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Sample records for childhood dilated cardiomyopathy

  1. Cushing's Disease Presented by Reversible Dilated Cardiomyopathy

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    Berna İmge Aydoğan; Demet Menekşe Gerede; Asena Gökçay Canpolat; Murat Faik Erdoğan

    2015-01-01

    Introduction. Dilated cardiomyopathy is rarely reported among CS patients especially without hypertension and left ventricular hypertrophy. Materials and Methods. We hereby report a Cushing’s syndrome case presenting with dilated cardiomyopathy. Results. A 48-year-old female patient was admitted to our clinic with severe proximal myopathy and dilated cardiomyopathy without ventricular hypertrophy. Cushing’s disease was diagnosed and magnetic-resonance imaging of the pituitary gland revealed a...

  2. Peripartum Cardiomyopathy as a Part of Familial Dilated Cardiomyopathy

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    van Spaendonck-Zwarts, Karin Y.; van Tintelen, J. Peter; van Veldhuisen, Dirk J.; van der Werf, Rik; Jongbloed, Jan D. H.; Paulus, Walter J.; Dooijes, Dennis; van den Berg, Maarten P.

    2010-01-01

    Background-Anecdotal cases of familial clustering of peripartum cardiomyopathy (PPCM) and familial occurrences of PPCM and idiopathic dilated cardiomyopathy (DCM) together have been observed, suggesting that genetic factors play a role in the pathogenesis of PPCM. We hypothesized that some cases of

  3. Peripartum cardiomyopathy as a part of familial dilated cardiomyopathy

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    K.Y. van Spaendonck-Zwarts (Karin); J.P. van Tintelen (Peter); D.J. van Veldhuisen (Dirk); R. van der Werf (Rik); J.D.H. Jongbloed (Jan); W.J. Paulus (Walter); D. Dooijes (Dennis); M.P. van den Berg (Maarten)

    2010-01-01

    textabstractBACKGROUND-: Anecdotal cases of familial clustering of peripartum cardiomyopathy (PPCM) and familial occurrences of PPCM and idiopathic dilated cardiomyopathy (DCM) together have been observed, suggesting that genetic factors play a role in the pathogenesis of PPCM. We hypothesized that

  4. Related factors of dilated cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    Guangyong Huang; Hang Gao; Xiangang Meng; Zhonghua Yan; Xiangquan Kong; Lexin Wang

    2009-01-01

    Objective To investigate the etiology and relative factors of dilated cardiomyopathy (DCM) in Chinese patients. Methods A case-control study was conducted to compare 233 patients with DCM in high-incidence areas (case group) and 150 patients with stable angina pectoris (control group). Life styles and history of diseases information was collected by questionaire; human anti-myocardial antibody IgG (AMA- IgG), human Coxsackie B virus IgG (CBV- IgG) and human adenovirus antibody IgG (ADV- lgG) were measured with ELISA. General chemical and toxicological indicators in drink water from high and low prevalence areas and serum trace elements also were compared. Results 1 ) Compared with the control group, the case group had more farmers (P < 0.01), with low average incomes (P < 0.01), higher alcohol consumption (P < 0.01) and higher incidence of the history of myocarditis (P < 0.01 ). 2) AMA-IgG, CBV-IgG and ADV-IgG levels were low and the positive rates ofAMA-IgG, CBV-IgG and ADV-IgG of patients with DCM were respectively 7.78%, 6.67% and 6.67%, no statistical significance comparing with those in the control group. 3) The content of iron (1.36±2.18 vs 0.39±0.67 mg/L, P<0.05) and manganese (0.384±0.35 vs 0.15±0.14, P<0.01 ) in drinking water of high-incidence areas was significantly higher than that in low-incidence areas. 4) The content of serum iron (69.14±57.8 vs 20.04±17.5 μ mol/L, P<0.01 ) and copper (25.74±4.2 vs 19.7±4.5 μmol/L, P<0.01) in the case group evidently exceeded the normal range and obviously higher than that in the control group. Conclusions 1) The incidence of some DCM is related with low incomes, high alcohol consumption and myocarditis. 2) These data do not support that DCM is related with persistent virus infection and autoimmunization; 3) Iron and manganese contents exceeding standards in drinking water and the high content of serum iron and copper is comparatively related with the incidence of DCM.

  5. Gallium-67 myocardial scintigraphy in dilated cardiomyopathy

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    Aoki, Toshikazu; Konishi, Tokuji; Koyama, Takao; Morita, Yuriko; Futagami, Yasuo; Hayashi, Takamaro; Hamada, Masayuki; Nakano, Takeshi

    1988-12-01

    Gallium-67 imaging has been employed clinically in the detection of malignant tumor or chronic inflammatory disease. In this study, we evaluated the usefulness of Gallium-67 myocardial imaging as an adjunct to endomyocardial biopsy in the diagnosis of myocarditis. Nine patients who had been diagnosed clinically as dilated cardiomyopathy underwent Gallium-67 myocardial imaging. Left ventricular endomyocardial biopsy was performed on all patients. Two had positive Gallium-67 imaging, but myocarditis was not proven in their tissue specimen. Two others with proven myocarditis had negative Gallium-67 imaging. These results suggest that Gallium-67 imaging is not always a useful tool to detect latent myocarditis in patients with dilated cardiomyopathy.

  6. Sheehan syndrome with reversible dilated cardiomyopathy.

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    Laway, Bashir A; Alai, Mohammad S; Gojwari, Tariq; Ganie, Mohd A; Zargar, Abdul Hamid

    2010-01-01

    Cardiac abnormalities in patients with Sheehan syndrome are uncommon. A case of Sheehan syndrome with dilated cardiomyopathy is presented in whom hormone replacement with levothyroxine and prednisolone resulted in complete recovery of cardiomyopathy. A 25-year-old woman presented with lactation failure, secondary amenorrhea, features of hypothyroidism and a hypocortisol state following severe postpartum hemorrhage after her last child birth. She also had smear positive pulmonary tuberculosis. After starting antitubercular treatment, she developed shock, suggestive of hypocortisol crisis. Hormonal investigations revealed evidence of panhypopitutarism and magnetic resonance imaging revealed partial empty sella. Meanwhile echocardiography revealed evidence of dilated cardiomyopathy (DCM). The patient was given replacement therapy in the form of glucocorticoids and levothyroxine in addition to antitubercular treatment. She improved and on follow-up over a period of 7 months, the DCM completely reversed. To our knowledge this is the first report of reversible DCM in a patient with Sheehan syndrome.

  7. Peripartum cardiomyopathy and dilated cardiomyopathy: different at heart

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    Ilse Anne Elise Bollen

    2015-01-01

    Full Text Available Peripartum cardiomyopathy (PPCM is a severe cardiac disease occurring in the last month of pregnancy or in the first 5 months after delivery and shows many similar clinical characteristics as dilated cardiomyopathy (DCM such as ventricle dilation and systolic dysfunction. While PPCM was believed to be DCM triggered by pregnancy, more and more studies show important differences between these diseases. While it is likely they share part of their pathogenesis such as increased oxidative stress and an impaired microvasculature, discrepancies seen in disease progression and outcome indicate there must be differences in pathogenesis as well. In this review, we compared studies in DCM and PPCM to search for overlapping and deviating disease etiology, pathogenesis and outcome in order to understand why these cardiomyopathies share similar clinical features but have different underlying pathologies.

  8. Reversible dilated cardiomyopathy due to subclinical hyperthyroidism.

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    Tsarouhas, Kostantinos; Kafantaris, Ioannis; Antonakopoulos, Athanasios; Vavetsi, Spiridoula; Pavlidis, Pavlos; Constantinou, Loizos L

    2010-01-01

    We present a patient without primary heart disease in whom subclinical hyperthyroidism was accompanied by manifestations of dilated cardiomyopathy, as evaluated by echocardiography, coronary angiography, and radionuclide ventriculography. His condition was reversed 6 months after conventional treatment (furosemide, carvedilol, angiotensin-converting-enzyme inhibitor and thiamazole administration). This patient represents an exceptional case, as overt congestive heart failure with left ventricular dilatation and depressed ventricular ejection fraction is not a common finding in patients with hyperthyroidism, let alone patients with subclinical hyperthyroidism and no underlying heart disease.

  9. Stroke and dilated cardiomyopathy associated with celiac disease

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    Murat; Dogan; Erdal; Peker; Eren; Cagan; Sinan; Akbayram; Mehmet; Acikgoz; Huseyin; Caksen; Abdurrahman; Uner; Yasar; Cesur

    2010-01-01

    Celiac disease(CD) is manifested by a variety of clinical signs and symptoms that may begin either in childhood or adult life.Neurological symptoms without signs of malabsorption have been observed for a long time in CD.In this report,an 8-year-old girl with CD presented with rarely seen dilated cardiomyopathy and stroke.The girl was admitted with left side weakness.Her medical history indicated abdominal distention,chronic diarrhea,failure to thrive,and geophagia.On physical examination,short stature,pale ...

