patients carrying this mutation were analyzed. Quantitative reverse transcriptase PCR and sequencing of these PCR products indicated that transcripts from the mutant allele were degraded by the nonsense-mediated mRNA decay (NMD) mechanism. The fact that no truncated mutant protein was detectable in western blot (WB) analysis strengthens the notion that the mutant transcript is efficiently degraded. Furthermore, WB analysis showed that the expression of lamin C protein was reduced by the expected approximately 50%. Clearly decreased lamin A and lamin C levels were also observed by immunofluorescence microscopy analysis. However, results from both WB and nano-liquid chromatography/mass spectrometry demonstrated that the levels of lamin A protein were more reduced suggesting an effect on expression of lamin A from the wild type allele. PCR analysis of the ratio of lamin A to lamin C transcripts showed unchanged relative amounts of lamin A transcript suggesting that the effect on the wild type allele was operative at the protein level. Immunofluorescence microscopy analysis showed no abnormal nuclear morphology of patient fibroblast cells. Based on these data, we propose that

  15. Mitochondrial cardiomyopathies

    Directory of Open Access Journals (Sweden)

    Ayman W. El-Hattab

    2016-07-01

    Full Text Available Mitochondria are found in all nucleated human cells and perform a variety of essential functions, including the generation of cellular energy. Mitochondria are under dual genome control. Only a small fraction of their proteins are encoded by mitochondrial DNA (mtDNA while more than 99% of them are encoded by nuclear DNA (nDNA. Mutations in mtDNA or mitochondria-related nDNA genes result in mitochondrial dysfunction leading to insufficient energy production required to meet the needs of various organs, particularly those with high energy requirements, including the central nervous system, skeletal and cardiac muscles, kidneys, liver, and endocrine system. Because cardiac muscles are one of the high energy demanding tissues, cardiac involvement occurs in mitochondrial diseases with cardiomyopathies being one of the most frequent cardiac manifestations found in these disorders. Cardiomyopathy is estimated to occur in 20-40% of children with mitochondrial diseases. Mitochondrial cardiomyopathies can vary in severity from asymptomatic status to severe manifestations including heart failure, arrhythmias, and sudden cardiac death. Hypertrophic cardiomyopathy is the most common type; however, mitochondrial cardiomyopathies might also present as dilated, restrictive, left ventricular noncompaction, and histiocytoid cardiomyopathies. Cardiomyopathies are frequent manifestations of mitochondrial diseases associated with defects in electron transport chain (ETC complexes subunits and their assembly factors, mitochondrial tRNAs, rRNAs, ribosomal proteins, and translation factors, mtDNA maintenance, and coenzyme Q10 synthesis. Other mitochondrial diseases with cardiomyopathies include Barth syndrome, Sengers syndrome, TMEM70-related mitochondrial complex V deficiency, and Friedreich ataxia.

  16. Relationship between evaluation by quantitative fatty acid myocardial scintigraphy and response to {beta}-blockade therapy in patients with dilated cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Ito, Tatsuo; Hoshida, Shiro; Nishino, Masami; Aoi, Toshiyuki; Egami, Yasuyuki; Takeda, Toshihiro; Kawabata, Masayoshi; Tanouchi, Jun; Yamada, Yoshio; Kamada, Takenobu [Div. of Cardiology, Osaka Rosai Hospital (Japan)

    2001-12-01

    Predicting the effect of {beta}-blockade therapy on the clinical outcome of patients with dilated cardiomyopathy (DCM) is difficult prior to the initiation of therapy. Myocardial fatty acid metabolism has been shown to be impaired in patients with DCM. We examined whether the extent of myocardial injury, as assessed by iodine-123 15-(p-iodophenyl)-3-R,S-methylpentadecanoic acid (BMIPP) myocardial scintigraphy, is related to the response of patients with DCM to {beta}-blockade therapy. Thirty-seven patients with DCM were examined using BMIPP myocardial scintigraphy before and after 6 months of treatment with metoprolol. Myocardial BMIPP uptake (%BM uptake) was estimated quantitatively as a percentage of the total injected count ratio. The left ventricular end-diastolic and end-systolic dimensions (LVDd, LVDs) and ejection fraction (LVEF) were also evaluated. The patients were divided into two groups according to their functional improvement (>10% elevation of LVEF) after 6 months of metoprolol therapy. Twenty-eight patients responded to the therapy, while nine did not. Prior to the therapy, no significant differences in LVDd, LVDs or LVEF were observed between the responders and non-responders. However, the %BM uptake was significantly lower in the non-responders than in the responders (1.0%{+-}0.2% vs 2.1%{+-}0.5%, P<0.001). The %BM uptake could be used to distinguish the responders from the non-responders with a sensitivity of 0.93 and a specificity of 1.00 at a threshold value of 1.4. After the metoprolol therapy, the %BM uptake improved significantly in the responders (2.5%{+-}0.5%, P<0.01) but did not change in the non-responders. These results indicate that myocardial BMIPP uptake could predict the response of DCM patients to {beta}-blockade therapy. (orig.)

  17. The significance of 201Tl/123I MIBG (metaiodobenzylguanidine) mismatched myocardial regions for predicting ventricular tachycardia in patients with idiopathic dilated cardiomyopathy

    International Nuclear Information System (INIS)

    Maeno, Masakazu; Ishida, Yoshio; Shimonagata, Tsuyoshi

    1993-01-01

    123 I-MIBG (MIBG) regional defects in myocardial regions with preserved 201 Tl (Tl) uptake have been observed in patients with idiopathic dilated cardiomyopathy (DCM). To evaluate whether the presence of Tl/MIBG mismatched regions is related to the occurrence of ventricular tachycardia (VT), we performed myocardial dual SPECT imaging with Tl (111 MBq) and MIBG (111 MBq) in 17 patients with DCM, 11 (Gp A) with and 6 (Gp B) without VT. Myocardial dual SPECT imaging was performed at 15 minutes after and 4 hours after the tracer injection. The regional tracer uptake was scored visually in 6 segments of the basal, middle, and apical short-axial images and in 2 apical segments of the midventricular vertical long-axial image by a four-point scoring system (0=normal, 1=moderate, 2=severe and 3=complete defect). Then, the severity of tracer maldistributions was assessed by the difference between total defect scores (TDSs) of Tl and MIBG (ΔTDS). TDS was not different between Gps A and B in both Tl and MIBG images. However, ΔTDS was larger in Gp A than in Gp B (13.5±6.5 vs. 5.8±3.0, p<0.05). Also, the number of segments with the mismatched tracer uptake was larger in Gp A than in Gp B (12.5±3.0 vs. 8.3±1.5, p<0.01). In the electrophysiologic study, we found that the fractionated area corresponded to the mismatched region in 3 of 5 patients in Gp A. These results suggest that regional sympathetic denervation is a possible factor which provocates VT, and myocardial dual SPECT imaging with Tl and MIBG is a useful method for predicting VT in patients with DCM. (author)

  18. Mitochondrial-related gene expression profiles suggest an important role of PGC-1alpha in the compensatory mechanism of endemic dilated cardiomyopathy

    International Nuclear Information System (INIS)

    He, Shu-Lan; Tan, Wu-Hong; Zhang, Zeng-Tie; Zhang, Feng; Qu, Cheng-Juan; Lei, Yan-Xia; Zhu, Yan-He; Yu, Han-Jie; Xiang, You-Zhang

    2013-01-01

    Keshan disease (KD) is an endemic dilated cardiomyopathy with unclear etiology. In this study, we compared mitochondrial-related gene expression profiles of peripheral blood mononuclear cells (PBMCs) derived from 16 KD patients and 16 normal controls in KD areas. Total RNA was isolated, amplified, labeled and hybridized to Agilent human 4×44k whole genome microarrays. Mitochondrial-related genes were screened out by the Third-Generation Human Mitochondria-Focused cDNA Microarray (hMitChip3). Quantitative real-time PCR, immunohistochemical and biochemical parameters related mitochondrial metabolism were conducted to validate our microarray results. In KD samples, 34 up-regulated genes (ratios≥2.0) were detected by significance analysis of microarrays and ingenuity systems pathway analysis (IPA). The highest ranked molecular and cellular functions of the differentially regulated genes were closely related to amino acid metabolism, free radical scavenging, carbohydrate metabolism, and energy production. Using IPA, 40 significant pathways and four significant networks, involved mainly in apoptosis, mitochondrion dysfunction, and nuclear receptor signaling were identified. Based on our results, we suggest that PGC-1alpha regulated energy metabolism and anti-apoptosis might play an important role in the compensatory mechanism of KD. Our results may lead to the identification of potential diagnostic biomarkers for KD in PBMCs, and may help to understand the pathogenesis of KD. Highlights: • Thirty-four up-regulated genes were detected in KD versus health controls. • Forty pathways and four networks were detected in KD. • PGC-1alpha regulated energy metabolism and anti-apoptosis in KD

  19. Efficacy of Pimobendan in the Prevention of Congestive Heart Failure or Sudden Death in Doberman Pinschers with Preclinical Dilated Cardiomyopathy (The PROTECT Study)

    Science.gov (United States)

    Summerfield, NJ; Boswood, A; O'Grady, MR; Gordon, SG; Dukes-McEwan, J; Oyama, MA; Smith, S; Patteson, M; French, AT; Culshaw, GJ; Braz-Ruivo, L; Estrada, A; O'Sullivan, ML; Loureiro, J; Willis, R; Watson, P

    2012-01-01

    Background The benefit of pimobendan in delaying the progression of preclinical dilated cardiomyopathy (DCM) in Dobermans is not reported. Hypothesis That chronic oral administration of pimobendan to Dobermans with preclinical DCM will delay the onset of CHF or sudden death and improve survival. Animals Seventy-six client-owned Dobermans recruited at 10 centers in the UK and North America. Methods The trial was a randomized, blinded, placebo-controlled, parallel group multicenter study. Dogs were allocated in a 1:1 ratio to receive pimobendan (Vetmedin capsules) or visually identical placebo. The composite primary endpoint was prospectively defined as either onset of CHF or sudden death. Time to death from all causes was a secondary endpoint. Results The proportion of dogs reaching the primary endpoint was not significantly different between groups (P = .1). The median time to the primary endpoint (onset of CHF or sudden death) was significantly longer in the pimobendan (718 days, IQR 441–1152 days) versus the placebo group (441 days, IQR 151–641 days) (log-rank P = 0.0088). The median survival time was significantly longer in the pimobendan (623 days, IQR 491–1531 days) versus the placebo group (466 days, IQR 236–710 days) (log-rank P = .034). Conclusion and Clinical Importance The administration of pimobendan to Dobermans with preclinical DCM prolongs the time to the onset of clinical signs and extends survival. Treatment of dogs in the preclinical phase of this common cardiovascular disorder with pimobendan can lead to improved outcome. PMID:23078651

  20. Evaluation of left ventricular function and volume in patients with dilated cardiomyopathy: Gated myocardial single-photon emission tomography (SPECT) versus echocardiography

    International Nuclear Information System (INIS)

    Berk, Fatma; Isgoren, S.; Demir, H.; Kozdag, G.; Ural, D.; Komsuoglu, B.

    2005-01-01

    Left ventricular function, volumes and regional wall motion provide valuable diagnostic information and are of long-term prognostic importance in patients with dilated cardiomyopathy (DCM). This study was designed to compare the effectiveness of 2D-echocardiography and gated single-photon emission tomography (SPECT) for evaluation of these parameters in patients with DCM. Gated SPECT and 2D-echocardiography were performed in 33 patients having DCM. Gated SPECT data, including left ventricular ejection fraction (LVEF), were processed using an automated algorithm. Standard technique was used for 2D-echocardiography. Regional wall motion was evaluated using both modalities and was scored by two independent observers using a 16-sement model with a 5-point scoring system. The overall agreement between the two imaging modalities for the assessment of regional wall motion was 56% (298/528 segments). With gated SPECT, LEVF, end-diastolic volume (EDV), and end-diastolic volume (EDV), and end-systolic volume (ESV) were 27+-9%, 217+-73mL, respectively, and 30.8%, 195+-58mL and, 137+-48 mL with echocardiography. The correlation between gated SPECT and 2-D-echocardiography was good (r=0.76, P<0.01) for the assessment of LVEF. The correlation for EDV and ESV were also good, but with wider limits of agreement (r=0.72, P<0.01 and r=0.73, P<0.01, respectively) and significantly higher values were obtained with gated SPECT (P<0.01). Gated SPECT and 2D-echocardiography correlate well for the assessment of LV function and LV volumes. Like 2D-echocardiography, gated SPECT provides reliable information about LV function and dimension with the additional advantage of perfusion data. (author)

  1. Relationship between myocardial extracellular space expansion estimated with post-contrast T1 mapping MRI and left ventricular remodeling and neurohormonal activation in patients with dilated cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Yoon, Ji Hyun; Son, Jung Woo; Chung, Hye Moon [Cardiology Division, Dept. of Internal Medicine, Yonsei University College of Medicine, Seoul (Korea, Republic of); and others

    2015-10-15

    Post-contrast T1 values are closely related to the degree of myocardial extracellular space expansion. We determined the relationship between post-contrast T1 values and left ventricular (LV) diastolic function, LV remodeling, and neurohormonal activation in patients with dilated cardiomyopathy (DCM). Fifty-nine patients with DCM (mean age, 55 ± 15 years; 41 males and 18 females) who underwent both 1.5T magnetic resonance imaging and echocardiography were enrolled. The post-contrast 10-minute T1 value was generated from inversion time scout images obtained using the Look-Locker inversion recovery sequence and a curve-fitting algorithm. The T1 sample volume was obtained from three interventricular septal points, and the mean T1 value was used for analysis. The N-Terminal pro-B-type natriuretic peptide (NT-proBNP) level was measured in 40 patients. The mean LV ejection fraction was 24 ± 9% and the post-T1 value was 254.5 ± 46.4 ms. The post-contrast T1 value was significantly correlated with systolic longitudinal septal velocity (s'), peak late diastolic velocity of the mitral annulus (a'), the diastolic elastance index (Ed, [E/e']/stroke volume), LV mass/volume ratio, LV end-diastolic wall stress, and LV end-systolic wall stress. In a multivariate analysis without NT-proBNP, T1 values were independently correlated with Ed (β = -0.351, p = 0.016) and the LV mass/volume ratio (β = 0.495, p = 0.001). When NT-proBNP was used in the analysis, NT-proBNP was independently correlated with the T1 values (β = -0.339, p = 0.017). Post-contrast T1 is closely related to LV remodeling, diastolic function, and neurohormonal activation in patients with DCM.

  2. Anti-KCNQ1 K⁺ channel autoantibodies increase IKs current and are associated with QT interval shortening in dilated cardiomyopathy.

    Science.gov (United States)

    Li, Jin; Seyler, Claudia; Wiedmann, Felix; Schmidt, Constanze; Schweizer, Patrick A; Becker, Rüdiger; Katus, Hugo A; Thomas, Dierk

    2013-06-01

    Autoimmune-associated proarrhythmia in dilated cardiomyopathy (DCM) is poorly understood. Given the significance of KCNQ1 potassium channels in heart rhythm disorders, we hypothesized that circulating anti-KCNQ1 autoantibodies directly modulate cardiac electrophysiology in DCM patients. The purpose of this pilot study was to characterize ion channel autoantibodies in DCM targeting the cardiac repolarizing K(+) current, IKs, and the underlying KCNQ1 potassium channel. One hundred and fifty DCM patients were screened for anti-KCNQ1 autoantibodies using an enzyme-linked immunosorbent assay. Autoantibodies targeting the extracellular pore domain of the KCNQ1 channel were detected in 6% of study patients. Seropositive individuals exhibited significantly shorter corrected QT intervals when compared with seronegative patients (371 ± 39.9 ms vs. 408 ± 47.9 ms; P = 0.036). There was no difference in clinical severity of heart failure between groups. The functional significance of anti-KCNQ1 antibodies was determined in human embryonic kidney 293 cells expressing KCNQ1/KCNE1 using the whole-cell patch clamp technique. IKs recordings demonstrated a 2.7-fold increase in mean current density on exposure to patients' sera containing anti-KCNQ1 antibodies in contrast to seronegative controls (8.74 ± 1.44 pA/pF vs. 3.26 ± 0.36 pA/pF; P = 0.003). IKs enhancement was not associated with increased KCNQ1 protein levels or altered cell surface expression of the channel. Anti-KCNQ1 autoantibodies found in a subgroup of DCM patients are associated with QT interval shortening and increased IKs current.

  3. Orage rythmique chez un patient porteur d’une cardiomyopathie dilatée et un défibrillateur automatique implantable (DAI)

    Science.gov (United States)

    Ikama, Stéphane Méo; Makani, Jospin; Ellenga-Mbolla, Bertrand; Ondze-Kafata, Louis Igor; Gombet, Thierry Raoul; Kimbally-Kaky, Gisèle

    2017-01-01

    Les arythmies ventriculaires graves sont fréquentes au cours de l’insuffisance cardiaque, mettant en jeu le pronostic vital du fait du risque accru de mort subite. Leur prise en charge efficace reste limitée en Afrique Subsaharienne, du fait des moyens limités ou non disponibles comme le défibrillateur automatique implantable (DAI). Nous rapportons l’observation d’un patient de 56 ans, porteur d’une cardiomyopathie dilatée non ischémique à fraction d’éjection du ventricule gauche (FEVG) très abaissée, et qui a bénéficié en 2012 de l’implantation d’un DAI en prévention primaire de mort subite pour des arythmies ventriculaires. Le traitement d’entretien associait un diurétique, un IEC, et un anti-vitamine K. Le patient a présenté au mois de novembre 2014 des épisodes itératifs de décharges électriques délivrées par le DAI, sans sensation de palpitations suggestives d’épisodes d’arythmies. L’examen clinique est pauvre, en particulier pas de signes d’insuffisance cardiaque. L’interrogation du DAI a objectivé de nombreux épisodes de tachycardie et fibrillation ventriculaires ayant justifié le traitement par ATP ou par chocs de 15 joules. Le patient est mis sous amiodarone et bêtabloquant. L’évolution a été favorable avec un recul de trois mois, marquée par la reprise d’une vie normale, sans nouvel épisode de choc. Les anti-arythmiques gardent une importance capitale en cas d’arythmies ventriculaires graves, même en présence d’un DAI. PMID:28761607

  4. Evaluation of therapy for dilated cardiomyopathy with heart failure by iodine-123 metaiodobenzyl-guanidine imaging. Comparison with heart rate variability power spectral analysis

    International Nuclear Information System (INIS)

    Li, Shou-lin; Ikeda, Jun; Takita, Tamotsu; Sekiguchi, Yohei; Demachi, Jun; Chikama, Hisao; Goto, Atsushi; Shirato, Kunio

    1998-01-01

    The relationship between the myocardial uptake of iodine-123 metaiodobenzylguanidine ( 123 I-MIBG) and heart rate variability parameters has not been determined. This study determined the relationship between the change in myocardial uptake of 123 I-MIBG and improvement in left ventricular function after treatment, to determine the usefulness of 123 I-MIBG imaging to assess the effect of therapy on heart failure due to dilated cardiomyopathy (DCM). 123 I-MIBG imaging and power spectral analysis of heart rate variability were performed before and after treatment in 17 patients with heart failure due to DCM. The following parameters were compared before and after treatment: New York Heart Association (NYHA) functional class, radiographic cardiothoracic ratio (CTR), blood pressure, echocardiographic data (left ventricular end-systolic (LVDs) and end-diastolic (LVDd) diameters, left ventricular ejection fraction (LVEF)), plasma concentrations of norepinephrine and epinephrine, heart rate variability power spectral analysis data (mean low frequency (MLF) and high frequency power (MHF)) and the myocardium to mediastinum activity ratio (MYO/M) obtained in early and late images, and washout rate calculated by anterior planar imaging of 123 I-MIBG. The NYHA functional class, LVEF, LVDs, CTR, MLF and MHF improved after treatment. Early MYO/M and late MYO/M improved after treatment. The rate of increase in late MYO/M was positively correlated with the rate of improvement of LVEF after treatment. Furthermore, the late MYO/M was negatively correlated with MLF. Washout rate revealed no correlation with hemodynamic parameters. These findings suggest that late MYO/M is more useful than washout rate to assess the effect of treatment on heart failure due to DCM. Furthermore, the 123 I-MIBG imaging and heart rate variability parameters are useful to assess the autonomic tone in DCM with heart failure. (author)

  5. A predictive model for canine dilated cardiomyopathy—a meta-analysis of Doberman Pinscher data

    Directory of Open Access Journals (Sweden)

    Siobhan Simpson

    2015-03-01

    Full Text Available Dilated cardiomyopathy is a prevalent and often fatal disease in humans and dogs. Indeed dilated cardiomyopathy is the third most common form of cardiac disease in humans, reported to affect approximately 36 individuals per 100,000 individuals. In dogs, dilated cardiomyopathy is the second most common cardiac disease and is most prevalent in the Irish Wolfhound, Doberman Pinscher and Newfoundland breeds. Dilated cardiomyopathy is characterised by ventricular chamber enlargement and systolic dysfunction which often leads to congestive heart failure. Although multiple human loci have been implicated in the pathogenesis of dilated cardiomyopathy, the identified variants are typically associated with rare monogenic forms of dilated cardiomyopathy. The potential for multigenic interactions contributing to human dilated cardiomyopathy remains poorly understood. Consistent with this, several known human dilated cardiomyopathy loci have been excluded as common causes of canine dilated cardiomyopathy, although canine dilated cardiomyopathy resembles the human disease functionally. This suggests additional genetic factors contribute to the dilated cardiomyopathy phenotype.This study represents a meta-analysis of available canine dilated cardiomyopathy genetic datasets with the goal of determining potential multigenic interactions relating the sex chromosome genotype (XX vs. XY with known dilated cardiomyopathy associated loci on chromosome 5 and the PDK4 gene in the incidence and progression of dilated cardiomyopathy. The results show an interaction between known canine dilated cardiomyopathy loci and an unknown X-linked locus. Our study is the first to test a multigenic contribution to dilated cardiomyopathy and suggest a genetic basis for the known sex-disparity in dilated cardiomyopathy outcomes.

  6. MR imaging in cardiomyopathies

    International Nuclear Information System (INIS)

    Miller, S.; Riessen, R.

    2005-01-01

    According to the WHO classification, cardiomyopathies are a group of diseases which are associated with myocardial dysfunction and can be classified either as primary or secondary cardiomyopathies. Genetic disorders have been identified in certain primary cardiomyopathies, however often the etiology remains unknown. The term ''secondary cardiomyopathy'' is used to specify diseases with the clinical indications of a cardiomyopathy, but can be attributed to a certain pathophysiological mechanism such as exposure to toxic substances, metabolic syndromes or systemic diseases. Based on morphological and functional criteria, primary cardiomyopathies are divided into dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), arrhythmogenic right ventricular cardiomyopathy (ARVC) and restrictive cardiomyopathy (RCM). During the last two decades MR imaging has emerged to a well established diagnostic tool for the understanding and treatment of cardiomyopathies. Morphological and functional information can be achieved with a high level of accuracy and reproducibility. Tissue alteration of the myocardium can be detected assessing regional contrast enhancement, T1- and T2-signal intensities and chemical shift phenomena. This article describes characteristic aspects of MR imaging for the diagnosis of primary and secondary cardiomyopathies. (orig.)

  7. 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.

  8. Effects of acute exercise on flow-mediated dilatation in healthy humans.

    Science.gov (United States)

    Dawson, Ellen A; Green, Daniel J; Cable, N Timothy; Thijssen, Dick H J

    2013-12-01

    Although the effects of exercise training on vascular function have been well studied, less is known about the effects of acute exercise bouts. This synthesis summarizes and integrates knowledge derived from papers relating acute impacts of exercise on artery function, specifically endothelial function assessed by flow-mediated dilatation (FMD). We propose that an immediate decrease in FMD ("nadir") occurs soon after exercise cessation and that this is followed by a (supra)normalization response. The magnitude of the nadir and (supra)normalization and duration of this biphasic pattern of response appears to be influenced by numerous factors, including the nature of the exercise stimulus (e.g., type, duration, intensity), the subject population (e.g., trained vs. untrained), and various methodological factors. The impact of these factors on the biphasic pattern are most likely mediated through stimuli that underpin altered FMD postexercise, including shear and oxidative stress, changes in arterial diameter, and antioxidant status. We propose that a combination of these stimuli act synergistically to balance the vasomotor responses postexercise. Finally, we discuss the potential (clinical) relevance of the biphasic response after acute exercise, as the immediate nadir may represent an essential response for subsequent training-induced adaptations but may also represent a transient period of increased cardiovascular risk leading to the "exercise paradox."

  9. Reduction of myocardial blood flow reserve in idiopathic dilated cardiomyopathy without overt heart failure and its relation with functional indices: an echo-Doppler and positron emission tomography study.

    Science.gov (United States)

    Morales, Maria-Aurora; Neglia, Danilo; L'Abbate, Antonio

    2008-08-01

    Myocardial blood flow during pharmacological vasodilatation is depressed in patients with idiopathic dilated cardiomyopathy even the in absence of overt heart failure; the extent of myocardial blood flow abnormalities is not predictable by left ventricular ejection fraction (LVEF) and diastolic dimensions. To assess whether myocardial blood flow impairment in idiopathic dilated cardiomyopathy without overt heart failure can be related to Doppler-derived dP/dt and to echocardiographically determined left ventricular end systolic stress - which is linked to myocardial blood flow reserve in advanced disease. Twenty-six patients, New York Heart Association Class I-II, (LVEF 37.4 +/- 1.4%, left ventricular diastolic dimensions 62.6 +/- 0.9 mm) underwent resting/dipyridamole [13N]NH3 flow positron emission tomography and an ultrasonic study. Regional myocardial blood flow values (ml/min per g) were computed from positron emission tomography data in 13 left ventricular (LV) myocardial regions and averaged to provide mean myocardial blood flow and myocardial blood flow reserve, defined as dipyridamole/resting mean myocardial blood flow ratio. Resting myocardial blood flow was 0.686 +/- 0.045, dipyridamole myocardial blood flow 1.39 +/- 0.15 and myocardial blood flow reserve 2.12 +/- 0.2, lower than in controls (P < 0.01). The ratio dP/dt was directly related to dipyridamole myocardial blood flow and myocardial blood flow reserve (r = 0.552 and 0.703, P < 0.005 and P < 0.0001); no relation was found between myocardial blood flow and LVEF left ventricular diastolic dimensions, and left ventricular end systolic stress. In idiopathic dilated cardiomyopathy patients without overt heart failure, the extent of myocardial blood flow reserve impairment is related to dP/dt but not to more classical indices of left ventricular function.

  10. Is it possible to differentiate between Takotsubo cardiomyopathy and acute anterior ST-elevation myocardial infarction?

    DEFF Research Database (Denmark)

    Vervaat, Fabienne E; Christensen, Thomas E; Smeijers, Loes

    2015-01-01

    INTRODUCTION: Several studies have investigated the ability of the twelve-lead electrocardiogram (ECG) to reliably distinguish Takotsubo cardiomyopathy (TC) from an acute anterior ST-segment elevation myocardial infarction (STEMI). In these studies, only ECG changes were required - ST-segment dev...... insufficient to reliably distinguish patients with TC from patients with an acute anterior STEMI. To definitely exclude the diagnosis of an acute anterior STEMI coronary angiography, which remains the gold standard, will need to be performed.......-segment deviation and/or T-wave inversion - in TC whereas in acute anterior STEMI, ECGs had to meet STEMI criteria. In the majority of these studies, patients of both genders were used even though TC predominantly occurs in women. The aim of this study is to see whether TC can be distinguished from acute anterior......VR+ST-segment elevation ≤1mm in lead V1, (2) frontal plane ST-vector and (3) mean amplitude of ST-segment deviation in each lead. RESULTS: The existing ECG criterion was less accurate (76%) than in the original study (95%), with a large difference in sensitivity (26% vs. 91%). Only a frontal plane ST-vector of 60° could...

  11. FasL expression in cardiomyocytes activates the ERK1/2 pathway, leading to dilated cardiomyopathy and advanced heart failure.

    Science.gov (United States)

    Huby, Anne-Cecile; Turdi, Subat; James, Jeanne; Towbin, Jeffrey A; Purevjav, Enkhsaikhan

    2016-02-01

    Increase in the apoptotic molecule Fas ligand (FasL) in serum and cardiomyocytes has been shown to be associated with progressive dilated cardiomyopathy (DCM) and congestive heart failure (CHF) in humans. However, the underlying mechanism(s) of FasL-related deterioration of heart function remain obscure. The aim of the present study is to determine roles of myocardial FasL in the activation of alternative pathways such as extracellular-signal-regulated kinase 1/2 (ERK1/2), inflammation or fibrosis and to identify effective treatments of progressive DCM and advanced CHF. Transgenic mice with cardiomyocyte-specific overexpression of FasL were investigated and treated with an ERK1/2 inhibitor (U-0126), losartan (los), prednisolone (pred) or placebo. Morpho-histological and molecular studies were subsequently performed. FasL mice showed significantly higher mortality compared with wild-type (WT) littermates due to DCM and advanced CHF. Prominent perivascular and interstitial fibrosis, increased interleukin secretion and diffuse CD3-positive cell infiltration were evident in FasL hearts. Up-regulation of the short form of Fas-associated death domain (FADD)-like interleukin 1β-converting enzyme (FLICE) inhibitory protein (s-FLIP), RIP (receptor-interacting protein) and ERK1/2 and down-regulation of transforming growth factor beta 1 (TGFβ1) and nuclear factor-κB (NF-κB) was determined in the myocardium, whereas expression of ERK1/2, periostin (Postn) and osteopontin increased in cardiac fibroblasts. U-0126 and los increased CHF survival by 75% compared with pred and placebo groups. U-0126 had both anti-fibrotic and anti-apoptotic effects, whereas los reduced fibrosis only. Myocardial FasL expression in mice activates differential robust fibrotic, apoptotic and inflammatory responses via ERK1/2 in cardiomyocytes and cardiac fibroblasts inducing DCM and CHF. Blocking the ERK1/2 pathway prevented progression of FasL-induced DCM and CHF by reducing fibrosis, inflammation

  12. Temporal change of myocardial tissue character is associated with left ventricular reverse remodeling in patients with dilated cardiomyopathy: A cardiovascular magnetic resonance study.

    Science.gov (United States)

    Nabeta, Takeru; Inomata, Takayuki; Fujita, Teppei; Iida, Yuichiro; Ikeda, Yuki; Sato, Takanori; Ishii, Shunsuke; Maekawa, Emi; Mizutani, Tomohiro; Naruke, Takashi; Koitabashi, Toshimi; Inoue, Yusuke; Ako, Junya

    2017-08-01

    Prognostic significance of temporal change in myocardial tissue characterization by cardiovascular magnetic resonance (CMR) has not been elucidated in patients with non-ischemic dilated cardiomyopathy (DCM). Sixty-eight patients with newly-diagnosed DCM who underwent CMR including late gadolinium enhancement (LGE) both at baseline and during follow-up period were enrolled. LGE score was defined by a signal intensity of ≥5 standard deviations above the remote reference myocardium mean. Left ventricular reverse remodeling (LVRR) defined as a LV ejection fraction increase of ≥10% and a decrease in indexed LV end-diastolic diameter of ≥10% compared to those at baseline was detected in 38% of the patients. There was no significant difference in LGE score between baseline and follow-up (5.8% vs. 7.3%; p=0.38). The change in LGE area (delta-LGE) was significantly lower in patients with LVRR than those without (-0.5%±3.4% vs. 3.0±7.4%; p=0.02). On the other hand, T2 ratio during the follow-up significantly reduced (1.95±0.48 vs. 1.67±0.56; p<0.01); however, there was no significant difference in the change in T2 ratio between patients with LVRR and those without (-0.29±0.73 vs. -0.27±0.66; p=0.88). Multivariate logistic analysis indicated that baseline LGE score [odds ratio; 0.78; 95% confidence interval (CI) 0.66 to 0.90; p<0.01] together with delta-LGE (odds ratio; 0.77; 95% CI 0.61 to 0.92; p=0.01) were independently associated with subsequent LVRR (p<0.01). The temporal change of LGE-CMR score during the clinical course was significantly correlated with following LVRR. Copyright © 2016 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.

  13. Prediction of left ventricular dilatation with thallium-201 SPET imaging after primary angioplasty in patients with acute myocardial infarction

    Energy Technology Data Exchange (ETDEWEB)

    Choi, Joon Young; Moon, Dae Hyuk; Shin, Jung Woo; Lee, Hee Kyung [Department of Nuclear Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul (Korea); Lee, Cheol Whan; Park, Seong-Wook; Hong, Myeong-Ki; Song, Jae-Kwan; Park, Seung-Jung [Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul (Korea)

    2002-06-01

    Progressive ventricular dilatation is an important prognostic factor in patients with acute myocardial infarction. We evaluated clinical, angiographic, echocardiographic and thallium-201 single-photon emission tomography (SPET) imaging variables predictive of the change in left ventricular volume during a 7-month follow-up period after primary angioplasty in patients with acute myocardial infarction. Thirty-six patients with first acute myocardial infarction treated with primary angioplasty within 12 h of onset underwent {sup 201}Tl SPET imaging (5.8{+-}2.1 days after angioplasty). Changes in left ventricular volume were assessed over the 7-month period. The left ventricle dilated significantly after angioplasty (P<0.001). Multivariate analysis revealed that the number of segments with {sup 201}Tl uptake <40% of peak activity was a single independent predictor of increase in end-diastolic volume index between 1 week and 7 months (R{sup 2}=0.41, P< 0.001). The presence of two or more segments with {sup 201}Tl uptake <40% predicted an increase in end-diastolic volume index of {>=}6 ml/m{sup 2} with positive and negative predictive values of 85% (17/20) and 75% (12/16), respectively. It is concluded that, following primary angioplasty in patients with acute myocardial infarction, the extent of myocardial infarction assessed by {sup 201}Tl SPET can identify those who will develop ventricular dilatation during the subsequent 7 months. (orig.)

  14. Co segregation of the m.1555A>G mutation in the MT-RNR1 gene and mutations in MT-ATP6 gene in a family with dilated mitochondrial cardiomyopathy and hearing loss: A whole mitochondrial genome screening.

    Science.gov (United States)

    Alila-Fersi, Olfa; Chamkha, Imen; Majdoub, Imen; Gargouri, Lamia; Mkaouar-Rebai, Emna; Tabebi, Mouna; Tlili, Abdelaziz; Keskes, Leila; Mahfoudh, Abdelmajid; Fakhfakh, Faiza

    2017-02-26

    Mitochondrial disease refers to a heterogeneous group of disorders resulting in defective cellular energy production due to dysfunction of the mitochondrial respiratory chain, which is responsible for the generation of most cellular energy. Because cardiac muscles are one of the high energy demanding tissues, mitochondrial cardiomyopathies is one of the most frequent mitochondria disorders. Mitochondrial cardiomyopathy has been associated with several point mutations of mtDNA in both genes encoded mitochondrial proteins and mitochondrial tRNA and rRNA. We reported here the first description of mutations in MT-ATP6 gene in two patients with clinical features of dilated mitochondrial cardiomyopathy. The mutational analysis of the whole mitochondrial DNA revealed the presence of m.1555A>G mutation in MT-RNR1 gene associated to the m.8527A>G (p.M>V) and the m.8392C>T (p.136P>S) variations in the mitochondrial MT-ATP6 gene in patient1 and his family members with variable phenotype including hearing impairment. The second patient with isolated mitochondrial cardiomyopathy presented the m.8605C>T (p.27P>S) mutation in the MT-ATP6 gene. The three mutations p.M1V, p.P27S and p.P136S detected in MT-ATP6 affected well conserved residues of the mitochondrial protein ATPase 6. In addition, the substitution of proline residue at position 27 and 136 effect hydrophobicity and structure flexibility conformation of the protein. Copyright © 2017 Elsevier Inc. All rights reserved.