  10. Dilated cardiomyopathy and inclusion body myositis.

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    Ballo, Piercarlo; Chiodi, Leandro; Cameli, Matteo; Malandrini, Alessandro; Federico, Antonio; Mondillo, Sergio; Zuppiroli, Alfredo

    2012-04-01

    Inclusion body myositis (IBM) is the most common inflammatory myopathy after 50 years of age. In contrast to polymyositis and dermatomyositis, in which cardiac involvement is relatively common, current evidences indicate that IBM is not associated with cardiac disease. We report the case of a patient with biopsy-proven IBM who developed heart failure and major ventricular arrhythmias secondary to dilated cardiomyopathy few months after the clinical onset of IBM, and in whom no pathophysiologic causes explaining cardiac enlargement and dysfunction were found by laboratory and instrumental investigations. The hypothesis of a pathophysiologic association between the two conditions is discussed.

  11. The Mutations Associated with Dilated Cardiomyopathy

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    Ruti Parvari

    2012-01-01

    Full Text Available Cardiomyopathy is an important cause of heart failure and a major indication for heart transplantation in children and adults. This paper describes the state of the genetic knowledge of dilated cardiomyopathy (DCM. The identification of the causing mutation is important since presymptomatic interventions of DCM have proven value in preventing morbidity and mortality. Additionally, as in general in genetic studies, the identification of the mutated genes has a direct clinical impact for the families and population involved. Identifying causative mutations immediately amplifies the possibilities for disease prevention through carrier screening and prenatal testing. This often lifts a burden of social isolation from affected families, since healthy family members can be assured of having healthy children. Identification of the mutated genes holds the potential to lead to the understanding of disease etiology, pathophysiology, and therefore potential therapy. This paper presents the genetic variations, or disease-causing mutations, contributing to the pathogenesis of hereditary DCM, and tries to relate these to the functions of the mutated genes.

  12. Shared Genetic Predisposition in Peripartum and Dilated Cardiomyopathies.

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    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.

  13. Patient with Eating Disorder, Carnitine Deficiency and Dilated Cardiomyopathy.

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    Fotino, A Domnica; Sherma, A

    2015-01-01

    Dilated cardiomyopathy is characterized by a dilated and poorly functioning left ventricle and can result from several different etiologies including ischemic, infectious, metabolic, toxins, autoimmune processes or nutritional deficiencies. Carnitine deficiency-induced cardiomyopathy (CDIM) is an uncommon cause of dilated cardiomyopathy that can go untreated if not considered. Here, we describe a 30-year-old woman with an eating disorder and recent percutaneous endoscopic gastrotomy (PEG) tube placement for weight loss admitted to the hospital for possible PEG tube infection. Carnitine level was found to be low. Transthoracic echocardiogram (TTE) revealed ejection fraction 15%. Her hospital course was complicated by sepsis from a peripherally inserted central catheter (PICC). She was discharged on a beta-blocker and carnitine supplementation. One month later her cardiac function had normalized. Carnitine deficiency-induced myopathy is an unusual cause of cardiomyopathy and should be considered in adults with decreased oral intake or malabsorption who present with cardiomyopathy.

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

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    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.

  15. Pneumatic dilatation for childhood achalasia.

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    Babu, R; Grier, D; Cusick, E; Spicer, R D

    2001-09-01

    Treatment of achalasia by pneumatic balloon dilatation (PBD) is well established in adults. Due to limited experience and the rarity of the condition in children, there are relatively few reports in the paediatric literature. Although PBD has been reported as a primary method of treatment, there are no reports of secondary PBD for childhood achalasia. Between 1995 and 1999, five patients underwent treatment for achalasia (age: 9-14 years, M:F = 4:1). The presenting symptoms were dysphagia (5). vomiting episodes (2), aspiration (1), food-bolus obstruction (1), and failure to thrive (1). In all patients a barium swallow and manometry were used to confirm the diagnosis. Three underwent primary PBD. Two who had previously undergone surgical myotomy underwent secondary PBD for recurrence of symptoms. Dilatation was performed using a 35-mm balloon with the child under general anaesthesia. Technical success was defined as demonstration of a waist under screening at lower pressures followed by abolition of the waist at higher pressures. In addition to reviewing our results, a systematic review of the literature was performed (Medline, Cochrane Library, Pubmed, Embase). Three patients (primary dilatation) showed excellent improvement after a single dilatation. In two cases (secondary dilatation) three and five attempts were required. No complications were encountered. The mean follow-up period was 2 years (1-3.5 years) and four patients remained asymptomatic, an overall success rate of 80%. The literature review revealed similar good results in most of the recent reports. Thus, PBD as a primary treatment for childhood achalasia has a success rate of 70%-90% with minimal side effects, short hospital stay, and good patient acceptability over an operation. We have also established the usefulness of this method as a secondary treatment when symptoms recur after surgery.

  16. Characteristic adaptations of the extracellular matrix in dilated cardiomyopathy

    NARCIS (Netherlands)

    Louzao-Martinez, Laura; Vink, Aryan; Harakalova, Magdalena; Asselbergs, Folkert W; Verhaar, Marianne C; Cheng, Caroline

    2016-01-01

    Dilated cardiomyopathy (DCM) is a relatively common heart muscle disease characterized by the dilation and thinning of the left ventricle accompanied with left ventricular systolic dysfunction. Myocardial fibrosis is a major feature in DCM and therefore it is inevitable that corresponding extracellu

  17. A Tension-Based Model Distinguishes Hypertrophic versus Dilated Cardiomyopathy.

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    Davis, Jennifer; Davis, L Craig; Correll, Robert N; Makarewich, Catherine A; Schwanekamp, Jennifer A; Moussavi-Harami, Farid; Wang, Dan; York, Allen J; Wu, Haodi; Houser, Steven R; Seidman, Christine E; Seidman, Jonathan G; Regnier, Michael; Metzger, Joseph M; Wu, Joseph C; Molkentin, Jeffery D

    2016-05-19

    The heart either hypertrophies or dilates in response to familial mutations in genes encoding sarcomeric proteins, which are responsible for contraction and pumping. These mutations typically alter calcium-dependent tension generation within the sarcomeres, but how this translates into the spectrum of hypertrophic versus dilated cardiomyopathy is unknown. By generating a series of cardiac-specific mouse models that permit the systematic tuning of sarcomeric tension generation and calcium fluxing, we identify a significant relationship between the magnitude of tension developed over time and heart growth. When formulated into a computational model, the integral of myofilament tension development predicts hypertrophic and dilated cardiomyopathies in mice associated with essentially any sarcomeric gene mutations, but also accurately predicts human cardiac phenotypes from data generated in induced-pluripotent-stem-cell-derived myocytes from familial cardiomyopathy patients. This tension-based model also has the potential to inform pharmacologic treatment options in cardiomyopathy patients.

  18. Titin gene mutations are common in families with both peripartum cardiomyopathy and dilated cardiomyopathy

    NARCIS (Netherlands)

    van Spaendonck-Zwarts, Karin Y.; Posafalvi, Anna; van den Berg, Maarten P.; Hilfiker-Kleiner, Denise; Bollen, Ilse A. E.; Sliwa, Karen; Alders, Marielle; AlMomani, Rowida; van Langen, Irene M.; van der Meer, Peter; Sinke, Richard J.; van der Velden, Jolanda; Van Veldhuisen, Dirk J.; van Tintelen, J. Peter; Jongbloed, Jan D. H.

    2014-01-01

    Aim Peripartum cardiomyopathy (PPCM) can be an initial manifestation of familial dilated cardiomyopathy (DCM). We aimed to identify mutations in families that could underlie their PPCM and DCM. Methods and results We collected 18 families with PPCM and DCM cases from various countries. We studied th

  19. X-Linked Dilated Cardiomyopathy: A Cardiospecific Phenotype of Dystrophinopathy

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    Akinori Nakamura

    2015-06-01

    Full Text Available X-linked dilated cardiomyopathy (XLDCM is a distinct phenotype of dystrophinopathy characterized by preferential cardiac involvement without any overt skeletal myopathy. XLDCM is caused by mutations of the Duchenne muscular dystrophy (DMD gene and results in lethal heart failure in individuals between 10 and 20 years. Patients with Becker muscular dystrophy, an allelic disorder, have a milder phenotype of skeletal muscle involvement compared to Duchenne muscular dystrophy (DMD and sometimes present with dilated cardiomyopathy. The precise relationship between mutations in the DMD gene and cardiomyopathy remain unclear. However, some hypothetical mechanisms are being considered to be associated with the presence of some several dystrophin isoforms, certain reported mutations, and an unknown dystrophin-related pathophysiological mechanism. Recent therapy for Duchenne muscular dystrophy, the severe dystrophinopathy phenotype, appears promising, but the presence of XLDCM highlights the importance of focusing on cardiomyopathy while elucidating the pathomechanism and developing treatment.

  20. 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.

  1. Dilated cardiomyopathy after electrical injury: report of two cases.

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    Buono, Lee M; DePace, Nicholas L; Elbaum, David M

    2003-05-01

    The specific etiologic factor and pathogenesis of most dilated cardiomyopathies have yet to be described definitively. Hypotheses of the etiologic factor of idiopathic dilated cardiomyopathy (DCM) abound. This report describes two patients with electrical injury in whom DCM developed after the electrical insult in the absence of other precipitating causes. Further histologic examination of myocardial tissue after electrical injury may reveal clues regarding the pathophysiology behind electrically induced DCM. Because electrical injury may be associated with myocardial dysfunction, short- and long-term evaluation of left ventricular function may be warranted.

  2. Dilated cardiomyopathy in an American cocker spaniel with taurine deficiency.

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    Gavaghan, B J; Kittleson, M D

    1997-12-01

    An American Cocker Spaniel with low plasma taurine concentration (taurine. Improvement in all echocardiographic indices were noted over a 22 week follow-up, most notably an increase in left ventricular shortening fraction to 20%, a decrease of E-point septal separation from 14 mm to 7 mm and marked left ventricular remodelling. This degree of improvement in myocardial function may represent a direct link between dilated cardiomyopathy in the American Cocker Spaniel and plasma taurine deficiency. Alternatively, this response may reflect a breed-related cardiomyopathy with a natural history and therapeutic response not commonly seen in the more common large breed cardiomyopathy presentations.

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

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    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.

  4. Reversible transition from a hypertrophic to a dilated cardiomyopathy

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    Spillmann, Frank; Kühl, Uwe; Van Linthout, Sophie; Dominguez, Fernando; Escher, Felicitas; Schultheiss, Heinz‐Peter; Pieske, Burkert

    2015-01-01

    Abstract We report the case of a 17‐year‐old female patient with known hypertrophic cardiomyopathy and a Wolff‐Parkinson‐White syndrome. She came to our department for further evaluation of a new diagnosed dilated cardiomyopathy characterized by an enlargement of the left ventricle and a fall in ejection fraction. Clinically, she complained about atypical chest pain, arrhythmic episodes with presyncopal events, and dyspnea (NYHA III) during the last 6 months. Non‐invasive and invasive examinations including magnetic resonance imaging, electrophysiological examinations, and angiography did not lead to a conclusive diagnosis. Therefore, endomyocardial biopsies (EMBs) were taken to investigate whether a specific myocardial disease caused the impairment of the left ventricular function. EMB analysis resulted in the diagnosis of a virus‐negative, active myocarditis. Based on this diagnosis, an immunosuppressive treatment with prednisolone and azathioprine was started, which led to an improvement of cardiac function and symptoms within 3 months after initiating therapy. In conclusion, we show that external stress triggered by myocarditis can induce a reversible transition from a hypertrophic cardiomyopathy to a dilated cardiomyopathy phenotype. This case strongly underlines the need for a thorough and invasive examination of heart failure of unknown causes, including EMB investigations as recommend by the actual ESC position statement. PMID:27774273

  5. Stress-echocardiography in idiopathic dilated cardiomyopathy: instructions for use

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    Neskovic Aleksandar N

    2005-02-01

    Full Text Available Abstract A number of studies have suggested that stress-echocardiography may be used for prognostic stratification in patients with idiopathic dilated cardiomyopathy. There is no consensus on which protocol or which measurements of left ventricular contractile reserve to use. The most frequently used protocol is low-dose dobutamine stress-echocardiography, and most commonly used measures of left ventricular systolic performance are ejection fraction, wall motion score index and cardiac power output. Stress-echocardiography has been shown to predict improvement in cardiac function in patients with recently diagnosed dilated cardiomyopathy, as well as to predict which patients will benefit from the treatment with beta-blockers. Most importantly, stress-echocardiography can identify patients with worse prognosis in terms of cardiac death and need for transplantation. Additionally, contractile reserve is closely correlated with maximal oxygen consumption and can even be used for further stratification in patients with maximal oxygen consumption between 10 and 14 ml/kg/min. Future studies are needed for head-to-head comparison of various protocols in an attempt to make standardization in the assessment of patients with dilated cardiomyopathy.

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

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    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 ...

  7. 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.

  8. A Family History of Dilated Cardiomyopathy Induced by Viral Myocarditis

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    Thomas Cognet

    2012-01-01

    Full Text Available Myocarditis can lead to acute heart failure, cardiogenic shock, or sudden death and later, dilated cardiomyopathy (DCM with chronic heart failure. We report the cases of two DCM induced by acute and past myocarditis in the same family and expressed by its two main complications within few weeks: an hemodynamic presentation as a fulminant myocarditis rapidly leading to cardiac tranplantation and a rythmologic presentation as an electrical storm leading to catheter ablation of ventricular tachycardia. These cases ask the question of the family predisposition to viral myocarditis leading to DCM.

  9. Dilated cardiomyopathy as part of familial dystrophia myotonica

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    Gadgaard, Tenna; Eiskjær, Hans; Jensen, Peter Kjestrup Axel;

    2014-01-01

    Dilated cardiomyopathy (DCM) is a condition characterized by non-ischaemic heart failure and is often hereditary. We present a family in which the proband had DCM in isolation while several relatives presented with myotonia, hypotonia, poly-hydramnion during pregnancy or a mental handicap....... The disease presentation and subsequent genetic investigations were consistent with a diagnosis of dystrophia myotonica. This case presentation illustrate that DCM may be part of a systemic condition and that familial investigations may have important implications for correct diagnosis, treatment...... and counseling....

  10. The Efficacy of L-Carnitine Treatment in Dilated Cardiomyopathy

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    DÖNDER, Emir

    1998-01-01

    This study was carried out to investigate clinical effects of treatment with the supplementation of L-carnitine in cases with dilated cardiomyopathy. B Mode, M-Mode, and continuous Doppler echocardiograms were applied with standard techniques in totally 28 patients assessed before treatment with L-carnitine and at the 1 st , 5 th , 10 th , 30 th , and 60 th days of the treatment. The diameter of the left ventricular endsystolic and end-diastolic have decreased with L-carnitine tre...

  11. Perturbation of NCOA6 Leads to Dilated Cardiomyopathy

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    Jae-il Roh

    2014-08-01

    Full Text Available Dilated cardiomyopathy (DCM is a progressive heart disease characterized by left ventricular dilation and contractile dysfunction. Although many candidate genes have been identified with mouse models, few of them have been shown to be associated with DCM in humans. Germline depletion of Ncoa6, a nuclear hormone receptor coactivator, leads to embryonic lethality and heart defects. However, it is unclear whether Ncoa6 mutations cause heart diseases in adults. Here, we report that two independent mouse models of NCOA6 dysfunction develop severe DCM with impaired mitochondrial function and reduced activity of peroxisome proliferator-activated receptor δ (PPARδ, an NCOA6 target critical for normal heart function. Sequencing of NCOA6-coding regions revealed three independent nonsynonymous mutations present in 5 of 50 (10% patients with idiopathic DCM (iDCM. These data suggest that malfunction of NCOA6 can cause DCM in humans.

  12. 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].

  13. Modeling GATAD1-Associated Dilated Cardiomyopathy in Adult Zebrafish

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    Jingchun Yang

    2016-01-01

    Full Text Available Animal models have played a critical role in validating human dilated cardiomyopathy (DCM genes, particularly those that implicate novel mechanisms for heart failure. However, the disease phenotype may be delayed due to age-dependent penetrance. For this reason, we generated an adult zebrafish model, which is a simpler vertebrate model with higher throughput than rodents. Specifically, we studied the zebrafish homologue of GATAD1, a recently identified gene for adult-onset autosomal recessive DCM. We showed cardiac expression of gatad1 transcripts, by whole mount in situ hybridization in zebrafish embryos, and demonstrated nuclear and sarcomeric I-band subcellular localization of Gatad1 protein in cardiomyocytes, by injecting a Tol2 plasmid encoding fluorescently-tagged Gatad1. We next generated gatad1 knock-out fish lines by TALEN technology and a transgenic fish line that expresses the human DCM GATAD1-S102P mutation in cardiomyocytes. Under stress conditions, longitudinal studies uncovered heart failure (HF-like phenotypes in stable KO mutants and a tendency toward HF phenotypes in transgenic lines. Based on these efforts of studying a gene-based inherited cardiomyopathy model, we discuss the strengths and bottlenecks of adult zebrafish as a new vertebrate model for assessing candidate cardiomyopathy genes.

  14. Molecular profiling of dilated cardiomyopathy that progresses to heart failure

    Science.gov (United States)

    Burke, Michael A.; Chang, Stephen; Wakimoto, Hiroko; Gorham, Joshua M.; Conner, David A.; Christodoulou, Danos C.; Parfenov, Michael G.; DePalma, Steve R.; Eminaga, Seda; Konno, Tetsuo; Seidman, Jonathan G.; Seidman, Christine E.