  15. A Systematic Review of Phenotypic Features Associated With Cardiac Troponin I Mutations in Hereditary Cardiomyopathies

    DEFF Research Database (Denmark)

    Mogensen, Jens; Hey, Thomas; Lambrecht, Sascha

    2015-01-01

    BACKGROUND: Genetic investigations have established that mutations in proteins of the contractile unit of the myocardium, known as the sarcomere, may be associated with hypertrophic cardiomyopathy (HCM), restrictive cardiomyopathy (RCM), and dilated cardiomyopathy (DCM). It has become clinical...

  16. Failure of frank-starling mechanism detected on radionuclide ambulatory monitoring (C-vest II) is associated with severe prognosis in ischemic dilatative cardiomyopathy

    International Nuclear Information System (INIS)

    Carboni, G.P.

    2004-01-01

    Background: Global perfusion scoring when combined to measures of left ventricular (LV) ejection fraction (EF), LV volumes and LV diastolic function during daily life activities could explain the mechanisms for severe prognosis IN ISCHEMIC DILATATIVE CARDIOMYOPATHY (ICMP). Methods: After a routine Tl-gated SPECT, 13 consecutive pts with ICMP in whom the indication for revascularization procedures had been excluded, mean age of 68 +-8 years, with < 30% LV EF and an abnormal exercise defect (ex AD) of an extent of 27+-17% pixels with reversibility (R) of 13+-25% pixels on polar maps were carefully selected. After 1 week time, global LV function was then continuously monitored by radionuclide ambulatory monitoring (VEST) and rest basal values were immediately validated against multigated blood pool analysis (MUGA). VEST recordings lasted for an average time of 242+-126 min. Results: When EFs measured on MUGA and VEST were compared there was any significant difference (30+-9% vs 27+-11%, p=ns),while Bland-Altman plotting revealed a mean difference /average of 0.09% and a SD difference of 0.2%. When on VEST, values at rest such as heart rate (HR), EFs, end-diastolic volumes (EDVs), end-systolic volumes (ESVs) and PFR were compared with the same values measured during stair climbing (SC) there was any difference in EDVs (104 +-5 mlvs 103+- 19 ml,p = ns) but not for HR (86+-12 bpmvs 102+-15 bpm,p < 0.05), ESVs (73+-11% vs 62+-17%, p < 0.04), EFs ( 30+-9% vs 38+-13%, p < 0.05) and PFR values (1.3+-0.3 edv/s vs 2.0+-0.6 edv/s, p < 0.02), respectively .A ratio of 38% (5/13) severe cardiovascular events (1 death , 1 stroke and 3 re-hospitalizations for heart failure ) was observed in a 4+-1 months follow-up.In a multiple regression model were then considered on VEST 10 variables (F-value = 217, significance of the model, p< 0.05): age, EFs, EDVs, ESVs and PFR values at rest, EDVs, ESVs and PFR values during SC, extent of ex AD and extent of R of ex AD.on T1-SPECT. Of these

  17. Sex impacts the flow-mediated dilation response to acute aerobic exercise in older adults.

    Science.gov (United States)

    Yoo, Jeung-Ki; Pinto, Michelle M; Kim, Han-Kyul; Hwang, Chueh-Lung; Lim, Jisok; Handberg, Eileen M; Christou, Demetra D

    2017-05-01

    There is growing evidence of sex differences in the chronic effect of aerobic exercise on endothelial function (flow-mediated dilation; FMD) in older adults, but whether there are sex differences also in the acute effect of aerobic exercise on FMD in older adults is unknown. The purpose of this study was to test the hypothesis that sex modulates the FMD response to acute aerobic exercise in older adults. Thirteen older men and fifteen postmenopausal women (67±1 vs. 65±2years, means±SE, P=0.6), non-smokers, free of major clinical disease, participated in this randomized crossover study. Brachial artery FMD was measured: 1) prior to exercise; 2) 20min after a single bout of high-intensity interval training (HIIT; 40min; 4×4 intervals 90% peak heart rate (HRpeak)), moderate-intensity continuous training (MICT; 47min 70% HRpeak) and low-intensity continuous training (LICT; 47min 50% HRpeak) on treadmill; and 3) following 60-min recovery from exercise. In older men, FMD was attenuated by 45% following HIIT (5.95±0.85 vs. 3.27±0.52%, P=0.003) and by 37% following MICT (5.97±0.87 vs. 3.73±0.47%, P=0.03; P=0.9 for FMD response to HIIT vs. MICT) and was normalized following 60-min recovery (P=0.99). In postmenopausal women, FMD did not significantly change in response to HIIT (4.93±0.55 vs. 6.31±0.57%, P=0.14) and MICT (5.32±0.62 vs. 5.60±0.68%, P=0.99). In response to LICT, FMD did not change in postmenopausal women nor older men (5.21±0.64 vs. 6.02±0.73%, P=0.7 and 5.70±0.80 vs. 5.55±0.67%, P=0.99). In conclusion, sex and exercise intensity influence the FMD response to acute aerobic exercise in older adults. Copyright © 2017 Elsevier Inc. All rights reserved.

  18. Monitorização eletrocardiográfica ambulatorial por 24-horas em cães com cardiomiopatia dilatada idiopática Twenty-four-hour ambulatory electrocardiographic monitoring in dogs with idiopathic dilated cardiomyopathy

    Directory of Open Access Journals (Sweden)

    F.L. Yamaki

    2007-12-01

    Full Text Available Caracterizou-se monitorização eletrocardiográfica ambulatorial por 24 horas (ou monitorização Holter em cães com cardiomiopatia dilatada idiopática, visando principalmente à detecção de arritmias ventriculares não detectadas pela eletrocardiografia convencional (de repouso. Para tanto, avaliaram-se 40 pacientes com diagnóstico de cardiomiopatia dilatada idiopática, por meio de exame físico e mensuração indireta da pressão arterial, além de exames eletrocardiográfico, ecocardiográfico, radiográfico de tórax e da monitorização Holter. Extra-sístoles ventriculares foram detectadas, por monitorização Holter, em 97,5% dos animais e taquicardia ventricular, em 45%. Não houve correlação entre o número de extra-sístoles ventriculares e a fração de encurtamento. Considerando as manifestações clínicas, apenas houve associação entre presença de taquicardia ventricular e histórico de síncopes. Conclui-se que a incidência de arritmias ventriculares em cães com cardiomiopatia dilatada idiopática é bastante alta, sendo a taquicardia ventricular relativamente freqüente, ocorrendo mais sob a forma não sustentada.This study aimed to characterize 24-hour ambulatory electrocardiographic monitoring (Holter monitoring in dogs with idiopathic dilated cardiomyopathy. Physical examination and indirect (Doppler blood pressure measurement, and also electrocardiography, thoracic radiography, echocardiography, and 24-hour ambulatory electrocardiographic exams were performed in 40 dogs with idiopathic dilated cardiomyopathy. Ventricular extrasystoles were detected in 97.5% of the animals, and ventricular tachycardia in 45%. No correlation between the number of ventricular extrasystoles and the shortening fraction was observed. Concerning the clinical symptoms, there was only association between the presence of ventricular tachycardia and past report of syncope. It was concluded that the incidence of ventricular arrhythmias is

  19. The role of echocardiography in diagnosis and follow up of patients with takotsubo cardiomyopathy or acute ballooning syndrome.

    Science.gov (United States)

    Naser, Nabil; Buksa, Marko; Kusljugic, Zumreta; Terzic, Ibrahim; Sokolovic, Sekib; Hodzic, Enisa

    2011-01-01

    The transient left ventricular apical ballooning syndrome, also known as takotsubo cardiomyopathy was first described in Japan approximately 20 years ago (Satoh and coworkers, 1991). It was later described elsewhere as well and is being increasingly recognized. Takotsubo Cardiomyopathy characterized by transient apical and midventricular LV dysfunction in the absence of significant coronary artery disease that is triggered by emotional or physical stress. Its name refers to a contraption used for catching octopuses and suggests the aspect assumed by the ventricle during the systole due to the typical regional wall motion abnormalities that occur after onset. Takotsubo cardiomiopathy occurring mainly in post-menopausal women, echocardiography in the Takotsubo cardiomyopathy reveals during its acute phase a ballooning resembling the octopus trap configuration--the apex and lateral ventricular segments are hypokinetic while the base is hyperkinetic--along with reduced ejection fraction. Ventricular function will usually recover within a few days/weeks. The objective of this study is to determine the role of echocardiography in detecting and establishing the diagnosis of Takotsubo cardiomiopathy in patients with suspect acute coronary syndrome and during the follow up period. The study covered 12 adult patients the majority are women (92%) who were subjected to echocardiography evaluation as part of the clinical cardiological examination due to suspect acute coronary syndrome or Takotsubo Stress Cardiomyopathy. The patients were examined on an ultrasound machine Philips iE 33 x Matrix, ATL HDI and GE Vived 7 equipped with all cardiologic probes for adults and multi-plan TEE probes. We evaluated clinical characteristics, LV systolic function, biomarkers, and prognosis in all patients. Among all the patients referred for Echocardiographic evaluation for left ventricle motion abnormalities with suspect acute coronary syndrome, the echo exam revealed 12 patients with acute

  20. Myocardial scintigraphy using iodine-123 15-(p-Iodophenyl)-3-R, S-methylpentadecanoic acid predicts the response to beta-blocker therapy in patients with dilated cardiomyopathy but does not reflect therapeutic effect

    Energy Technology Data Exchange (ETDEWEB)

    Yoshinaga, Keiichiro; Tahara, Minoru; Torii, Hiroyuki; Akimoto, Masaki [Kagoshima City Medical Association Hopital (Japan); Kihara, Koichi; Tei, Chuwa

    2000-05-01

    Myocardial fatty acid metabolism is disturbed in patients with idiopathic dilated cardiomyopathy. Myocardial scintigraphy using iodine-123 15-(p-iodophenyl)-3-R, S-methylpentadecanoic acid (BMIPP) was used to assess the response to {beta}-blocker therapy in 19 patients with dilated cardiomyopathy. BMIPP myocardial scintigraphy was performed before and 6 months after initiating {beta}-blocker therapy with metoprolol. Cardiac BMIPP uptake was assessed as the total defect score (TDS) and heart-to-mediastinum activity (H/M) ratio. Patients were classified retrospectively as responders with an improvement of at least one functional class (New York Heart Association) or an increase in ejection fraction of {>=}0.10 at 6 months, or as nonresponders meeting neither criterion. Responders had a significantly better pretreatment TDS (p<0.005) and H/M ratio (p<0.0001) than nonresponders. TDS exhibited no significant changes over 6 months in either group (responders: 13.2{+-}3.7 vs 12.5{+-}3.3; nonresponders: 20.8{+-}6.5 vs 20.5{+-}3.0). Responders showed no significant changes in H/M ratio (2.47{+-}0.28 vs 2.43{+-}0.42); paradoxically, nonresponders showed a significant increase from 1.82{+-}0.11 to 2.10{+-}0.19 (p<0.05), suggesting that {beta}-blocker therapy protected the myocardial fatty acid metabolism even in the absence of clinical improvement. BMIPP myocardial scintigraphy provides a prediction of response to {beta}-blocker treatment, but does not reflect the therapeutic effect in responders at 6 months. (author)

  1. Scorpion-related cardiomyopathy and acute pulmonary edema in a child who is stung by Leiurus abdullahbayrami

    Directory of Open Access Journals (Sweden)

    Mehmet Dokur

    2017-09-01

    Full Text Available Venom of Leiurus abdullahbayrami (Scorpiones: Buthidae is an extremely toxic one and it stimulates voltage-gated sodium and potassium channels. In case of a stung by this scorpion; excessive catecholamine release occur and it impairs left ventricle contractility and consequently a heart failure occurs (scorpion sting-related cardiomyopathy. In addition to this cardiac-induced acute pulmonary, edema may occur in severe cases too. An 11-year-old male child who was stung by a scorpion (species: Leiurus abdullahbayrami consulted to the Emergency Room. Even after 7 h of scorpion envenomation he was confused and having hallucinations. Besides he was dyspneic, tachycardic, hypotensive and got worse in overall situation due to cardiogenic pulmonary edema. These clinical findings are concordant with the Level III scorpion envenomation (major systemic manifestations. Positive inotropic agents, diuretics and antiagregant agents used on supportive therapy in his treatment. After 2 weeks he get recovered and discharged from the pediatric intensive care unit. This research is conducted by thinking emergency physicians should learn that Leiurus abdullahbayrami envenomation can cause scorpion-related cardiomyopathy and acute pulmonary edema especially in children. Keywords: Leiurus, Scorpionism, Cardiomyopathy, Pulmonary edema

  2. Acute pulmonary edema caused by takotsubo cardiomyopathy in a pregnant woman undergoing transvaginal cervical cerclage

    OpenAIRE

    Lee, Jae-Young; Kwon, Hyun-Jung; Park, Sang-Wook; Lee, Yu-Mi

    2017-01-01

    Abstract Background: The physiological changes associated with pregnancy may predispose pregnant women to pulmonary edema. Other known causes of pulmonary edema during pregnancy include tocolytic drugs, preeclampsia, eclampsia, and peripartum cardiomyopathy. Methods: We describe a rare case of pulmonary edema caused by takotsubo cardiomyopathy in a pregnant woman at 14 weeks of gestation who was undergoing emergency transvaginal cervical cerclage. Results: Intraoperative chest radiography rev...

  3. Mitochondrial 12S ribosomal RNA A1555G mutation associated with cardiomyopathy and hearing loss following high-dose chemotherapy and repeated aminoglycoside exposure

    DEFF Research Database (Denmark)

    Skou, Anne-Sofie; Tranebjærg, Lisbeth; Jensen, Tim

    2014-01-01

    A 19-month-old girl with the A1555G mitochondrial mutation in the 12S ribosomal RNA gene and acute myelogenous leukemia developed dilated cardiomyopathy and bilateral sensorineural hearing loss before undergoing allogeneic stem cell transplantation. She had received gentamicin during episodes...

  4. Therapeutic effect of co-enzyme Q10 on idiopathic dilated cardiomyopathy: assessment by iodine-123 labelled 15-(p-iodophenyl)-3(R,S)-methylpentadecanoic acid myocardial single-photon emission tomography

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Yong-ih [Department of Internal Medicine, Nishiyodo Hospital, Nishiyodo (Japan); Sawada, Yoshihiro [Department of Internal Medicine, Nishiyodo Hospital, Nishiyodo (Japan); Fujiwara, Go [Department of Radiology, Nishiyodo Hospital, Nishiyodo (Japan); Chiba, Hiroshi [Department of Internal Medicine, Mimihara General Hospital, Mimihara (Japan); Nishimura, Tsunehiko [Division of Tracer Kinetics, Biomedical Research Center, Osaka University Medical School, Osaka (Japan)

    1997-06-10

    It has been reported that myocardial mitochondrial function can be improved by the administration of co-enzyme Q10 (CoQ10). Recently, iodine-123 labelled 15-(p-iodophenyl)-3-(R,S)-methylpentadecanoic acid (BMIPP) was developed for metabolic imaging using single-photon emission tomography (SPET). This study was conducted to determine whether the therapeutic effects of CoQ10 on idiopathic dilated cardiomyopathy can be evaluated by BMIPP myocardial SPET. Fifteen patients, comprising 14 men and one woman (mean age: 64{+-}12 years), were examined. CoQ10 was administered at 30 mg/day for a period of 35.7{+-}12.4 days. BMIPP myocardial SPET was carried out before and after CoQ10 treatment. The count ratio of the heart (H) to the upper mediastinum (M) (H/M ratio) was calculated using a region of interest method with anterior planar imaging. Representative short-axis tomograms were divided into 27 segments (three slices x nine segments). Each segmental score was analysed semiquantitatively using a four-point scoring system (normal=0, mild low uptake=1, severe low uptake=2, defect=3). The H/M ratio showed a significant improvement, from 2.39{+-}0.39 to 2.54{+-}0.47, after treatment (P<0.05). The BMIPP total defect score after CoQ10 treatment was significantly decreased to 10.1{+-}4.3, compared to 13.9{+-}4.5 without CoQ10 treatment (P<0.001). However, the percent fractional shortening measured using echocardiography was not significantly different before and after CoQ treatment (19.2{+-}8.1 vs 19.7{+-}7.1). BMIPP myocardial SPET was confirmed to be sensitive in evaluating the therapeutic effects of CoQ10 in patients with idiopathic dilated cardiomyopathy. This method is unique, since the therapeutic effects can be estimated from the perspective of metabolic SPET imaging. (orig.). With 5 figs., 1 tab.

  5. Intracoronary administration of autologous bone marrow-derived progenitor cells in a critically ill two-yr-old child with dilated cardiomyopathy.

    Science.gov (United States)

    Rupp, Stefan; Bauer, Jürgen; Tonn, Torsten; Schächinger, Volker; Dimmeler, Stefanie; Zeiher, Andreas M; Schranz, Dietmar

    2009-08-01

    DCM is the most common cardiomyopathy in childhood. Effectiveness of anticongestive therapy is limited in most cases and about one-third of children diagnosed with DCM die or receive heart transplantation within the first year after diagnosis. Cardiac stem cell transplantation has become a promising therapy to treat heart failure in adult patients. Based on these promising results, the cardiac stem cell therapy might also represent a new therapeutic option particularly in young children. The present case documents for the first time intracoronary administration of autologous bone marrow-derived progenitor cells in a critically ill two-yr-old child with severe heart failure caused by DCM. Because of progressive worsening of the clinical condition despite maximal anticongestive treatment, the decision to perform autologous stem cell therapy was made. Cardiac stem cell therapy proved to be technically feasible, was associated with improvement in cardiac function, and might represent an option before heart transplantation in children with severe heart failure.

  6. Acute gastric dilatation and acute pancreatitis in a patient with an eating disorder: solving a chicken and egg situation.

    Science.gov (United States)

    Kim, Hyung Hun; Park, Seun Ja; Park, Moo In; Moon, Won

    2011-01-01

    A 26-year-old woman with an eating disorder presented to the emergency department with severe abdominal pain following binge eating. A plain film X-ray demonstrated a huge dilatation of the stomach with a high air-fluid level. Serum amylase was 2,265 IU/L, and serum lipase was 2,001 IU/L. Abdominopelvic computed tomography scan revealed a massive gastric dilatation and completely compressed duodenum. The distended right colonic loop and small bowel loops were reduced to the pelvic area and the displaced small bowel and mesenteries tightly pulled on the mesenteric vasculature. After nasogastric tube decompression and irrigation, her abdominal pain subsided. On the 15th day after admission, a follow-up abdominopelvic computed tomography scan demonstrated mild edematous changes of the pancreas compatible with pancreatitis.

  7. ACUTE INTRAVENOUS VERSUS CHRONIC ORAL-DRUG EFFECTS OF VERAPAMIL ON LEFT-VENTRICULAR DIASTOLIC FUNCTION IN PATIENTS WITH HYPERTROPHIC CARDIOMYOPATHY

    NARCIS (Netherlands)

    POSMA, JL; BLANKSMA, PK; VANDERWALL, E; LIE, KI

    1994-01-01

    The effects of acute intravenous (i.v.) versus long-term oral(p.o.) verapamil administration on diastolic function were studied in 20 patients with hypertrophic cardiomyopathy (HCM). We used a tip manometer catheter to record left ventricular pressure (LVP) tracings and a nuclear probe to measure LV

  8. Pregnancy in women with a cardiomyopathy: Outcomes and predictors from a retrospective cohort.

    Science.gov (United States)

    Billebeau, Gilles; Etienne, Martin; Cheikh-Khelifa, Riadh; Vauthier-Brouzes, Daniele; Gandjbakhch, Estelle; Isnard, Richard; Nizard, Jacky; Komajda, Michel; Dommergues, Marc; Charron, Philippe

    2017-10-25

    Pregnancies in women with pre-existing cardiomyopathies are considered at high risk for complications. However, few data are available to characterize their natural history and predict the outcome. Our aim was to evaluate the prevalence and predictors of acute cardiac and obstetric events in women with a cardiomyopathy during pregnancy, excluding peripartum cardiomyopathy. In this retrospective study in a referral centre for cardiomyopathies, we included 43 consecutive pregnancies in 36 women with dilated, hypertrophic, arrhythmogenic right ventricular or tachycardia-induced cardiomyopathy, or left ventricular non-compaction. We observed a major cardiovascular event during 15 pregnancies (35%), including three cardiac deaths, which occurred in patients who did not follow our usual early multidisciplinary protocol. The Carpreg score was predictive of maternal complication rate (67%, 36% and 31% in women with a Carpreg score of 2, 1 and 0, respectively). However, major cardiac complications occurred in four women with no risk factors. Left ventricular ejection fraction alone, gradient in hypertrophic cardiomyopathy, the Zahara score and the modified World Health Organization score appeared to be less discriminant than Carpreg for maternal outcome. There were two intrauterine fetal deaths, nine premature deliveries (23%), 17 low neonatal birth weights (40%) and 11 cases of hypoglycaemia (26%). Pregnancy in women with a cardiomyopathy is at high risk for both women and neonates. The highest risks are observed in women who do not benefit from early multidisciplinary team management, and in patients with dilated cardiomyopathy. Our findings suggest that the Carpreg score is the most appropriate predictor of maternal complications, although the stratification might be improved. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

  9. Acute Gastric Dilatation: A Transient Cause of Hepatic Portal Venous Gas—Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Satya B. Allaparthi

    2013-01-01

    Full Text Available Gastric pneumatosis (GP and hepatic portal venous gas (HPVG have typically been thought of as an ominous radiological sign associated with a grave prognosis, and the observation of HPVG on plain abdominal radiography, ultrasonography, or computed tomography is viewed as a significant finding. It is often associated with severe or potentially lethal conditions warranting urgent diagnosis and possible surgical intervention. Early studies of HPVG based on plain abdominal radiography found an associated mortality rate of 75% primarily due to ischemic bowel. However, modern abdominal computed tomography (CT has resulted in the detection of HPVG in an increased proportion of nonfatal and benign conditions. We report a nonfatal case of HPVG in a patient with Noonan’s syndrome due to acute gastric dilatation in the setting of gastric outlet obstruction caused by a congenital band that is extremely rare in adults.

  10. Myocardial regenerative therapy using a scaffold-free skeletal-muscle-derived cell sheet in patients with dilated cardiomyopathy even under a left ventricular assist device: a safety and feasibility study.

    Science.gov (United States)

    Yoshikawa, Yasushi; Miyagawa, Shigeru; Toda, Koichi; Saito, Atsuhiro; Sakata, Yasushi; Sawa, Yoshiki

    2018-02-01

    Despite promising experimental results, clinically, intramyocardial myoblast injection failed to reverse remodeling and it induced arrhythmogenicity. In contrast, scaffold-free skeletal muscle-derived cell (SC) sheets attenuated cardiac dysfunction and arrhythmogenicity via paracrine effects. We report the first clinical trial of SC sheet implantation (SCSI) conducted in four patients with dilated cardiomyopathy (DCM) supported by a left ventricular assist device (LVAD). SC sheets were made from muscle fibers and multi-layered SC sheets were applied to the left ventricular (LV) anterolateral surface via left thoracotomy. There were no major cardiac adverse events. Ventricular arrhythmia decreased in all except one patient, in whom global LV function did not improve. The LV volume decreased and LV ejection fraction improved in all except the same patient. Systolic wall thickening, reflecting regional wall motion, improved in the sheet-implanted areas, and vessels in the LV apex increased in all patients, suggesting angiogenesis. The LVAD was successfully removed in two patients. SCSI induced reverse remodeling and angiogenesis, and improved LV function, allowing LVAD removal in two patients, although functional recovery failed to improve in the one non-responder, even with angiogenesis. SCSI is a promising regenerative therapy for DCM patients responsive to this strategy, even with LVAD assistance.

  11. Acute gastric dilatation and volvulus in a free-living polar bear

    Science.gov (United States)

    Amstrup, Steven C.; Nielsen, Carol A.

    1989-01-01

    A large, adult male polar bear (Ursus maritimus) was found dead on a barrier island north of Prudhoe Bay, Alaska (USA), in June 1987. There were no external signs of trauma. A twisted distended stomach, distinctive parenchymal and fascial congestion, and significant difficulty in repositioning the anterior abdominal organs, indicated that gastric dilatation-volvulus (GDV) was the proximate cause of death. Polar bears frequently consume large quantities of food at one time and have large stomachs that are well adapted to periodic gorging. The scarcity of food in winter and early spring, combined with voluntary fasting and protracted vigorous activity during the breeding season in late spring may have predisposed this bear to GDV. The relationship between GDV and postprandial exercise emphasizes the need for a better understanding of how the present human invasion of arctic habitats may influence polar bear activities.

  12. “Reverse McConnell’s Sign”: Interpreting Interventricular Hemodynamic Dependency and Guiding the Management of Acute Heart Failure during Takotsubo Cardiomyopathy

    OpenAIRE

    Kan Liu; Zhongxia Sun; Tiemin Wei

    2015-01-01

    Although most patients with Takotsubo cardiomyopathy (TTC) have benign clinical course and prognosis, TTC can induce acute heart failure and hemodynamic instability. TTC mimics the clinical features of acute anterior wall myocardial infarction (AMI). Bedside clinicians often have a diagnostic dilemma when cardiac catheterization and angiography are either contraindicated or can cause potential adverse consequences. Misdiagnosing TTC as AMI will lead to initiation of harmful pharmacological or...

  13. Hipotiroidismo, miocardiopatía dilatada y síndrome nefrótico durante el embarazo Hypothyroidism, dilated cardiomyopathy and nephrotic syndrome during pregnancy

    Directory of Open Access Journals (Sweden)

    Ariel K. Saad

    2011-02-01

    Full Text Available El hipotiroidismo en el embarazo es infrecuente, pero cuando ocurre suele asociarse con complicaciones maternas y fetales. Se presenta el caso de una mujer joven sin antecedentes de enfermedad cardiovascular que consulta por ortopnea, dolor torácico y edema de miembros inferiores. Los exámenes pusieron en evidencia la existencia de insuficiencia cardíaca, hipotiroidismo, síndrome nefrótico e insuficiencia renal. El eco-Doppler mostró dilatación de las cuatro cavidades cardíacas con deterioro grave de la función sistólica. El tratamiento con levotiroxina por vía intravenosa mejoró el cuadro clínico y los parámetros de laboratorio. Se analizan los efectos de la hormona tiroidea sobre el aparato cardiovascular y se comentan los mecanismos fisiopatológicos de la insuficiencia cardíaca en el embarazo.Hypothyroidism during pregnancy is infrequent, but its presence is associated with maternal and fetal complications. We present the case of a young pregnant woman with no previous history of cardiovascular disease, who consulted for orthopnea, chest pain and edema in both legs. Laboratory tests demonstrated a hypothyroid condition and a nephrotic syndrome with renal failure. The echo-Doppler exam showed a four chamber dilatation with systolic dysfunction. Treatment with intravenous levothyroxine improved her medical condition. We analyze the effects of thyroid hormone on the heart and vascular system and discuss the pathophysiologic mechanisms of heart failure during pregnancy.

  14. Anabolic steroids abuse-induced cardiomyopathy and ischaemic stroke in a young male patient.

    Science.gov (United States)

    Shamloul, Reham Mohammed; Aborayah, Ahmed Fathy; Hashad, Assem; Abd-Allah, Foad

    2014-02-26

    We report a case of a 37-year-old man presented with acute stroke and hepatorenal impairment which were associated with anabolic-androgenic steroids (AAS) abuse over 2 years. Despite the absence of apparent symptoms and signs of congestive heart failure at presentation, an AAS-induced dilated cardiomyopathy with multiple thrombi in the left ventricle was attributed to be the underlying cause of his condition. Awareness of the complications of AAS led to the prompt treatment of the initially unrecognised dilated cardiomyopathy, and improved the liver and kidney functions. However, the patient was exposed to a second severe ischaemic event, which led to his death. This unique and complex presentation of AAS complications opens for better recognition and treatment of their potentially fatal effects.

  15. Como o ecocardiograma pode ser útil em predizer a morte em crianças com cardiomiopatia dilatada idiopática? How can the echocardiogram be useful for predicting death in children with idiopathic dilated cardiomyopathy?

    Directory of Open Access Journals (Sweden)

    Vitor Manuel Pereira Azevedo

    2004-06-01

    Full Text Available OBJETIVO: Determinar os fatores preditores ecocardiográficos de morte em crianças com cardiomiopatia dilatada idiopática. MÉTODOS: Estudo retrospectivo de 148 crianças com cardiomiopatia dilatada idiopática diagnosticadas entre setembro/1979 a março/2003. Critérios para inclusão: insuficiência cardíaca e redução da contratilidade no ecocardiograma, na ausência de cardiopatia congênita ou secundária. Foram analisados 470 exames, durante 244,8 meses de evolução. Parâmetros analisados: dimensão do átrio esquerdo (DAE, relação átrio esquerdo/aorta (DAE/Ao, dimensão sistólica (DSVE e diastólica (DDVE do VE, massa do VE (massa, dimensão do ventrículo direito (DVD, fração de ejeção do VE (FE, percentagem de encurtamento do VE (%EC, gravidade da insuficiência das valvas atrioventriculares e da valva pulmonar e medida da pressão sistólica (PsVD e diastólica (PdVD do VD. Foi considerado significativo erro alfa OBJECTIVE: To determine the echocardiographic predicting factors of death in children with idiopathic dilated cardiomyopathy. METHODS: A retrospective study of 148 children with idiopathic dilated cardiomyopathy diagnosed between September 1979 and March 2003 was carried out. The inclusion criteria were as follows: heart failure and a reduction in contractility on the echocardiogram in the absence of congenital or secondary heart disease. Four hundred and seventy examinations during a period of 244.8 months of evolution were analyzed. The following parameters were assessed: left atrial dimension (LAD; left atrium/aorta ratio (LAD/Ao; left ventricular systolic (LVSD and diastolic (LVDD dimensions; left ventricular mass (LVmass; right ventricular dimension (RVD; left ventricular ejection fraction (LVEF; left ventricular shortening fraction (% SH; severity of the insufficiency of the atrioventricular and pulmonary valves; and right ventricular systolic (RVSP and diastolic (RVDP pressures. The significance level

  16. Usefulness of 123I-metaiodobenzylguanidine myocardial scintigraphy for predicting the effectiveness of β-blockers in patients with dilated cardiomyopathy from the standpoint of long-term prognosis

    International Nuclear Information System (INIS)

    Fujimoto, Shinichiro; Inoue, Aritomo; Hisatake, Shinji; Yamashina, Shohei; Yamashina, Hisayo; Nakano, Hajime; Yamazaki, Junichi

    2004-01-01

    The usefulness of 123 I-metaiodobenzylguanidine (MIBG) myocardial scintigraphy in predicting the effectiveness of β-blocker therapy in dilated cardiomyopathy (DCM) was investigated from the standpoint of long-term prognosis. The subjects were 53 DCM patients in whom β-blockers had been successfully introduced and used for 6 months or longer. When symptoms were stable before the introduction of β-blockers and for up to 1 year thereafter, MIBG myocardial single-photon emission computed tomography was performed and the images analysed to obtain the extent score (EXT), severity score (SEV) and washout rate (WR). At the same time, echocardiography was performed to measure left ventricular ejection fraction (LVEF). Thereafter, patients were placed under observation for an average of 1,314±986 days, with the occurrence of cardiac events as the endpoint. The degree of improvement in WR after introduction of β-blockers was a significant predictor of cardiac events. In fact, none of the patients whose improvement in WR was valued at 10 or higher had cardiac events. Accordingly, using improvement in WR of 10 as the cut-off value, the patients were divided into two groups, ''improved'' and ''unimproved''. There were significant differences between the groups in respect of early EXT, early SEV and WR before the introduction of β-blockers. As regards predictors of WR improvement, multivariate logistic regression analysis demonstrated that early EXT, WR and LVEF were significant predictors. This study shows that, from the standpoint of long-term prognosis, DCM patients who would benefit the most from β-blocker therapy are those with low early EXT and early SEV and high WR before β-blocker introduction regardless of LVEF values. (orig.)

  17. Survival and echocardiographic evaluation of dogs with idiopathic dilated cardiomyopathy treated with carvedilol Avaliação ecocardiográfica e de sobrevida de cães com cardiomiopatia dilatada idiopática tratados com carvedilol

    Directory of Open Access Journals (Sweden)

    E.C. Soares

    2010-06-01

    Full Text Available Sixty dogs with idiopathic dilated cardiomyopathy were randomly treated with traditional therapy - digitalis, diuretics, angiotensin-converting inhibitors - (group A or treated with these drugs plus carvedilol (group B. Echocardiographic variables were measured before and after 3, 13, 26, and 52 weeks of treatment or until death. Comparisons between groups and time were performed. No significant differences between groups were found in the most of the echocardiographic variables. The left ventricular end-systolic diameter indexed to body surface area (LVESDi increased significantly in the group A dogs compared to the group B animals. The survival of groups A and B dogs were not different (P-value=0.1137. In conclusion, the stability of the LVESDi observed in the group treated with carvedilol may represent the beneficial effect over the ventricular remodeling.Sessenta cães com cardiomiopatia dilatada idiopática receberam, aleatoriamente, tratamento convencional - digitálicos, diuréticos, inibidores da enzima conversora de angiotensina - (grupo A ou esses fármacos mais carvedilol (grupo B. As variáveis ecocardiográficas foram avaliadas antes e depois de três, 13, 26 e 52 semanas de tratamento ou até o óbito. Não foram encontradas diferenças significativas entre os grupos de animais quanto à maioria das variáveis ecocardiográficas. O diâmetro sistólico final do ventrículo esquerdo indexado à superfície corpórea (DSVEi aumentou de forma significativa no grupo A quando comparado ao grupo B. Não se observou diferença na sobrevida dos grupos A e B (P=0,1137. Concluiu-se que a estabilização do DSVEi no grupo tratado com carvedilol pode representar o efeito benéfico deste fármaco sobre o remodelamento ventricular.

  18. Missense mutation Lys18Asn in dystrophin that triggers X-linked dilated cardiomyopathy decreases protein stability, increases protein unfolding, and perturbs protein structure, but does not affect protein function.

    Science.gov (United States)

    Singh, Surinder M; Bandi, Swati; Shah, Dinen D; Armstrong, Geoffrey; Mallela, Krishna M G

    2014-01-01

    Genetic mutations in a vital muscle protein dystrophin trigger X-linked dilated cardiomyopathy (XLDCM). However, disease mechanisms at the fundamental protein level are not understood. Such molecular knowledge is essential for developing therapies for XLDCM. Our main objective is to understand the effect of disease-causing mutations on the structure and function of dystrophin. This study is on a missense mutation K18N. The K18N mutation occurs in the N-terminal actin binding domain (N-ABD). We created and expressed the wild-type (WT) N-ABD and its K18N mutant, and purified to homogeneity. Reversible folding experiments demonstrated that both mutant and WT did not aggregate upon refolding. Mutation did not affect the protein's overall secondary structure, as indicated by no changes in circular dichroism of the protein. However, the mutant is thermodynamically less stable than the WT (denaturant melts), and unfolds faster than the WT (stopped-flow kinetics). Despite having global secondary structure similar to that of the WT, mutant showed significant local structural changes at many amino acids when compared with the WT (heteronuclear NMR experiments). These structural changes indicate that the effect of mutation is propagated over long distances in the protein structure. Contrary to these structural and stability changes, the mutant had no significant effect on the actin-binding function as evident from co-sedimentation and depolymerization assays. These results summarize that the K18N mutation decreases thermodynamic stability, accelerates unfolding, perturbs protein structure, but does not affect the function. Therefore, K18N is a stability defect rather than a functional defect. Decrease in stability and increase in unfolding decrease the net population of dystrophin molecules available for function, which might trigger XLDCM. Consistently, XLDCM patients have decreased levels of dystrophin in cardiac muscle.

  19. Morphometric Documentation of a High Prevalence of Left Ventricular Dilated Cardiomyopathy in Both Clinically Normal and Cyanotic Mature Commercial Broiler Breeder Roosters with Comparisons to Market-Age Broilers.

    Science.gov (United States)

    Wilson, Floyd D; Magee, Danny L; Jones, Kelli H; Baravik-Munsell, Erica; Cummings, Timothy S; Wills, Robert W; Pace, Lanny W

    2016-09-01

    Previous studies documented the common occurrence of transitory cyanosis and echocardiographic aortic insufficiency in mature commercial broiler breeder roosters. During further investigations, we observed a high prevalence of hearts exhibiting extensive dilation of the left ventricle chamber compatible with dilated left ventricular cardiomyopathy present in both cyanotic and normal subpopulations. We conducted quantitative studies focused on documentation of cardiac ventricle parameters by using simple gross morphometric methods performed on formalin-fixed hearts obtained from both clinically normal roosters and those exhibiting variable transitory cyanosis, echocardiographic aortic insufficiency, or both. A high prevalence of often dramatic left ventricular dilation reflected in enlarged left ventricular chamber areas and elevated left ventricle-to-total ventricle area ratios was morphometrically documented. However, no statistically significant differences in the occurrence of ventricular abnormalities were observed between normal and cyanotic roosters. Age-associated changes were also demonstrated by comparative morphometric studies on hearts from normal market-age broilers (average age of 7 wk) and those of mature roosters (average age of 42 wk). Elevation in both left and right ventricular weight-to-total heart weight ratios dramatically increased with aging. In addition, values (average ± SD) for the left ventricle chamber area-to-total ventricle area ratios increased from 3.2 ± 2.0% in broilers up to 10.0 ± 8.8% in roosters. None of the normal broilers studied demonstrated left ventricular volume ratios above 10%, whereas 33% of the roosters had left ventricular volume ratios above 10%, including 13% with ratios of 20% or higher. However, the left ventricle wall area-to-body weight ratios were much closer for the two age groups (0.85 ± 0.18 cm(2)/kg in broilers and 0.79 ± 0.13 cm(2)/kg in roosters). Also, the standard right ventricle-to-total ventricle

  20. Autologous bone marrow mononuclear cell delivery to dilated ...

    African Journals Online (AJOL)

    Autologous bone marrow mononuclear cell delivery to dilated cardiomyopathy patients: A clinical trial. PLN Kaparthi, G Namita, LK Chelluri, VSP Rao, PK Shah, A Vasantha, SK Ratnakar, K Ravindhranath ...

  1. Acute effects of sildenafil on flow mediated dilatation and cardiovascular autonomic nerve function in type 2 diabetic patients.

    Science.gov (United States)

    Stirban, Alin; Laude, Dominique; Elghozi, Jean-Luc; Sander, Denise; Agelink, Marcus W; Hilz, Max J; Ziegler, Dan

    2009-02-01

    Sildenafil, frequently used as on demand medication for the treatment of erectile dysfunction (ED), has been suggested to improve endothelial function but also to alter blood pressure (BP) and induce sympathetic activation. In people with type 2 diabetes mellitus (T2DM), a high-risk population, the safety profile and the effects on endothelial function of a maximal sildenafil dose (100 mg) have not been investigated and therefore constituted the aim of our study. A double-blind, placebo-controlled, cross-over trial using a single dose of 100 mg sildenafil or placebo has been conducted in 40 subjects with T2DM without known CVD. Haemodynamic parameters, flow mediated dilatation (FMD) in brachial artery, cardiovascular autonomic function tests and spontaneous baroreflex sensitivity (BRS) were measured. Sixty minutes after administration of sildenafil but not placebo, a fall of supine systolic blood pressure (SBP) (-5.41 +/- 1.87 vs. + 0.54 +/- 1.71 mmHg) and diastolic blood pressure (DBP) (-4.46 +/- 1.13 vs. + 0.89 +/- 0.94 mmHg), as well as orthostatic SBP (-7.41 +/- 2.35 vs. + 0.94 +/- 2.06 mmHg) and DBP (-5.65 +/- 1.45 vs. + 1.76 +/- 1.00 mmHg) during standing occurred, accompanied by an increase in heart rate (+1.98 +/- 0.69 vs. - 2.42 +/- 0.59 beats/min) (all p < 0.01 vs. placebo). Changes in BP to standing up, FMD, time domain and frequency domain indices of heart rate variability (HRV) and BRS were comparable between sildenafil and placebo. Sildenafil administered at a maximum single dose to T2DM men results in a mild increase in heart rate and decrease in BP, but it induces neither an acute improvement of FMD nor any adverse effects on orthostatic BP regulation, HRV and BRS. Copyright (c) 2008 John Wiley & Sons, Ltd.

  2. Relationship between late ventricular potentials and myocardial {sup 123}I-metaiodobenzylguanidine scintigraphy in patients with dilated cardiomyopathy with mild to moderate heart failure: results of a prospective study of sudden death events

    Energy Technology Data Exchange (ETDEWEB)

    Kasama, Shu [Gunma University Graduate School of Medicine, Department of Medicine and Biological Science (Cardiovascular Medicine), Gunma (Japan); Cardiovascular Hospital of Central Japan (Kitakanto Cardiovascular Hospital), Department of Cardiovascular Medicine, Gunma (Japan); Toyama, Takuji; Kaneko, Yoshiaki; Kurabayashi, Masahiko [Gunma University Graduate School of Medicine, Department of Medicine and Biological Science (Cardiovascular Medicine), Gunma (Japan); Iwasaki, Toshiya; Sumino, Hiroyuki; Kumakura, Hisao; Minami, Kazutomo; Ichikawa, Shuichi [Cardiovascular Hospital of Central Japan (Kitakanto Cardiovascular Hospital), Department of Cardiovascular Medicine, Gunma (Japan); Matsumoto, Naoya [Nihon University School of Medicine, Department of Cardiology, Tokyo (Japan); Sato, Yuichi [Health Park Clinic, Department of Imaging, Gunma (Japan)

    2012-06-15

    Late ventricular potentials (LPs) are considered to be useful for identifying patients with heart failure at risk of developing ventricular arrhythmias. {sup 123}I-metaiodobenzylguanidine (MIBG) scintigraphy, which is used to evaluate cardiac sympathetic activity, has demonstrated cardiac sympathetic denervation in patients with malignant ventricular tachyarrhythmias. This study was undertaken to clarify the relationship between LPs and {sup 123}I-MIBG scintigraphy findings in patients with dilated cardiomyopathy (DCM). A total of 56 patients with DCM were divided into an LP-positive group (n = 24) and an LP-negative group (n = 32). During the compensated period, the delayed heart/mediastinum count (H/M) ratio, delayed total defect score (TDS), and washout rate (WR) were determined from {sup 123}I-MIBG images and plasma brain natriuretic peptide (BNP) concentrations were measured. Left ventricular end-diastolic volume (LVEDV), left ventricular end-systolic volume (LVESV), and left ventricular ejection fraction (LVEF) were simultaneously determined by echocardiography. LVEDV, LVESV, LVEF and plasma BNP concentrations were similar in the two groups. However, TDS was significantly higher (35 {+-} 8 vs. 28 {+-} 6, p < 0.005), the H/M ratio was significantly lower (1.57 {+-} 0.23 vs. 1.78 {+-} 0.20, p < 0.005), and the WR was significantly higher (60 {+-} 14% vs. 46 {+-} 12%, p < 0.001) in the LP-positive than in the LP-negative group. The average follow-up time was 4.5 years, and there were nine sudden deaths among the 56 patients (16.1%). In logistic regression analysis, the incidences of sudden death events were similar in those LP-negative with WR <50%, LP-negative with WR {>=}50% and LP-positive with WR <50% (0%, 10.0% and 14.3%, respectively), but was significantly higher (41.2%) in those LP-positive with WR {>=}50% (p < 0.01, p < 0.05, and p < 0.05, respectively). The present study demonstrated that the values of cardiac {sup 123}I-MIBG scintigraphic parameters

  3. Tratamento de insuficiência cardíaca com benazepril em cães com cardiomiopatia dilatada e endocardiose Treatment of congestive heart failure with benazepril in dogs with dilated cardiomyopathy and endocardiosis

    Directory of Open Access Journals (Sweden)

    P.M. Pereira

    2005-09-01

    Full Text Available Foram avaliados os efeitos clínicos do benazepril, um inibidor da enzima de conversão da angiotensina de ação prolongada, em cães com insuficiência cardíaca congestiva (ICC secundária à endocardiose de mitral ou cardiomiopatia dilatada. O medicamento foi administrado na dose de 0,25 a 0,5mg/kg/dia. Diuréticos, digitálicos e f��rmacos antiarrítmicos foram usados de acordo com a necessidade de cada paciente. Exames físico, radiográfico e eletrocardiográfico foram realizados nos dias 0, 7, 28 e 56. A gasometria arterial e a bioquímica sérica foram avaliadas nos dias 0 e 56. Os sinais de dispnéia e o estado geral dos pacientes melhoraram em todos os cães após o início do tratamento. Houve diminuição na freqüência da tosse e não houve alterações no eletrocardiograma, exceto pela diminuição na amplitude e na duração da onda P. Nenhum efeito colateral foi observado. Conclui-se que o benazepril é um inibidor da enzima de conversão da angiotensina, eficaz e bem tolerado no tratamento da ICC no cão.Clinical effects of benazepril, a long acting angiotensin-converting enzyme (ACEi, in dogs with naturally-occurring congestive heart failure (CHF caused by mitral endocardiosis or dilated cardiomyopathy were studied. The drug was given orally at a dose of 0.25 to 0.5mg/kg/day. Diuretics, digitalics, and antiarrhtyhmic drugs were given as needed. Physical, radiographic, and eletrocardiographic examination were performed at days 0, 7, 28, and 56. Serum biochemistry and arterial blood gases were obtained at days 0 and 56. Signs of dyspnea and general condition improved in all dogs. Cough decreased in frequency. The electrocardiogram did not change with benazepril use except for a decrease in P wave amplitude and duration. No adverse effects related to the use of benazepril were observed. Benazepril is an effective and well tolerated ACEi for the treatment of CHF in dogs.

  4. Acute extrahepatic infectious or inflammatory diseases are a cause of transient mosaic pattern on CT and MR imaging related to sinusoidal dilatation of the liver

    International Nuclear Information System (INIS)

    Ronot, Maxime; Vilgrain, Valerie; Kerbaol, Anne; Rautou, Pierre-Emmanuel; Valla, Dominique-Charles; Brancatelli, Giuseppe; Bedossa, Pierre; Cazals-Hatem, Dominique

    2016-01-01

    To report the association of a mosaic enhancement pattern on contrast-enhanced CT or MR imaging and hepatic sinusoidal dilatation (SD) with acute inflammatory conditions affecting extrahepatic organs. From 2007 to 2012, patients with acute inflammatory diseases who underwent contrast-enhanced CT and/or MRI of the liver with a mosaic enhancement pattern were selected. Clinico-biological and other imaging features were collected at diagnosis and during follow-up. Sixteen patients were included (15 women, median age 27 years; range 18-68). Five women (33 %) were receiving oral contraceptives. Acute inflammatory diseases included pyelonephritis (n = 10), pancreatitis (n = 2), pneumonia (n = 1), septicemia (n = 1), active Crohn's disease (n = 1), and infectious colitis (n = 1). Median white blood cell count was 13,250 cells/μL (range 11,500-18,000 cells/μL) and CRP level 94 mg/L (range 60-121 mg/L). Mosaic enhancement pattern was present in the whole liver and was prominent in the subcapsular areas. Four patients underwent liver biopsy confirming SD. Eleven patients underwent follow-up imaging showing normalized aspect in 9/11 patients after a median of 2 months. Acute diseases of extrahepatic organs, associated with a marked systemic inflammatory syndrome should be added to the list of conditions causing a reversible hepatic sinusoidal dilatation as manifested by a mosaic enhancement pattern on contrast-enhanced CT or MR imaging. (orig.)

  5. Atypical aortic coarctation as a cause of a cardiomyopathy

    OpenAIRE

    Alsemgeest, F.; Kamp, O.; Marcu, C.B.

    2015-01-01

    Atypical locations for aortic coarctation have been previously described. However, to our knowledge, no case has been described of a rapidly progressive dilated cardiomyopathy caused by an atypical coarctation, with a rapid normalisation of ventricular function after treatment.

  6. Acute pulmonary edema caused by takotsubo cardiomyopathy in a pregnant woman undergoing transvaginal cervical cerclage: A case report.

    Science.gov (United States)

    Lee, Jae-Young; Kwon, Hyun-Jung; Park, Sang-Wook; Lee, Yu-Mi

    2017-01-01

    The physiological changes associated with pregnancy may predispose pregnant women to pulmonary edema. Other known causes of pulmonary edema during pregnancy include tocolytic drugs, preeclampsia, eclampsia, and peripartum cardiomyopathy. We describe a rare case of pulmonary edema caused by takotsubo cardiomyopathy in a pregnant woman at 14 weeks of gestation who was undergoing emergency transvaginal cervical cerclage. Intraoperative chest radiography revealed severe pulmonary edema and echocardiography indicated moderate left ventricular dysfunction with akinesia of the mid to apical left ventricular wall segment, which is reflective of takotsubo cardiomyopathy. With early detection and appropriate management, the patient was stabilized in a relatively short period of time. Based on her clinical signs and symptoms, we suspect that the pulmonary edema was caused by takotsubo cardiomyopathy.

  7. Recombinant α-Klotho may be prophylactic and therapeutic for acute to chronic kidney disease progression and uremic cardiomyopathy.

    Science.gov (United States)

    Hu, Ming Chang; Shi, Mingjun; Gillings, Nancy; Flores, Brianna; Takahashi, Masaya; Kuro-O, Makoto; Moe, Orson W

    2017-05-01

    α-Klotho is highly expressed in the kidney, and its extracellular domain is cleaved and released into the circulation. Chronic kidney disease (CKD) is a state of α-Klotho deficiency, which exerts multiple negative systemic effects on numerous organs including the cardiovascular system. Since acute kidney injury (AKI) greatly escalates the risk of CKD development, we explored the effect of α-Klotho on prevention and treatment on post-AKI to CKD progression and cardiovascular disease. Therein, ischemia reperfusion injury-induced AKI was followed by early administration of recombinant α-Klotho or vehicle starting one day and continued for four days after kidney injury (CKD prevention protocol). A CKD model was generated by unilateral nephrectomy plus contralateral ischemia reperfusion injury. Late administration of α-Klotho in this model was started four weeks after injury and sustained for 12 weeks (CKD treatment protocol). The prevention protocol precluded AKI to CKD progression and protected the heart from cardiac remodeling in the post-AKI model. One important effect of exogenous α-Klotho therapy was the restoration of endogenous α-Klotho levels long after the cessation of exogenous α-Klotho therapy. The treatment protocol still effectively improved renal function and attenuated cardiac remodeling in CKD, although these parameters did not completely return to normal. In addition, α-Klotho administration also attenuated high phosphate diet-induced renal and cardiac fibrosis, and improved renal and cardiac function in the absence of pre-existing renal disease. Thus, recombinant α-Klotho protein is safe and efficacious, and might be a promising prophylactic or therapeutic option for prevention or retardation of AKI-to-CKD progression and uremic cardiomyopathy. Copyright © 2017 International Society of Nephrology. Published by Elsevier Inc. All rights reserved.

  8. A Small Molecule Inhibitor of Sarcomere Contractility Acutely Relieves Left Ventricular Outflow Tract Obstruction in Feline Hypertrophic Cardiomyopathy.

    Directory of Open Access Journals (Sweden)

    Joshua A Stern

    Full Text Available Hypertrophic cardiomyopathy (HCM is an inherited disease of the heart muscle characterized by otherwise unexplained thickening of the left ventricle. Left ventricular outflow tract (LVOT obstruction is present in approximately two-thirds of patients and substantially increases the risk of disease complications. Invasive treatment with septal myectomy or alcohol septal ablation can improve symptoms and functional status, but currently available drugs for reducing obstruction have pleiotropic effects and variable therapeutic responses. New medical treatments with more targeted pharmacology are needed, but the lack of preclinical animal models for HCM with LVOT obstruction has limited their development. HCM is a common cause of heart failure in cats, and a subset exhibit systolic anterior motion of the mitral valve leading to LVOT obstruction. MYK-461 is a recently-described, mechanistically novel small molecule that acts at the sarcomere to specifically inhibit contractility that has been proposed as a treatment for HCM. Here, we use MYK-461 to test whether direct reduction in contractility is sufficient to relieve LVOT obstruction in feline HCM. We evaluated mixed-breed cats in a research colony derived from a Maine Coon/mixed-breed founder with naturally-occurring HCM. By echocardiography, we identified five cats that developed systolic anterior motion of the mitral valve and LVOT obstruction both at rest and under anesthesia when provoked with an adrenergic agonist. An IV MYK-461 infusion and echocardiography protocol was developed to serially assess contractility and LVOT gradient at multiple MYK-461 concentrations. Treatment with MYK-461 reduced contractility, eliminated systolic anterior motion of the mitral valve and relieved LVOT pressure gradients in an exposure-dependent manner. Our findings provide proof of principle that acute reduction in contractility with MYK-461 is sufficient to relieve LVOT obstruction. Further, these studies

  9. Peripartum cardiomyopathy

    International Nuclear Information System (INIS)

    Velasquez V, Jorge E; Duque R, Mauricio

    2008-01-01

    Peripartum cardiomyopathy is a clinical entity with a variable frequency according to the zone of the study. It is characterized by a systolic dysfunction of the left ventricle and posterior appearance of heart failure symptoms that occur during the last month of pregnancy and the first post-partum months. Its etiology isn't still clear, but different theories are proposed based on inflammatory, infectious and autoimmune processes. Alterations related to oxidative stress that could largely explain this pathology were recently described. Its clinical presentation has a big similitude with all other causes of heart failure although atypical presentations have been described. Its diagnosis requires a high suspicion level and must be considered in any woman with symptoms of heart failure during the peripartum. The conventional treatment of chronic heart failure that includes beta-blockers, angiotensin converting enzyme inhibitors and diuretics, in addition to the advances in diagnosis and management of acute heart failure, allowed changing the history of the disease by lowering mortality and recovering systolic function of the left ventricle. Gestations posterior to the development of this entity will depend on the complete recovery of heart function without lowering the risk of recurrence. There still remain many questions to answer in areas like etiology, risk factors, treatment and prognosis markers that may allow to prevent and to manage in an appropriate and safe way both the mother and her son.

  10. Differential diagnosis at admission between Takotsubo cardiomyopathy and acute apical-anterior myocardial infarction in postmenopausal women.

    Science.gov (United States)

    Zorzi, Alessandro; Baritussio, Anna; ElMaghawry, Mohamed; Siciliano, Mariachiara; Migliore, Federico; Perazzolo Marra, Martina; Iliceto, Sabino; Corrado, Domenico

    2016-08-01

    Takotsubo cardiomyopathy (TTC) typically affects postmenopausal women and clinically presents with chest pain, ST-segment elevation, elevated cardiac enzymes and apical left ventricular (LV) wall motion abnormalities that mimic 'apical-anterior' acute myocardial infarction (AMI). This study assessed whether at-admission clinical evaluation helps in differential diagnosis between the two conditions. The study compared at-admission clinical, electrocardiographic (ECG) and echocardiographic findings of 31 women (median age 67 years, interquartile range (IQR) 62-76) with typical TTC and 30 women (median age 73 years, IQR 61-81) with apical-anterior AMI due to acute occlusion of the mid/distal left anterior descending coronary artery. Women with TTC significantly more often showed PR-segment depression (62% versus 3%, p<0.001), J-waves (26% versus 3%, p=0.03), maximum ST-segment elevation ⩽2 mm (84% versus 37%, p<0.001) and ST-segment elevation in lead II (42% versus 10%, p=0.01) than those with AMI. At multivariate analysis, PR-segment depression (odds ratio (OR)=37.2, 95% confidence interval (CI)=3.4-424, p=0.002) and maximum ST-segment elevation ⩽2 mm (OR=11.1, 95% CI=1.7-99.4, p=0.01) remained the only independent predictors of TTC and the co-existence of both parameters excluded AMI with a 100% specificity. The two groups did not differ with regard to age, first troponin-I value, echocardiographic LV ejection fraction and distribution of hypo/akinetic LV segments. At-admission electrocardiogram (but no clinical, laboratory and echocardiographic features) allows differential diagnosis between TTC and apical-anterior AMI in postmenopausal women. The combination of PR-segment depression and mild (⩽2 mm) ST-segment elevation predicted TTC with greater accuracy than traditional parameters such as localisation of ST-segment elevation and reciprocal ST-segment depression. © The European Society of Cardiology 2015.

  11. O papel do acúmulo de colágeno no interstício miocárdico na sobrevida dos pacientes com cardiomiopatia dilatada idiopática e chagásica The role of storage of interstitial myocardial collagen on the overlife rate of patients with idiopathic and chagasic dilated cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Vera Lopes Nunes

    2006-12-01

    Full Text Available OBJETIVO: Avaliar a correlação entre um marcador estrutural do miocárdio e a sobrevida dos pacientes com cardiomiopatia dilatada. MÉTODOS: Mediante realização da biópsia endomiocárdica e exame ecocardiográfico foram estudados 9 indivíduos sem doença estrutural miocárdica (controle e 45 pacientes com cardiomiopatia dilatada grave de etiologia idiopática (MCDI e chagásica (MCDC. Foi analisada a correlação entre a quantidade de colágeno miocárdico intersticial (FVCI e a sobrevida desses pacientes, se a FVCI diferia entre as etiologias, e se a fibrose interferia na função e geometria do miocárdio. RESULTADOS: Foi observado que a FVCI foi 15 vezes maior nos cardiomiopatas em relação ao grupo-controle, mas não diferiu em relação às MCDI e MCDC (*p OBJECTIVE: To find out whether there is a correlation between a myocardial structural marker and the overlife rate of patients with dilated cardiomyopathy. METHODS: Using endomyocardial biopsy and 2D-echocardiogram, we studied nine patients with no changes in myocardial structure (control and 45 patients with severe dilated cardiomyopathy of idiopathic etiology (IDCM and of Chagasic etiology (CDCM. We analyzed the correlation between the quantity of interstitial myocardial collagen (ICVF and the overlife rates of these patients. We also evaluated the difference in ICVF between these groups and whether fibrosis interfered on the geometry and function of the myocardium. RESULTS: We observed that ICVF was 15 times higher in cardiomyopathy patients than in the control group, but there was no difference in ICVF between CDCM and IDCM (*p < 0.001 patients. There was no correlation between ICVF and the overlife rate in cardiomyopathy patients (IDCM p = 0.249, and CDCM p = 0.587. We observed a significant correlation between ICVF and left ventricular ejection fraction (LVEF only for IDCM. There was no correlation between ICVF and left ventricular diastolic diameter in either etiology

  12. Update on Myocarditis and Inflammatory Cardiomyopathy: Reemergence of Endomyocardial Biopsy.

    Science.gov (United States)

    Dominguez, Fernando; Kühl, Uwe; Pieske, Burkert; Garcia-Pavia, Pablo; Tschöpe, Carsten

    2016-02-01

    Myocarditis is defined as an inflammatory disease of the heart muscle and is an important cause of acute heart failure, sudden death, and dilated cardiomyopathy. Viruses account for most cases of myocarditis or inflammatory cardiomyopathy, which could induce an immune response causing inflammation even when the pathogen has been cleared. Other etiologic agents responsible for myocarditis include drugs, toxic substances, or autoimmune conditions. In the last few years, advances in noninvasive techniques such as cardiac magnetic resonance have been very useful in supporting diagnosis of myocarditis, but toxic, infectious-inflammatory, infiltrative, or autoimmune processes occur at a cellular level and only endomyocardial biopsy can establish the nature of the etiological agent. Furthermore, after the generalization of immunohistochemical and viral genome detection techniques, endomyocardial biopsy provides a definitive etiological diagnosis that can lead to specific treatments such as antiviral or immunosuppressive therapy. Endomyocardial biopsy is not commonly performed for the diagnosis of myocarditis due to safety reasons, but both right- and left endomyocardial biopsies have very low complication rates when performed by experienced operators. This document provides a state-of-the-art review of myocarditis and inflammatory cardiomyopathy, with special focus on the role of endomyocardial biopsy to establish specific treatments. Copyright © 2015 Sociedad Española de Cardiología. Published by Elsevier España, S.L.U. All rights reserved.

  13. Arrhythmogenic right ventricular cardiomyopathy in a dog : case report

    Directory of Open Access Journals (Sweden)

    A.J. Möhr

    2000-07-01

    Full Text Available An 8-month-old Labrador retriever bitch was evaluated for sudden-onset, progressive abdominal distension. Physical examination revealed an exaggerated inspiratory effort, severe ascites, bilateral jugular vein distension, and hypokinetic femoral arterial pulses. Thoracic auscultation detected tachycardia with muffled heart sounds, without audible cardiac murmurs. Thoracic radiographs identified severe right ventricular enlargement and pleural effusion. The electrocardiogram was consistent with incomplete right bundle branch block or right ventricular enlargement. Echocardiography demonstrated severe right ventricular and atrial dilation, secondary tricuspid regurgitation, and thinning and hypocontractility of the right ventricular myocardium. Left heart chamber sizes were slightly decreased, with normal left ventricular contractility. Adiagnosis of arrhythmogenic right ventricular cardiomyopathy was reached, based on the characteristic clinical, electrocardiographic, radiographic and echocardiographic findings, and the exclusion of other causes of isolated right ventricular failure. Treatment effected good control of clinical signs, until acutely decompensated congestive right heart failure led to euthanasia after 4 months. Arrhythmogenic right ventricular cardiomyopathy is a well-described clinical entity in humans, and has previously been documented in 3 male dogs. The condition is characterised by progressive fibro-adipose replacement of right ventricular myocardium, while the left ventricle usually remains unaffected. It should be considered a differential diagnosis in any young dog presented with isolated right heart failure, syncope, or unexplained ventricular tachyarrhythmias. This article reports the 1st case of arrhythmogenic right ventricular cardiomyopathy in a female dog, and highlights its echocardiographic features.

  14. Paschke Dilations

    Directory of Open Access Journals (Sweden)

    Abraham Westerbaan

    2017-01-01

    Full Text Available In 1973 Paschke defined a factorization for completely positive maps between C*-algebras. In this paper we show that for normal maps between von Neumann algebras, this factorization has a universal property, and coincides with Stinespring's dilation for normal maps into B(H.

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

    International Nuclear Information System (INIS)

    Nishimura, Tsunehiko; Nagata, Seiki; Sakakibara, Hiroshi

    1988-01-01

    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. (author)

  16. Daunorubicin Down-Regulates the Expression of Stem Cell Markers and Factors Involved in Stem Cell Migration and Homing in Rat Heart in Subchronic but not Acute Cardiomyopathy.

    Science.gov (United States)

    Srankova, Jasna; Doka, Gabriel; Pivackova, Lenka; Mesarosova, Lucia; Kyselovic, Jan; Klimas, Jan; Krenek, Peter

    2016-11-01

    We tested the hypothesis that daunorubicin (DAU) cardiotoxicity alters expression of cytokines involved in stem cell migration and homing. Male Wistar rats were treated with daunorubicin to induce acute DAU cardiomyopathy (6 × 3 mg/kg, i.p., every 48 hr, DAU-A) or subchronic DAU cardiomyopathy (15 mg/kg, i.v., DAU-C). The left ventricle was catheterized. The animals were killed 48 hr (DAU-A) and 8 weeks (DAU-C) after the last dose of DAU. Expression of foetal genes (Nppa, Nppb), isomyosins (Myh6, Myh7), sources of oxidative stress (Abcb8, gp91phox), cytokines (Sdf-1, Cxcr4, Scf, Vegf, Hgf, Igf-1), markers of cardiac progenitor (c-kit, Atnx-1), endothelial progenitor (CD34, CD133) and mesenchymal (CD44, CD105) stem cells were determined by qRT-PCR in left ventricular tissue. Reduced body-weight, decreased left ventricular weight and function, and elevated Nppa, Nppb, Myh7 were observed in both models. Myh6 decreased only in DAU-C, which had a 35% mortality. Up-regulated gp91phox and down-regulated Abcb8 in DAU were present only in DAU-C where we observed markedly decreased expressions of Scf and Vegf as well as expressions of stem cell markers. Down-regulation of cytokines and stem cell markers may reflect impaired chemotaxis, migration and homing of stem cells and tissue repair in the heart in subchronic but not acute model of DAU cardiomyopathy. © 2016 Nordic Association for the Publication of BCPT (former Nordic Pharmacological Society).

  17. Determinants of Thyrotoxic Cardiomyopathy Recovery

    Directory of Open Access Journals (Sweden)

    Lucia Oliveros-Ruiz

    2013-01-01

    Full Text Available The purpose was to evaluate the effect of the disease duration prior to treatment, thyroid hormones level, or both on the reversibility of dilated cardiomyopathy. Between January 2006 and December 2010, a longitudinal study with a 6 months follow-up was carried on. One hundred and seventy patients with hyperthyroidism were referred to the cardiologist, and 127 had a 6 months followup after antithyroid treatment and were evaluated by echocardiography. Dilated cardiomyopathy reversibility criteria were established according to echocardiographic parameters. Complete reversibility existed when all parameters were met, partial reversibility when LVEF was ≥55% plus two or three other parameters, and no reversibility when LVEF was ≤55% regardless of other parameters. The results showed that echocardiography parameters related to the regression of myocardial mass were associated with a disease duration shorter than 10.38 months. This was the main predictive variable for reversal of dilated cardiomyopathy, followed by β-blocker treatment, and the last predictive variable was the serum level of free triiodothyronine. This study showed that the effect on the myocardium related to thyrotoxicosis was associated with the disease duration before treatment.

  18. Stem Cell-Based Therapies in Chagasic Cardiomyopathy.

    Science.gov (United States)

    de Carvalho, Antonio Carlos Campos; Carvalho, Adriana Bastos

    2015-01-01

    Chagas disease is caused by Trypanosoma cruzi and can lead to a dilated cardiomyopathy decades after the prime infection by the parasite. As with other dilated cardiomyopathies, conventional pharmacologic therapies are not always effective and as heart failure progresses patients need heart transplantation. Therefore alternative therapies are highly desirable and cell-based therapies have been investigated in preclinical and clinical studies. In this paper we review the main findings of such studies and discuss future directions for stem cell-based therapies in chronic chagasic cardiomyopathy.

  19. MR imaging in cardiomyopathies; MR-tomographische Diagnostik von Kardiomyopathien

    Energy Technology Data Exchange (ETDEWEB)

    Miller, S. [Radiologische Universitaetsklinik Tuebingen (Germany); Riessen, R. [Tuebingen Univ. (Germany). Medizinische Klinik

    2005-11-15

    According to the WHO classification, cardiomyopathies are a group of diseases which are associated with myocardial dysfunction and can be classified either as primary or secondary cardiomyopathies. Genetic disorders have been identified in certain primary cardiomyopathies, however often the etiology remains unknown. The term ''secondary cardiomyopathy'' is used to specify diseases with the clinical indications of a cardiomyopathy, but can be attributed to a certain pathophysiological mechanism such as exposure to toxic substances, metabolic syndromes or systemic diseases. Based on morphological and functional criteria, primary cardiomyopathies are divided into dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), arrhythmogenic right ventricular cardiomyopathy (ARVC) and restrictive cardiomyopathy (RCM). During the last two decades MR imaging has emerged to a well established diagnostic tool for the understanding and treatment of cardiomyopathies. Morphological and functional information can be achieved with a high level of accuracy and reproducibility. Tissue alteration of the myocardium can be detected assessing regional contrast enhancement, T1- and T2-signal intensities and chemical shift phenomena. This article describes characteristic aspects of MR imaging for the diagnosis of primary and secondary cardiomyopathies. (orig.)

  20. Peripartum Cardiomyopathy

    African Journals Online (AJOL)

    pregnant women. The pathophysiology is poorly understood. Echocardiography is central to diagnosis of PPCM and effective treatment monitoring in patients of PPCM. The outcome ... Keywords: Cardiomyopathy, Echocardiography, Peripartum, Pregnancy ... complicated by pre‑eclampsia, gestational hypertension,.

  1. Restrictive Cardiomyopathy

    Science.gov (United States)

    ... can be mistaken for a condition called constrictive pericarditis. This condition causes the sac-like membrane around ... inflamed and thickened. Surgery can usually correct constrictive pericarditis. On the other hand, restrictive cardiomyopathy cannot be ...