    2016-01-01

    Dilated cardiomyopathy (DCM) is defined by progressive functional and structural changes. We performed RNA-seq at different stages of disease to define molecular signaling in the progression from pre-DCM hearts to DCM and overt heart failure (HF) using a genetic model of DCM (phospholamban missense mutation, PLNR9C/+). Pre-DCM hearts were phenotypically normal yet displayed proliferation of nonmyocytes (59% relative increase vs. WT, P = 8 × 10–4) and activation of proinflammatory signaling with notable cardiomyocyte-specific induction of a subset of profibrotic cytokines including TGFβ2 and TGFβ3. These changes progressed through DCM and HF, resulting in substantial fibrosis (17.6% of left ventricle [LV] vs. WT, P = 6 × 10–33). Cardiomyocytes displayed a marked shift in metabolic gene transcription: downregulation of aerobic respiration and subsequent upregulation of glucose utilization, changes coincident with attenuated expression of PPARα and PPARγ coactivators -1α (PGC1α) and -1β, and increased expression of the metabolic regulator T-box transcription factor 15 (Tbx15). Comparing DCM transcriptional profiles with those in hypertrophic cardiomyopathy (HCM) revealed similar and distinct molecular mechanisms. Our data suggest that cardiomyocyte-specific cytokine expression, early fibroblast activation, and the shift in metabolic gene expression are hallmarks of cardiomyopathy progression. Notably, key components of these profibrotic and metabolic networks were disease specific and distinguish DCM from HCM. PMID:27239561

  15. Dobutamine stress echocardiographyin distinguishing ischemic from nonischemic dilated cardiomyopathy

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    Miloradović Vladimir

    2005-01-01

    Full Text Available Introduction The aim of this study was to evaluate the diagnostic accuracy of dobutamine stress echocardiography for detection of coronary artery disease in patients with dilated cardiomyopathy. Detection of regional wall motion abnormalities at rest does not reliably distinguish ischemic from nonischemic cardiomyopathy. Material and methods To distinguish between ischemic and nonischemic dilated cardiomyopathy (DCM, we studied 50 patients with left ventricular dysfunction (20 ischemic and 30 nonischemic, detected by coronary angiography using dobutamine stress echocardiography. Echocardiographic images were obtained at baseline, low and paek dose of dobutamine. Rest and stress left ventricular wall motion scores were derived from analysis of regional wall motion. Results Dobutamine infusion was terminated after achievement of the target heart rate or maximal protocol dose in 16 (80% patients with ischemic heart disease and in 23 (73.3% patients with nonischemic heart disease. At rest, there were more normal segments (p<0.001 and a trend toward more akinetic segments (p, not significant per ischemic than per nonischemic DCM patients. However, either at rest or with low-dose dobutamine, individual data largely overlapped. At peak dose, in ischemic DCM, regional contraction worsened in many normal or dyssinergic regions at rest (in some cases after inprovement with low-dose dobutamine; in contrast, in nonischemic DCM, further mild impovement was observed in a variable number of left ventricular areas. Thus, with peak-dose dobutamine, more akinetic and less normal segments were present per ishemic than per nonischemic DCM patient (both, p<0.001. A value of six or more akinetic segments was 90% sensitive and 98% specific for ischemic DCM. Conclusions Our data show that analysis of regional contraction by dobutamine stress echocardiography can distinguish between.

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

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    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

  17. Defining the molecular genetic basis of idiopathic dilated cardiomyopathy.

    Science.gov (United States)

    Olson, T M; Keating, M T

    1997-02-01

    Dilated cardiomyopathy (DCM) is a significant health care problem. The etiology is idiopathic in approximately half of the patients. Recognition that 20%-25% of idiopathic DCM cases are familial has advanced the hypothesis that single gene defects are important in the disease's pathogenesis. General linkage analyses in rare, large DCM families have determined the chromosome location of five idiopathic DCM genes. Candidate-gene mutational analyses in more typical, small pedigrees represent an alternative strategy for DCM gene identification. Human molecular genetics will play a fundamental role in defining pathogenic mechanisms for DCM with the prospect of new, molecular-based diagnostic and therapeutic approaches. (Trends Cardiovasc Med 1997;7:60-63). © 1997, Elsevier Science Inc.

  18. Kidney infarction in Friedreich's ataxia with dilated cardiomyopathy.

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    Evangelopoulos, Dimitrios Stergios; Pirvu, Tatiana Nataly; Exadaktylos, Aristomenis; Kohl, Sandro

    2012-09-30

    A 37-year-old man with advanced Friedreich's ataxia was referred to our emergency department with acute exacerbated abdominal pain of unclear aetiology. Laboratory tests showed slightly increased inflammatory parameters, elevated troponin and B-type natriuretic peptide, as well as minimal proteinuria. Transthoracic echocardiography revealed a pre-existing dilated cardiomyopathy. Abdominal sonography showed no pathological alterations. Owing to persistent pain under analgesia, a contrast-enhanced CT-abdomen was performed, which revealed a non-homogeneous perfusion deficit of the right kidney, although neither abdominal vascular alteration, cardiac thrombus, deep vein thrombosis nor a patent foramen ovale could be detected. Taking all clinical and radiological results into consideration, the current incident was diagnosed as a thromboembolic kidney infarction. As a consequence, lifelong oral anticoagulation was initiated.

  19. Viral Myocarditis and Dilated Cardiomyopathy: Etiology and Pathogenesis.

    Science.gov (United States)

    Huber, Sally A

    2016-01-01

    Myocarditis is an inflammation of the myocardium which often follows microbial infections and is a significant cause of sudden unexpected death in the young (myocarditis and has been found to be of limited value in lymphocytic myocarditis. The relatively limited response might reflect the need for host immunity to control persistent virus infection in the heart which may be the predominant cause of the chronic myocarditis and dilated cardiomyopathy. Treating the persistent virus infection with interferon-beta improved cardiac function in a clinical trial. However, classic immunosuppressive drugs, such as cyclosporine A and cyclophosphamide, are not effective against all types of immunity and experimental myocarditis models have shown that certain immunopathogenic forms of the disease are resistant to these immunosuppressive agents. Understanding the molecular mechanisms underlying the pathogenesis of this disease and the various infectious agents which can cause it will be essential for developing effective therapeutic agents.

  20. 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.

  1. Epidemiology and genetics of dilated cardiomyopathy in the Indian context

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    Ushasree B

    2009-07-01

    Full Text Available Background: Dilated cardiomyopathy (DCM still remains to be a poorly understood and less analyzed group of cardiac-muscle disorders when compared to hypertrophic cardiomyopathy (HCM. Also, the vast clinical heterogeneity among the patients has rendered the small and isolated kindred studies less informative on the genetics and epidemiology of DCM. Aim of the study: The study aimed at understanding the epidemiology and genetics of DCMs in the Indian context. Materials and methods/ Statistical analysis: One hundred seven DCM patients and 105 healthy individuals were included in the study for epidemiological and genetic risk factor identification and to fit the possible mode of inheritance. Single′s ascertainment methodology for segregation analysis and Penrose frequency estimates were followed to evaluate for the role of specific epidemiological factors in the disease etiology. Chi-square analysis was carried out to interpret the results statistically. Results and Conclusion: Our study suggests that epidemiological factors like gender, age at onset and vegetarian diet in conjunction with sarcomere gene mutations may play a role in the disease expression. Similarly, segregation analysis for the possible mode of inheritance showed a deviation from the autosomal dominant mode of inheritance, strengthening the underlying genetic heterogeneity of DCM.

  2. 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.

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

    Science.gov (United States)

    Del Corsso, C; Campos de Carvalho, A C

    2011-05-01

    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.

  4. [The thickness/radius ratio (t/r) in patients with dilated and hypertrophic cardiomyopathy].

    Science.gov (United States)

    Guadalajara, J F; Valenzuela, F; Martínez Sánchez, C; Huerta, D

    1990-01-01

    We studied 17 patients with cardiomyopathy (10 hypertrophic and 7 dilated). With two-dimensional echocardiography, we obtained a short axis view at the level of papillary muscle. We calculated the ratio between thickness (h), of ventricular wall and cavity's radius (r) in diastole and systole (h/r ratio). Hypertrophic cardiomyopathy has a high h/r ratio in diastole (inappropriate hypertrophy), hypercontractility and low and systolic wall stress. Dilated cardiomyopathy has a low diastolic h/r ratio (inadequate hypertrophy) with low contractility and elevated end-systolic, wall stress. We discuss the mechanisms and consequences of different patterns of hypertrophy on the ventricular performance.

  5. Common susceptibility variants examined for association with dilated cardiomyopathy.

    Science.gov (United States)

    Rampersaud, Evadnie; Kinnamon, Daniel D; Hamilton, Kara; Khuri, Sawsan; Hershberger, Ray E; Martin, Eden R

    2010-03-01

    Rare mutations in more than 20 genes have been suggested to cause dilated cardiomyopathy (DCM), but explain only a small percentage of cases, mainly in familial forms. We hypothesised that more common variants may also play a role in increasing genetic susceptibility to DCM, similar to that observed in other common complex disorders. To test this hypothesis, we performed case-control analyses on all DNA polymorphic variation identified in a resequencing study of six candidate DCM genes (CSRP3, LDB3, MYH7, SCN5A, TCAP, and TNNT2) conducted in 289 unrelated white probands with DCM of unknown cause and 188 unrelated white controls. In univariate analyses, we identified associated common variants at LDB3 site 10779, LDB3 site 57877, MYH7 sites 16384 and 17404, and TCAP sites 140 and 1735. Multivariate analyses to examine the joint effects of multiple gene variants confirmed univariate results for MYH7 and TCAP and identified a block of nine variants in MYH7 that was strongly associated with DCM. Common variants in genes known to be causative of DCM may play a role in genetic susceptibility to DCM. Our results suggest that examination of common genetic variants may be warranted in future studies of DCM and other Mendelian-like disorders.

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

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    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.