  2. Substituição da valva mitral com tração dos músculos papilares em pacientes com miocardiopatia dilatada Mitral valve replacement with chordae tendineae preservation, traction and fixation in end-stage dilated cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Fabio Antonio Gaiotto

    2007-03-01

    Full Text Available OBJETIVO: Avaliar a geometria e a função do ventrículo esquerdo (VE após a troca mitral com tração e fixação dos papilares, em portadores de insuficiência cardíaca terminal com insuficiência mitral secundária. MÉTODO: Dos 20 pacientes avaliados, 70% eram homens, com idade média de 50,2 anos e 55% recebiam inotrópicos. A fração de ejeção (FEVE foi menor que 30% em todos; 85% estavam em classe funcional (CF IV. Dezoito receberam próteses de pericárdio bovino e dois, mecânicas. Os períodos considerados foram: 3, 6, 12 e 18 meses. As variáveis consideradas: volume sistólico do VE (VS, a FEVE, os diâmetros sistólico e diastólico finais (DSF e DDF e os volumes sistólico e diastólico finais (VSF e VDF. No estudo estatístico, empregou-se da análise de variância (AV e o teste de Friedmann (F. A sobrevida foi aferida pelo método de Kaplan-Meyer. RESULTADOS: Dois (10% faleceram no período imediato. A sobrevida no primeiro ano foi de 85%, no segundo, 44%, no terceiro, 44%, no quarto, 44% e no quinto, 44%. A comparação entre pré e 3 meses, empregando-se a AV, não revelou alteração significativa para o VS (p=0,086. Houve acréscimo da FEVE (p=0,008 e decréscimo do DDF (p=0,038; do DSF (p=0,008; do VDF (p=0,029 e do VSF (p=0,009. Os momentos pré, 3 e 6 meses, com o teste F, não revelaram alterações. Entre os momentos pré, 3 meses e final, empregando-se a AV, não houve significância. CONCLUSÃO: Há melhora da FEVE, dos VDF, VSF, DDF e DSF; até o terceiro mês. A partir de então, as variáveis permanecem estáveis.OBJECTIVE: This study aimed at evaluating results of mitral valve replacement using a new technique of complete chordae tendineae adjustment for left ventricular remodeling. METHODS: Twenty end-stage idiopathic dilated cardiomyopathy patients with severe functional mitral valve regurgitation underwent mitral valve replacement. Seventeen (85% were in functional class IV. Both anterior and posterior

  3. Clínica de cães com cardiomiopatia dilatada idiopática, tratados ou não com carvedilol Clinic of dogs with dilated cardiomyopathy (DCM treated or not by carvedilol

    Directory of Open Access Journals (Sweden)

    Moacir Leomil Neto

    2011-04-01

    -arrhythmics. Carvedilol is a third generation non-selective β-blocker which blocks equally and competitively (β1, β2 and α1 receptors. Produces an evident peripheral vasodilation, exerts anti-oxidative effects, removing free radicals of oxygen and preventing lipidic peroxydation of cardiac membranes, and the loss of myocytes and arrhythmias, as well as reducing mortality rate in human patients. The aim of the present study was to evaluate by physical examination, electrocardiography, radiography, and echocardiography the evolution of dogs with dilated cardiomyopathy (DCM treated by conventional therapy associated to carvedilol. Forty-nine dogs with DCM were divided in two groups: group NT: treated with conventional therapy, and group T: treated with conventional therapy associated to carvedilol. The animals were submitted to clinical and complementary examinations during one year. The results demonstrated that carvedilol therapy presented good tolerability on the dose of 0.3mg kg-1 each 12 hours, prolonged lifetime of the dogs in 30.9%, did not alter systolic or diastolic pressure, reduced heart frequency after three weeks of treatment, significantly enhanced shortening and ejection fractions after six months of treatment, did not promote radiographic or E-septum distance alterations, decreased patients letality, as demonstrated by improvement of clinical score and functional class (heart failure according to NYHA of the animals, obtained three weeks after the beginning of cavedilol therapy.

  4. A Case of Acute ST-Segment Elevation Myocardial Infarction Mimicking Stress Induced Cardiomyopathy; Demonstration of Typical Echocardiographic Finding Correlated with Unusual Distribution of Left Anterior Descending Coronary Artery

    OpenAIRE

    Shin, Sung Kyun; Jin, Seon-Ah; Park, Yong Kyu; Park, Jae-Hyeong

    2010-01-01

    Stress-induced cardiomyopathy (SCMP) is diagnosed in 1-2% of patients presenting with symptoms suggestive of acute coronary syndrome. Because of sharing many common clinical features with SCMP, acute ST-segment elevation myocardial infarction (STEMI) can be misdiagnosed as SCMP. However, it can be associated with fatal outcome of the patient. Also, diagnosis of SCMP seems to be always challenging to clinicians, especially in the decision of taking coronary angiography which is still invasive ...

  5. Takotsubo (Stress Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Justin J Hourmozdi

    2017-01-01

    Full Text Available History of present illness: A 59-year-old male presented to the emergency department in shock from pneumonia. The patient was initially afebrile, pulse rate 120 beats per minute, blood pressure 117/69 mmHg, respiratory rate 42 breaths per minute, pulse oximetry 94% on a non-rebreather mask and a lactate of 14 mmol/L. He became progressively more hypotensive despite fluid resuscitation and was started on norepinephrine. Shortly after, the patient developed torsades de pointes that was terminated with intravenous push magnesium. His initial ECG had shown sinus tachycardia; however, repeat ECG showed ST-segment elevation in the inferolateral leads and the patient had troponin I elevation that peaked at 16 ng/mL. Significant findings: Bedside echocardiography showed the findings consistent with Takotsubo cardiomyopathy. Echocardiographic images are shown in subxiphoid (A and apical four chamber (B views. Note the apical ballooning appearance (asterisk of the left ventricle (LV. Discussion: Formal echocardiography confirmed features classic for Takotsubo (stress cardiomyopathy, including globally depressed left ventricle (LV ejection fraction, systolic apical ballooning appearance of the LV, mid and apical segments of LV depression, and hyper kinesis of the basal walls. Takotsubo cardiomyopathy is a syndrome known to cause ST-segment elevation on ECG, transient LV dysfunction, and dysrhythmia in the absence of acute obstructive coronary disease. There is no consensus on diagnostic criteria; however, these criteria are commonly used: 1 transient hypokinesis, akinesis, or dyskinesis in the LV mid-segments with or without apical involvement; regional wall motion abnormalities that extend beyond a single epicardial vascular distribution; and frequently, but not always, a stressful trigger 2 the absence of acute coronary disease or angiographic evidence of acute plaque rupture 3 new ECG abnormalities (ST-segment elevation and/or T-wave inversion or modest

  6. Sincronia ventricular em portadores de miocardiopatia dilatada e indivíduos normais: avaliação através da ventriculografia radioisotópica Ventricular synchrony in patients with dilated cardiomyopathy and normal individuals: assessment by radionuclide ventriculography

    Directory of Open Access Journals (Sweden)

    Simone Cristina S. Brandão

    2007-05-01

    Full Text Available OBJETIVO: Estabelecer parâmetros de sincronia intra- e interventricular em indivíduos normais e compará-los aos de pacientes com miocardiopatia dilatada com e sem distúrbios de condução ao eletrocardiograma (ECG. MÉTODOS: Três grupos de pacientes foram incluídos no estudo: 18 indivíduos (G1 sem cardiopatia e com ECG normal (52+/-12 anos, 29% masculinos; 50 portadores de miocardiopatia dilatada e disfunção ventricular esquerda grave, sendo 20 pacientes (G2 com QRS 120 ms (57+/-12 anos, 60% masculinos. Todos foram submetidos à ventriculografia radioisotópica (VR. Para avaliar dissincronia intraventricular esquerda foi estudada a largura do histograma de fase e para avaliar dissincronia interventricular foi medida a diferença da média do ângulo de fase entre o ventrículo direito e o esquerdo (DifDE. RESULTADOS: As frações de ejeção do ventrículo esquerdo (FEVEs foram: 62±6% (G1, 27±6% (G2 e 22±7% (G3 e do VD foram: 46 ± 4% (G1, 38±9%(G2 e 37±9% (G3. A avaliação da largura do histograma de fase foi de: 89±18 ms (G1, 203±54 ms (G2 e 312±130 ms (G3, pOBJECTIVE: To establish the parameters of intra- and interventricular synchrony in normal individuals and to compare them with patients with dilated cardiomyopathy with and without conduction disorders shown in the electrocardiogram (ECG examination. METHODS: Three groups of patients were included in this study: 18 individuals (G1 with no cardiomyopathy and with a normal ECG (52±12 years, 29% male; 50 patients with dilated cardiomyopathy and severe left ventricular dysfunction, with 20 patients (G2 presenting QRS 120ms (57±12 years, 60% male. All patients underwent RV. Evaluation of left intraventricular dyssynchrony was carried out with the measurement of the phase histogram width and interventricular dyssynchrony was evaluated by the difference of the mean phase angle between the right and left ventricles (RLDif. RESULTS: Left ventricle ejection fractions (LVEFs were

  7. Intravenously Delivered Mesenchymal Stem Cells: Systemic Anti-Inflammatory Effects Improve Left Ventricular Dysfunction in Acute Myocardial Infarction and Ischemic Cardiomyopathy.

    Science.gov (United States)

    Luger, Dror; Lipinski, Michael J; Westman, Peter C; Glover, David K; Dimastromatteo, Julien; Frias, Juan C; Albelda, M Teresa; Sikora, Sergey; Kharazi, Alex; Vertelov, Grigory; Waksman, Ron; Epstein, Stephen E

    2017-05-12

    Virtually all mesenchymal stem cell (MSC) studies assume that therapeutic effects accrue from local myocardial effects of engrafted MSCs. Because few intravenously administered MSCs engraft in the myocardium, studies have mainly utilized direct myocardial delivery. We adopted a different paradigm. To test whether intravenously administered MSCs reduce left ventricular (LV) dysfunction both post-acute myocardial infarction and in ischemic cardiomyopathy and that these effects are caused, at least partly, by systemic anti-inflammatory activities. Mice underwent 45 minutes of left anterior descending artery occlusion. Human MSCs, grown chronically at 5% O 2 , were administered intravenously. LV function was assessed by serial echocardiography, 2,3,5-triphenyltetrazolium chloride staining determined infarct size, and fluorescence-activated cell sorting assessed cell composition. Fluorescent and radiolabeled MSCs (1×10 6 ) were injected 24 hours post-myocardial infarction and homed to regions of myocardial injury; however, the myocardium contained only a small proportion of total MSCs. Mice received 2×10 6 MSCs or saline intravenously 24 hours post-myocardial infarction (n=16 per group). At day 21, we harvested blood and spleens for fluorescence-activated cell sorting and hearts for 2,3,5-triphenyltetrazolium chloride staining. Adverse LV remodeling and deteriorating LV ejection fraction occurred in control mice with large infarcts (≥25% LV). Intravenous MSCs eliminated the progressive deterioration in LV end-diastolic volume and LV end-systolic volume. MSCs significantly decreased natural killer cells in the heart and spleen and neutrophils in the heart. Specific natural killer cell depletion 24 hours pre-acute myocardial infarction significantly improved infarct size, LV ejection fraction, and adverse LV remodeling, changes associated with decreased neutrophils in the heart. In an ischemic cardiomyopathy model, mice 4 weeks post-myocardial infarction were

  8. Exome Sequencing Identifies a Novel LMNA Splice-Site Mutation and Multigenic Heterozygosity of Potential Modifiers in a Family with Sick Sinus Syndrome, Dilated Cardiomyopathy, and Sudden Cardiac Death.

    Directory of Open Access Journals (Sweden)

    Michael V Zaragoza

    Full Text Available The goals are to understand the primary genetic mechanisms that cause Sick Sinus Syndrome and to identify potential modifiers that may result in intrafamilial variability within a multigenerational family. The proband is a 63-year-old male with a family history of individuals (>10 with sinus node dysfunction, ventricular arrhythmia, cardiomyopathy, heart failure, and sudden death. We used exome sequencing of a single individual to identify a novel LMNA mutation and demonstrated the importance of Sanger validation and family studies when evaluating candidates. After initial single-gene studies were negative, we conducted exome sequencing for the proband which produced 9 gigabases of sequencing data. Bioinformatics analysis showed 94% of the reads mapped to the reference and identified 128,563 unique variants with 108,795 (85% located in 16,319 genes of 19,056 target genes. We discovered multiple variants in known arrhythmia, cardiomyopathy, or ion channel associated genes that may serve as potential modifiers in disease expression. To identify candidate mutations, we focused on ~2,000 variants located in 237 genes of 283 known arrhythmia, cardiomyopathy, or ion channel associated genes. We filtered the candidates to 41 variants in 33 genes using zygosity, protein impact, database searches, and clinical association. Only 21 of 41 (51% variants were validated by Sanger sequencing. We selected nine confirmed variants with minor allele frequencies G, a novel heterozygous splice-site mutation as the primary mutation with rare or novel variants in HCN4, MYBPC3, PKP4, TMPO, TTN, DMPK and KCNJ10 as potential modifiers and a mechanism consistent with haploinsufficiency.

  9. Cardiorespiratory fitness modulates the acute flow-mediated dilation response following high-intensity but not moderate-intensity exercise in elderly men.

    Science.gov (United States)

    Bailey, Tom G; Perissiou, Maria; Windsor, Mark; Russell, Fraser; Golledge, Jonathan; Green, Daniel J; Askew, Christopher D

    2017-05-01

    Impaired endothelial function is observed with aging and in those with low cardiorespiratory fitness (V̇o 2peak ). Improvements in endothelial function with exercise training are somewhat dependent on the intensity of exercise. While the acute stimulus for this improvement is not completely understood, it may, in part, be due to the flow-mediated dilation (FMD) response to acute exercise. We examined the hypothesis that exercise intensity alters the brachial (systemic) FMD response in elderly men and is modulated by V̇o 2peak Forty-seven elderly men were stratified into lower (V̇o 2peak = 24.3 ± 2.9 ml·kg -1 ·min -1 ; n = 27) and higher fit groups (V̇o 2peak = 35.4 ± 5.5 ml·kg -1 ·min -1 ; n = 20) after a test of cycling peak power output (PPO). In randomized order, participants undertook moderate-intensity continuous exercise (MICE; 40% PPO) or high-intensity interval cycling exercise (HIIE; 70% PPO) or no-exercise control. Brachial FMD was assessed at rest and 10 and 60 min after exercise. FMD increased after MICE in both groups {increase of 0.86% [95% confidence interval (CI), 0.17-1.56], P = 0.01} and normalized after 60 min. In the lower fit group, FMD was reduced after HIIE [reduction of 0.85% (95% CI, 0.12-1.58), P = 0.02] and remained decreased at 60 min. In the higher fit group, FMD was unchanged immediately after HIIE and increased after 60 min [increase of 1.52% (95% CI, 0.41-2.62), P exercise control, FMD was reduced in both groups after 60 min ( P = 0.05). Exercise intensity alters the acute FMD response in elderly men and V̇o 2peak modulates the FMD response following HIIE but not MICE. The sustained decrease in FMD in the lower fit group following HIIE may represent a signal for vascular adaptation or endothelial fatigue. NEW & NOTEWORTHY This study is the first to show that moderate-intensity continuous cycling exercise increased flow-mediated dilation (FMD) transiently before normalization of FMD after 1 h, irrespective of

  10. Dilating Eye Drops

    Science.gov (United States)

    ... Frequently Asked Questions Español Condiciones Chinese Conditions Dilating Eye Drops En Español Read in Chinese What are dilating eye drops? Dilating eye drops contain medication to enlarge ( ...

  11. A Case of Acute ST-Segment Elevation Myocardial Infarction Mimicking Stress Induced Cardiomyopathy; Demonstration of Typical Echocardiographic Finding Correlated with Unusual Distribution of Left Anterior Descending Coronary Artery.

    Science.gov (United States)

    Shin, Sung Kyun; Jin, Seon-Ah; Park, Yong Kyu; Park, Jae-Hyeong

    2010-09-01

    Stress-induced cardiomyopathy (SCMP) is diagnosed in 1-2% of patients presenting with symptoms suggestive of acute coronary syndrome. Because of sharing many common clinical features with SCMP, acute ST-segment elevation myocardial infarction (STEMI) can be misdiagnosed as SCMP. However, it can be associated with fatal outcome of the patient. Also, diagnosis of SCMP seems to be always challenging to clinicians, especially in the decision of taking coronary angiography which is still invasive and even risky. Here, we present a case with acute STEMI mimicking SCMP as a result of anatomical variation of coronary circulation. In this patient, prompt and early coronary angiography and stent implantation was very helpful.

  12. Cirrhotic cardiomyopathy

    DEFF Research Database (Denmark)

    Wiese, Signe; Hove, Jens D; Bendtsen, Flemming

    2014-01-01

    Cirrhosis is known to cause alterations in the systemic haemodynamic system. Cirrhotic cardiomyopathy designates a cardiac dysfunction that includes impaired cardiac contractility with systolic and diastolic dysfunction, as well as electromechanical abnormalities in the absence of other known...... causes of cardiac disease. This condition is primarily revealed by inducing physical or pharmacological stress, but echocardiography is excellent at revealing diastolic dysfunction and might also be used to detect systolic dysfunction at rest. Furthermore, measurement of circulating levels of cardiac...

  13. RESTRICTIVE CARDIOMYOPATHY AND SECONDARY CONGESTIVE HEART FAILURE IN A MCDOWELL'S CARPET PYTHON (MORELIA SPILOTA MCDOWELLI).

    Science.gov (United States)

    Schilliger, Lionel; Chetboul, Valérie; Damoiseaux, Cécile; Nicolier, Alexandra

    2016-12-01

    Echocardiography is an established and noninvasive diagnostic tool used in herpetologic cardiology. Various cardiac lesions have been previously described in reptiles with the exception of restrictive cardiomyopathy. In this case report, restrictive cardiomyopathy and congestive heart failure associated with left atrial and sinus venosus dilation were diagnosed in a 2-yr-old captive lethargic McDowell's carpet python ( Morelia spilota mcdowelli), based on echocardiographic, Doppler, and histopathologic examinations. This cardiomyopathy was also associated with thrombosis within the sinus venosus.

  14. Simultaneous interstitial pneumonitis and cardiomyopathy induced by venlafaxine

    Directory of Open Access Journals (Sweden)

    Pedro Gonçalo Ferreira

    2014-06-01

    Full Text Available Venlafaxine is a serotonin-norepinephrine reuptake inhibitor used as an antidepressant. Interindividual variability and herb-drug interactions can lead to drug-induced toxicity. We report the case of a 35-year-old female patient diagnosed with synchronous pneumonitis and acute cardiomyopathy attributed to venlafaxine. The patient sought medical attention due to dyspnea and dry cough that started three months after initiating treatment with venlafaxine for depression. The patient was concomitantly taking Centella asiatica and Fucus vesiculosus as phytotherapeutic agents. Chest CT angiography and chest X-ray revealed parenchymal lung disease (diffuse micronodules and focal ground-glass opacities and simultaneous dilated cardiomyopathy. Ecocardiography revealed a left ventricular ejection fraction (LVEF of 21%. A thorough investigation was carried out, including BAL, imaging studies, autoimmune testing, right heart catheterization, and myocardial biopsy. After excluding other etiologies and applying the Naranjo Adverse Drug Reaction Probability Scale, a diagnosis of synchronous pneumonitis/cardiomyopathy associated with venlafaxine was assumed. The herbal supplements taken by the patient have a known potential to inhibit cytochrome P450 enzyme complex, which is responsible for the metabolization of venlafaxine. After venlafaxine discontinuation, there was rapid improvement, with regression of the radiological abnormalities and normalization of the LVEF. This was an important case of drug-induced cardiopulmonary toxicity. The circumstantial intake of inhibitors of the CYP2D6 isoenzyme and the presence of a CYP2D6 slow metabolism phenotype might have resulted in the toxic accumulation of venlafaxine and the subsequent clinical manifestations. Here, we also discuss why macrophage-dominant phospholipidosis was the most likely mechanism of toxicity in this case.

  15. Arrhythmogenic cardiomyopathy in a patient with a rare loss-of-function KCNQ1 mutation.

    Science.gov (United States)

    Xiong, Qinmei; Cao, Qing; Zhou, Qiongqiong; Xie, Jinyan; Shen, Yang; Wan, Rong; Yu, Jianhua; Yan, Sujuan; Marian, Ali J; Hong, Kui

    2015-01-23

    Ventricular tachycardia (VT) is a common manifestation of advanced cardiomyopathies. In a subset of patients with dilated cardiomyopathy, VT is the initial and the cardinal manifestation of the disease. The molecular genetic basis of this subset of dilated cardiomyopathy is largely unknown. We identified 10 patients with dilated cardiomyopathy who presented with VT and sequenced 14 common causal genes for cardiomyopathies and arrhythmias. Functional studies included cellular patch clamp, confocal microscopy, and immunoblotting. We identified nonsynonymous variants in 4 patients, including a rare missense p.R397Q mutation in the KCNQ1 gene in a 60-year-old man who presented with incessant VT and had mild cardiac dysfunction. The p.R397Q mutation was absent in an ethnically matched control group, affected a conserved amino acid, and was predicted by multiple algorithms to be pathogenic. Co-expression of the mutant KCNQ1 with its partner unit KCNE1 was associated with reduced tail current density of slowly activating delayed rectifier K(+) current (IKs). The mutation reduced membrane localization of the protein. Dilated cardiomyopathy with an initial presentation of VT may be a forme fruste of arrhythmogenic cardiomyopathy caused by mutations in genes encoding the ion channels. The findings implicate KCNQ1 as a possible causal gene for arrhythmogenic cardiomyopathy. © 2015 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley Blackwell.

  16. Fatores prognósticos e evolução da função ventricular em 5 anos de seguimento da ventriculectomia parcial esquerda no tratamento da cardiomiopatia dilatada Prognostic factors in the follow-up of patients with idiopathic dilated cardiomyopathy submitted to partial left ventriculectomy

    Directory of Open Access Journals (Sweden)

    Luiz Felipe P. MOREIRA

    2001-12-01

    Full Text Available OBJETIVO: Nesta investigação, os resultados tardios da ventriculectomia parcial esquerda, associada à correção da insuficiência das valvas atrioventriculares, foram estudados em 43 pacientes portadores de cardiomiopatia dilatada. CASUÍSTICA E MÉTODOS: Os pacientes estavam em classe funcional III (18 ou IV (25 no pré-operatório, sendo que 7 pacientes foram operados na vigência de choque cardiogênico. A redução cirúrgica do volume do ventrículo esquerdo (VE foi associada à anuloplastia mitral em 32 pacientes e à substituição daquela valva em 3. Em 10 pacientes, também foi realizada plastia de valva tricúspide. Doze pacientes foram submetidos ao implante de desfibriladores automáticos. RESULTADOS: Ocorreram 9 (20,9% óbitos hospitalares. O tempo de seguimento pós-operatório variou entre dois e 68 meses, com média de 34,2 meses. Aos seis meses de seguimento, 8 pacientes estavam em classe funcional I, 13 em classe II, 3 em classe III e 1 em classe IV (pOBJECTIVE: Partial left ventriculectomy has been performed in patients with severe cardiomyopathies. The purpose of this investigation is to document the clinical effects of this procedure, associated with mitral insufficiency correction, in 43 patients with idiopathic dilated cardiomyopathy. METHODS: Eighteen patients were in New York Heart Association class III and 25 were in persistent class IV. Seven of these patients were operated on in cardiogenic shock. The procedure was associated with mitral anuloplasty in 32 patients and with mitral replacement in three. Ten patients were also submitted to De Vega tricuspid valve anuloplasty. Automatic cardioverter-defibrillators were implanted in 12 patients. RESULTS: Nine (20.9% patients died during the hospital period. The follow-up time ranged from two to 57 months, with a mean of 28.3 months. At six months of follow-up, eight patients were in functional class I, 13 patients in class II, three patients in class III e one patient

  17. Reversible catecholamine-induced cardiomyopathy due to pheochromocytoma: case report.

    Science.gov (United States)

    Satendra, Milan; de Jesus, Cláudia; Bordalo e Sá, Armando L; Rosário, Luís; Rocha, José; Bicha Castelo, Henrique; Correia, Maria José; Nunes Diogo, António

    2014-03-01

    Pheochromocytoma is a tumor originating from chromaffin tissue. It commonly presents with symptoms and signs of catecholamine excess, such as hypertension, tachycardia, headache and sweating. Cardiovascular manifestations include catecholamine-induced cardiomyopathy, which may present as severe left ventricular dysfunction and congestive heart failure. We report a case of pheochromocytoma which was diagnosed following investigation of dilated cardiomyopathy. We highlight the dramatic symptomatic improvement and reversal of cardiomyopathy, with recovery of left ventricular function after treatment. Copyright © 2013 Sociedade Portuguesa de Cardiologia. Published by Elsevier España. All rights reserved.

  18. Two different cardiomyopathies in a single patient : hypertrophic cardiomyopathy and left ventricular noncompaction.

    Science.gov (United States)

    Sunbul, M; Ozben, B; Mutlu, B

    2013-05-01

    Hypertrophic cardiomyopathy is a complex and relatively common genetic disorder characterized by left ventricular (LV) hypertrophy, usually associated with a nondilated and hyperdynamic chamber with heterogeneous phenotypic expression and clinical course. On the other hand, LV noncompaction is an uncommon cardiomyopathy characterized by the persistence of fetal myocardium with a pattern of prominent trabecular meshwork and deep intertrabecular recesses, systolic dysfunction, and LV dilatation. We report a 29-year-old man with these two different inherent conditions. Our case raises the possibility of a genetic mutation common to these two clinical entities or different gene mutations existing in the same individual.

  19. Implantable cardioverter defibrillator therapy in young patients with cardiomyopathies and channelopathies: a single Italian centre experience.

    Science.gov (United States)

    Migliore, Federico; Silvano, Maria; Zorzi, Alessandro; Bertaglia, Emanuele; Siciliano, Mariachiara; Leoni, Loira; De Franceschi, Pietro; Iliceto, Sabino; Corrado, Domenico

    2016-07-01

    This study was designed to prospectively evaluate the risk-benefit ratio of implantable cardioverter defibrillator (ICD) therapy in young patients with cardiomyopathies and channelopathies. The study population included 96 consecutive patients [68 men, median age 27 (22-32) years] with cardiomyopathies, such as arrhythmogenic right ventricular cardiomyopathy (n = 35), dilated cardiomyopathy (n = 17), hypertrophic cardiomyopathy (n = 15), Brugada syndrome (n = 14), idiopathic ventricular fibrillation (n = 5), left ventricular noncompaction (n = 4), long-QT syndrome (n = 4) and short-QT syndrome (n = 2), who were 18-35 years old at the time of ICD implantation. During a mean follow-up of 72.6 ± 53.3 months, one patient with end-stage hypertrophic cardiomyopathy died because of acute heart failure, and 11 patients underwent orthotopic heart transplantation. Twenty patients (20.8%) had a total of 38 appropriate ICD interventions (4%/year), and 26 patients (27.1%) experienced a total of 49 adverse ICD-related events (5.4%/year), including 23 inappropriate ICD interventions occurring in nine patients (9.4%) and 26 device-related complications requiring surgical revision occurring in 20 patients (20.8%). Lead failure/fracture requiring lead extraction was the most common complication (n = 9). A threshold for ICD therapy less than 300 ms was associated with a borderline significant lower probability of inappropriate ICD interventions (hazard ratio = 0.2; 95% confidence interval 0.02-1.2; P = 0.07), whereas underweight status was an independent predictor of device-related complications (hazard ratio = 5.4; 95% confidence interval 1.5-19.4; P = 0.01). In young patients with cardiomyopathies and channelopathies, ICD therapy provided life-saving protection by effectively terminating life-threatening ventricular arrhythmias. However, because ICD-related adverse events are common, the risk/benefit ratio should be

  20. Metabolic imaging of patients with cardiomyopathy

    International Nuclear Information System (INIS)

    Geltman, E.M.

    1991-01-01

    The cardiomyopathies comprise a diverse group of illnesses that can be characterized functionally by several techniques. However, the delineation of derangements of regional perfusion and metabolism have been accomplished only relatively recently with positron emission tomography (PET). Regional myocardial accumulation and clearance of 11C-palmitate, the primary myocardial substrate under most conditions, demonstrate marked spatial heterogeneity when studied under fasting conditions or with glucose loading. PET with 11C-palmitate permits the noninvasive differentiation of patients with nonischemic from ischemic dilated cardiomyopathy, since patients with ischemic cardiomyopathy demonstrate large zones of intensely depressed accumulation of 11C-palmitate, probably reflecting prior infarction. Patients with hypertrophic cardiomyopathy and Duchenne's muscular dystrophy demonstrate relatively unique patterns of myocardial abnormalities of perfusion and metabolism. The availability of new tracers and techniques for the evaluation of myocardial metabolism (11C-acetate), perfusion (H2(15)O), and autonomic tone (11-C-hydroxyephedrine) should facilitate further understanding of the pathogenesis of the cardiomyopathies

  1. 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.

  2. Smooth muscle LDL receptor-related protein-1 deletion induces aortic insufficiency and promotes vascular cardiomyopathy in mice.

    Directory of Open Access Journals (Sweden)

    Joshua E Basford

    Full Text Available Valvular disease is common in patients with Marfan syndrome and can lead to cardiomyopathy. However, some patients develop cardiomyopathy in the absence of hemodynamically significant valve dysfunction, suggesting alternative mechanisms of disease progression. Disruption of LDL receptor-related protein-1 (Lrp1 in smooth muscle cells has been shown to cause vascular pathologies similar to Marfan syndrome, with activation of smooth muscle cells, vascular dysfunction and aortic aneurysms. This study used echocardiography and blood pressure monitoring in mouse models to determine whether inactivation of Lrp1 in vascular smooth muscle leads to cardiomyopathy, and if so, whether the mechanism is a consequence of valvular disease. Hemodynamic changes during treatment with captopril were also assessed. Dilation of aortic roots was observed in young Lrp1-knockout mice and progressed as they aged, whereas no significant aortic dilation was detected in wild type littermates. Diastolic blood pressure was lower and pulse pressure higher in Lrp1-knockout mice, which was normalized by treatment with captopril. Aortic dilation was followed by development of aortic insufficiency and subsequent dilated cardiomyopathy due to valvular disease. Thus, smooth muscle cell Lrp1 deficiency results in aortic dilation and insufficiency that causes secondary cardiomyopathy that can be improved by captopril. These findings provide novel insights into mechanisms of cardiomyopathy associated with vascular activation and offer a new model of valvular cardiomyopathy.

  3. RARE CASES OF HYPERTROPHIC CARDIOMYOPATHY: VARIANTS AND CLINICAL OBSERVATIONS

    Directory of Open Access Journals (Sweden)

    V. Yu. Zimina

    2015-09-01

    Full Text Available Hypertrophic cardiomyopathy belongs to a group of hereditary diseases due to sarcomere gene mutation. This abnormality is characterized by the development of symmetric or asymmetric hypertrophy of left ventricular myocardium with its normal contractile function or hypercontractility. Authors provide a brief overview of variants of hypertrophic cardiomyopathy and phenocopies of this disease, when structural changes in the heart are not the result of classic sarcomere gene mutation. In patients with some phenocopies concentric left ventricular hypertrophy can transform into its dilatation with reduced contractility. Such variant of hypertrophic cardiomyopathy is presented in the first clinical observation. The second case shows that hypertrophic cardiomyopathy can be one of the symptoms of the disease with other reasons for poor outcome.

  4. RARE CASES OF HYPERTROPHIC CARDIOMYOPATHY: VARIANTS AND CLINICAL OBSERVATIONS

    Directory of Open Access Journals (Sweden)

    V. Yu. Zimina

    2014-01-01

    Full Text Available Hypertrophic cardiomyopathy belongs to a group of hereditary diseases due to sarcomere gene mutation. This abnormality is characterized by the development of symmetric or asymmetric hypertrophy of left ventricular myocardium with its normal contractile function or hypercontractility. Authors provide a brief overview of variants of hypertrophic cardiomyopathy and phenocopies of this disease, when structural changes in the heart are not the result of classic sarcomere gene mutation. In patients with some phenocopies concentric left ventricular hypertrophy can transform into its dilatation with reduced contractility. Such variant of hypertrophic cardiomyopathy is presented in the first clinical observation. The second case shows that hypertrophic cardiomyopathy can be one of the symptoms of the disease with other reasons for poor outcome.

  5. Pnematic Dilation in Achalasia

    Directory of Open Access Journals (Sweden)

    Maximilian Bittinger

    2001-01-01

    Full Text Available Pneumatic dilation is the most common first-line therapy for the treatment of achalasia. The aim of dilation is a controlled disruption of circular muscle fibres of the lower esophageal sphincter to reduce the functional obstruction. Several types of dilators and different dilation techniques are used, but the achieved results are similar. The mean success rate is about 80% in the short term, but some patients need redilation in the further course (particularly young patients. Best long term results are obtained if the lower esophageal sphincter pressure can be reduced below 10 mmHg. Major complications are rare after pneumatic dilation; the most serious complication is esophageal perforation, which occurs at a mean rate of about 2.5%. Considering the pros and cons of other effective forms of treatment of achalasia (esophagomyotomy and intrasphincteric injection of botulinum toxin, pneumatic dilation is still the treatment of choice in the majority of patients with achalasia.

  6. X-linked cardiomyopathy is heterogeneous

    Energy Technology Data Exchange (ETDEWEB)

    Wilson, M.J.; Sillence, D.O.; Mulley, J.C. [and others

    1994-09-01

    Two major loci of X-linked cardiomyopathy have been mapped by linkage analysis. The gene for X-linked dilated cardiomyopathy (XLCM) is mapped to the dystrophin locus at Xp21, while Barth syndrome has been localised to distal Xq28. XLCM usually presents in juvenile males with no skeletal disease but decreased dystrophin in cardiac muscle. Barth syndrome most often presents in infants and is characterized by skeletal myopathy, short stature and neutropenia in association with cardiomyopathy of variable severity. Prior to carrier or prenatal diagnosis in a family, delineation of the cardiomyopathy locus involved is essential. We report the linkage mapping of a large kindred in which several male infants have died with hypertrophic cardiomyopathy. There is a family history of unexplained death of infant males less than 6 months old over 4 generations. Features of Barth syndrome such as short stature, skeletal myopathy and neutropenia have not been observed. Genotyping at 10 marker loci in Xq28 has revealed significant pairwise lod scores with the cardiomyopathy phenotype at DXS52 (Z=2.21 at {theta}=0.0), at markers p26 and p39 near DXS15 (Z=2.30 at {theta}=0.0) and at F8C (Z=2.24 at {theta}=0.0). A recombinant detected with DXS296 defines the proximal limit to the localization. No recombinants were detected at any of the loci distal to DXS296. The most distal marker in Xq28, DXS1108, is within 500 kb of the telomere. As the gene in this family is localized to Xq28, it is possible that this disorder is an allelic variant at the Barth syndrome locus.

  7. Avaliação hemodinâmica e evolução clínica de crianças portadoras de cardiomiopatia dilatada grave candidatas a transplante cardíaco Hemodynamic evaluation and clinical outcome of children candidates for heart transplantation with severe dilated cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Estela Azeka

    1998-11-01

    were calculated. RESULTS: Ten (41.6% children were transplanted (group A, 5 (20.8% are still waiting heart transplantation (group B and 9 (37.6% died (group C. The mean age of the B and C group were significantly different. The PVRI, MPAP and TPG were significant lower in group A than group C (p=0.01; p=0.044 and p=0.023 respectively. CONCLUSION: The profile of the hemodynamic parameters of children with severe dilated cardiomyopathy was compatible with the clinical findings. The age was the only difference comparing groups B and C. PVRI, MPAP and TPG were significant factors between group A and C. The older the patient, the worse was the prognosis.