  7. A Case Report of Cardiac Amyloidosis Initially Managed as Dilated Cardiomyopathy: Missing the obvious!

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    Yerramareddy Vijaya Chandra

    2016-06-01

    Full Text Available Amyloidosis is a rare disorder with uncertain incidence; however, in UK and US population, AL amyloidosis, the most frequently diagnosed type, has an annual incidence of 6 to 10 cases per million. [1] The deposition of amyloid, the extracellular proteinaceous material, in the tissues results in a group of disorders called amyloidoses. [2] The most commonly deposited amyloid material in various organ systems including heart are light chains, transthyretin and serum amyloid A. [2] One of the challenges in diagnosing amyloidosis early is that it commonly manifests with nonspeci c symptoms of fatigue and weight loss. The diagnosis is generally considered only when symptoms are traceable to a speci c organ. [3] Cardiac amyloidosis presents initially with mild LV diastolic dysfunction, progressing to classical restrictive cardiomyopathy and nally even dilated cardiomyopathy like stage with end-stage heart failure. The disease can be mistaken in the early stages with hypertrophic cardiomyopathy and hypertensive heart disease and in the late stages with the common-garden variety of dilated cardiomyopathy. Here, we describe a case of cardiac amyloidosis, initially diagnosed and managed as dilated cardiomyopathy with inadequate response to management. Amyloidosis; 2D ECHO; Dilated Cardiomyopathy

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

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    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.

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

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    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.

  10. Acute dilated cardiomyopathy in a patient with beriberi and cryoglobulinaemic vasculitis: an unusual potential complication of two rare disorders.

    Science.gov (United States)

    Tejedor, Ana; Solé, Manel; Prieto-González, Sergio; Alba, Marco Antonio; Grau, Josep Maria; Cid, Maria Cinta; Hernández-Rodríguez, José

    2014-01-01

    We report the case of a 45-year-old patient who presented with acute dilated cardiomyopathy. During admission the patient was consecutively diagnosed with cryoglobulinaemic vasculitis and beriberi. In both diseases, cardiac involvement may occur as dilated cardiomyopathy. Thiamin deficiency was the final cause for the severe cardiac manifestations (cardiac acute beriberi or Shoshin syndrome), which returned to normal after thiamin supplementation.

  11. Non-invasive evaluation of arrhythmic risk in dilated cardiomyopathy:From imaging to electrocardiographic measures

    Institute of Scientific and Technical Information of China (English)

    Massimo; Iacoviello; Francesco; Monitillo

    2014-01-01

    Malignant ventricular arrhythmias are a major adverse event and worsen the prognosis of patients affected by ischemic and non-ischemic dilated cardiomyopathy.The main parameter currently used to stratify arrhythmic risk and guide decision making towards the implantation of a cardioverter defibrillator is the evaluation of the left ventricular ejection fraction.However,this strategy is characterized by several limitations and consequently additional parameters have been suggested in order to improve arrhythmic risk stratification.The aim of this review is to critically revise the prognostic significance of non-invasive diagnostic tools in order to better stratify the arrhythmic risk prognosis of dilated cardiomyopathy patients.

  12. Pregnancy-Associated Cardiomyopathy in Survivors of Childhood Cancer

    Science.gov (United States)

    Hines, Melissa R.; Mulrooney, Daniel A.; Hudson, Melissa M.; Ness, Kirsten K.; Green, Daniel M.; Howard, Scott C.; Krasin, Matthew; Metzger, Monika L.

    2015-01-01

    Purpose Current information regarding pregnancy-associated cardiomyopathy among women treated for childhood cancer is insufficient to appropriately guide counseling and patient management. This study aims to characterize its prevalence within a large cohort of females exposed to cardiotoxic therapy. Methods Retrospective cohort study of female cancer survivors treated at St. Jude Children’s Research Hospital between 1963 and 2006, at least 5 years from diagnosis, ≥ 13 years old at last follow-up, and with at least one successful pregnancy. Pregnancy-associated cardiomyopathy was defined as shortening fraction < 28% or ejection fraction < 50% or treatment for cardiomyopathy during or up to 5 months after completion of pregnancy. Results Among 847 female cancer survivors with 1554 completed pregnancies only 3 (0.3%) developed pregnancy-associated cardiomyopathy, 40 developed non-pregnancy-associated cardiomyopathy either 5 months post-partum (n=14), or prior to pregnancy (n=26). Among those with cardiomyopathy prior to pregnancy (n=26), cardiac function deteriorated during pregnancy in 8 patients (3 patients with normalization of cardiac function prior to pregnancy, 3 with persistently abnormal cardiac function, and 2 for whom resolution of cardiomyopathy was unknown prior to pregnancy). Patients that developed cardiomyopathy recevied a higher median dose of anthracyclines compared to those that did not (321 mg/m2 versus 164 mg/m2; p< 0.01). Conclusions Pregnancy-associated cardiomyopathy in childhood cancer survivors is rare. Implications for cancer survivors Most female childhood cancer survivors will have no cardiac complications during or after childbirth, however those with a history of cardiotoxic therapies should be followed carefully during pregnancy particularly those with a history of anthracycline exposures and if they had documented previous or current subclinical or symptomatic cardiomyopathy. Female childhood cancer survivors with a history of

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

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    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.

  14. Dilated Cardiomyopathy in a Rio Grande Wild Turkey (Meleagris gallopavo intermedia) in Southern Utah, USA, 2013.

    Science.gov (United States)

    Frame, David D; Kelly, E Jane; Van Wettere, Arnaud

    2015-07-01

    A male Rio Grande Wild Turkey (Meleagris gallopavo intermedia) living in semidomestication was submitted for necropsy. Emaciation, a greatly enlarged heart, and chronic passive congestion of the liver were present. Dilated cardiomyopathy occurs in domestic turkey flocks but has not been reported in Wild Turkeys.

  15. [Clinical case of the month. Cardiac complications of acromegaly: a rare cause of dilated cardiomyopathy].

    Science.gov (United States)

    Devoitille, A; Beckers, A; Piérard, L A

    2012-04-01

    Acromegaly is a disease characterized by chronic growth hormone hypersecretion. Cardiovascular complications represent the main cause of death. We present here a rare case of dilated cardiomyopathy whose diagnosis revealed an acromegaly. This will provide the opportunity to review an uncommon disease and its recently reassessed prevalence.

  16. 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

  17. 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.

    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 biops

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

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    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. 

  19. 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 cardiomyopa

  20. Congenital posterior pole cataract and adult onset dilating cardiomyopathy: expanding the phenotype of αB-crystallinopathies.

    Science.gov (United States)

    van der Smagt, J J; Vink, A; Kirkels, J H; Nelen, M; ter Heide, H; Molenschot, M M C; Weger, R A; Schellekens, P A W; Hoogendijk, J; Dooijes, D

    2014-04-01

    Mutations in the αB-crystallin gene (CRYAB) have been reported in desmin-related myopathies, with or without cardiac involvement. Mutations in this gene have also been documented in large multi-generation families with autosomal dominant congenital posterior pole cataract (CPPC). In these congenital cataract families no cardiac or muscular phenotype was reported. This report describes a family with an unusual read-through mutation in CRYAB, leading to the elongation of the normal αB-crystallin protein with 19 amino acid residues. Affected family members combine a CPPC with an adult onset dilated cardiomyopathy (DCM), thereby expanding the αB-crystallinopathy phenotype. Repolarisation abnormalities preceded the onset of cardiomyopathy and were already present in childhood. No skeletal myopathy was observed. This report illustrates that congenital cataract can be a prelude to more severe disease even outside the context of inborn errors of metabolism. The identification of a CRYAB mutation in this family supports the notion that mutations in this gene are a rare cause of genetically determined DCM. The combined congenital cataract/cardiomyopathy phenotype adds to our understanding of the complex phenotypic spectrum of αB-crystallinopathies.

  1. Familial Dilated Cardiomyopathy Caused by a Novel Frameshift in the BAG3 Gene.

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    Rocio Toro

    Full Text Available Dilated cardiomyopathy, a major cause of chronic heart failure and cardiac transplantation, is characterized by left ventricular or biventricular heart dilatation. In nearly 50% of cases the pathology is inherited, and more than 60 genes have been reported as disease-causing. However, in 30% of familial cases the mutation remains unidentified even after comprehensive genetic analysis. This study clinically and genetically assessed a large Spanish family affected by dilated cardiomyopathy to search for novel variations.Our study included a total of 100 family members. Clinical assessment was performed in alive, and genetic analysis was also performed in alive and 1 deceased relative. Genetic screening included resequencing of 55 genes associated with sudden cardiac death, and Sanger sequencing of main disease-associated genes. Genetic analysis identified a frame-shift variation in BAG3 (p.H243Tfr*64 in 32 patients. Genotype-phenotype correlation identified substantial heterogeneity in disease expression. Of 32 genetic carriers (one deceased, 21 relatives were clinically affected, and 10 were asymptomatic. Seventeen of the symptomatic genetic carriers exhibited proto-diastolic septal knock by echocardiographic assessment.We report p.H243Tfr*64_BAG3 as a novel pathogenic variation responsible for familial dilated cardiomyopathy. This variation correlates with a more severe phenotype of the disease, mainly in younger individuals. Genetic analysis in families, even asymptomatic individuals, enables early identification of individuals at risk and allows implementation of preventive measures.