  8. EBCT in diagnosis on primary cardiomyopathy

    International Nuclear Information System (INIS)

    Li Xiangmin; Zhou Xuhui; Meng Quanfei; Li Weiduo; Peng Qian; Tan Zhiyu

    2000-01-01

    Objective: To evaluate the diagnostic value of electron beam CT (EBCT) in primary cardiomyopathy (PCM). Methods: EBCT including coronary scanning and heart movie study was performed in 15 patients. The diagnosis of PCM was established by clinical and imaging findings or/and pathologic examination. Results: BECT findings related to the classification of PCM drawn up by WHO/ISFC were as follows: (1) dilated PCM (8 cases): dilated left ventricle (LV) was found in 7 of 8 cases and dilated right ventricle (RV) in the remaining one. Hypokinetic contraction of the LV wall with (250 ± 101) ml LVEDV and (18.9 ±6.6)% LVEF presented in the patients with dilated LV. (2) Hypertrophic PCM (5 cases): Hypertrophic ventricular septum (16.5 ± 2.3) mm was demonstrated in all cases, hypertrophy of the LV wall or the apex in 3 of 5 cases, and stenosis of the LV outflow tract in 2 cases. (3) Restrictive PCM (2 cases): Thickening of endocardium with lower density than cardiac muscle, reduced RV, obliteration of apex, hypokinetic contraction of the RV wall, and dilatation of right atrium (RA) with parietal thrombus formation of RA was identified in one patient. Both the LV and the RV were involved, with enlargement of atrium. Conclusion: The findings of PCM on EBCT seem to reflect pathologic and functional changes of PCM, and may be useful for diagnosis and classification of PCM

  9. Cardiovascular magnetic resonance in hypertrophic cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Shiozaki, Afonso Akio; Parga, Jose Rodrigues; Arteaga, Edmundo; Rochitte, Carlos Eduardo [Sao Paulo Univ. (USP), SP (Brazil). Instituto do Coracao. Setor de Tomografia Computarizada e Ressonancia Magnetica Cardiovascular]. E-mail: rochitte@incor.usp.br; Kim, Raymond J. [Duke Cardiovascular Magnetic Resonance Center, Durham, NC (United States); Tassi, Eduardo Marinho [Diagnosticos da America S.A., Rio de Janeiro, RJ (Brazil). Sector of Cardiovascular Magnetic Resonance and Computed Tomography

    2007-03-15

    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. 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)

  11. Genetic advances in sarcomeric cardiomyopathies: state of the art.

    Science.gov (United States)

    Ho, Carolyn Y; Charron, Philippe; Richard, Pascale; Girolami, Francesca; Van Spaendonck-Zwarts, Karin Y; Pinto, Yigal

    2015-04-01

    Genetic studies in the 1980s and 1990s led to landmark discoveries that sarcomere mutations cause both hypertrophic and dilated cardiomyopathies. Sarcomere mutations also likely play a role in more complex phenotypes and overlap cardiomyopathies with features of hypertrophy, dilation, diastolic abnormalities, and non-compaction. Identification of the genetic cause of these important conditions provides unique opportunities to interrogate and characterize disease pathogenesis and pathophysiology, starting from the molecular level and expanding from there. With such insights, there is potential for clinical translation that may transform management of patients and families with inherited cardiomyopathies. If key pathways for disease development can be identified, they could potentially serve as targets for novel disease-modifying or disease-preventing therapies. By utilizing gene-based diagnostic testing, we can identify at-risk individuals prior to the onset of clinical disease, allowing for disease-modifying therapy to be initiated early in life, at a time that such treatment may be most successful. In this section, we review the current application of genetics in clinical management, focusing on hypertrophic cardiomyopathy as a paradigm; discuss state-of-the-art genetic testing technology; review emerging knowledge of gene expression in sarcomeric cardiomyopathies; and discuss both the prospects, as well as the challenges, of bringing genetics to medicine. © The Author 2015. Published by Oxford University Press on behalf of the European Society of Cardiology.

  12. [Congestive cardiomyopathy in addiction to clobenzorex, an anorexigenic drug].

    Science.gov (United States)

    Cornaert, P; Camblin, J; Graux, P; Anaye, B; Dutoit, A; Croccel, L

    1986-04-01

    Cardiac failure caused by high doses of amphetamine-like drugs is rare. We report a case of decompensated congestive cardiomyopathy occurring in a 29 year old woman addicted to clobenzorex (Dinintel). This patient had been taking 5 to 7 capsules per day for 5 years. No other cause of cardiac failure was detected. A rapid improvement was obtained by digitalis and diuretic therapy; no further episodes of cardiac failure were observed after one year. However, the drug could not be completely withdrawn and echocardiography has shown increasing left ventricular dilatation. The possible mechanisms of amphetamine induced myocardial toxicity are discussed and the analogy with the group of adrenergic cardiomyopathies is underlined.

  13. Comparison of myocardial thallium and β-methyl iodophenyl pentadecanoic acid (BMIPP) distribution in cardiomyopathy hamster

    International Nuclear Information System (INIS)

    Nishimura, Tsunehiko; Sago, Masayoshi

    1989-01-01

    The usefulness of fatty acid imaging in the detection of cardiomyopathy was evaluated by comparing thallium and BMIPP myocardial distribution in Bio 14.6 Syrian Hamster (25 week ages). Autoradiography was performed in 3 using 3.7 MBq (100 μCi) of 125 I-BMIPP and 37 MBq (1 mCi) of 201 TlCl. In vivo pin-hole imaging was performed in 3 using 37 MBq (1 mCi) of 123 I-BMIPP and 37 MBq (1 mCi) of 201 TlCl. In all cardiomyopathy hamsters, decreased uptake of BMIPP compared to that of thallium was demonstrated. These findings suggest dilated cardiomyopathy is associated with severe focal alternation in the substrate used for the performance of myocardial work. In conclusion, myocardial imaging using BMIPP may be useful for early detection of myocardial degeneration compared to thallium in patients with dilated cardiomyopathy. (author)

  14. Multimodality imaging in apical hypertrophic cardiomyopathy

    Science.gov (United States)

    Parisi, Rosario; Mirabella, Francesca; Secco, Gioel Gabrio; Fattori, Rossella

    2014-01-01

    Apical hypertrophic cardiomyopathy (AHCM) is a relatively rare morphologic variant of HCM in which the hypertrophy of myocardium is localized to the left ventricular apex. Symptoms of AHCM might vary from none to others mimic coronary artery disease including acute coronary syndrome, thus resulting in inappropriate hospitalization. Transthoracic echocardiography is the first-line imaging technique for the diagnosis of hypertrophic cardiomyopathies. However, when the hypertrophy of the myocardium is localized in the ventricular apex might results in missed diagnosis. Aim of this paper is to review the different imaging techniques used for the diagnosis of AHCM and their role in the detection and comprehension of this uncommon disease. PMID:25276293

  15. Genetics Home Reference: X-linked dilated cardiomyopathy

    Science.gov (United States)

    ... of this condition can include an irregular heartbeat (arrhythmia), shortness of breath, extreme tiredness (fatigue), and swelling ... Celebrates Its 15th Anniversary National DNA Day 2018 Newborn Screening Saves Lives Act Turns 10 All Bulletins ...

  16. Resolution of dilated cardiomyopathy in an adolescent with change ...

    African Journals Online (AJOL)

    She underwent evaluation for pulmonary tuberculosis ... mild pedal edema. Her weight was 42kg (between 5th. -10th percentile) and height=152cm (between 5th -10th. Percentile). Her cardiovascular exam was remarkable for tachycardia, small volume .... and treatment with potentially cardiotoxic zidovudine in the setting of ...

  17. Cardiovascular genetics : Technological advancements and applicability for dilated cardiomyopathy

    NARCIS (Netherlands)

    Kummeling, G. J M; Baas, A. F.; Harakalova, M.; van der Smagt, J. J.; Asselbergs, F. W.

    2015-01-01

    Genetics plays an important role in the pathophysiology of cardiovascular diseases, and is increasingly being integrated into clinical practice. Since 2008, both capacity and cost-efficiency of mutation screening of DNA have been increased magnificently due to the technological advancement obtained

  18. Dilated cardiomyopathy in a child with abdominal neuroblastoma ...

    African Journals Online (AJOL)

    spironolactone, furosemide, digoxin and ramipril for cardiac optimisation. Six months later, after a reduction of the tumour size to 3.6 × 2.6 × 3.4 centimetres, she was scheduled for surgical excision of the tumour. There was an improvement of cardiac function (ejection fraction of 41%) in the interim period. Her blood pressure ...

  19. Resolution of dilated cardiomyopathy in an adolescent with change ...

    African Journals Online (AJOL)

    Full Text: EMAIL FREE FULL TEXT EMAIL FREE FULL TEXT · DOWNLOAD FULL TEXT DOWNLOAD FULL TEXT · http://dx.doi.org/10.4314/ahs.v15i1.39 · AJOL African Journals Online. HOW TO USE AJOL... for Researchers · for Librarians · for Authors · FAQ's · More about AJOL · AJOL's Partners · Terms and Conditions of ...

  20. Characteristics of syncope in patients with dilated cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Abdel-Hadi Rami

    2016-04-01

    Conclusion: In patients with DCM, syncope is a relatively rare finding. Cardiac causes (arrhythmias and conduction disorders are responsible for the majority of cases. Risk factors for syncope in these patients remain to be determined.

  1. Childhood dilated cardiomyopathy in Jos, Nigeria | Bode-Thomas ...

    African Journals Online (AJOL)

    Another child suffered a cerebro-vascular accident (CVA) before the diagnosis was made. Conclusions: DCM should be considered early in young children with long-standing or recurrent CHF. A high index of suspicion, early diagnosis and appropriate management should reduce morbidity, prevent complications and ...

  2. Genetic engineering and therapy for inherited and acquired cardiomyopathies.

    Science.gov (United States)

    Day, Sharlene; Davis, Jennifer; Westfall, Margaret; Metzger, Joseph

    2006-10-01

    The cardiac myofilaments consist of a highly ordered assembly of proteins that collectively generate force in a calcium-dependent manner. Defects in myofilament function and its regulation have been implicated in various forms of acquired and inherited human heart disease. For example, during cardiac ischemia, cardiac myocyte contractile performance is dramatically downregulated due in part to a reduced sensitivity of the myofilaments to calcium under acidic pH conditions. Over the last several years, the thin filament regulatory protein, troponin I, has been identified as an important mediator of this response. Mutations in troponin I and other sarcomere genes are also linked to several distinct inherited cardiomyopathic phenotypes, including hypertrophic, dilated, and restrictive cardiomyopathies. With the cardiac sarcomere emerging as a central player for such a diverse array of human heart diseases, genetic-based strategies that target the myofilament will likely have broad therapeutic potential. The development of safe vector systems for efficient gene delivery will be a critical hurdle to overcome before these types of therapies can be successfully applied. Nonetheless, studies focusing on the principles of acute genetic engineering of the sarcomere hold value as they lay the essential foundation on which to build potential gene-based therapies for heart disease.

  3. Pediatric cardiomyopathy: importance of genetic and metabolic evaluation

    Science.gov (United States)

    Kindel, Steven J.; Miller, Erin M.; Gupta, Resmi; Cripe, Linda H.; Hinton, Robert B.; Spicer, Robert L.; Towbin, Jeffrey A.; Ware, Stephanie M.

    2012-01-01

    Background Cardiomyopathy is a heterogeneous disease with a strong genetic component. A research-based pediatric cardiomyopathy registry (PCMR) identified familial, syndromic, or metabolic causes in 30% of children. However, these results pre-dated clinical genetic testing. Methods and Results We determined the prevalence of familial, syndromic, or metabolic causes in eighty-three consecutive unrelated patients referred for genetic evaluation of cardiomyopathy from 2006–2009. Seventy-six percent of probands (n=63) were categorized as familial, syndromic, or metabolic. Forty-three percent (n=18) of hypertrophic cardiomyopathy (HCM) patients had mutations in sarcomeric genes, with MYH7 and MYBPC3 mutations predominating. Syndromic (17%, n=7) and metabolic (26%, n=11) causes were frequently identified in HCM patients. The metabolic subgroup was differentiated by decreased endocardial shortening fraction on echocardiography. Dilated cardiomyopathy (DCM) patients had similar rates of syndromic (20%, n=5) and metabolic (16%, n=4) causes, but fewer familial cases (24%, n=6) than HCM patients. Conclusions The cause of cardiomyopathy is identifiable in a majority of affected children. An underlying metabolic or syndromic cause is identified in greater than 35% of children with HCM or DCM. Identification of etiology is important for management, family based risk assessment, and screening. PMID:22555271

  4. Percutaneous dilatational tracheostomy

    DEFF Research Database (Denmark)

    Johnsen, R.

    2015-01-01

    Introduction Since the introduction and development of percutaneous dilatational tracheostomy (PDT), this procedure is accepted and incorporated in ICUs worldwide. In spite of obvious benefits for the patients, who obtain more comfort and mobility and less use of sedatives, the procedure also...... providers' notes are entered. When searching for -percutaneous dilatation tracheostomy' in the electronic system, we found all patients who had undergone this specific procedure. Afterwards we analyzed each of these patients' hospital records, looking for any periprocedure or postprocedure complications...

  5. Suspected acute myocardial infarction in a dystrophin-deficient dog.

    Science.gov (United States)

    Schneider, Sarah Morar; Coleman, Amanda Erickson; Guo, Lee-Jae; Tou, Sandra; Keene, Bruce W; Kornegay, Joe N

    2016-06-01

    Golden retriever muscular dystrophy (GRMD) is a model for the genetically homologous human disease, Duchenne muscular dystrophy (DMD). Unlike the mildly affected mdx mouse, GRMD recapitulates the severe DMD phenotype. In addition to skeletal muscle involvement, DMD boys develop cardiomyopathy. While the cardiomyopathy of DMD is typically slowly progressive, rare early episodes of acute cardiac decompensation, compatible with myocardial infarction, have been described. We report here a 7-month-old GRMD dog with an apparent analogous episode of myocardial infarction. The dog presented with acute signs of cardiac disease, including tachyarrhythmia, supraventricular premature complexes, and femoral pulse deficits. Serum cardiac biomarkers, cardiac-specific troponin I (cTnI) and N-terminal prohormone of B-type natriuretic peptide (NT-proBNP), were markedly increased. Echocardiography showed areas of hyperechoic myocardial enhancement, typical of GRMD cardiomyopathy. Left ventricular dyskinesis and elevated cTnI were suggestive of acute myocardial damage/infarction. Over a 3-year period, progression to a severe dilated phenotype was observed. Copyright © 2016 Elsevier B.V. All rights reserved.

  6. Cardiomyopathy in Vici syndrome

    African Journals Online (AJOL)

    Solaf M. Elsayed

    2018-01-12

    Jan 12, 2018 ... Cardiomyopathy in Vici syndrome. Dear Editor,. A 7 month old boy was referred to our clinic with provisional diagnosis of Pompe disease because of his Echocardiographic find- ings of hypertrophic cardiomyopathy. Although the patient had severe hypotonia and increased CPK level characteristics of ...

  7. Epidemiology of cardiomyopathy – A Clinical and Genetic Study of Restrictive Cardiomyopathy: The EPOCH-R Study

    Directory of Open Access Journals (Sweden)

    Mitali Kapoor

    2017-01-01

    Full Text Available Introduction: Restrictive cardiomyopathy (RCM is characterized by diastolic dysfunction, biatrial enlargement, and normal or near-normal systolic function. RCM is the rarest kind among cardiomyopathies with a severe outcome. Methods: Here, we present the clinical outcomes of thirty RCM patients recruited from a tertiary care unit of India, All India Institute of Medical Sciences, New Delhi. For clinical assessment, patients underwent electrocardiogram, echocardiography, and cardiac catheterization, and endomyocardial biopsy whenever required. Results: Out of 190 patients with cardiomyopathy, 100 had dilated cardiomyopathy, 60 had hypertrophic cardiomyopathy, and 30 had idiopathic RCM and were recruited for the study. Out of these thirty patients, 63.3% were males. A maximum number of patients were diagnosed in their second to third decade of life. Atrial fibrillation (73.3% and ST-T abnormalities (76.6% were common. Most of the patients showed the early age of onset with symptoms emerging in the first and second decades of life. Shortness of breath and fatigue were found to be common symptoms. No familial cases were found. Conclusion: RCM in India is a sporadic disease, rare, and occurs in the young. Prognosis of RCM is still worse than any other cardiomyopathy.

  8. Mutational Analysis of GATA4 and NKX2.5 Genes in Dilated ...

    African Journals Online (AJOL)

    Purpose: To evaluate the lipid profile abnormalities and association of the GATA4 (1232 C→T) and. NKX 2.5 (73C→T) gene polymorphisms with Dilated cardiomyopathy (DCM) among Chinese population. Methods: The blood samples were collected from the Outpatient Department (OPD) of Cardiology Unit,. Zhengzhou ...

  9. Genetics Home Reference: arrhythmogenic right ventricular cardiomyopathy

    Science.gov (United States)

    ... cardiomyopathy Merck Manual Consumer Version: Cardiomyopathy Merck Manual Consumer Version: Overview of Abnormal Heart Rhythms My46 Trait Profile Orphanet: Arrhythmogenic right ventricular cardiomyopathy Orphanet: Familial isolated ...

  10. Takotsubo Cardiomyopathy in Intensive Care Unit: Prevention, Diagnosis and Management

    Directory of Open Access Journals (Sweden)

    Hannah Masoud

    2016-01-01

    Full Text Available Accurate diagnosis of Takotsubo Cardiomyopathy has substantial prognostic implications in an intensive care unit, given its increased mortality risk and association with life-threatening complications. This report seeks to discuss diagnostic modalities that can be useful in accurately differentiating Takotsubo Cardiomyopathy from Acute Coronary Syndrome, and also briefly discuss prevention and management of this cardiomyopathy in an intensive care unit. For critically ill Takotsubo patients, intensive clinicians can consider establishment of diagnosis by specific electrocardiograph changes, distinctive marked release of cardiac enzymes, characteristic echocardiograph findings, as well as invasive coronary angiography or noninvasive cardiac magnetic imaging.

  11. Interação entre especialidades: miocardiopatia dilatada e neoplasia de mama HER2 positiva Interacción entre especialidades: miocardiopatía dilatada y neoplasia de mama HER2 positiva Interaction between specialties: dilated cardiomyopathy and HER2-positive breast Cancer

    Directory of Open Access Journals (Sweden)

    Solange Moraes Sanches

    2010-01-01

    en la miocardiopatía dilatada, de una forma muy interesante.Basic research may result in unexpected benefit in terms of progress in the understanding of mechanisms responsible for different diseases and their potential treatment alternatives. This is seen, for instance, when a specific situation, defined in clinical practice, may be translated into laboratory findings which suggest a new therapy for an unrelated disease, representing the inverse of the more usual bench-to-bedside path. During the past few years, the use of the monoclonal antibody trastuzumab, in the adjuvant and therapeutic context, has become of fundamental importance in the treatment of breast cancer with amplification/overexpression of HER2, resulting in significant increase in survival rates. The observation that trastuzumab also induces cardiotoxicity, and the identification of mechanisms involved in this side effect, have allowed the investigation of these factors as a therapeutic alternative for dilated cardiomyopathy, in a highly interesting fashion.

  12. Dystrophic Cardiomyopathy: Complex Pathobiological Processes to Generate Clinical Phenotype

    Directory of Open Access Journals (Sweden)

    Takeshi Tsuda

    2017-09-01

    Full Text Available Duchenne muscular dystrophy (DMD, Becker muscular dystrophy (BMD, and X-linked dilated cardiomyopathy (XL-DCM consist of a unique clinical entity, the dystrophinopathies, which are due to variable mutations in the dystrophin gene. Dilated cardiomyopathy (DCM is a common complication of dystrophinopathies, but the onset, progression, and severity of heart disease differ among these subgroups. Extensive molecular genetic studies have been conducted to assess genotype-phenotype correlation in DMD, BMD, and XL-DCM to understand the underlying mechanisms of these diseases, but the results are not always conclusive, suggesting the involvement of complex multi-layers of pathological processes that generate the final clinical phenotype. Dystrophin protein is a part of dystrophin-glycoprotein complex (DGC that is localized in skeletal muscles, myocardium, smooth muscles, and neuronal tissues. Diversity of cardiac phenotype in dystrophinopathies suggests multiple layers of pathogenetic mechanisms in forming dystrophic cardiomyopathy. In this review article, we review the complex molecular interactions involving the pathogenesis of dystrophic cardiomyopathy, including primary gene mutations and loss of structural integrity, secondary cellular responses, and certain epigenetic and other factors that modulate gene expressions. Involvement of epigenetic gene regulation appears to lead to specific cardiac phenotypes in dystrophic hearts.

  13. IL-33 Independently Induces Eosinophilic Pericarditis and Cardiac Dilation: ST2 Improves Cardiac Function

    Science.gov (United States)

    Abston, Eric D.; Barin, Jobert G.; Cihakova, Daniela; Bucek, Adriana; Coronado, Michael J.; Brandt, Jessica E.; Bedja, Djahida; Kim, Joseph B.; Georgakopoulos, Dimitrios; Gabrielson, Kathleen L.; Mitzner, Wayne; Fairweather, DeLisa

    2013-01-01

    Background Interleukin (IL)-33 via its receptor ST2 protects the heart from myocardial infarct and hypertrophy in animal models, but paradoxically increases autoimmune disease. In this study we examined the effect of IL-33 or ST2 administration on autoimmune heart disease. Methods and Results We used pressure volume relationships and isoproterenol challenge to assess the effect of recombinant (r)IL-33 or rST2 (e.g. soluble ST2) administration on the development of autoimmune coxsackievirus (CVB3) myocarditis and dilated cardiomyopathy (DCM) in male BALB/c mice. rIL-33 treatment significantly increased acute perimyocarditis (p=0.006) and eosinophilia (p=1.3×10−5), impaired cardiac function (maximum ventricular power p=0.0002), and increased ventricular dilation (end diastolic volume p=0.01). rST2 treatment prevented eosinophilia and improved heart function compared to rIL-33 treatment (ejection fraction, p=0.009). Neither treatment altered viral replication. rIL-33 increased IL-4, IL-33, IL-1β and IL-6 levels in the heart during acute myocarditis. To determine whether IL-33 altered cardiac function on its own, we administered rIL-33 to undiseased mice and found that rIL-33 induced eosinophilic pericarditis and adversely affected heart function. We used cytokine knockout mice to determine that this effect was due to IL-33-mediated signaling but not IL-1β or IL-6. Conclusions We show for the first time that IL-33 induces eosinophilic pericarditis while sST2 prevents eosinophilia and improves systolic function, and that IL-33 independently adversely affects heart function via the IL-33 receptor. PMID:22454393

  14. Eating and arterial endothelial function: a meta-analysis of the acute effects of meal consumption on flow-mediated dilation.

    Science.gov (United States)

    Thom, N J; Early, A R; Hunt, B E; Harris, R A; Herring, M P

    2016-11-01

    Given that endothelial dysfunction precedes atherosclerotic cardiovascular disease, exploring the parameters that modify postprandial flow-mediated dilation (FMD) is important for public health. The objectives of the study are to estimate the population effect of meal ingestion on FMD and to determine how the effect varied based on patient characteristics and modifiable methodological features. Articles published before June 2015 were located using MEDLINE, PubMed and Web of Science. One hundred fifty-four effects were derived from 78 articles involving 2,548 subjects were selected. Included articles required measurement of FMD in adults before and after meal ingestion. Effects were analysed using an unstandardized mean gain random effects model, and significant moderators were analysed using meta-regression. Meal consumption significantly reduced FMD by a heterogeneous mean effect size delta (Δ) of -2.03 (95% CI: [-2.28, -1.77]), an ~2% reduction in FMD. FMD reductions were larger among normal weight individuals, males, those with a cardio-metabolic disorder, those with elevated baseline FMD, and individuals with impaired glucose tolerance at baseline. Macronutrient meal ingestion significantly reduced FMD, an effect that was moderated by body mass index, sex and two-way interactions between disease status and both baseline FMD and baseline blood glucose levels. © 2016 World Obesity.

  15. Takotsubo (Stress) Cardiomyopathy

    Science.gov (United States)

    ... the American Heart Association Cardiology Patient Page Takotsubo (Stress) Cardiomyopathy Scott W. Sharkey , John R. Lesser , Barry ... heart contraction has returned to normal. Importance of Stress In 85% of cases, takotsubo is triggered by ...

  16. Mutation Glu82Lys in lamin A/C gene is associated with cardiomyopathy and conduction defect

    International Nuclear Information System (INIS)

    Wang Hu; Wang Jizheng; Zheng Weiyue; Wang Xiaojian; Wang Shuxia; Song Lei; Zou Yubao; Yao Yan; Hui Rutai

    2006-01-01

    Dilated cardiomyopathy is a form of heart muscle disease characterized by impaired systolic function and ventricular dilation. The mutations in lamin A/C gene have been linked to dilated cardiomyopathy. We screened genetic mutations in a large Chinese family of 50 members including members with dilated cardiomyopathy and found a Glu82Lys substitution mutation in the rod domain of the lamin A/C protein in eight family members, three of them have been diagnosed as dilated cardiomyopathy, one presented with heart dilation. The pathogenic mechanism of lamin A/C gene defect is poorly understood. Glu82Lys mutated lamin A/C and wild type protein was transfected into HEK293 cells. The mutated protein was not properly localized at the inner nuclear membrane and the emerin protein, which interacts with lamin A/C, was also aberrantly distributed. The nuclear membrane structure was disrupted and heterochromatin was aggregated aberrantly in the nucleus of the HEK293 cells stably transfected with mutated lamin A/C gene as determined by transmission electron microscopy

  17. Contemporary trends in the epidemiology and management of cardiomyopathy and pericarditis in sub‐Saharan Africa

    Science.gov (United States)

    Mayosi, Bongani M

    2007-01-01

    Heart failure in sub‐Saharan Africans is mainly due to non‐ischaemic causes, such as hypertension, rheumatic heart disease, cardiomyopathy and pericarditis. The two endemic diseases that are major contributors to the clinical syndrome of heart failure in Africa are cardiomyopathy and pericarditis. The major forms of endemic cardiomyopathy are idiopathic dilated cardiomyopathy, peripartum cardiomyopathy and endomyocardial fibrosis. Endomyocardial fibrosis, which affects children, has the worst prognosis. Other cardiomyopathies have similar epidemiological characteristics to those of other populations in the world. HIV infection is associated with occurrence of HIV‐associated cardiomyopathy in patients with advanced immunosuppression, and the rise in the incidence of tuberculous pericarditis. HIV‐associated tuberculous pericarditis is characterised by larger pericardial effusion, a greater frequency of myopericarditis, and a higher mortality than in people without AIDS. Population‐based studies on the epidemiology of heart failure, cardiomyopathy and pericarditis in Africans, and studies of new interventions to reduce mortality, particularly in endomyocardial fibrosis and tuberculous pericarditis, are needed. PMID:17890693

  18. Takotsubo cardiomyopathy in amiodarone-induced hyperthyroidism

    OpenAIRE

    Ismael Capel; Elisabet Tasa-Vinyals; Albert Cano-Palomares; Irene Bergés-Raso; Lara Albert; Mercedes Rigla; Assumpta Caixàs

    2017-01-01

    Summary Takotsubo cardiomyopathy (TC) is an atypical, severe but reversible form of acute heart insufficiency. It typically presents with left ventricular failure, transient apical and mid-segments hypokinesis, absence of significant coronary stenosis and new electrographic abnormalities and/or elevation in serum cardiac enzymes. Although TC (?broken heart syndrome?) has classically been associated with emotional trauma, evidence suggests that other precipitants might exist, including iatroge...

  19. New perspectives in Hypertrophic Cardiomyopathy

    NARCIS (Netherlands)

    M.J.M. Kofflard (Marcel)

    1998-01-01

    textabstractHypertrophic cardiomyopathy is a primary cardiac disorder with a heterogeneous expression. Although relatively uncommon, the disease has been studied extensively as appears from the numerous studies that have explored specific facets of hypertrophic cardiomyopathy. This review will focus

  20. Exercise Rehabilitation in Pediatric Cardiomyopathy

    OpenAIRE

    Somarriba, Gabriel; Extein, Jason; Miller, Tracie L.

    2008-01-01

    Children with cardiomyopathy carry significant risk of morbidity and mortality. New research and technology have brought about significant advancements to the diagnosis and clinical management of children with cardiomyopathy. However, currently heart transplantation remains the standard of care for children with symptomatic and progressive cardiomyopathy. Cardiovascular rehabilitation programs have yielded success in improving cardiac function, overall physical activity, and quality of life i...

  1. Stimulant-related Takotsubo cardiomyopathy.

    Science.gov (United States)

    Butterfield, Mike; Riguzzi, Christine; Frenkel, Oron; Nagdev, Arun

    2015-03-01

    Takotsubo cardiomyopathy (TC) is a rare but increasingly recognized mimic of acute coronary syndrome. Patients present with angina,ST-segment changes on electrocardiogram (both elevations and depressions),and rapid rises in cardiac biomarkers. Many kinds of stressful events have been associated with TC, but only a handful of drug-related cases have previously been reported. We describe the case of a 58-year-old woman who developed TC 2 days after crack cocaine use, a diagnosis first suggested as bedside echocardiography in the emergency department.Recognition of the classic echocardiographic appearance of TC—apical hypokinesis causing “ballooning” of the left ventricle during systole—may greatly assist providers in the early identification of this condition.

  2. Nutritional deficiency of selenium secondary to weight loss (bariatric) surgery associated with life-threatening cardiomyopathy.

    Science.gov (United States)

    Boldery, Rachel; Fielding, George; Rafter, Tony; Pascoe, Andrew L; Scalia, Gregory M

    2007-04-01

    Nutritional deficiencies of vitamins and minerals have been associated with reversible and irreversible cardiomyopathic processes. Selenium deficient dilated cardiomyopathy, first described in 1935 in the Keshan Province of China, was sometimes reversed with selenium supplementation. In the past three decades, selenium deficient cardiomyopathy has re-emerged in western medicine secondary to gastrointestinal disorders, long-term total parenteral nutrition (TPN) and gastrointestinal surgery. This report describes a case of selenium deficient cardiomyopathy secondary to the bariatric (weight loss) surgical operation bilio-pancreatic diversion (Scopinaro procedure). This patient presented with life-threatening heart failure nine months after this surgery, having lost 100 kg of body weight. Multiple nutritional deficiencies were detected and corrected including severely depleted selenium levels. Cardiac function and the clinical scenario improved dramatically over three weeks. Screening patients at risk of malnourishment for selenium deficiency as a potential cause of cardiomyopathy is indicated.

  3. HYPERTROPHIC CARDIOMYOPATHY IN MULTIMORBIDITY

    Directory of Open Access Journals (Sweden)

    A. I. Lakhonina

    2016-06-01

    Full Text Available Aspects of diagnosis, difficulties in the diagnosis and optimal therapeutic strategies in patient with hypertrophic cardiomyopathy and comorbid conditions such as arterial hypertension, ischemic heart disease, dyslipidemia, diabetes mellitus type 2, stenosis of the left renal artery, obesity are reviewed on the example of clinical case. Hypertrophic cardiomyopathy combined with multimorbidity conditions requires a high-quality medical management, where the main goal is to improve the quality and duration of patient's life. This goal is being achieved by optimizing patient's lifestyle and assigning only the minimum amount of medications. Necessity of careful diagnosis of hypertrophic cardiomyopathy, evaluation of the risk of sudden death and search of optimal treatment in patients with multimorbidity pathology are demonstrated in clinical case.

  4. Treatment of Chagas cardiomyopathy.

    Science.gov (United States)

    Botoni, Fernando A; Ribeiro, Antonio Luiz P; Marinho, Carolina Coimbra; Lima, Marcia Maria Oliveira; Nunes, Maria do Carmo Pereira; Rocha, Manoel Otávio C

    2013-01-01

    Chagas' disease (ChD), caused by the protozoa Trypanosoma cruzi (T. cruzi), was discovered and described by the Brazilian physician Carlos Chagas in 1909. After a century of original description, trypanosomiasis still brings much misery to humanity and is classified as a neglected tropical disease prevalent in underdeveloped countries, particularly in South America. It is an increasing worldwide problem due to the number of cases in endemic areas and the migration of infected subjects to more developed regions, mainly North America and Europe. Despite its importance, chronic chagas cardiomyopathy (CCC) pathophysiology is yet poorly understood, and independently of its social, clinical, and epidemiological importance, the therapeutic approach of CCC is still transposed from the knowledge acquired from other cardiomyopathies. Therefore, the objective of this review is to describe the treatment of Chagas cardiomyopathy with emphasis on its peculiarities.

  5. Cardiomyopathy induced by anthracycline

    International Nuclear Information System (INIS)

    Quiroz, Isabel; Espinoza, Gerson; Poveda, Maria; Flores, Walter

    2002-01-01

    Anthracycline cardiomyopathy is less frequently encountered nowadays, due to the well recognized dose limitations and cardiac monitoring protocols used by chemotherapy centers. However, it is a condition that will persist due to the sensitivity of some patients to these drugs and the necessity for large doses to be used for certain individuals. We have demonstrated the benefit of angiotensin converting enzyme inhibitor therapy and would consider introducing these compounds at the earliest opportunity. The use of probucol and vitamins as antioxidants capable of preventing the onset of cardiomyopathy in humans appears to require further investigation but may significantly reduce the incidence of this condition in the future. (The author)

  6. Athlete's heart or hypertrophic cardiomyopathy?

    Science.gov (United States)

    Lauschke, Jörg; Maisch, Bernhard

    2009-02-01

    Intensive endurance training is able to cause a distinct pattern of functional and structural changes of the cardiovascular system. In an unknown proportion of athletes a so called "athlete's heart" develops. There is an overlap between this type of physiologic cardiac hypertrophy and mild forms of hypertrophic cardiomyopathy (HCM), the most common genetic disorder of the cardiovascular system with a prevalence of 0.2%. HCM is caused by mutations in 14 genes coding for sarcomere proteins. In the literature up to 50% of cases of sudden cardiac death (SCD) in younger sportsmen were connected to hypertrophic cardiomyopathy. It is therefore the most common cause of SCD in highly trained young athletes. Because of this data a great interest in distinguishing these two diagnoses exists. Apart from clinical examination and some non-specific ECG-changes, Echocardiography is the method of choice. The athlete's heart shows an eccentric biventricular hypertrophy with wall thicknesses under 15 mm and a moderately dilated left ventricle (LVEDD up to 58 mm). HCM is commonly characterized by asymmetric left ventricular hypertrophy with a reduced LV-diameter. In up to 70% of cases left ventricular outflow tract obstruction is evident during stress echocardiography. Systolic function is normal in highly trained athletes and the majority of HCM patients as well. There are important differences regarding diastolic filling patterns. Physiological hypertrophy is consistent with a normal diastolic function with even increased early diastolic filling. In case of HCM diastolic dysfunction (mostly relaxation disturbances) occurs in the majority of patients and is therefore inconsistent with an athlete's heart. If the diagnosis could not be stated using echocardiography, methods like cardiac-MRI, metabolic exercise testing, histological studies of endomyocardial biopsies and genetic testing can provide further information. A correct diagnosis may on the one hand prevent some athletes from

  7. [Cardiac magnetic resonance in acute myocarditis: a new non-invasive diagnostic gold standard?].

    Science.gov (United States)

    Monney, Pierre; Locca, Didier; Muzzarelli, Stefano; Hullin, Roger; Jeanrenaud, Xavier; Schwitter, Juerg

    2012-05-30

    Acute myocarditis was until recently one of the most difficult diagnoses in cardiology. The spectrum of signs and symptoms is very wide, the usual non-invasive tests lack specificity and the myocardial biopsy is only performed in a minority of cases to confirm the diagnosis. Due to its unique ability to directly image myocardial necrosis, fibrosis and oedema, cardiac magnetic resonance (CMR) is now considered the primary tool for noninvasive assessment of patients with suspected myocarditis. CMR is also useful for monitoring disease activity under treatment. Myocarditis has been associated with the development of dilated cardiomyopathy; CMR could play a role in the follow-up of such cases to detect the progression toward a dilatative phenotype. Precise mapping of myocardial lesions with cardiac MRI is invaluable to guide myocardial biopsy and increase its diagnostic yield by improving sensitivity.

  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. Diagnosis of cardiomyopathies: tips and tricks for internists and general practitioners

    Directory of Open Access Journals (Sweden)

    Giuseppe Palmiero

    2017-04-01

    Full Text Available Cardiomyopathies are little known to internists and general practitioners (GPs, and not always able to arouse the interest of cardiologists. Probably, this happens because cardiomyopathies are perceived as rare and complex disorders, a prerogative of a few dedicated centers. This may partly explain why the diagnosis of cardiomyopathy is often missed and, consequently, why cardiomyopathies are largely underdiagnosed. Internists and general practitioners should have an interest in these conditions, because cardiomyopathies are not as rare as generally perceived, and because their complexity can be unravelled with knowledge and methodology. Cardiomyopathies are defined as myocardial disorders in which the heart is structurally and functionally abnormal in the absence of coronary artery disease or abnormal loading conditions. Irrespective of the cardiac imaging technique used, a limited number of phenotypes are defined based on ventricular morphology and function. These basic phenotypes include hypertrophic, dilated, restrictive and right ventricular arrhythmogenic cardiomyopathies. Aim of this review is to describe a simplified approach to the detection of the underlying causes of specific phenotypes. We will focus our attention on the basic phenotypes, presenting a diagnostic work-up and a suggestive clinical case for each phenotype.

  10. Examining the Psychosocial Impact of Genetic Testing for Cardiomyopathies.

    Science.gov (United States)

    Wynn, Julia; Holland, David T; Duong, Jimmy; Ahimaz, Priyanka; Chung, Wendy K

    2017-12-15

    Inherited cardiomyopathies, including hypertrophic cardiomyopathy (HCM) and dilated cardiomyopathy (DCM), are the most common monogenic cause of cardiac disease and can rarely lead to sudden cardiac death (SCD). They are characterized by incomplete and age-dependent penetrance and are usually initially symptomatic in adulthood yet can present in childhood as well. Over 20 genes have been identified to cause HCM, and more than 40 genes are known to cause DCM. Genetic testing for these genes has been integrated into medical care; however, the psychological impact of genetic testing and the impact of the uncertainty that comes with receiving these results have not been well studied. This study surveyed 90 adult probands and relatives with a personal or family history of cardiomyopathy from a single hospital-based cardiac genetic program to determine the psychosocial impact of genetic testing for cardiomyopathies. Standardized psychological instruments including an adapted Multidimensional Impact of Cancer Risk Assessment (aMICRA), Impact of Event Scale (IES), and Satisfaction with Decision (SWD) scales were utilized. Patients with positive genetic test results had higher scores for intrusive thoughts, avoidance, and distress when compared to those with negative genetic test results and were also more likely to make or plan to make life changes because of the results of their genetic testing. Satisfaction with the decision to undergo genetic testing was similar regardless of genetic test results. The results of this study provide insight into the patient experience of genetic testing for cardiomyopathies and how these experiences are associated with genetic test results and cardiac history.

  11. Cardiomyopathy in Vici syndrome

    African Journals Online (AJOL)

    Solaf M. Elsayed

    2018-01-12

    Jan 12, 2018 ... The syndrome was first described in 1988, in two brothers with ACC, bilateral cataracts, ... syndrome with brain anomalies, cardiomyopathy, and severe intellectual disability. Case Reports in Genetics 2011, Volume 2011. [6] Finocchi A, Angelino G, Cantarutti N, Corbari M, Bevivino E, Cascioli S, et al.

  12. Cardiomyopathy Following Latrodectus Envenomation

    Directory of Open Access Journals (Sweden)

    Levine, Michael

    2010-12-01

    Full Text Available Latrodectus envenomations are common throughout the United States and the world. While many envenomations can result in catecholamine release with resultant hypertension and tachycardia, myocarditis is very rare. We describe a case of a 22- year-old male who sustained a Latrodectus envenomation complicated by cardiomyopathy. [West J Emerg Med. 2010; 11(5:521-523.

  13. Hypertrophic Cardiomyopathy Association

    Science.gov (United States)

    ... 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 ...

  14. [Plasma selenium and peripartum cardiomyopathy in Bamako, Mali].

    Science.gov (United States)

    Cénac, A; Touré, K; Diarra, M B; Sergeant, C; Jobic, Y; Sanogo, K; Dembele, M; Fayol, V; Simonoff, M

    2004-01-01

    Peripartum heart failure due to unexplained dilated cardiomyopathy is a common disorder as Savannak-Sahelian Africa. One of the many suspected risk factors identified is selenium deficiency. The purpose of this study was to measure plasma selenium levels in patients with peripartum heart failure due to cardiomyopathy in Bamako, Republic of Mali and compare data with healthy Sahalian women with the same obstetrical status. Plasma selenium was measured in a patient group consisting of 28 Malian women presenting peripartum heart failure and in a control group of 28 healthy breast-feeding Nigerien women of comparable age. The criteria for matching the two groups was parity (similar number of deliveries) since multiparity is a risk factor for peripartum cardiomyopathy. The Wilcoxon test (nonparametric) was used to compare the 2 groups considering up value < 0.05 as significant. Plasma selenium was significantly lower in patients from Mali than in controls from Niger (65 +/- 17 ng/ml vs. 78 +/- 17 ng/ml, p = 0.01). The results of this study showing lower plasma selenium in Bamako patients with peripartum cardiomyopathy than in a matching healthy control population confirms the previous data from the Niamey study.

  15. Takotsubo cardiomyopathy after a dancing session: a case report.

    Science.gov (United States)

    Kaballo, Mohammed A; Yousif, Abdelazim; Abdelrazig, Awadalla M; Ibrahim, Ammar A; Hennessy, Terence G

    2011-10-31

    Stress-induced (Takotsubo) cardiomyopathy is a rare form of cardiomyopathy which presents in a manner similar to that of acute coronary syndrome. This sometimes leads to unnecessary thrombolysis therapy. The pathogenesis of this disease is still poorly understood. We believe that reporting all cases of Takotsubo cardiomyopathy will contribute to a better understanding of this disease. Here, we report a patient who, in the absence of any recent stressful events in her life, developed the disease after a session of dancing. A 69-year-old Caucasian woman presented with features suggestive of acute coronary syndrome shortly after a session of dancing. Echocardiography and a coronary angiogram showed typical features of Takotsubo cardiomyopathy and our patient was treated accordingly. Eight weeks later, her condition resolved completely and the results of echocardiography were totally normal. Takotsubo cardiomyopathy, though transient, is a rare and serious condition. Although it is commonly precipitated by stressful life events, these are not necessarily present. Our patient was enjoying one of her hobbies (that is, dancing) when she developed the disease. This case has particular interest in medicine, especially for the specialties of cardiology and emergency medicine. We hope that it will add more information to the literature about this rare condition.

  16. Cardiogenic shock in Takotsubo cardiomyopathy: a focus on management.

    Science.gov (United States)

    Doucet, Katie M; Labinaz, Marino X; Beauchesne, Luc M; Burwash, Ian G

    2015-01-01

    Takotsubo cardiomyopathy has become a well recognized mimicker of acute coronary syndrome. Patients generally do well, although a minority can develop life-threatening complications. We present a case of 1 such patient in a branched self-assessment format designed to challenge the reader's clinical management skills. Copyright © 2015 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.

  17. CARDIOMYOPATHIE DU POSTPARTUM: A propos de cinq cas au ...

    African Journals Online (AJOL)

    The main presenting symptom was congestive heart failure with acute pulmonary edema. In all cases, the chest x-ray showed ... The treatment administered to these patients included bed rest, salt restriction, digitalics, ... The main issues concerning the management of postpartum cardiomyopathy are raised in this article.

  18. Production of aldosterone in cardiac tissues of healthy dogs and with dilated myocardiopathy

    OpenAIRE

    Reynoso-Palomar, Alejandro; Mena-Aguilar, Georgina; Cruz-García, Marisol; Pastelín-Rojas, César; Villa-Mancera, Abel

    2017-01-01

    Background and Aim: Aldosterone is a hormone, belonging to the group of mineralocorticoids, mainly synthesized in the adrenal cortex, basically its function is to regulate blood pressure and sodium potassium levels in the body; high levels of this hormone have harmful effects in the organism and mainly in the heart in chronic form. Dilated cardiomyopathy is a progressive disease of heart muscle that is characterized by ventricular chamber enlargement and contractile dysfunction, is one of the...

  19. Fluid dynamics of dilatant fluid

    DEFF Research Database (Denmark)

    Nakanishi, Hiizu; Nagahiro, Shin-ichiro; Mitarai, Namiko

    2012-01-01

    A dense mixture of granules and liquid often shows a severe shear thickening and is called a dilatant fluid. We construct a fluid dynamics model for the dilatant fluid by introducing a phenomenological state variable for a local state of dispersed particles. With simple assumptions for an equation...

  20. Roentgenoendovascular dilatation of brachycephalic arteries

    International Nuclear Information System (INIS)

    Rabkin, I.Kh.; Kachel, R.; Glazer, F.; Matevosov, A.L.; Dzhoraev, I.G.; Medizinische Akademie, Erfurt

    1988-01-01

    The authors reported the technique, methods of and indications for roentgenoendovascular dilation in stenotic and occlusive lesions of the brachycephalic branches of the aorta. A total of 102 vascular dilations were perfomed in 76 patients resulting in a good angiographic and clinical effect. In 2 patients the first world prosthetics was performed using an original coiled nitinol prosthesis

  1. Reversible ischaemia in hypertrophic cardiomyopathy.

    OpenAIRE

    Thomson, H.; Fong, W.; Stafford, W.; Frenneaux, M.

    1995-01-01

    Atypical and typical chest pains are common symptoms in patients with hypertrophic cardiomyopathy. Some of these chest pains seem to be caused by ischaemia. It is difficult to objectively demonstrate ischaemia in hypertrophic cardiomyopathy. The first line treatment for chest pain considered to be ischaemic in patients with hypertrophic cardiomyopathy is the use of either a beta blocker or calcium blocker. Septal myectomy can be effective in patients with symptoms refractory to conventional t...

  2. The Cardiomyopathy Registry of the EURObservational Research Programme of the European Society of Cardiology: baseline data and contemporary management of adult patients with cardiomyopathies.

    Science.gov (United States)

    Charron, Philippe; Elliott, Perry M; Gimeno, Juan R; Caforio, Alida L P; Kaski, Juan Pablo; Tavazzi, Luigi; Tendera, Michal; Maupain, Carole; Laroche, Cécile; Rubis, Pawel; Jurcut, Ruxandra; Calò, Leonardo; Heliö, Tiina M; Sinagra, Gianfranco; Zdravkovic, Marija; Kavoliuniene, Aušra; Felix, Stephan B; Grzybowski, Jacek; Losi, Maria-Angela; Asselbergs, Folkert W; García-Pinilla, José Manuel; Salazar-Mendiguchia, Joel; Mizia-Stec, Katarzyna; Maggioni, Aldo P

    2018-01-24

    The Cardiomyopathy Registry of the EURObservational Research Programme is a prospective, observational, and multinational registry of consecutive patients with four cardiomyopathy subtypes: hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), arrhythmogenic right ventricular cardiomyopathy (ARVC), and restrictive cardiomyopathy (RCM). We report the baseline characteristics and management of adults enrolled in the registry. A total of 3208 patients were enrolled by 69 centres in 18 countries [HCM (n = 1739); DCM (n = 1260); ARVC (n = 143); and RCM (n = 66)]. Differences between cardiomyopathy subtypes (P < 0.001) were observed for age at diagnosis, history of familial disease, history of sustained ventricular arrhythmia, use of magnetic resonance imaging or genetic testing, and implantation of defibrillators. When compared with probands, relatives had a lower age at diagnosis (P < 0.001), but a similar rate of symptoms and defibrillators. When compared with the Long-Term phase, patients of the Pilot phase (enrolled in more expert centres) had a more frequent rate of familial disease (P < 0.001), were more frequently diagnosed with a rare underlying disease (P < 0.001), and more frequently implanted with a defibrillator (P = 0.023). Comparing four geographical areas, patients from Southern Europe had a familial disease more frequently (P < 0.001), were more frequently diagnosed in the context of a family screening (P < 0.001), and more frequently diagnosed with a rare underlying disease (P < 0.001). By providing contemporary observational data on characteristics and management of patients with cardiomyopathies, the registry provides a platform for the evaluation of guideline implementation. Potential gaps with existing recommendations are discussed as well as some suggestions for improvement of health care provision in Europe. Published on behalf of the European Society of Cardiology. All rights reserved.

  3. Inferior ST-Elevation Myocardial Infarction Associated with Takotsubo Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Oliver Koeth

    2010-01-01

    Full Text Available Takotsubo cardiomyopathy (TCM is usually characterized by transient left ventricular apical ballooning. Due to the clinical symptoms which include chest pain, electrocardiographic changes, and elevated myocardial markers, Takotsubo cardiomyopathy is frequently mimicking ST-elevation myocardial infarction in the absence of a significant coronary artery disease. Otherwise an acute occlusion of the left anterior descending coronary artery can produce a typical Takotsubo contraction pattern. ST-elevation myocardial infarction (STEMI is frequently associated with emotional stress, but to date no cases of STEMI triggering TCM have been reported. We describe a case of a female patient with inferior ST-elevation myocardial infarction complicated by TCM.

  4. Hypertrophic Cardiomyopathy: A Review

    OpenAIRE

    Houston, Brian A; Stevens, Gerin R

    2015-01-01

    Hypertrophic cardiomyopathy (HCM) is a global disease with cases reported in all continents, affecting people of both genders and of various racial and ethnic origins. Widely accepted as a monogenic disease caused by a mutation in 1 of 13 or more sarcomeric genes, HCM can present catastrophically with sudden cardiac death (SCD) or ventricular arrhythmias or insidiously with symptoms of heart failure. Given the velocity of progress in both the fields of heart failure and HCM, we present a revi...

  5. 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.

  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. Emergency Physicians Are Able to Detect Right Ventricular Dilation With Good Agreement Compared to Cardiology.

    Science.gov (United States)

    Rutz, Matt A; Clary, Julie M; Kline, Jeffrey A; Russell, Frances M

    2017-07-01

    Focused cardiac ultrasound (FOCUS) is a useful tool in evaluating patients presenting to the emergency department (ED) with acute dyspnea. Prior work has shown that right ventricular (RV) dilation is associated with repeat hospitalizations and shorter life expectancy. Traditionally, RV assessment has been evaluated by cardiologist-interpreted comprehensive echocardiography. The primary goal of this study was to determine the inter-rater reliability between emergency physicians (EPs) and a cardiologist for determining RV dilation on FOCUS performed on ED patients with acute dyspnea. This was a prospective, observational study at two urban academic EDs; patients were enrolled if they had acute dyspnea and a computed tomographic pulmonary angiogram without acute disease. All patients had an EP-performed FOCUS to assess for RV dilation. RV dilation was defined as an RV to left ventricular ratio greater than 1. FOCUS interpretations were compared to a blinded cardiologist FOCUS interpretation using agreement and kappa statistics. Of 84 FOCUS examinations performed on 83 patients, 17% had RV dilation. Agreement and kappa, for EP-performed FOCUS for RV dilation were 89% (95% confidence interval [CI] 80-95%) and 0.68 (95% CI 0.48-0.88), respectively. Emergency physician sonographers are able to detect RV dilation with good agreement when compared to cardiology. These results support the wider use of EP-performed FOCUS to evaluate for RV dilation in ED patients with dyspnea. © 2017 by the Society for Academic Emergency Medicine.

  8. Role of left ventricular twist mechanics in cardiomyopathies, dance of the helices.

    Science.gov (United States)

    Kauer, Floris; Geleijnse, Marcel Leonard; van Dalen, Bastiaan Martijn

    2015-08-26

    Left ventricular twist is an essential part of left ventricular function. Nevertheless, knowledge is limited in "the cardiology community" as it comes to twist mechanics. Fortunately the development of speckle tracking echocardiography, allowing accurate, reproducible and rapid bedside assessment of left ventricular twist, has boosted the interest in this important mechanical aspect of left ventricular deformation. Although the fundamental physiological role of left ventricular twist is undisputable, the clinical relevance of assessment of left ventricular twist in cardiomyopathies still needs to be established. The fact remains; analysis of left ventricular twist mechanics has already provided substantial pathophysiological understanding on a comprehensive variety of cardiomyopathies. It has become clear that increased left ventricular twist in for example hypertrophic cardiomyopathy may be an early sign of subendocardial (microvascular) dysfunction. Furthermore, decreased left ventricular twist may be caused by left ventricular dilatation or an extensive myocardial scar. Finally, the detection of left ventricular rigid body rotation in noncompaction cardiomyopathy may provide an indispensible method to objectively confirm this difficult diagnosis. All this endorses the value of left ventricular twist in the field of cardiomyopathies and may further encourage the implementation of left ventricular twist parameters in the "diagnostic toolbox" for cardiomyopathies.

  9. DKA-Induced Takotsubo Cardiomyopathy in Patient with Known HOCM

    Directory of Open Access Journals (Sweden)

    Ayla Gordon

    2017-01-01

    Full Text Available The first published case of Diabetic Ketoacidosis-induced Takotsubo cardiomyopathy was in 2009. Our patient is the 1st reported case of Diabetic Ketoacidosis- (DKA- induced Takotsubo cardiomyopathy (TC in a patient with known hypertrophic cardiomyopathy (HOCM in the United States. In the literature, there are only two examples linking DKA to TC; however, this report focuses on the biochemical and physiological causes of TC in a patient with known HOCM and new-onset DKA. TC in previously diagnosed HOCM poses particular complications. With the above patient’s baseline outflow tract obstruction due to septal hypertrophy, the acute reduction in EF due to TC resulted in transient drop in brain perfusion and, therefore, syncope.

  10. Segmental dilatation of the ileum

    Directory of Open Access Journals (Sweden)

    Tune-Yie Shih

    2017-01-01

    Full Text Available A 2-year-old boy was sent to the emergency department with the chief problem of abdominal pain for 1 day. He was just discharged from the pediatric ward with the diagnosis of mycoplasmal pneumonia and paralytic ileus. After initial examinations and radiographic investigations, midgut volvulus was impressed. An emergency laparotomy was performed. Segmental dilatation of the ileum with volvulus was found. The operative procedure was resection of the dilated ileal segment with anastomosis. The postoperative recovery was uneventful. The unique abnormality of gastrointestinal tract – segmental dilatation of the ileum, is described in details and the literature is reviewed.

  11. Vessel dilatation in coronary angiograms

    Energy Technology Data Exchange (ETDEWEB)

    Hinterauer, L.; Goebel, N.

    1983-11-01

    Amongst 166 patients with aneurysms, ectasia or megaloarteries shown on coronary angiograms, 86.1% had dilated vessels as part of generalised coronary sclerosis (usually in patients with three-vessel disease). In 9%, dilatation was of iatrogenic origin and in 4.8% it was idiopathic. One patient had Marfan's syndrome. Amongst 9 000 patients, there were eight with megalo-arteries without stenosis; six of these had atypical angina and three suffered an infarct. Patients with definite dilatation of the coronary artery and stagnation of contrast flow required treatment.

  12. Vessel dilatation in coronary angiograms

    International Nuclear Information System (INIS)

    Hinterauer, L.; Goebel, N.

    1983-01-01

    Amongst 166 patients with aneurysms, ectasia or megaloarteries shown on coronary angiograms, 86.1% had dilated vessels as part of generalised coronary sclerosis (usually in patients with three-vessel disease). In 9%, dilatation was of iatrogenic origin and in 4.8% it was idiopathic. One patient had Marfan's syndrome. Amongst 9 000 patients, there were eight with megalo-arteries without stenosis; six of these had atypical angina and three suffered an infarct. Patients with definite dilatation of the coronary artery and stagnation of contrast flow required treatment. (orig.) [de

  13. Value of cardiovascular MR in diagnosing left ventricular non-compaction cardiomyopathy and in discriminating between other cardiomyopathies

    Energy Technology Data Exchange (ETDEWEB)

    Grothoff, Matthias; Lehmkuhl, Lukas; Gutberlet, Matthias [University of Leipzig - Heart Center, Department of Diagnostic and Interventional Radiology, Leipzig (Germany); Pachowsky, Milena [Klinik fuer Strahlenheilkunde, Charite, Campus Virchow-Klinikum, Berlin (Germany); Hoffmann, Janine [University of Leipzig, Department of Obstetrics, Leipzig (Germany); Posch, Maximilian [Department of Cardiothoracic Surgery, Deutsches Herzzentrum Berlin, Berlin (Germany); Klaassen, Sabine [Experimental and Clinical Research Center, Charite Medical Faculty and Max Delbrueck Center for Molecular Medicine, Berlin (Germany)

    2012-12-15

    To analyse the value of cardiovascular magnetic resonance (CMR)-derived myocardial parameters to differentiate left ventricular non-compaction cardiomyopathy (LVNC) from other cardiomyopathies and controls. We retrospectively analysed 12 patients with LVNC, 11 with dilated and 10 with hypertrophic cardiomyopathy and compared them to 24 controls. LVNC patients had to fulfil standard echocardiographic criteria as well as additional clinical and imaging criteria. Cine steady-state free precession and late gadolinium enhancement (LGE) imaging was performed. The total LV myocardial mass index (LV-MMI), compacted (LV-MMI{sub compacted}), non-compacted (LV-MMI{sub non-compacted}), percentage LV-MM{sub non-compacted}, ventricular volumes and function were calculated. Data were compared using analysis of variance and Dunnett's test. Additionally, semi-quantitative segmental analyses of the occurrence of increased trabeculation were performed. Total LV-MMI{sub non-compacted} and percentage LV-MM{sub non-compacted} were discriminators between patients with LVCN, healthy controls and those with other cardiomyopathies with cut-offs of 15 g/m{sup 2} and 25 %, respectively. Furthermore, trabeculation in basal segments and a ratio of non-compacted/compacted myocardium of {>=}3:1 were criteria for LVNC. A combination of these criteria provided sensitivities and specificities of up to 100 %. None of the LVNC patients demonstrated LGE. Absolute CMR quantification of the LV-MMI{sub non-compacted} or the percentage LV-MM{sub non-compacted} and increased trabeculation in basal segments allows one to reliably diagnose LVNC and to differentiate it from other cardiomyopathies. (orig.)

  14. New perspectives in Hypertrophic Cardiomyopathy

    OpenAIRE

    Kofflard, Marcel

    1998-01-01

    textabstractHypertrophic cardiomyopathy is a primary cardiac disorder with a heterogeneous expression. Although relatively uncommon, the disease has been studied extensively as appears from the numerous studies that have explored specific facets of hypertrophic cardiomyopathy. This review will focus on the anatomic abnormalities, the prevalence, symptoms and clinical outcome, therapeutic interventions and genetic mutations responsible for the disease.

  15. [Myocardial regional thickness in patients with and without cardiomyopathy assessed by cardiac magnetic resonance].

    Science.gov (United States)

    de Zan, Macarena; Carrascosa, Patricia; Deviggiano, Alejandro; Capuñay, Carlos; Rodríguez-Granillo, Gastón A

    To explore regional differences in myocardial wall thickness (WT) among the most prevalent cardiomyopathies and in individuals without structural heart disease using cardiac magnetic resonance. Patients older than 18 years referred to cardiac magnetic resonance during the period between January 2014 and September 2014, with a diagnosis of hypertrophic cardiomyopathy, idiopathic dilated cardiomyopathy, ischemic cardiomyopathy, and myocarditis were retrospectively selected from our database. One hundred twenty patients patients were included. The control group had an average WT of 5.9±1.1mm, with a WT index of 2.9±0.8. Significantly lower mean WT in the apical segments were identified in both the control group (basal 6.7±1.3 vs. mid 6.0±1.3 vs. apical 4.6±1.0mm, P<.0001) and in all evaluated cardiomyopathies (hypertrophic cardiomyopathy: basal 10.5±2.4 vs. mid 10.8±2.7 vs. apical 7.3±3.3mm, P<.0001; idiopathic dilated cardiomyopathy: basal 7.7±1.7 vs. mid 7.6±1.3 vs. apical 5.4±1.3mm, P<.0001; ischemic cardiomyopathy: basal 7.4±1.7 vs. mid 7.5±1.9 vs. apical 5.5±1.8mm, P<.0001; myocarditis: basal 7.1±1.5 vs. mid 6.4±1.1 vs. apical 5.1±0.8, P<.0001). Significant gender differences were also evident regarding the mean WT both in the control group (male 6.5±2.1 vs. female 5.2±1.7mm, P<.0001), as in hypertrophic cardiomyopathy (10.5±5.3 vs. 8.5±5.7mm, P<.0001) and myocarditis (6.6±2.0 vs. 5.2±1.6mm, P<.0001). We found a relatively high prevalence of segments commonly deemed thinned among patients without structural heart disease. We also observed a marked asymmetry and longitudinal gradient in wall thickness both in controls and in the various cardiomyopathies evaluated. Copyright © 2016 Instituto Nacional de Cardiología Ignacio Chávez. Publicado por Masson Doyma México S.A. All rights reserved.

  16. Cardiomyopathy in the pediatric patients

    Directory of Open Access Journals (Sweden)

    Shi-Min Yuan

    2018-04-01

    Full Text Available Pediatric cardiomyopathies are a group of myocardial diseases with complex taxonomies. Cardiomyopathy can occur in children at any age, and it is a common cause of heart failure and heart transplantation in children. The incidence of pediatric cardiomyopathy is increasing with time. They may be associated with variable comorbidities, which are most often arrhythmia, heart failure, and sudden death. Medical imaging technologies, including echocardiography, cardiac magnetic resonance, and nuclear cardiology, are helpful in reaching a diagnosis of cardiomyopathy. Nevertheless, endomyocardial biopsy is the final diagnostic method of diagnosis. Patients warrant surgical operations, such as palliative operations, bridging operations, ventricular septal maneuvers, and heart transplantation, if pharmaceutical therapies are ineffective. Individual therapeutic regimens due to pediatric characteristics, genetic factors, and pathogenesis may improve the effects of treatment and patients' survival. Key Words: cardiomyopathy, classification, pediatrics

  17. Dilatancy in Slow Granular Flows

    Science.gov (United States)

    Kabla, Alexandre J.; Senden, Tim J.

    2009-06-01

    When walking on wet sand, each footstep leaves behind a temporarily dry impression. This counterintuitive observation is the most common illustration of the Reynolds principle of dilatancy: that is, a granular packing tends to expand as it is deformed, therefore increasing the amount of porous space. Although widely called upon in areas such as soil mechanics and geotechnics, a deeper understanding of this principle is constrained by the lack of analytical tools to study this behavior. Using x-ray radiography, we track a broad variety of granular flow profiles and quantify their intrinsic dilatancy behavior. These measurements frame Reynolds dilatancy as a kinematic process. Closer inspection demonstrates, however, the practical importance of flow induced compaction which competes with dilatancy, leading more complex flow properties than expected.

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

    Energy Technology Data Exchange (ETDEWEB)

    Uehara, Toshiisa [Osaka Univ., Suita (Japan). Medical School; Ishida, Yoshio; Hayashida, Kohei [and others

    1998-04-01

    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)

  19. The Use of a Novel Heart Failure Agent in the Treatment of Pregnancy-Associated Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Vamsi C. Gaddipati

    2017-01-01

    Full Text Available Peripartum cardiomyopathy is an uncommon, pregnancy-related form of dilated cardiomyopathy that is associated with development of new-onset left ventricular dysfunction. Its etiology is presently unknown, but current standard of care involves the use of typical drug therapy for the treatment of heart failure. Pregnancy-associated cardiomyopathy (PACM is a similar condition that refers to patients who develop such symptoms prior to the last month of pregnancy. We report the case of a nulliparous Caucasian female who develops early, severe PACM during her first pregnancy with postpartum persistence of New York Heart Association class II-III symptoms despite medical therapy. The use of the novel heart failure agent, sacubitril/valsartan (Entresto, is initiated with near-complete resolution of her symptoms.

  20. Peripartum cardiomyopathy in Denmark

    DEFF Research Database (Denmark)

    Ersbøll, Anne S; Johansen, Marianne; Damm, Peter

    2017-01-01

    and searched for cardiomyopathy and heart failure ICD-10 diagnoses in a period of nine months before to 12 months after a delivery from 1 January 2005 through 31 December 2014. Diagnoses were validated and additional data were extracted from patient charts. A total of 61 women met the inclusion criteria...... equalling 1 in 10 149 deliveries. The majority recovered left ventricular systolic function within one year, but 14.8% suffered a major adverse event with 3.3% mortality, 8.2% mechanical circulatory support requirement and/or heart transplantation and 4.9% persistent severe heart failure. Half of the women...

  1. Recent advances of {sup 123}I-BMIPP myocardial SPECT for evaluating ischemic heart disease and cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Nishimura, T. [Tracer Kinetics and Nuclear Medicine, Osaka Univ., Medical School (Japan)

    1998-12-31

    Recent advances of {sup 123}I-BMIPP myocardial SPECT for evaluating ischemic heart disease and cardiomyopathy were reviewed. The dissociation between BMIPP and thallium defects was frequently observed in patients with acute myocardial infarction with successful reperfusion. The degree and improvement of perfusion/metabolism mismatch may reflect subsequent recovery from postischemic wall motion abnormality. BMIPP myocardial SPECT is also sensitive for detecting myocardial ischemia in acute coronary syndrome. BMIPP defects were observed at sites that corresponded to hypertrophic cardiomyopathy. On the other hand, the dissociation between BMIPP and thallium defects was not observed frequently in dilated cardiomyopathy. However, the heart to the mediastinum ratio and defect score were significantly decreased after coenzyme Q10 treatment. It was confirmed to be sensitive in evaluating the therapeutic effect. Furthermore, the basic mechanisms of BMIPP uptake were also reviewed in relation to BMIPP metabolism and longchain fatty acid transporter (CD36). (orig.) [Deutsch] Es wird eine Uebersicht ueber neueste Entwicklungen der {sup 123}I-BMIPP-Myokard-SPECT fuer die Diagnostik ischaemischer Herzerkrankungen und der Kardiomyopathie praesentiert. Regelmaessig wurde eine Dissoziation zwischen BMIPP- und Thallium-Defekten bei Patienten mit akutem Myokardinfarkt und erfolgreicher Reperfusion beobachtet. Die BMIPP-Aufnahme ist also im erfolgreich therapierten Myokard unmittelbar nach der Reperfusion gestoert, obwohl die Durchblutung wiederhergestellt wurde. Der Grad und die Entwicklung der Perfusions-Stoffwechsel-Mismatche repraesentieren eine spaetere Erholung von der postischaemischen Wandbewegungsstoerung. Die Ruhe-BMIPP-SPECT ist ein sehr sensitives Verfahren, um die Myokardischaemie beim akuten Koronarsyndrom zu erfassen. Diese Methoden sind unter dem Aspekt der Myokardvitalitaet beim Notfallpatienten fuer das Patientenmanagement wertvoll. In 80% der Patienten mit

  2. Stress Cardiomyopathy in the Setting of COPD Exacerbation

    Directory of Open Access Journals (Sweden)

    Kevin Landefeld

    2015-10-01

    Full Text Available Introduction. Stress cardiomyopathy, or takotsubo cardiomyopathy, is an acute, reversible left ventricular dysfunction usually initiated by a psychological or physical stress. We report this case of stress cardiomyopathy following a chronic obstructive pulmonary disease exacerbation and the subsequent treatment. Case Description. A 49-year-old white female with a history of chronic obstructive pulmonary disease presented to the emergency room via emergency medical services with worsening severe shortness of breath and productive cough for 2 weeks but denied any chest pain on arrival. On presentation, she was noted to be tachypneic, using her accessory muscles and with bilateral coarse expiratory wheezing on lung auscultation. Initial electrocardiogram demonstrated sinus tachycardia. She was treated with multiple albuterol treatments. Soon afterwards, the course was complicated by hypoxic respiratory failure eventually requiring intubation. Her repeat electrocardiogram showed acute changes consistent with myocardial infarction, and an echocardiograph demonstrated apical akinesia with an ejection fraction of 25% to 30%. The patient was urgently taken for cardiac catheterization, which showed no angiographic evidence of coronary artery disease. Three days after initial presentation, a repeat transthoracic echocardiogram showed overall left ventricular systolic function improvement. Discussion. This case provided a unique look at the difficulty of balancing catecholamines in a patient with bronchospasm and stress cardiomyopathy.

  3. Improvement of exercise capacity and left ventricular diastolic function with metoprolol XL after acute myocardial infarction

    DEFF Research Database (Denmark)

    Poulsen, S H; Jensen, S E; Egstrup, K

    2000-01-01

    BACKGROUND: Left ventricular (LV) diastolic function predicts and correlates with exercise capacity. Beta-blockers improve exercise capacity and LV diastolic function in patients with severe LV systolic dysfunction in dilated cardiomyopathy. However, information on the effect of metoprolol XL...... on exercise capacity in relation to LV diastolic function in patients with mild to moderate LV systolic dysfunction after acute myocardial infarction is limited. METHODS: In a randomized, double-blind, placebo-controlled study of 77 patients, a subgroup of 59 patients with mild to moderate LV systolic...... dysfunction after acute myocardial infarction were given metoprolol XL (n = 29) or placebo (n = 30). The effects of metoprolol XL on exercise capacity in relation to effects on LV diastolic filling were studied. Two-dimensional Doppler echocardiography and maximal symptom limited bicycle test were performed...

  4. Human herpesvirus 6-induced inflammatory cardiomyopathy in immunocompetent children

    Directory of Open Access Journals (Sweden)

    Surabhi Reddy

    2017-01-01

    Full Text Available Over the last decade, human herpesvirus 6 (HHV-6 has been implicated in the etiology of pediatric myocarditis and subsequent dilated cardiomyopathy (DCM. This review provides an overview of recent literature investigating the pathophysiological relevance of HHV-6 in inflammatory cardiomyopathy. We examined 11 cases of previously published pediatric myocarditis and/or DCM associated with HHV-6 and also our experience of detection of virus particles in vascular endothelium of HHV-6 positive endomyocardial biopsy tissue by electron microscopy. The exact role of the presence of HHV-6 and its load remains controversial as the virus is also found in the heart of healthy controls. Therefore, the question remains open whether and how cardiac HHV-6 may be of pathogenetic importance. Quantitative polymerase chain reaction or mRNA testing allows differentiation between low-level latent virus found in asymptomatic myocardium and active HHV-6 infection. Although only a small number of pediatric cases have been reported in literature, HHV-6 should be considered as a causative agent of inflammatory cardiomyopathy, especially in children under three who might be experiencing a primary infection. Future studies are needed to establish a threshold for determining active infection in biopsy samples and the role of coinfections other cardiotropic viruses.