  2. Comparison of Calcitonin Gene Related Peptide Level between Children with Dilated Cardiomyopathy and Control Group

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    Noormohammad Noori

    2015-06-01

    Full Text Available Background: Dilated cardiomyopathy is revealed with left ventricular dilatation and systolic dysfunction. Objectives: This study aimed to compare the children with dilated cardiomyopathy and control group regarding the level of Calcitonin Gene Related Peptide (CGRP and its relationship with echocardiography findings Patients and Methods: This case-control study was conducted on 37 children with dilated cardiomyopathy and free of any clinical symptoms and 37 healthy age- and sex-matched children referring to Ali-e-Asghar and Ali Ebne Abitaleb hospitals in Zahedan, Iran. After taking history, echocardiography was performed for both groups. The data were analyzed using the SPSS statistical software and appropriate statistical tests. Results: The two groups were significantly different regarding most of the echocardiographic parameters (P < 0.05. Also, a significant difference was found between the two groups concerning the mean CGRP levels (P = 0.001. Among echocardiographic parameters, CGRP was directly related to Interventricular Septal dimension in Systole (IVSS (P = 0.022, R = 0.375. However, no significant relationship was observed between CGRP level and Ross classification. Conclusions: The findings of this study showed an increase in CGRP serum levels in the case group. Besides, a direct correlation was observed between CGRP level and IVSS.

  3. 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 blo...

  4. Sheehan's syndrome with reversible dilated cardiomyopathy: A case report and brief overview.

    Science.gov (United States)

    Islam, A K M Monwarul; Hasnat, Mohammad A; Doza, Fatema; Jesmin, Humayra

    2014-04-01

    Sheehan's syndrome is a rare condition characterized by post-partal panhypopituitarism due to necrosis of adenohypophysis resulting from severe post-partum hemorrhage. Lethargy, amenorrhea and failure of lactation are the usual presenting features. Cardiac involvement in Sheehan's syndrome is rare. The case presented here describes dilated cardiomyopathy in a 36-year-old lady who failed to respond adequately to the standard anti-failure treatment. Further investigation revealed the diagnosis of Sheehan's syndrome. Besides other manifestations, cardiac function reverted to normal after giving replacement therapy with glucocorticoid, levothyroxine and sex hormone. Physicians, specially those in developing countries, should have high index of suspicion for the diagnosis of Sheehan's syndrome while dealing with a case of 'peripartal dilated cardiomyopathy'. Persistent amenorrhea and failure of lactation may be important clues in this context. Timely diagnosis and appropriate treatment can lessen the sufferings of the patients.

  5. Inter- Not Intraindividual Differences in sTWEAK Levels Predict Functional Deterioration and Mortality in Patients with Dilated Cardiomyopathy

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    Kai-Uwe Jarr

    2014-01-01

    Full Text Available Background. TNF-like weak inducer of apoptosis (TWEAK has been reported to predict mortality in patients with dilated cardiomyopathy. However, whether it can be used as a biomarker for disease monitoring or rather represents a risk factor for disease progression remains unclear. Aim of the Study. To evaluate the potential of sTWEAK as a biomarker in patients with dilated cardiomyopathy. Results. We conducted a serial study of sTWEAK levels in 78 patients with dilated cardiomyopathy. Soluble TWEAK levels predicted not only a combined mortality/heart transplantation endpoint after 4 years (P=0.0001, but also the risk for clinical deterioration (P=0.0001. Compared to NT-proBNP, sTWEAK remained relatively stable in individual patients on follow-up indicating that inter- rather than intraindividual differences in sTWEAK levels predicted outcome. Finally, neither did the scavenger receptor sCD163 correlate with sTWEAK levels nor did its determination add additional information on outcome in patients with dilated cardiomyopathy. Conclusion. Soluble TWEAK levels in patients with dilated cardiomyopathy may not be of value for disease monitoring but may represent a risk factor for disease progression and death. Further research will be necessary to elucidate the exact role of sTWEAK as a potential modulator of immune response in the setting of dilated cardiomyopathy.

  6. TRANSURETHRAL RESECTION OF PROSTRATE IN DILATED CARDIOMYOPATHY PATIENT WITH LOW EJECTION FRACTION

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    Prabhavathi

    2014-06-01

    Full Text Available Dilated cardiomyopathy (DCM patient undergoing non-cardiac surgery poses a challenge for the anesthesiologist to manage it efficiently. DCM is usually accompanied by progressive congestive cardiac failure (CCF and life threatening arrhythmias. The anesthesiologist must have the idea of its haemodynamics, diagnostic evaluations, treatment modalities and more so regarding various drug interactions during anesthesia. We managed this case with combined low dose spinal epidural anesthesia with dexmeditomedine as additive.

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

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    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.

  8. Reversible dilated cardiomyopathy: into the thaumaturgy of the heart - Part 2

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    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 second part will deal with chemotherapy-induced cardiomyopathy, alcohol- related cardiomyopathy, myocarditis and peripartum cardiomyopathy. 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.

  9. 3-Methylglutaconyl-Coenzyme-A Hydratase Deficiency and the Development of Dilated Cardiomyopathy

    Science.gov (United States)

    Spergel, Craig D.; Milko, Mariya; Edwards, Christopher; Steinhoff, Jeff P.

    2014-01-01

    A 25-year-old Canadian male with a history of 3-methylglutaconyl-coenzyme-A hydratase deficiency, also known as 3-methylglutaconic aciduria type I, a very rare inborn error of metabolism, presented with respiratory distress, nausea, vomiting and signs of multisystem organ failure due to a suspected underlying infectious process. An electrocardiogram revealed bilateral atrial enlargement and an elevated brain natriuretic peptide on the initial laboratory studies, which prompted a more thorough cardiac workup. The transthoracic echocardiogram revealed a dilated cardiomyopathy with severe systolic dysfunction. The deficient enzyme present in this patient is involved in the pathway of leucine catabolism and is particularly important in various tissues for energy production and sterol synthesis. The dilated cardiomyopathy in this patient possibly had a variety of potential mechanisms including: a mitochondrial myopathy due to the deficiency of this enzyme leading to a defect in energy production inside cardiac myocytes; or a direct toxicity from 3-methylglutaconic acid (3-MGA) and its toxic metabolites; or a cardiac dysfunction due to a variety of other potential mechanisms. In conclusion, this patient’s clinical presentation suggested that 3-methylglutaconyl-CoA hydratase deficiency could cause a severe dilated cardiomyopathy and heart failure.

  10. 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; Giraldo, Alejandro; 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.

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

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    Jesús Talavera

    2015-01-01

    Full Text Available 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.

  12. A novel mitochondrial tRNA gene mutation in a chinese family with dilated cardiomyopathy and sensorineural deafness

    Institute of Scientific and Technical Information of China (English)

    Xianghong Wu; Xiumei Xie; Guotian Ma; Guoju Sun; Xiaobin Chen

    2006-01-01

    Objective: To determine whether a mutation of mitochondrial DNA induces familial dilated cardiomyopathy in Chinese families with cardiomyopathy, and analyzed the correlation between the genotype and phenotype. Methods: Affected members in three Chinese families of the familial dilated cardiomyopathy underwent clinical evaluation and DNA analysis. Polymerase chain reaction and direct DNA sequencing were used to screen for mitochondrial DNA mutation. The type of mtDNA vairations and clinical situation were analysed on the patients with mitochondrial DNA mutation. Results: The mitochondrial A3434G mutation was identified in one of the three families,the 3434 th nucleotide A was replaced by G, which led to change of amino acid. No mutations were identified in the clinically unaffected members of the family and all members of the other two families.Conclusion: This study indicates that the mitochondrial A3434G mutation maybe related with familial dilated cardiomyopathy and deafness.

  13. Potential genetic predisposition for anthracycline-associated cardiomyopathy in families with dilated cardiomyopathy

    NARCIS (Netherlands)

    Wasielewski, Marijke; van Spaendonck-Zwarts, Karin Y; Westerink, Nico-Derk L; Jongbloed, Jan D H; Postma, Aleida; Gietema, Jourik A; van Tintelen, J Peter; van den Berg, Maarten P

    2014-01-01

    OBJECTIVE: Anthracyclines are successfully used in cancer treatment, but their use is limited by their cardiotoxic side effects. Several risk factors for anthracycline-associated cardiomyopathy (AACM) are known, yet the occurrence of AACM in the absence of these known risk factors suggests that othe

  14. Furthering the link between the sarcomere and primary cardiomyopathies: restrictive cardiomyopathy associated with multiple mutations in genes previously associated with hypertrophic or dilated cardiomyopathy.