  5. Clinical and Prognostic Profiles of Cardiomyopathies Caused by Mutations in the Troponin T Gene.

    Science.gov (United States)

    Ripoll-Vera, Tomás; Gámez, José María; Govea, Nancy; Gómez, Yolanda; Núñez, Juana; Socías, Lorenzo; Escandell, Ángela; Rosell, Jorge

    2016-02-01

    Mutations in the troponin T gene (TTNT2) have been associated in small studies with the development of hypertrophic cardiomyopathy characterized by a high risk of sudden death and mild hypertrophy. We describe the clinical course of patients carrying mutations in this gene. We analyzed the clinical characteristics and prognosis of patients with mutations in the TNNT2 gene who were seen in an inherited cardiac disease unit. Of 180 families with genetically studied cardiomyopathies, 21 families (11.7%) were identified as having mutations in TNNT2: 10 families had Arg92Gln, 5 had Arg286His, 3 had Arg278Cys, 1 had Arg92Trp, 1 had Arg94His, and 1 had Ile221Thr. Thirty-three additional genetic carriers were identified through family assessment. The study included 54 genetic carriers: 56% were male, and the mean average age was 41 ± 17 years. There were 33 cases of hypertrophic cardiomyopathy, 9 of dilated cardiomyopathy, and 1 of noncompaction cardiomyopathy, and maximal myocardial thickness was 18.5 ± 6mm. Ventricular dysfunction was present in 30% of individuals and a history of sudden death in 62%. During follow-up, 4 patients died and 14 (33%) received a defibrillator (8 probands, 6 relatives). Mean survival was 54 years. Carriers of Arg92Gln had early disease development, high penetrance, a high risk of sudden death, a high rate of defibrillator implantation, and a high frequency of mixed phenotype. Mutations in the TNNT2 gene were more common in this series than in previous studies. The clinical and prognostic profiles depended on the mutation present. Carriers of the Arg92Gln mutation developed hypertrophic or dilated cardiomyopathy and had a significantly worse prognosis than those with other mutations in TNNT2 or other sarcomeric genes. Copyright © 2015 Sociedad Española de Cardiología. Published by Elsevier España, S.L.U. All rights reserved.

  6. Trastuzumab (Herceptin)-associated cardiomyopathy presented as new onset of complete left bundle-branch block mimicking acute coronary syndrome: a case report and literature review.

    Science.gov (United States)

    Tu, Chung-Ming; Chu, Kai-Ming; Yang, Shin-Ping; Cheng, Shu-Mung; Wang, Wen-Been

    2009-09-01

    Trastuzumab (Herceptin) is well documented in reducing suffering and mortality from breast cancer. The clinically most important side effect of Herceptin is cardiotoxicity, which is reported in 2.6% to 4.5% of patients receiving trastuzumab alone and in as many as 27% of patients when trastuzumab is combined with an anthracycline in metastatic disease. We reported the case of a 50-year-old woman who presented to our emergency department (ED) because of chest pain and shortness of breath. On physical examination, holosystolic murmur over apex could be heard. Pulmonary and abdominal examinations were unremarkable. Twelve-lead electrocardiography showed sinus tachycardia and new onset of complete left bundle-branch block. Emergent transthoracic echocardiography revealed generalized hypokinesia of left ventricle and akinesia over interventricular septum and apex. She subsequently underwent immediate coronary angiography that revealed normal coronary angiography, and left ventriculogram revealed generalized hypokinesia with severe left ventricle dysfunction with ejection fraction of 33%. During right heart catheterization and endomyocardial biopsy, cardiac tamponade developed and was successfully relieved by pericardial window. She was discharged event-free 3 weeks later with conservative treatment. Although new onset of complete left bundle-branch block in a patient with chest pain may be acute coronary syndrome, careful review of medicine history is mandatory to avoid unnecessary procedure and complications.

  7. Takotsubo Cardiomyopathy With Significant Coronary Stenosis and Atrioventricular Conduction Block: A Rare Case Report With 3 Year Follow-Up

    OpenAIRE

    Saadatifar, Hakimeh; Khoshhal Dehdar, Fahimeh; Saadatifar, Samira; Moshkani Farahani, Maryam

    2015-01-01

    Introduction Takotsubo cardiomyopathy (TCMP) is a rare acute cardiomyopathy characterized by acute chest pain syndrome, similar to myocardial infarction, except that no significant stenosis is observed on coronary angiography in patients with this condition; these findings aid the diagnosis of TCMP. Case Presentation We discuss an unusual case of TCMP in a 45-year-old woman with complete heart block and significant coronary artery...

  8. Isolated cardiomyopathy caused by a DMD nonsense mutation in somatic mosaicism: genetic normalization in skeletal muscle.

    Science.gov (United States)

    Juan-Mateu, J; Paradas, C; Olivé, M; Verdura, E; Rivas, E; González-Quereda, L; Rodríguez, M J; Baiget, M; Gallano, P

    2012-12-01

    X-linked dilated cardiomyopathy is a pure cardiac dystrophinopathy phenotype mainly caused by DMD mutations that present a specific transcription effect in cardiac tissue. We report a 26-year-old male who presented with severe dilated cardiomyopathy and high creatine kinase. The patient did not complain of skeletal muscle weakness. A muscle biopsy showed mild dystrophic changes and a low proportion of dystrophin-negative fibres. A molecular study identified a nonsense DMD mutation (p.Arg2098X) in somatic mosaicism. The ratio of mutant versus normal allele in blood and skeletal muscle suggests selective pressure against mutant muscle cells, a process known as genetic normalization. We hypothesize that this process may have mitigated skeletal muscle symptoms in this patient. This is the second report of a DMD somatic mosaic with evidence of genetic normalization in muscle. Somatic DMD mutations should be considered in patients presenting with idiopathic dilated cardiomyopathy. © 2011 John Wiley & Sons A/S. Published by Blackwell Publishing Ltd.

  9. Hypertrophic cardiomyopathy: a review.

    Science.gov (United States)

    Hensley, Nadia; Dietrich, Jennifer; Nyhan, Daniel; Mitter, Nanhi; Yee, May-Sann; Brady, MaryBeth

    2015-03-01

    Hypertrophic cardiomyopathy (HCM) is a relatively common disorder that anesthesiologists encounter among patients in the perioperative period. Fifty years ago, HCM was thought to be an obscure disease. Today, however, our understanding and ability to diagnose patients with HCM have improved dramatically. Patients with HCM have genotypic and phenotypic variability. Indeed, a subgroup of these patients exhibits the HCM genotype but not the phenotype (left ventricular hypertrophy). There are a number of treatment modalities for these patients, including pharmacotherapy to control symptoms, implantable cardiac defibrillators to manage malignant arrhythmias, and surgical myectomy and septal ablation to decrease the left ventricular outflow obstruction. Accurate diagnosis is vital for the perioperative management of these patients. Diagnosis is most often made using echocardiographic assessment of left ventricular hypertrophy, left ventricular outflow tract gradients, systolic and diastolic function, and mitral valve anatomy and function. Cardiac magnetic resonance imaging also has a diagnostic role by determining the extent and location of left ventricular hypertrophy and the anatomic abnormalities of the mitral valve and papillary muscles. In this review on hypertrophic cardiomyopathy for the noncardiac anesthesiologist, we discuss the clinical presentation and genetic mutations associated with HCM, the critical role of echocardiography in the diagnosis and the assessment of surgical interventions, and the perioperative management of patients with HCM undergoing noncardiac surgery and management of the parturient with HCM.

  10. 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.)

  11. Microfibrillar cardiomyopathy: A rare case

    Directory of Open Access Journals (Sweden)

    Narender Kumar

    2011-01-01

    Full Text Available Microfibrillar cardiomyopathy is a very rare cause of restrictive cardiomyopathy (RCM. The index case was a male patient who presented with shortness of breath and pedal edema. Further clinical investigations favored a clinical diagnosis of RCM. An endomyocardial biopsy revealed subendocardial and interstitial hyaline eosinophillic material resembling amyloid that did not stain with Congo red. An electron microscopic examination showed that this material was composed of twisted linear and bundles of tangled microfibrils. The etiology of the microfibrillar deposition is currently unknown. The pathologists should entertain the diagnosis of microfibrillar cardiomyopathy in suspected cases of amyloidosis that are negative for Congo red.

  12. Genetics of cardiomyopathies in children

    Directory of Open Access Journals (Sweden)

    Matteo Vatta

    2011-08-01

    Full Text Available Cardiomyopathies are diseases of the heart muscle leading to heart failure and/or an increased risk of arrhythmogenic sudden cardiac death. These disorders represent a major cause of morbidity and mortality in children. In childhood forms of cardiomyopathy, genetic etiologies are frequent, but non-genetic or acquired causes, such viral infection, also play a significant role. In the last twenty years, the genetic causes of cardiomyopathies have been increasingly identified and clinical correlations are beginning to be defined. Here we present an overview of the recent advances in our understanding of the genetics of cardiomyopathies in children and what is known about the pathophysiological mechanisms underlying these gene-related forms of disease.

  13. Selective decrease of components of the creatine kinase system and ATP synthase complex in chronic Chagas disease cardiomyopathy.

    Directory of Open Access Journals (Sweden)

    Priscila Camillo Teixeira

    2011-06-01

    Full Text Available BACKGROUND: Chronic Chagas disease cardiomyopathy (CCC is an inflammatory dilated cardiomyopathy with a worse prognosis than other cardiomyopathies. CCC occurs in 30 % of individuals infected with Trypanosoma cruzi, endemic in Latin America. Heart failure is associated with impaired energy metabolism, which may be correlated to contractile dysfunction. We thus analyzed the myocardial gene and protein expression, as well as activity, of key mitochondrial enzymes related to ATP production, in myocardial samples of end-stage CCC, idiopathic dilated (IDC and ischemic (IC cardiomyopathies. METHODOLOGY/PRINCIPAL FINDINGS: Myocardium homogenates from CCC (N=5, IC (N=5 and IDC (N=5 patients, as well as from heart donors (N=5 were analyzed for protein and mRNA expression of mitochondrial creatine kinase (CKMit and muscular creatine kinase (CKM and ATP synthase subunits aplha and beta by immunoblotting and by real-time RT-PCR. Total myocardial CK activity was also assessed. Protein levels of CKM and CK activity were reduced in all three cardiomyopathy groups. However, total CK activity, as well as ATP synthase alpha chain protein levels, were significantly lower in CCC samples than IC and IDC samples. CCC myocardium displayed selective reduction of protein levels and activity of enzymes crucial for maintaining cytoplasmic ATP levels. CONCLUSIONS/SIGNIFICANCE: The selective impairment of the CK system may be associated to the loss of inotropic reserve observed in CCC. Reduction of ATP synthase alpha levels is consistent with a decrease in myocardial ATP generation through oxidative phosphorylation. Together, these results suggest that the energetic deficit is more intense in the myocardium of CCC patients than in the other tested dilated cardiomyopathies.

  14. Microvascular dysfunction in nonfailing arrhythmogenic right ventricular cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Paul, Matthias [University Hospital Muenster, Department of Cardiology and Angiology, Muenster (Germany); University Hospital Muenster, Institute for Genetics of Heart Diseases, Muenster (Germany); Rahbar, Kambiz; Kies, Peter; Schober, Otmar [University Hospital Muenster, Department of Nuclear Medicine, Muenster (Germany); Gerss, Joachim [University of Muenster, Institute of Biostatistics and Clinical Research, Muenster (Germany); Schaefers, Klaus; Schaefers, Michael [University of Muenster, European Institute for Molecular Imaging - EIMI, Muenster (Germany); Breithardt, Guenter [University Hospital Muenster, Department of Cardiology and Angiology, Muenster (Germany); Schulze-Bahr, Eric [University Hospital Muenster, Institute for Genetics of Heart Diseases, Muenster (Germany); Wichter, Thomas [Marienhospital Osnabrueck, Department of Cardiology, Niels-Stensen-Kliniken, Osnabrueck (Germany)

    2012-03-15

    Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a nonischaemic cardiomyopathy and leading cause of sudden death in the young. It has been shown that microvascular dysfunction reflected by an impaired myocardial blood flow (MBF) response to stress is present in patients with other forms of nonischaemic cardiomyopathy, e.g. dilated cardiomyopathy, and that the reduced MBF may be related to a poor prognosis. Therefore, we quantified MBF, coronary flow reserve and coronary vascular resistance in patients with nonfailing ARVC using H{sub 2}{sup 15} O and PET. In ten male patients with ARVC (mean age 49 {+-} 14 years), MBF was quantified at rest and during adenosine-induced hyperaemia using H{sub 2}{sup 15} O PET. Results were compared with those obtained in 20 age-matched healthy male control subjects (mean age 46 {+-} 14 years). Resting MBF was not significantly different between patients with ARVC and controls (MBF{sub rest} 1.19 {+-} 0.29 vs. 1.12 {+-} 0.20 ml/min/ml). However, hyperaemic MBF was significantly lower in patients with ARVC than in controls (2.60 {+-} 0.96 vs. 3.68 {+-} 0.84 ml/min/ml; p = 0.005). Consequently, patients with ARVC had a significantly lower coronary flow reserve than control subjects (2.41 {+-} 1.34 vs. 3.39 {+-} 0.93; p = 0.030). In addition, hyperaemic coronary vascular resistance was increased in patients with ARVC (36.79 {+-} 12.91 vs. 26.31 {+-} 6.49 mmHg x ml{sup -1} x min x ml; p = 0.007), but was found to be unchanged at rest. In this small well-characterized cohort of patients with nonfailing ARVC, we found a significantly reduced hyperaemic MBF and increased coronary vascular resistance. Further studies are necessary to corroborate this potential new functional aspect of the pathophysiological mechanisms underlying ARVC. (orig.)

  15. New insights into cirrhotic cardiomyopathy

    DEFF Research Database (Denmark)

    Møller, Søren; Hove, Jens D; Dixen, Ulrik

    2013-01-01

    Cirrhotic cardiomyopathy designates a cardiac dysfunction, which includes reduced cardiac contractility with systolic and diastolic dysfunction, and presence of electrophysiological abnormalities in particular prolongation of the QT interval. Several pathophysiological mechanisms including reduced...... beta-receptor function seem involved in the autonomic and cardiac dysfunction. Cirrhotic cardiomyopathy can be revealed by tissue Doppler imaging but is best demasked by physical or pharmacological stress. Liver transplantation may revert cardiac dysfunction but surgery and shunt insertion may also...

  16. Tachycardia-Induced Cardiomyopathy in a 12-Year-Old Child With Long QT Syndrome

    Directory of Open Access Journals (Sweden)

    Ghandi

    2016-05-01

    Full Text Available Introduction Tachycardia-induced cardiomyopathy (TIC is a ventricular dysfunction secondary to chronic and persistent tachycardia that can regress partially or completely following heart rate normalization. Paroxysmal atrial tachycardia and permanent junctional reciprocating tachycardia are two types of frequent arrhythmias that can cause cardiomyopathy in children. Case Presentation A 12-year-old child with obesity (body mass index > 26.8 was admitted with fatigue, pallor and tachypnea to the clinic. He had palpitation for the past 24 hours. On the cardiac auscultation, holosystolic 2/6 murmur was heard in the apex as well as gallop rhythm. Electrocardiogram revealed heart rate of 150 - 160 bpm and negative P waves in II, III and AVF leads. The echocardiography revealed dilated cardiomyopathy with an ejection fraction of 30%. Conclusions Diagnosis of tachycardia-induced cardiomyopathy in children is important, since appropriate treatment improves the prognosis. Every child with recurrent and persistent palpitation with the first episode of congestive heart failure should be evaluated for tachycardia- induced cardiomyopathy.

  17. Arrhythmogenic right ventricular cardiomyopathy.

    Science.gov (United States)

    Soni, Roopali; Oade, Yvette

    2011-08-24

    Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare inherited disease of the heart muscle that causes ventricular tachyarrhythmias and sudden death in young people and athletes. It results in fibrofatty replacement of the right ventricle, and the subepicardial region of the left ventricle. It is the most common cause of sudden cardiac death in young people after hypertrophic heart disease. Diagnosis can be difficult and at present there is no cure for ARVC. Prevention of sudden death is the most important management strategy. Paediatricians need to be aware of the possibility of ARVC in adolescents and young adults presenting with palpitations, fatigue, syncope or cardiac arrest. The authors present two cases of apparently healthy teenage boys who died suddenly and unexpectedly. Postmortem examination of the myocardium was strongly suggestive of ARVC in both cases.

  18. [Peripartum cardiomyopathy: a case report].

    Science.gov (United States)

    Karchmer-Krivitzky, S; Espinosa-Fernández, R; Sánchez-Aranda, A; LópezRioja, M J; Monzalbo-Núñez, D

    2016-08-01

    Peripartum cardiomyopathy also known as cardiomyopathy associated with pregnancy, is rarely a cause of heart failure, it affects pregnant or puerperal women in the first 5 months. Although the first case reported was in 1849, it was recognized until 1930. In 2010 the European Society of Cardiology Working Group on peripartum cardiomyopathy, defined this pathology as an idiopathic cardiomyopathy that affects pregnant women between the third trimester and five months after delivery. Characterized by a left ventricular failure with an ejection fraction of ≤45% and an end-diastolic dimension ≥2.7 cm/m2 , in absence of an identifiable cause of heart failure. We report a case of a 39-year-old patient, diagnosed with a peripartum cardiomyopathy in the early puerperium, characterized by hypertension, tachycardia, dyspnea and oxygen desaturation. The transesophageal echocardiogram reported heart failure, a hypokinetic left ventricle and a ventricular failure with an ejection fraction <40%. We could not identify an other cause to justify heart failure. Multidisciplinary management was administered successfully. The importance of this article relies in the fact that eripartum cardiomyopathy has a high morbidity and mortality. The impact of this pathology is unknow in our country. Here we establish and discuss the multidisciplinary management held in our hospital with this specific patient in order to improve the prognosis on future occasions.

  19. DIFFERENTIAL DIAGNOSIS OF HYPERTROPHIC CARDIOMYOPATHY

    Directory of Open Access Journals (Sweden)

    I. V. Leontyeva

    2017-01-01

    Full Text Available Hypertrophic cardiomyopathy is the most common form of cardiomyopathy, occurring in childhood, occurring when a gene is mutated that encodes proteins of sarcomeric and non-sarcomeric complexes. The diagnosis of the disease is based on the data of echocardiography, revealing structural changes in the heart muscle according to the type of hypertrophy, while the genesis of these changes remains unclear. The causes of hypertrophic cardiomyopathy in childhood are diverse. Of great importance is the early diagnosis of metabolic forms of hypertrophic cardiomyopathy, so in some cases regress of hypertrophy is possible against the background of enzyme-substitution or other drug therapy. The article presents a clinical (cardiac and extracardiac symptoms and laboratory markers of hypertrophic cardiomyopathy with mutations of genes of proteins of the sarcomeric complex, congenital metabolic disorders (glycogenoses, lysosomal pathology, fatty acid metabolism disorders, and mitochondrial diseases, genetic syndromes (Noonan, LEOPARD, Costello, cardio-fascial-cutaneous, neuromuscular diseases. The criteria for differential diagnosis of genetic forms of hypertrophic cardiomyopathy and myocardial hypertrophy in athletes are presented. 

  20. Hypertrophic Cardiomyopathy Complicated by Pulmonary Edema in the Postpartum Period

    Directory of Open Access Journals (Sweden)

    Kate Hanneman

    2013-01-01

    Full Text Available We report the case of a 42-year-old patient with hypertrophic cardiomyopathy (HCM who presented to the emergency department with severe shortness of breath one week following uneventful cesarean delivery. Thoracic CT ruled out pulmonary embolus and confirmed pulmonary edema. Asymmetric interventricular septal thickening was clearly identified, demonstrating that the heart may be evaluated even on a non-ECG gated study. Acute pulmonary edema in the postpartum period is an unusual clinical presentation of HCM.

  1. Reproducibility and genital sparing with a vaginal dilator used for female anal cancer patients.

    Science.gov (United States)

    Briere, Tina Marie; Crane, Christopher H; Beddar, Sam; Bhosale, Priya; Mok, Henry; Delclos, Marc E; Krishnan, Sunil; Das, Prajnan

    2012-08-01

    Acute vulvitis, acute urethritis, and permanent sexual dysfunction are common among patients treated with chemoradiation for squamous cell carcinoma of the anal canal. Avoidance of the genitalia may reduce sexual dysfunction. A vaginal dilator may help delineate and displace the vulva and lower vagina away from the primary tumor. The goal of this study was to evaluate the positional reproducibility and vaginal sparing with the use of a vaginal dilator. Ten female patients treated with IMRT for anal cancer were included in this study. A silicone vaginal dilator measuring 29 mm in diameter and 114 mm in length was inserted into the vagina before simulation and each treatment. The reproducibility of dilator placement was investigated with antero-posterior and lateral images acquired daily. Weekly cone beam CT (CBCT) imaging was used to confirm coverage of the GTV, which was typically posterior and inferior to the dilator apex. Finally, a planning study was performed to compare the vaginal doses for these 10 patients to a comparable group of 10 female patients who were treated for anal cancer with IMRT without vaginal dilators. The absolute values of the location of the dilator apex were 7.0 ± 7.8mm in the supero-inferior direction, 7.5 ± 5.5 mm in the antero-posterior, and 3.8 ± 3.1mm in the lateral direction. Coverage of the GTV and CTV was confirmed from CBCT images. The mean dose to the vagina was lower by 5.5 Gy, on average, for the vaginal dilator patients, compared to patients treated without vaginal dilators. The vaginal dilator tended to be inserted more inferiorly during treatment than during simulation. For these ten patients, this did not compromise tumor coverage. Combined with IMRT treatment planning, use of a vaginal dilator could allow for maximum sparing of female genitalia for patients undergoing radiation therapy for anal cancer. Published by Elsevier Ireland Ltd.

  2. Current Indications for Implantable Cardioverter Defibrillators in Non-Ischemic Cardiomyopathies and Channelopathies.

    Science.gov (United States)

    González-Torrecilla, Esteban; Arenal, Angel; Atienza, Felipe; Datino, Tomás; Bravo, Loreto; Ruiz, Pablo; Ávila, Pablo; Fernández-Avilés, Francisco

    2015-01-01

    Current indications for implantable cardioverter defibrillators (ICDs) in patients with channelopathies and cardiomyopathies of non-ischemic origin are mainly based on non-randomized evidence. In patients with nonischemic dilated cardiomyopathy (NIDCM), there is a tendency towards a beneficial effect on total mortality of ICD therapy in patients with significant left ventricular (LV) dysfunction. Although an important reduction in sudden cardiac death (SCD) seems to be clearly demonstrated in these patients, a net beneficial effect on total mortality is unclear mostly in cases with good functional status. Risk stratification has been changing over the last two decades in patients with hypertrophic cardiomyopathy (HCM). Its risk profile has been delineated in parallel with the beneficial effect of ICD in high risk patients. Observational results based on "appropriate" ICD interventions do support its usefulness both in primary and secondary SCD prevention in these patients. Novel risk models quantify the rate of sudden cardiac death in these patients on individual basis. Less clear risk stratification is available for cases of arrhythmogenic right ventricular cardiomyopathy (ARVC) and in other uncommon familiar cardiomyopathies. Main features of risk stratification vary among the different channelopathies (long QT syndrome -LQTS-, Brugada syndrome, etc) with great debate on the management of asymptomatic patients. For most familiar cardiomyopathies, ICD therapy is the only accepted strategy in the prevention of SCD. So far, genetic testing has a limited role in risk evaluation and management of the individual patient. This review aims to summarize these criticisms and to refine the current indications of ICD implantation in patients with cardiomyopathies and major channelopathies.

  3. Cardiomyopathy in Patients With Hereditary Bullous Epidermolysis.

    Science.gov (United States)

    Batalla, A; Vicente, A; Bartrons, J; Prada, F; Fortuny, C; González-Enseñat, M A

    In recent decades, an association has been reported between epidermolysis bullosa (EB) and dilated cardiomyopathy (DC). DC is typically in an advanced phase when detected, leading to a poorer prognosis. Our objective was to determine the prevalence of DC in patients with EB seen in Hospital San Joan de Déu in Barcelona, Spain, between May 1986 and April 2015. This was a descriptive, cross-sectional chart-review study in which we recorded the type and main subtypes of EB and the presence or absence of DC. Fifty-seven patients with EB were found, 19 with EB simplex, 10 with junctional EB, 27 with dystrophic EB (14 dominant dystrophic and 13 recessive dystrophic), and just 1 with Kindler syndrome. DC was detected in only 2 patients with recessive dystrophic EB. Twenty-three patients had presented factors that could have had a causal relationship with the potential onset of DC. DC is a possible complication of EB, particularly in recessive dystrophic EB. Periodic follow-up should be performed to make an early diagnosis and start treatment. Copyright © 2017 AEDV. Publicado por Elsevier España, S.L.U. All rights reserved.

  4. Cardiogenic Shock due to Psychosis-Induced Inverted Takotsubo Cardiomyopathy Bridged-to-Recovery with a Percutaneous Left Ventricular Assist Device

    Directory of Open Access Journals (Sweden)

    Ravi Korabathina

    2016-01-01

    Full Text Available Inverted Takotsubo cardiomyopathy, a less common variant in the spectrum of stress-induced cardiomyopathy, is increasingly being reported. This report describes an acute psychiatric illness leading to the onset of this syndrome. The patient presented here developed cardiogenic shock but successfully recovered with the use of a percutaneous left ventricular assist device.

  5. Brain Abscess after Esophageal Dilatation

    DEFF Research Database (Denmark)

    Gaïni, S; Grand, M; Michelsen, J

    2007-01-01

    with malaise, progressive lethargy, fever, aphasia and hemiparesis. Six days before she had been treated with esophageal dilatation for a stricture caused by accidental ingestion of caustic soda. The brain abscess was treated with surgery and antibiotics. She recovered completely. This clinical case...

  6. Takotsubo cardiomyopathy: A known unknown foe of asthma.

    Science.gov (United States)

    Kotsiou, Ourania S; Douras, Alexandros; Makris, Demosthenes; Mpaka, Nikoleta; Gourgoulianis, Konstantinos I

    2017-10-01

    Patients with uncontrolled asthma are at a greater risk of asthma attacks requiring emergency room visits or hospital admissions. Takotsubo cardiomyopathy is potentially a significant complication in a course of status asthmaticus. We describe a 43-year-old female patient who presented with status asthmaticus that was further complicated with takotsubo cardiomyopathy. Recognizing apical ballooning syndrome is challenging in patients with a history of respiratory disease because the symptoms of the last entity may complicate the diagnostic approach. It is difficult to distinguish clinically apical ballooning syndrome from the acute airway exacerbation itself. Both asthma and takotsubo cardiomyopathy share the same clinical presentation with dyspnea and chest tightness. In our patient, the electrocardiographic abnormalities, the rapidly reversible distinctive characteristics of echocardiography, and the modest elevation of serum cardiac biomarkers levels, in combination with the presence of a stress trigger (severe asthma attack), strongly supported the diagnosis of broken heart syndrome. Clinicians should re-evaluate asthma management and be aware of the complications associated with asthma attacks such as stress-induced cardiomyopathy.

  7. Psychological Features of Takotsubo Cardiomyopathy: Report of Four Cases.

    Science.gov (United States)

    Jenab, Yaser; Hashemi, Seyedeh Roghaieh; Ghaffari-Marandi, Neda; Zafarghandi, Hoda; Shahmansouri, Nazila

    2017-04-01

    Takotsubo or stress-induced cardiomyopathy is a cardiomyopathy in which the patient has a sudden onset, reversible left ventricular systolic dysfunction without any significant coronary artery disease. Four women, who were at a mean age of 64 years and suffered from chest pain exacerbated by emotional stress, were admitted as cases of acute coronary syndrome and were completely evaluated through precise history taking, physical examination, and ECG. Coronary angiography or coronary multidetector computed tomography was used to exclude significant coronary artery disease. In these patients with confirmed Takotsubo cardiomyopathy, in addition to the Diagnostic and Statistical Manual of the American Psychiatric Association (DSM-IV) criteria, a 71-item form of the Minnesota Multiphasic Personality Inventory (MMPI)-Mini-Mult-was employed for psychological assessment. The main common elevated scale was hypochondriasis. Individuals with high scores on this scale are obsessed with themselves, especially in regard to their body, and often use their disease symptoms in order to manipulate others. They are mainly passive aggressive, critical, and demanding, which stems from their lack of effective verbal abilities as a means of communication, specifically when it comes to anger or hostility expression. To the best of our knowledge, there is no available study evaluating patients with Takotsubo cardiomyopathy using the Mini-Mult questionnaire for psychological assessment.

  8. Pheochromocytoma presenting as takotsubo-like cardiomyopathy following delivery.

    Science.gov (United States)

    Jóźwik-Plebanek, Katarzyna; Pęczkowska, Mariola; Klisiewicz, Anna; Wrzesiński, Kazimierz; Prejbisz, Aleksander; Niewada, Maciej; Kabat, Marek; Szperl, Małgorzata; Eisenhofer, Graeme; Lenders, Jacques W; Januszewicz, Andrzej

    2014-12-01

    Diagnosis of pheochromocytoma during pregnancy can be difficult, and the tumor carries an unfavorable prognosis if not diagnosed and treated in a timely manner. To present a case of Takotsubo-like cardiomyopathy characterized by transient left ventricular apical ballooning due to pheochromocytoma following delivery. A few hours after Caesarean section, a 32-year-old Caucasian female presented with pulmonary edema followed by cardiac arrest with echocardiographic and ventriculographic evidence of reversible acute myocardial failure characteristic of Takotsubo-like cardiomyopathy. A previously unrecognized adrenal pheochromocytoma was found during her clinical work-up. Left ventricle (LV) function normalized after surgical removal of the tumor, which was carried out after implementing an alpha-adrenoreceptor blockade. Hemorrhagic necrosis of the pheochromocytoma was seen on histopathologic analysis; this may have triggered the sequence of events leading to the development of Takotsubo-like cardiomyopathy and hemodynamic collapse. To the best of our knowledge, this is the first reported case of Takotsubo-like cardiomyopathy related to pheochromocytoma following delivery. This emphasizes the increased cardiovascular risk if pheochromocytoma is not diagnosed and treated in a timely manner, especially during pregnancy.

  9. Risk of Cardiomyopathy in Younger Persons With a Family History of Death from Cardiomyopathy

    DEFF Research Database (Denmark)

    Ranthe, Mattis F; Carstensen, Lisbeth; Øyen, Nina

    2015-01-01

    BACKGROUND: Recommendations for presymptomatic screening of relatives of cardiomyopathy patients are based on findings from tertiary centers. Cardiomyopathy inheritance patterns are fairly well understood, but how cardiomyopathy in younger persons (<50 years) aggregates in families at the populat......BACKGROUND: Recommendations for presymptomatic screening of relatives of cardiomyopathy patients are based on findings from tertiary centers. Cardiomyopathy inheritance patterns are fairly well understood, but how cardiomyopathy in younger persons (families...... at the population level is unclear. In a nationwide cohort, we examined the risk of cardiomyopathy by family history of premature death (... ascertained family history of premature (

  10. Women's experiences of Takotsubo cardiomyopathy in a short-term perspective--a qualitative content analysis.

    Science.gov (United States)

    Dahlviken, Rønnaug M; Fridlund, Bengt; Mathisen, Lars

    2015-06-01

    Takotsubo cardiomyopathy is a reversible condition mimicking acute myocardial infarction. The phenomenon is associated with emotional and physical stressful trigger events. Evidence-based patient counselling should be based on disease-specific knowledge of patient experiences. The aim of the study was to describe women's experiences of Takotsubo cardiomyopathy in a short-term perspective. The study design was explorative and descriptive. Semi-structured interviews were conducted with 14 women diagnosed with Takotsubo cardiomyopathy, 1 day to 9 months after hospitalisation. The transcriptions underwent qualitative content analysis. The main theme that emerged was Takotsubo cardiomyopathy as a continuous process of making sense and adapting. To begin with, understanding and coping with signs and symptoms were described as having a diversity of signs and symptoms, taking actions towards signs and symptoms, receiving treatment for suspected ST/non ST-elevation myocardial infarction diagnosis and finally being diagnosed with Takotsubo cardiomyopathy. Understanding the context of illness was expressed as getting treated for Takotsubo cardiomyopathy diagnosis and having previous stressful conditions of life. The changing perspective that emanated was a combination of having prospects and expectations and experiencing limitations. Finally, managing to live with Takotsubo cardiomyopathy was manifested as returning home with the illness and receiving follow-up health care. Information on regaining prior health status and capacity within a short-term perspective may not be accurate. These women struggle and require education and counselling from healthcare professionals to comprehend and manage having a Takotsubo cardiomyopathy diagnosis. Women experiencing Takotsubo cardiomyopathy may be a target group for holistic and individual health care with a longer duration of follow-up. © 2014 Nordic College of Caring Science.

  11. Takotsubo cardiomyopathy in amiodarone-induced hyperthyroidism

    Directory of Open Access Journals (Sweden)

    Ismael Capel

    2017-02-01

    Full Text Available Takotsubo cardiomyopathy (TC is an atypical, severe but reversible form of acute heart insufficiency. It typically presents with left ventricular failure, transient apical and mid-segments hypokinesis, absence of significant coronary stenosis and new electrographic abnormalities and/or elevation in serum cardiac enzymes. Although TC (‘broken heart syndrome’ has classically been associated with emotional trauma, evidence suggests that other precipitants might exist, including iatrogenic and thyroid-mediated forms. Thyroid disease is a relatively common comorbidity in TC patients. We report a case of TC in a postmenopausal female with no history of emotional trauma or other potential precipitant factors who was diagnosed with amiodarone-induced hyperthyroidism during her hospital stay. Though some case reports of thyroidrelated TC exist, we are not aware of any other reported case of TC precipitated by amiodarone-induced hyperthyroidism.

  12. High resolution systematic digital histological quantification of cardiac fibrosis and adipose tissue in phospholamban p.Arg14del mutation associated cardiomyopathy.