    Science.gov (United States)

    Caleshu, Colleen; Sakhuja, Rahul; Nussbaum, Robert L; Schiller, Nelson B; Ursell, Philip C; Eng, Celeste; De Marco, Teresa; McGlothlin, Dana; Burchard, Esteban González; Rame, J Eduardo

    2011-09-01

    Mutations in genes that encode components of the sarcomere are well established as the cause of hypertrophic and dilated cardiomyopathies. Sarcomere genes, however, are increasingly being associated with other cardiomyopathies. One phenotype more recently recognized as a disease of the sarcomere is restrictive cardiomyopathy (RCM). We report on two patients with RCM associated with multiple mutations in sarcomere genes not previously associated with RCM. Patient 1 presented with NYHA Class III/IV heart failure at 22 years of age. She was diagnosed with RCM and advanced heart failure requiring heart transplantation. Sequencing of sarcomere genes revealed previously reported homozygous p.Glu143Lys mutations in MYL3, and a novel heterozygous p.Gly57Glu mutation in MYL2. The patient's mother is a double heterozygote for these mutations, with no evidence of cardiomyopathy. Patient 2 presented at 35 years of age with volume overload while hospitalized for oophorectomy. She was diagnosed with RCM and is being evaluated for heart transplantation. Sarcomere gene sequencing identified homozygous p.Asn279His mutations in TPM1. The patient's parents are consanguineous and confirmed heterozygotes. Her father was diagnosed with HCM at 42 years of age. This is the first report of mutations in TPM1, MYL3, and MYL2 associated with primary, non-hypertrophied RCM. The association of more sarcomere genes with RCM provides further evidence that mutations in the various sarcomere genes can cause different cardiomyopathy phenotypes. These cases also contribute to the growing body of evidence that multiple mutations have an additive effect on the severity of cardiomyopathies.

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

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    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.

  16. Feasibility of Bone Marrow Stromal Cells Autologous Transplantation for Dilated Cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    ZHOU Cheng; YANG Chenyuan; XIAO Shiliang; FEI Hongwen

    2007-01-01

    The feasibility of bone marrow stromal cells autologous transplantation for rabbit model of dilated cardiomyopathy induced by adriamycin was studied. Twenty rabbits received 2 mg/kg of adriamycin intravenously once a week for 8 weeks (total dose, 16 mg/kg) to induce the cardiomyopathy model with the monitoring of cardiac function by transthoracic echocardiography. Marrow stromal cells were isolated from cell-transplanted group rabbits and were culture-expanded on the 8th week. On the 10th week, cells were labeled with 4,6-diamidino-2-phenylindole (DAPI), and then injected into the myocardium of the same rabbits. The results showed that viable cells labeled with DAPI could be identified in myocardium at 2nd week after transplantation. Histological findings showed the injury of the myocardium around the injection site was relieved with less apoptosis and more expression of bcl-2. The echocardiography found the improvement of local tissue movement from (2.12±0.51) cm/s to (3.81±0.47) cm/s (P<0.05) around the inject site, but no improvement of heart function as whole. It was concluded bone marrow stromal cells transplantation for dilated cardiomyopathy was feasibe. The management of cells in vitro, the quantity and the pattern of the cells transplantation and the action mechanism still need further research.

  17. Late gadolinium enhanced cardiovascular magnetic resonance of lamin A/C gene mutation related dilated cardiomyopathy

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    Peuhkurinen Keijo

    2011-06-01

    Full Text Available Abstract Background The purpose of this study was to identify early features of lamin A/C gene mutation related dilated cardiomyopathy (DCM with cardiovascular magnetic resonance (CMR. We characterise myocardial and functional findings in carriers of lamin A/C mutation to facilitate the recognition of these patients using this method. We also investigated the connection between myocardial fibrosis and conduction abnormalities. Methods Seventeen lamin A/C mutation carriers underwent CMR. Late gadolinium enhancement (LGE and cine images were performed to evaluate myocardial fibrosis, regional wall motion, longitudinal myocardial function, global function and volumetry of both ventricles. The location, pattern and extent of enhancement in the left ventricle (LV myocardium were visually estimated. Results Patients had LV myocardial fibrosis in 88% of cases. Segmental wall motion abnormalities correlated strongly with the degree of enhancement. Myocardial enhancement was associated with conduction abnormalities. Sixty-nine percent of our asymptomatic or mildly symptomatic patients showed mild ventricular dilatation, systolic failure or both in global ventricular analysis. Decreased longitudinal systolic LV function was observed in 53% of patients. Conclusions Cardiac conduction abnormalities, mildly dilated LV and depressed systolic dysfunction are common in DCM caused by a lamin A/C gene mutation. However, other cardiac diseases may produce similar symptoms. CMR is an accurate tool to determine the typical cardiac involvement in lamin A/C cardiomyopathy and may help to initiate early treatment in this malignant familiar form of DCM.

  18. Predictive value of mitral annular calcification for the diagnosis of coronary artery disease in patients with dilated cardiomyopathy.

    Science.gov (United States)

    Dincer, I; Ozdol, C; Dandachi, R; Akyurek, O; Atmaca, Y; Kiliçkap, M; Erol, C; Oral, D

    2001-08-01

    Mitral annulus calcification (MAC) is an independent predictor of coronary artery disease (CAD). The present study was designed to determine whether an association exists between MAC and CAD in patients with dilated cardiomyopathy. Among the 286 patients with MAC on echocardiographic examination who underwent coronary angiography, 55 patients with echocardiographic findings of dilated cardiomyopathy (group I) were compared to 60 age-matched controls without MAC and an echocardiographic diagnosis of dilated cardiomyopathy (group II) who underwent coronary angiography during the same time. There were no differences in echocardiographic findings between two groups. The prevalence of CAD was higher in group I when compared to group II (74% vs 28%, pMAC (p=0.001), diabetes mellitus (p=0.048), and history of anginal chest pain (p=0.009) are the independent predictors for the presence of CAD in patients with dilated cardiomyopathy. In conclusion, MAC may be a marker for the presence of coronary artery disease in patients with dilated cardiomyopathy.

  19. Intravenous Cardiac Stem Cell-Derived Exosomes Ameliorate Cardiac Dysfunction in Doxorubicin Induced Dilated Cardiomyopathy

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    Adam C. Vandergriff

    2015-01-01

    Full Text Available Despite the efficacy of cardiac stem cells (CSCs for treatment of cardiomyopathies, there are many limitations to stem cell therapies. CSC-derived exosomes (CSC-XOs have been shown to be responsible for a large portion of the regenerative effects of CSCs. Using a mouse model of doxorubicin induced dilated cardiomyopathy, we study the effects of systemic delivery of human CSC-XOs in mice. Mice receiving CSC-XOs showed improved heart function via echocardiography, as well as decreased apoptosis and fibrosis. In spite of using immunocompetent mice and human CSC-XOs, mice showed no adverse immune reaction. The use of CSC-XOs holds promise for overcoming the limitations of stem cells and improving cardiac therapies.

  20. 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).

  1. Knock-out of nexilin in mice leads to dilated cardiomyopathy and endomyocardial fibroelastosis.

    Science.gov (United States)

    Aherrahrou, Zouhair; Schlossarek, Saskia; Stoelting, Stephanie; Klinger, Matthias; Geertz, Birgit; Weinberger, Florian; Kessler, Thorsten; Aherrahrou, Redouane; Moreth, Kristin; Bekeredjian, Raffi; Hrabě de Angelis, Martin; Just, Steffen; Rottbauer, Wolfgang; Eschenhagen, Thomas; Schunkert, Heribert; Carrier, Lucie; Erdmann, Jeanette

    2016-01-01

    Cardiomyopathy is one of the most common causes of chronic heart failure worldwide. Mutations in the gene encoding nexilin (NEXN) occur in patients with both hypertrophic and dilated cardiomyopathy (DCM); however, little is known about the pathophysiological mechanisms and relevance of NEXN to these disorders. Here, we evaluated the functional role of NEXN using a constitutive Nexn knock-out (KO) mouse model. Heterozygous (Het) mice were inter-crossed to produce wild-type (WT), Het, and homozygous KO mice. At birth, 32, 46, and 22 % of the mice were WT, Het, and KO, respectively, which is close to the expected Mendelian ratio. After postnatal day 6, the survival of the Nexn KO mice decreased dramatically and all of the animals died by day 8. Phenotypic characterizations of the WT and KO mice were performed at postnatal days 1, 2, 4, and 6. At birth, the relative heart weights of the WT and KO mice were similar; however, at day 4, the relative heart weight of the KO group was 2.3-fold higher than of the WT group. In addition, the KO mice developed rapidly progressive cardiomyopathy with left ventricular dilation and wall thinning and decreased cardiac function. At day 6, the KO mice developed a fulminant DCM phenotype characterized by dilated ventricular chambers and systolic dysfunction. At this stage, collagen deposits and some elastin deposits were observed within the left ventricle cavity, which resembles the features of endomyocardial fibroelastosis (EFE). Overall, these results further emphasize the role of NEXN in DCM and suggest a novel role in EFE.

  2. The role of sarcomere gene mutations in patients with idiopathic dilated cardiomyopathy

    DEFF Research Database (Denmark)

    Møller, Daniel Vega; Andersen, Paal Skytt; Hedley, Paula

    2009-01-01

    We investigated a Danish cohort of 31 unrelated patients with idiopathic dilated cardiomyopathy (IDC), to assess the role that mutations in sarcomere protein genes play in IDC. Patients were genetically screened by capillary electrophoresis single strand conformation polymorphism and subsequently...... by bidirectional DNA sequencing of conformers in the coding regions of MYH7, MYBPC3, TPM1, ACTC, MYL2, MYL3, TNNT2, CSRP3 and TNNI3. Eight probands carried disease-associated genetic variants (26%). In MYH7, three novel mutations were found; in MYBPC3, one novel variant and two known mutations were found...