    Directory of Open Access Journals (Sweden)

    Johannes M I H Gho

    Full Text Available Myocardial fibrosis can lead to heart failure and act as a substrate for cardiac arrhythmias. In dilated cardiomyopathy diffuse interstitial reactive fibrosis can be observed, whereas arrhythmogenic cardiomyopathy is characterized by fibrofatty replacement in predominantly the right ventricle. The p.Arg14del mutation in the phospholamban (PLN gene has been associated with dilated cardiomyopathy and recently also with arrhythmogenic cardiomyopathy. Aim of the present study is to determine the exact pattern of fibrosis and fatty replacement in PLN p.Arg14del mutation positive patients, with a novel method for high resolution systematic digital histological quantification of fibrosis and fatty tissue in cardiac tissue. Transversal mid-ventricular slices (n = 8 from whole hearts were collected from patients with the PLN p.Arg14del mutation (age 48±16 years; 4 (50% male. An in-house developed open source MATLAB script was used for digital analysis of Masson's trichrome stained slides (http://sourceforge.net/projects/fibroquant/. Slides were divided into trabecular, inner and outer compact myocardium. Per region the percentage of connective tissue, cardiomyocytes and fatty tissue was quantified. In PLN p.Arg14del mutation associated cardiomyopathy, myocardial fibrosis is predominantly present in the left posterolateral wall and to a lesser extent in the right ventricular wall, whereas fatty changes are more pronounced in the right ventricular wall. No difference in distribution pattern of fibrosis and adipocytes was observed between patients with a clinical predominantly dilated and arrhythmogenic cardiomyopathy phenotype. In the future, this novel method for quantifying fibrosis and fatty tissue can be used to assess cardiac fibrosis and fatty tissue in animal models and a broad range of human cardiomyopathies.

  13. Oversampling of wavelet frames for real dilations

    DEFF Research Database (Denmark)

    Bownik, Marcin; Lemvig, Jakob

    2012-01-01

    We generalize the Second Oversampling Theorem for wavelet frames and dual wavelet frames from the setting of integer dilations to real dilations. We also study the relationship between dilation matrix oversampling of semi-orthogonal Parseval wavelet frames and the additional shift invariance gain...

  14. Balloon dilatation of iatrogenic urethral strictures

    International Nuclear Information System (INIS)

    Acunas, B.; Acunas, G.; Gokmen, E.; Celik, L.

    1988-01-01

    Balloon dilatation of the urethra was performed in five patients with iatrogenic urethral strictures. The urethral strictures were successfully negotiated and dilated in all patients. Redilatation became necessary in a period ranging from 3 to 10 months. The authors believe that balloon dilatation of the urethra can be safely and successfully performed; the procedure produces minimal trauma and immediate relief of symptoms. (orig.)

  15. An unusual cardiomyopathy after physical stress in a child.

    Science.gov (United States)

    Fabi, Marianna; Testa, Gabriella; Gesuete, Valentina; Balducci, Anna; Ragni, Luca

    2013-01-01

    Takotsubo cardiomyopathy, or broken heart syndrome, is characterized by transient left ventricular dysfunction associated to chest pain, elevation of cardiac enzymes, and electrocardiographic changes, mimicking an acute coronary syndrome, especially in older women after a physical or emotional stress. It is extremely infrequent in children as well as after infective stress. We described a celiac 4-year-old girl, following a gluten-free diet, who developed features of cardiac failure few days after episodes of acute diarrhea with fever. The patient was treated with oral anticongestive therapy and intravenous immunoglobulins, and she had a dramatic and rapid improvement; echocardiographic features normalized in 48 hours. © 2012 Wiley Periodicals, Inc.

  16. HYPERTROPHIC OBSTRUCTIVE CARDIOMYOPATHY

    Directory of Open Access Journals (Sweden)

    A. G. Osiev

    2015-01-01

    Full Text Available Hypertrophic cardiomyopathy (HCMP is a relatively common disease with genetic predisposition, that is widely spread irrespective of gender, race or ethnicity. The cause of this pathology are mutations of genes encoding synthesis of contracting proteins. Degree and type of mutations define clinical manifestation of the disease and its prognosis. HCMP is classified according to four main criteria: depending on morphology, presence of left ventricular outlet obstruction, pressure gradient and hemodynamic parameters. Its prevalence amounts to 1:500, and in the recent years mortality has decreased significantly to 1%. Main symptoms of HCMP include dyspnoea, dizziness, syncope, angina, and heart arrhythmias. HCMP does not manifest obligatorily with all above mentioned signs and symptoms. Presence and severity of any symptoms depend on morphological particulars of the disease. Particular attention should be paid to arrhythmias, with atrial fibrillation among them, that may cause hazardous and occasionally lethal complications. Electrocardiography and echocardiography are recognized as the “golden standard” of HCMP diagnostics, while in the recent years, magnetic resonance imaging has become a highly informative diagnostic tool. Radionuclide diagnostics is used less frequently, while physical examination and assessments have been moving backwards. At present, main strategies in HCMP include medical treatment with β-blockers, calcium antagonists, angiotensin-converting enzyme inhibitors and anti-arrhythmics. There are two techniques for surgical treatment, i.e. myectomy by Morrow and alcohol septal ablation that is becoming increasingly popular. The article reviews literature on state-of-the-art diagnostics and treatment of HCMP patients.

  17. Peripartum cardiomyopathy - case series.

    Science.gov (United States)

    Prasad, Gowri Sayi; Bhupali, Ashok; Prasad, Sayi; Patil, Ajit N; Deka, Yashodhan

    2014-01-01

    To study the pattern of presentation, course of disease and outcome of pregnancy in Peripartum Cardiomyopathy. A prospective study of sixteen cases of PPCM was conducted at Apple Saraswati Multispecialty Hospital and Dr. D.Y. Patil Medical College and Hospital, Kolhapur, Maharashtra, India from January 2006 to December 2012. Data included age distribution, parity, gestational age, symptoms and risk factors. Medical management and pregnancy outcome were documented. Serial echocardiography data was compiled for a period of one year. In our study 9/16 (56%) were primigravidae, 4/16 (25%) had pre-eclamsia and 6/16 (35%) had co-existing hypertension. The difference in Echocardiography parameters observed between recovered and non-recovered patients was significant: Left Ventricular End diastolic dimension (5.6 cm vs 6.06 cm), Left Ventricular Ejection Fraction (28.7% vs 22.4%) and Left Ventricular fractional shortening (17.5% vs 13.4%). Thirteen out of sixteen patients were followed up for a period of one year out of which 61% (8/13) patients recovered completely. There was one mortality. PPCM is a diagnosis of exclusion. Majority were young primigravidae presenting postnatally. Pre-eclampsia and hypertension were risk factors. ECHO parameters were reliable predictors of recovery. Future pregnancies are better avoided. Copyright © 2014 Cardiological Society of India. Published by Elsevier B.V. All rights reserved.

  18. Balloon dilatations of esophageal strictures

    Energy Technology Data Exchange (ETDEWEB)

    Seo, Jeong Jin; Juhng, Seon Kwan; Kim, Jae Kyu; Chung, Hyon De [Chonnam National University College of Medicine, Seoul (Korea, Republic of)

    1990-04-15

    Most benign esophageal strictures can be successfully dilated with conventional bougienage technique. But occasionally strictures are so tight, lengthy, or sometimes irregular that this technique fail, and surgical intervention is required. Since 1974 Gruentzig balloon catheter has succeed when used for strictures in the cardiac and peripheral vasculatures, the biliary and urinary tracts, the colon of neonates after inflammatory disease and also in the esophagus. Fluoroscopically guided balloon catheters were used to dilate 30 esophageal strictures in 30 patients over 3 years at Department of Diagnostic Radiology, Chonnam University, College of Medicine. The distribution of age was from 7 years to 71 days and the ratio of male to female was 15:15. The causes of benign stricture (23 cases) were post-operative strictures (13), chemical (4), achalasia (3), chronic inflammation (2), esophageal rupture (1) and those of malignant stricture (7 cases) were post-radiation stricture of primary esophageal cancer (6) and metastatic esophageal cancer (1). The success rate of procedure was 93% (28/30). The causes of failure were the failure of passage of stricture due to markedly dilated proximal segment of esophagus (1 case) and too long segment of stricture (1 case). Complication of procedure was the diverticular-formation of esophagus in 3 cases, but has no clinical significance in follow-up esophagography. In conclusion, fluoroscopically guided balloon dilation of esophageal stricture appears to be safe, effective treatment and may be have theoretical advantages over conventional bougienage and also should be considered before other methods of treatment are used.

  19. Balloon dilatations of esophageal strictures

    International Nuclear Information System (INIS)

    Seo, Jeong Jin; Juhng, Seon Kwan; Kim, Jae Kyu; Chung, Hyon De

    1990-01-01

    Most benign esophageal strictures can be successfully dilated with conventional bougienage technique. But occasionally strictures are so tight, lengthy, or sometimes irregular that this technique fail, and surgical intervention is required. Since 1974 Gruentzig balloon catheter has succeed when used for strictures in the cardiac and peripheral vasculatures, the biliary and urinary tracts, the colon of neonates after inflammatory disease and also in the esophagus. Fluoroscopically guided balloon catheters were used to dilate 30 esophageal strictures in 30 patients over 3 years at Department of Diagnostic Radiology, Chonnam University, College of Medicine. The distribution of age was from 7 years to 71 days and the ratio of male to female was 15:15. The causes of benign stricture (23 cases) were post-operative strictures (13), chemical (4), achalasia (3), chronic inflammation (2), esophageal rupture (1) and those of malignant stricture (7 cases) were post-radiation stricture of primary esophageal cancer (6) and metastatic esophageal cancer (1). The success rate of procedure was 93% (28/30). The causes of failure were the failure of passage of stricture due to markedly dilated proximal segment of esophagus (1 case) and too long segment of stricture (1 case). Complication of procedure was the diverticular-formation of esophagus in 3 cases, but has no clinical significance in follow-up esophagography. In conclusion, fluoroscopically guided balloon dilation of esophageal stricture appears to be safe, effective treatment and may be have theoretical advantages over conventional bougienage and also should be considered before other methods of treatment are used

  20. Heart transplantation for Chagas cardiomyopathy.

    Science.gov (United States)

    Ramalho, Ana Rita; Prieto, David; Antunes, Pedro; Franco, Fátima; Antunes, Manuel J

    2017-11-01

    Chagas disease is an endemic disease in Latin America that is increasingly found in non-endemic areas all over the world due to the flow of migrants from Central and South America. We present the case of a Brazilian immigrant in Portugal who underwent orthotopic heart transplantation for end-stage Chagas cardiomyopathy. Immunosuppressive therapy included prednisone, mycophenolate mofetil and tacrolimus. Twelve months after the procedure she is asymptomatic, with good graft function, and with no evidence of complications such as graft rejection, opportunistic infections, neoplasms or reactivation of Trypanosoma cruzi infection. By reporting the first case in Portugal of heart transplantation for Chagas cardiomyopathy, we aim to increase awareness of Chagas disease as an emerging global problem and of Chagas cardiomyopathy as a serious complication for which heart transplantation is a valuable therapeutic option. Copyright © 2017 Sociedade Portuguesa de Cardiologia. Publicado por Elsevier España, S.L.U. All rights reserved.

  1. Takotsubo Cardiomyopathy in the Setting of Tension Pneumothorax

    Directory of Open Access Journals (Sweden)

    Michael Gale

    2015-01-01

    Full Text Available Background. Takotsubo cardiomyopathy is defined as a transient left ventricular dysfunction, usually accompanied by electrocardiographic changes. The literature documents only two other cases of Takotsubo cardiomyopathy in the latter setting. Methods. A 78-year-old female presented to the ED with severe shortness of breath, hypertension, and tachycardia. On physical exam, heart sounds (S1 and S2 were regular and wheezing was noticed bilaterally. We found laboratory results with a WBC of 20.0 (103/μL, troponin of 16.52 ng/mL, CK-mb of 70.6%, and BNP of 177 pg/mL. The patient was intubated for acute hypoxemic respiratory failure. A chest X-ray revealed a large left-sided tension pneumothorax. Initial echocardiogram showed apical ballooning with a LVEF of 10–15%. A cardiac angiography revealed normal coronary arteries with no coronary disease. After supportive treatment, the patient’s condition improved with a subsequent echocardiogram showing a LVEF of 60%. Conclusion. The patient was found to have Takotsubo cardiomyopathy in the setting of a tension pneumothorax. The exact mechanisms of ventricular dysfunction have not been clarified. However, multivessel coronary spasm or catecholamine cardiotoxicity has been suggested to have a causative role. We suggest that, in our patient, left ventricular dysfunction was induced by the latter mechanism related to the stress associated with acute pneumothorax.

  2. Improvement of exercise capacity and left ventricular diastolic function with metoprolol XL after acute myocardial infarction

    DEFF Research Database (Denmark)

    Poulsen, S H; Jensen, S E; Egstrup, K

    2000-01-01

    BACKGROUND: Left ventricular (LV) diastolic function predicts and correlates with exercise capacity. Beta-blockers improve exercise capacity and LV diastolic function in patients with severe LV systolic dysfunction in dilated cardiomyopathy. However, information on the effect of metoprolol XL...... dysfunction after acute myocardial infarction were given metoprolol XL (n = 29) or placebo (n = 30). The effects of metoprolol XL on exercise capacity in relation to effects on LV diastolic filling were studied. Two-dimensional Doppler echocardiography and maximal symptom limited bicycle test were performed...... on days 5 through 7 and after 3 months. RESULTS: Maximal exercise capacity increased in the metoprolol XL group (124 +/- 30 W vs 135 +/- 29 W) compared with placebo (125 +/- 31 W vs 126 +/- 34 W) (P

  3. Prevalence and Risk Factors of Stress Cardiomyopathy After Convulsive Status Epilepticus in ICU Patients.

    Science.gov (United States)

    Belcour, Dominique; Jabot, Julien; Grard, Benjamin; Roussiaux, Arnaud; Ferdynus, Cyril; Vandroux, David; Vignon, Philippe

    2015-10-01

    Although stress cardiomyopathy has been described in association with epilepsy, its frequency in patients with convulsive status epilepticus remains unknown. Accordingly, we sought to determine the prevalence and risk factors of stress cardiomyopathy in patients admitted to the ICU for convulsive status epilepticus. Prospective, descriptive, single-center study. Medical-surgical ICU of a teaching hospital. Thirty-two consecutive ventilated patients (21 men; age, 50 ± 18 yr; Simplified Acute Physiology Score II, 53 ± 15; Sequential Organ Failure Assessment, 6 ± 2) hospitalized in the ICU for convulsive status epilepticus. None. Hemodynamic parameters, transthoracic echocardiography, biological data, and electrocardiogram were obtained serially on ICU admission (H0), and after 6, 12, 24, and 48 hours of hospitalization (H6, H12, H24, and H48). Stress cardiomyopathy was defined as a 20% decrease in left ventricular ejection fraction between H0 or H6 and H48. Stress cardiomyopathy was diagnosed in 18 patients (56%; 95% CI, 38-74%). Mean left ventricular ejection fraction, left ventricular stroke index and cardiac index were initially (at H0 or H6 according to lowest individual values) significantly reduced in stress cardiomyopathy patients (45 ± 14% vs 61 ± 6%, p ICU for convulsive status epilepticus. Accordingly, these patients should be screened for stress cardiomyopathy and monitored if they present with hemodynamic compromise.

  4. Selenium- and zinc-deficient cardiomyopathy in human intestinal malabsorption: preliminary results of selenium/zinc infusion.

    Science.gov (United States)

    Frustaci, Andrea; Sabbioni, Enrico; Fortaner, Salvador; Farina, Massimo; del Torchio, Riccardo; Tafani, Marco; Morgante, Emanuela; Ciriolo, Maria Rosa; Russo, Matteo A; Chimenti, Cristina

    2012-02-01

    Patients with intestinal malabsorption may develop cardiac dysfunction the origin of which is often unclear. We sought to investigate the pathogenesis of dilated cardiomyopathy in human malabsorption. Eighteen patients with intestinal bypass as treatment for severe obesity and cardiomyopathy underwent endomyocardial biopsy. Biopsies were processed by histology, electron microscopy, polymerase chain reaction (PCR) for cardiotropic viruses, instrumental neutron activation analysis (INAA) of 33 myocardial trace elements, and assessment of glutathione peroxidase (GPX) activity and LC3-II expression. Histology and electron microscopy showed hypertrophy/degeneration of cardiomyocytes with pronounced cell autophagy and high expression of LC3-II. PCR was negative for viral genomes. INAA showed severe myocardial selenium (Se) and zinc (Zn) deficiency and reduced GPX activity vs. both patients with idiopathic dilated cardiomyopathy and normal controls. Se and Zn were added to antifailing heart therapy in 10 patients (group A1) agreeing to a control biopsy, and the response was compared with that of 8 patients (group A2) on supportive therapy alone. After 6 months, myocardial normalization of Se, Zn, LC3-II, and GPX in group A1 was associated with recovery of cardiomyocyte degeneration and autophagy, and significant improvement in cardiac dimension and function, that remained unchanged in group A2. A reversible Se- and Zn-deficient cardiomyopathy may occur in patients with intestinal malabsorption. It is characterized by decline of myocardial antioxidant reserve, oxidative damage of cell membranes, and enhanced cell autophagy.

  5. Value of transient dilation of the left ventricular cavity on stress thallium scintigraphy

    Energy Technology Data Exchange (ETDEWEB)

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

    1991-02-01

    This study was undertaken to evaluate the value of transient dilation of the left ventricular cavity on stress thallium scintigraphy in 80 patients with ischemic heart disease (IHD) and 50 with hypertrophic cardiomyopathy (HCM). Twenty persons without either coronary artery stenosis or heart disease were served as controls. Areas surrounded by maximum count points on the line of each 10deg on the short axis slice through the mid-cavity of the left ventricle were obtained at 10 minutes and at 3 hours after exercise. Transient dilation index (TDI) was obtained by dividing the area on early image by that on delayed image. TDI was significantly higher in patients with two or three vessel disease in the IHD group than the control group. High TDI was observed in 8% for one vessel disease, 40% for two vessel disease, and 80% for three vessel disease, contributing to the detection of multivessel IHD. In the HCM group of 80 patients, 24 (48%) had high TDI which was frequently associated with a history of chest pain and positive ECG findings at exercise. When these 24 HCM patients underwent exercise blood pool scintiscanning, left ventricular enddiastolic volume was similar before and at 10 minutes after exercise. These findings suggest that transient dilation of the left ventricular cavity after exercise may reflect subendocardial ischemia in both IHD and HCM. TDI would become a useful indicator for transient dilation of the left ventricular cavity. (N.K.).

  6. Left Ventricular Non-compaction Cardiomyopathy - A Case Report

    Directory of Open Access Journals (Sweden)

    Timea Szakacs Xantus

    2015-06-01

    Full Text Available Background: Left non-compaction cardiomyopathy (LVNC or “spongy myocardium” is a relatively rare primary genetic cardiomyopathy, characterized by prominent wall trabeculations and intertrabecular recesses which communicate with the ventricular cavity. It appears in isolated form or coexists with other congenital heart diseases and/or systemic abnormalities. Material and method: A 28-year-old woman was admitted with exertional dyspnoea, palpitations, non-specific chest pain and progressive fatigue on exertion. In her family history sudden cardiac-related deaths at young age are present. Cardiovascular system examination revealed tachycardia, intermittent extrabeats. The rest EKG showed sinusal tachycardia (105 bpm, negative T-waves in DII, DIII, aVF, V4-V6. Consecutive 24 hours Holter EKG monitoring revealed nonsustained ventricular tachycardia, paroxysmal atrial fibrillation, isolated ventricular extrasystoles. Echocardiography showed left ventricular systolic dysfunction (LVEF:30-35%, slight LV enlargement, normal right ventricle and small left ventricle (LV trabeculae in the apical area. Cardiac MRI demonstrated dilated LV and the presence of the trabeculations of LV walls suggestive for non-compaction cardiomyopathy. A combined treatment for heart failure and cardiac arrhythmias was initiated with good clinical results. Patient was scheduled for an implantable cardioverter defibrillator “life-saving”. Conclusions: The symptoms of heart failure and cardiac arrhythmias should be considered important in apparently healthy young patients. Besides intensive medical treatment is indicated the implantation of an ICD “life-saving” and in advanced cases heart transplantation. Even if the electrocardiographic findings are non specific for noncompaction, a complete diagnostic evaluation is important, including sophisticated imaging techniques, a screening of first-degree relatives, and an extensive clinical, and genetic appreciation by a

  7. Desmoplakin truncations and arrhythmogenic left ventricular cardiomyopathy: characterizing a phenotype.

    Science.gov (United States)

    López-Ayala, Jose María; Gómez-Milanés, Ivan; Sánchez Muñoz, Juan José; Ruiz-Espejo, Francisco; Ortíz, Martín; González-Carrillo, Josefa; López-Cuenca, David; Oliva-Sandoval, M J; Monserrat, Lorenzo; Valdés, Mariano; Gimeno, Juan R

    2014-12-01

    Risk stratification for sudden death in arrhythmogenic right ventricular cardiomyopathy (ARVC) is challenging in clinical practice. We lack recommendations for the risk stratification of exclusive left-sided phenotypes. The aim of this study was to investigate genotype-phenotype correlations in patients carrying a novel DSP c.1339C>T, and to review the literature on the clinical expression and the outcomes in patients with DSP truncating mutations. Genetic screening of the DSP gene was performed in 47 consecutive patients with a phenotype of either an ARVC (n = 24) or an idiopathic dilated cardiomyopathy (DCM), who presented with ventricular arrhythmias or a family history of sudden death (n = 23) (aged 40 ± 19 years, 62% males). Three unrelated probands with DCM were found to be carriers of a novel mutation (c.1339C>T). Cascade family screening led to the identification of 15 relatives who are carriers. Penetrance in c.1339C>T carriers was 83%. Sustained ventricular tachycardia was the first clinical manifestation in six patients and nine patients were diagnosed with left ventricular impairment (two had overt severe disease and seven had a mild dysfunction). Cardiac magnetic resonance revealed left ventricular involvement in nine cases and biventricular disease in three patients. Extensive fibrotic patterns in six and non-compaction phenotype in five patients were the hallmark in imaging. DSP c.1339C>T is associated with an aggressive clinical phenotype of left-dominant arrhythmogenic cardiomyopathy and left ventricular non-compaction. Truncating mutations in desmoplakin are consistently associated with aggressive phenotypes and must be considered as a risk factor of sudden death. Since ventricular tachycardia occurs even in the absence of severe systolic dysfunction, an implantable cardioverter-defibrillator should be indicated promptly. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2014. For permissions please

  8. Left Atrial Function in Patients with Chronic Chagasic Cardiomyopathy.

    Science.gov (United States)

    Fragata, Claudia da Silva; Matsumoto, Afonso Y; Ramires, Felix J A; Fernandes, Fabio; Buck, Paula de Cássia; Salemi, Vera Maria C; Nastari, Luciano; Mady, Charles; Ianni, Barbara Maria

    2015-07-01

    Chagas disease is a cause of dilated cardiomyopathy, and information about left atrial (LA) function in this disease still lacks. To assess the different LA functions (reservoir, conduit and pump functions) and their correlation with the echocardiographic parameters of left ventricular (LV) systolic and diastolic functions. 10 control subjects (CG), and patients with Chagas disease as follows: 26 with the indeterminate form (GI); 30 with ECG alterations (GII); and 19 with LV dysfunction (GIII). All patients underwent M-mode and two-dimensional echocardiography, pulsed-wave Doppler and tissue Doppler imaging. Reservoir function (Total Emptying Fraction: TEF): (p <0.0001), lower in GIII as compared to CG (p = 0.003), GI (p <0.001) and GII (p <0.001). Conduit function (Passive Emptying Fraction: PEF): (p = 0.004), lower in GIII (GIII and CG, p = 0.06; GI and GII, p = 0.06; and GII and GIII, p = 0.07). Pump function (Active Emptying Fraction: AEF): (p = 0.0001), lower in GIII as compared to CG (p = 0.05), GI (p<0.0001) and GII (p = 0.002). There was a negative correlation of E/e' (average) with the reservoir and pump functions (TEF and AEF), and a positive correlation of e' (average) with s' wave (both septal and lateral walls) and the reservoir, conduit and pump LA functions. An impairment of LA functions in Chagas cardiomyopathy was observed.

  9. Left Atrial Function in Patients with Chronic Chagasic Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Claudia da Silva Fragata

    2015-01-01

    Full Text Available Background: Chagas disease is a cause of dilated cardiomyopathy, and information about left atrial (LA function in this disease still lacks. Objective: To assess the different LA functions (reservoir, conduit and pump functions and their correlation with the echocardiographic parameters of left ventricular (LV systolic and diastolic functions. Methods: 10 control subjects (CG, and patients with Chagas disease as follows: 26 with the indeterminate form (GI; 30 with ECG alterations (GII; and 19 with LV dysfunction (GIII. All patients underwent M-mode and two-dimensional echocardiography, pulsed-wave Doppler and tissue Doppler imaging. Results: Reservoir function (Total Emptying Fraction: TEF: (p <0.0001, lower in GIII as compared to CG (p = 0.003, GI (p <0.001 and GII (p <0.001. Conduit function (Passive Emptying Fraction: PEF: (p = 0.004, lower in GIII (GIII and CG, p = 0.06; GI and GII, p = 0.06; and GII and GIII, p = 0.07. Pump function (Active Emptying Fraction: AEF: (p = 0.0001, lower in GIII as compared to CG (p = 0.05, GI (p<0.0001 and GII (p = 0.002. There was a negative correlation of E/e’ average with the reservoir and pump functions (TEF and AEF, and a positive correlation of e’ average with s’ wave (both septal and lateral walls and the reservoir, conduit and pump LA functions. Conclusion: An impairment of LA functions in Chagas cardiomyopathy was observed.

  10. Animal models of hypertrophic cardiomyopathy

    NARCIS (Netherlands)

    Maass, Alexander; Leinwand, Leslie A.

    Familial hypertrophic cardiomyopathy (FHC) is an autosomal-dominant disease that is both clinically and genetically heterogeneous. Disease-causing mutations have been found in eight genes encoding structural components of the thick and thin filament systems of the cardiac myocyte; it has therefore

  11. Improving Outcomes in Hypertrophic Cardiomyopathy

    NARCIS (Netherlands)

    P.A. Vriesendorp (Pieter)

    2016-01-01

    markdownabstractImproving outcomes in hypertrophic cardiomyopathy (HCM) is focused on the improvement of the therapeutic strategies for patients with HCM. First it demonstrates that individual patient selection in patients with obstructive and symptomatic HCM can lead to near normal life-expectancy;

  12. Cor triatriatum and hypertrophic cardiomyopathy.

    Science.gov (United States)

    Pellaton, Cyril; O'Mahony, Costas; Ludman, Andrew J; Sekhri, Neha; Mohiddin, Saidi

    2016-12-01

    : Cor triatriatum is a rare congenital anomaly known to be associated with other inherited heart diseases. We present a nonrestrictive cor triatriatum sinistrum associated with hypertrophic cardiomyopathy to illustrate how different multimodality noninvasive imaging techniques complement each other and can help with the diagnosis. To the best of our knowledge, this coexistence has not been previously reported.

  13. Synchronous advanced pulmonary tuberculosis and acute virus myocarditis mimicked wegener granulomatosis in a 26-year-old man: A case report

    Directory of Open Access Journals (Sweden)

    Pešut Dragica P.

    2016-01-01

    Full Text Available Introduction. Tuberculosis patients are rarely asymptomatic. Acute virus myocarditis presents with a wide range of symptoms, from mild dyspnea or chest pain to cardiogenic shock and death. Case Outline. A 26-year-old Caucasian man non-smoker presented with one-week history of lower extremities’ swelling. The patient’s medical history also revealed a two-day episode of subfebrile temperature with scanty hemoptysis three weeks prior to admission. The episode had not provoked him to seek medical care. Physical examination revealed generalized oedema, and laboratory analysis showed signs of acute renal insufficiency. Enlarged heart and hilar shadows, bilateral massive cavitary pulmonary opacities and pleural effusion were found at chest radiography. Sputum smears were Mycobacteria negative on direct microscopy. Electrocardiogram changes and echocardiography were suggestive of acute myocarditis with dilated cardiomyopathy. IgM titer to adenovirus was positive. Under diuretics, angiotensin-converting-enzyme inhibitor, beta-blocker, antibiotics and bed rest, fast heart compensation and renal function repair were achieved. Radiographic pulmonary changes promptly regressed except for a cavity in the right upper lobe. Bronchial aspirate from the affected lobe was Mycobacteria positive on direct microscopy and culture positive for Mycobacterium tuberculosis. Standard anti-tuberculosis drug regimen led to recovery. Conclusion. In the unusual common existence of two diseases whose presentation initially mimicked Wegener’s granulomatosis, acute dilated cardiomyopathy contributed to pulmonary tuberculosis detection. To prevent diagnostic delay in tuberculosis, further efforts in population education are necessary together with continual medical education. [Projekat Ministarstva nauke Republike Srbije, br. 175095

  14. Mutations in hotspot region of MYH7 gene exon 23 associated with restrictive cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Mitali Kapoor

    2017-05-01

    Full Text Available Restrictive cardiomyopathy (RCM is characterized by restrictive filling of the ventricles. The association between the variable expressivity and age at onset of disease and disease complexity with double and compound heterozygous state is associated with severity of disease phenotype in recent reports. Sharing of variants of sarcomere genes across cardiomyopathies has implication in clinical expression of different clinical phenotypes. The present study reports Sanger DNA sequencing of MYH7 gene, exon 23 from 30 unrelated RCM patients and 15 primary relatives from sporadic families with the hypothesis that RCM has common etiology with hypertrophic cardiomyopathy (HCM. Rare variant E949K and a de novo compound heterozygous mutation (p.E902K and p.D906N, in two RCM patients with early onset and no ventricular hypertrophy were found. These variants wereabsent in 50 dilated cardiomyopathy, 50 HCM patients and 15 primary relatives screened. The present report of rare and compound heterozygosity cases will further provide basis for the complexity and variable expressivity of phenotypes in patients in such complex diseases. The possible reasons for this phenotypic heterogeneity would be the presence of any other mutations in same chromosome or in different chromosome which is modifying the outcome of the causal mutation.

  15. Clinical review: Percutaneous dilatational tracheostomy

    Science.gov (United States)

    Al-Ansari, Mariam A; Hijazi, Mohammed H

    2006-01-01

    As the number of critically ill patients requiring tracheotomy for prolonged ventilation has increased, the demand for a procedural alternative to the surgical tracheostomy (ST) has also emerged. Since its introduction, percutaneous dilatational tracheostomies (PDT) have gained increasing popularity. The most commonly cited advantages are the ease of the familiar technique and the ability to perform the procedure at the bedside. It is now considered a viable alternative to (ST) in the intensive care unit. Evaluation of PDT procedural modifications will require evaluation in randomized clinical trials. Regardless of the PDT technique, meticulous preoperative and postoperative management are necessary to maintain the excellent safety record of PDT. PMID:16356203

  16. Adderall induced inverted-Takotsubo cardiomyopathy.

    Science.gov (United States)

    Alsidawi, Said; Muth, James; Wilkin, James

    2011-11-15

    Takotsubo Cardiomyopathy (TTC), also known as stress-induced cardiomyopathy, was initially described in Japan in 1990. Both illicit and prescription drugs have added to the growing list of insulting stressors. We describe an interesting case of atypical TTC triggered by adderall overdose. A 19-year-old female was brought to the Emergency Department after ingesting 30 Adderall tablets. She was complaining of pressure like chest pain and shortness of breath. Her cardiac enzymes were elevated but the electrocardiogram was unremarkable. Echocardiography identified an ejection fraction (EF) of 25-30% with severe hypokinesis of the base and a preserved apex. Cardiac angiography demonstrated normal coronary arteries with an EF of 35%, hyperkinetic apex and akinetic base consistent with the diagnosis of inverted-TTC. Her symptoms resolved in 24 hrs. Repeat echocardiogram performed 3 days later showed an EF of 60% with no regional wall motion abnormalities. TTC can be identified as a rapid development of severe and reversible left ventricular dysfunction extending beyond the territory of a single epicardial coronary artery in the absence of coronary artery disease or pheochromocytoma. Clinical presentation can be challenging and very hard to distinguish from acute myocardial infarction. Medication induced-TTC has been reported. In our case, the patient overdosed on Adderall which is a sympathomimetic medication. Cardiac imaging identified wall motion abnormalities consistent with inverted type TTC. Restoration of left ventricular function within days confirms the diagnosis of TTC. In conclusion, this case offers an interesting insight into the pathophysiology of TTC. Copyright © 2011 Wiley Periodicals, Inc.

  17. Percutaneous Dilational Tracheotomy in Solid-Organ Transplant Recipients.

    Science.gov (United States)

    Ozdemirkan, Aycan; Ersoy, Zeynep; Zeyneloglu, Pinar; Gedik, Ender; Pirat, Arash; Haberal, Mehmet

    2015-11-01

    Solid-organ transplant recipients may require percutaneous dilational tracheotomy because of prolonged mechanical ventilation or airway issues, but data regarding its safety and effectiveness in solid-organ transplant recipients are scarce. Here, we evaluated the safety, effectiveness, and benefits in terms of lung mechanics, complications, and patient comfort of percutaneous dilational tracheotomy in solid-organ transplant recipients. Medical records from 31 solid-organ transplant recipients (median age of 41.0 years [interquartile range, 18.0-53.0 y]) who underwent percutaneous dilational tracheotomy at our hospital between January 2010 and March 2015 were analyzed, including primary diagnosis, comorbidities, duration of orotracheal intubation and mechanical ventilation, length of intensive care unit and hospital stays, the time interval between transplant to percutaneous dilational tracheotomy, Acute Physiology and Chronic Health Evaluation II score, tracheotomy-related complications, and pulmonary compliance and ratio of partial pressure of arterial oxygen to fraction of inspired oxygen. The median Acute Physiology and Chronic Health Evaluation II score on admission was 24.0 (interquartile range, 18.0-29.0). The median interval from transplant to percutaneous dilational tracheotomy was 105.5 days (interquartile range, 13.0-2165.0 d). The only major complication noted was left-sided pneumothorax in 1 patient. There were no significant differences in ratio of partial pressure of arterial oxygen to fraction of inspired oxygen before and after procedure (170.0 [interquartile range, 102.2-302.0] vs 210.0 [interquartile range, 178.5-345.5]; P = .052). However, pulmonary compliance results preprocedure and postprocedure were significantly different (0.020 L/cm H2O [interquartile range, 0.015-0.030 L/cm H2O] vs 0.030 L/cm H2O [interquartile range, 0.020-0.041 L/cm H2O); P = .001]). Need for sedation significantly decreased after tracheotomy (from 17 patients [54.8%] to

  18. Dynamical and hamiltonian dilations of stochastic processes

    International Nuclear Information System (INIS)

    Baumgartner, B.; Gruemm, H.-R.

    1982-01-01

    This is a study of the problem, which stochastic processes could arise from dynamical systems by loss of information. The notions of ''dilation'' and ''approximate dilation'' of a stochastic process are introduced to give exact definitions of this particular relationship. It is shown that every generalized stochastic process is approximately dilatable by a sequence of dynamical systems, but for stochastic processes in full generality one needs nets. (Author)

  19. Aneurysmatic dilatation of the vena portae

    International Nuclear Information System (INIS)

    Dewes, W.; Gormanns, R.; Spangenberger, W.; Staedtisches Krankenhaus Ko