  3. Improvement of cardiac function after kidney transplantation with dilated cardiomyopathy and long dialysis vintage.

    Science.gov (United States)

    Mimura, Imari; Kawarazaki, Hiroo; Momose, Toshimitsu; Shibagaki, Yugo; Fujita, Toshiro

    2009-12-01

    Patients with long dialysis vintage have low cardiac output for various reasons. Although kidney transplantation is known to improve cardiac mortality, patients are sometimes evaluated as contraindicated for transplantation because of cardiac risk. We successfully performed kidney transplantation for a patient with a long dialysis vintage and dilated cardiomyopathy. Sequential (123)I-metaiodobenzylguanidine ((123)I-MIBG) scintigraphy suggested that amelioration of uraemia improved cardiac function. Kidney transplantation for patients with severely impaired cardiac function is safe and effective under careful perioperative monitoring irrespective of dialysis vintage. Sequential (123)I-MIBG scintigraphy can be used as an evaluation tool for the improvement in cardiac function.

  4. QT Dispersion Level and Its Clinical Significance.in Dilated Cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    2000-01-01

    Objective To evaluate the clinical significance of QT dis persion (QTd, QTcd) in dilated cardiomyopathy (DCM). Methods QTd and QTcd were measured on simultaneously recording 12-lead electrocardiograms ( ECGs) in 60 DCM patients and cormpared with 60 healthy subjects. Results The values of QTd and QTcd in DCM were significantly higher than those in control group ( P 110 ms and QTd≤110 ms(P0.05). Conclusion The values of QTd and QTcd increased in DCM patients were susceptive index for monitoring maligant VA in DCM, also important prognostic markers of CSD. QTd was correlated with NY HA functional class but not with EF and FS.

  5. Investigation of HSP60 gene expression in mRNA level in heart at dilated cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Riabenko D. V.

    2009-02-01

    Full Text Available The expression of HSP60 in the mRNA level in human hearts at the end-stage of dilated cardiomyopathy (DCM as well as in the hearts of mice with disease model similar to human DCM was investigated. We observed a significant increase in the Hsp60 mRNA level at the beginning of the disease and decrease to a normal level at the end stage. As the Hsp60 level was increased during the disease up to the end stage we can presume some changes in the regulation of Hsp60 synthesis or its degradation at DCM progression

  6. Sheehan’s syndrome with reversible dilated cardiomyopathy: A case report and brief overview

    Science.gov (United States)

    Islam, A.K.M. Monwarul; Hasnat, Mohammad A.; Doza, Fatema; Jesmin, Humayra

    2014-01-01

    Sheehan’s syndrome is a rare condition characterized by post-partal panhypopituitarism due to necrosis of adenohypophysis resulting from severe post-partum hemorrhage. Lethargy, amenorrhea and failure of lactation are the usual presenting features. Cardiac involvement in Sheehan’s syndrome is rare. The case presented here describes dilated cardiomyopathy in a 36-year-old lady who failed to respond adequately to the standard anti-failure treatment. Further investigation revealed the diagnosis of Sheehan’s syndrome. Besides other manifestations, cardiac function reverted to normal after giving replacement therapy with glucocorticoid, levothyroxine and sex hormone. Physicians, specially those in developing countries, should have high index of suspicion for the diagnosis of Sheehan’s syndrome while dealing with a case of ‘peripartal dilated cardiomyopathy’. Persistent amenorrhea and failure of lactation may be important clues in this context. Timely diagnosis and appropriate treatment can lessen the sufferings of the patients. PMID:24719543

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  10. Exenatide improves glucose homeostasis and prolongs survival in a murine model of dilated cardiomyopathy.

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    Arpita Kalla Vyas

    Full Text Available BACKGROUND: There is growing awareness of secondary insulin resistance and alterations in myocardial glucose utilization in congestive heart failure. Whether therapies that directly target these changes would be beneficial is unclear. We previously demonstrated that acute blockade of the insulin responsive facilitative glucose transporter GLUT4 precipitates acute decompensated heart failure in mice with advanced dilated cardiomyopathy. Our current objective was to determine whether pharmacologic enhancement of insulin sensitivity and myocardial glucose uptake preserves cardiac function and survival in the setting of primary heart failure. METHODOLOGY/PRINCIPAL FINDINGS: The GLP-1 agonist exenatide was administered twice daily to a murine model of dilated cardiomyopathy (TG9 starting at 56 days of life. TG9 mice develop congestive heart failure and secondary insulin resistance in a highly predictable manner with death by 12 weeks of age. Glucose homeostasis was assessed by measuring glucose tolerance at 8 and 10 weeks and tissue 2-deoxyglucose uptake at 75 days. Exenatide treatment improved glucose tolerance, myocardial GLUT4 expression and 2-deoxyglucose uptake, cardiac contractility, and survival over control vehicle-treated TG9 mice. Phosphorylation of AMP kinase and AKT was also increased in exenatide-treated animals. Total myocardial GLUT1 levels were not different between groups. Exenatide also abrogated the detrimental effect of the GLUT4 antagonist ritonavir on survival in TG9 mice. CONCLUSION/SIGNIFICANCE: In heart failure secondary insulin resistance is maladaptive and myocardial glucose uptake is suboptimal. An incretin-based therapy, which addresses these changes, appears beneficial.

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

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    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.

  12. Evaluation of 15 candidate genes for dilated cardiomyopathy in the Newfoundland dog.

    Science.gov (United States)

    Wiersma, Anje C; Stabej, Polona; Leegwater, Peter A J; Van Oost, Bernard A; Ollier, William E; Dukes-McEwan, Joanna

    2008-01-01

    Dilated cardiomyopathy (DCM) is a disease of the myocardium, which causes heart failure and premature death. It has been described in humans and several domestic animals. In the Newfoundland dog, DCM is an autosomal dominant disease with late onset and reduced penetrance. We analyzed 15 candidate genes for their involvement in DCM in the Newfoundland dog. Polymorphic microsatellite markers and single Nucleotide Polymorphisms were genotyped in 4 families of Newfoundland dogs segregating dilated cardiomyopathy for the genes encoding alpha-cardiac actin (ACTC), caveolin (CAVI), cysteine-rich protein 3 (CSRP3), LIM-domain binding factor 3 (LDB3), desmin (DES), lamin A/C (LMNA), myosin heavy polypeptide 7 (MYH7), delta-sarcoglycan (SGCD), troponin I (TNNTI3), troponin T (TNNT2), alpha-tropomyosin (TPMI), titin (TTN) and vinculin (VCL). A Logarithm of the odds (LOD) score of less than -2.0 in 2-point linkage analysis indicated exclusion of all but 2 genes, encoding CSRP3 and DES. A (LOD) score between -1.5 and -2.0 for CSRP3 and DES makes these genes unlikely causes of DCM in this dog breed. For the phospholamban (PLN) and titin cap (TTN) genes, a direct mutation screening approach was used. DNA sequence analysis of all exons showed no evidence that these genes are involved in DCM in the Newfoundland dog.

  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. Dilated Cardiomyopathy

    Science.gov (United States)

    ... including those caused by bacteria, viruses, fungi and parasites Drug and alcohol abuse Certain cancer medications Exposure ... blood pressure, low white blood cell count, and kidney or liver problems. Angiotensin II receptor blockers. These ...

  15. Mutation analysis of the phospholamban gene in 315 South Africans with dilated, hypertrophic, peripartum and arrhythmogenic right ventricular cardiomyopathies.

    Science.gov (United States)

    Fish, Maryam; Shaboodien, Gasnat; Kraus, Sarah; Sliwa, Karen; Seidman, Christine E; Burke, Michael A; Crotti, Lia; Schwartz, Peter J; Mayosi, Bongani M

    2016-02-26

    Cardiomyopathy is an important cause of heart failure in Sub-Saharan Africa, accounting for up to 30% of adult heart failure hospitalisations. This high prevalence poses a challenge in societies without access to resources and interventions essential for disease management. Over 80 genes have been implicated as a cause of cardiomyopathy. Mutations in the phospholamban (PLN) gene are associated with dilated cardiomyopathy (DCM) and severe heart failure. In Africa, the prevalence of PLN mutations in cardiomyopathy patients is unknown. Our aim was to screen 315 patients with arrhythmogenic right ventricular cardiomyopathy (n = 111), DCM (n = 95), hypertrophic cardiomyopathy (n = 40) and peripartum cardiomyopathy (n = 69) for disease-causing PLN mutations by high resolution melt analysis and DNA sequencing. We detected the previously reported PLN c.25C > T (p.R9C) mutation in a South African family with severe autosomal dominant DCM. Haplotype analysis revealed that this mutation occurred against a different haplotype background to that of the original North American family and was therefore unlikely to have been inherited from a common ancestor. No other mutations in PLN were detected (mutation prevalence = 0.2%). We conclude that PLN is a rare cause of cardiomyopathy in African patients. The PLN p.R9C mutation is not well-tolerated, emphasising the importance of this gene in cardiac function.

  16. Can serum tenascin-c be used as a marker of inflammation in patients with dilated cardiomyopathy?

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    Alyaa A. Kotby

    2014-03-01

    Conclusions: Serum-Tenascin Level was significantly increased in patients with idiopathic dilated cardiomyopathies. This increase was noted in acute and chronic cases, with significant difference between both being higher in the acute cases, and associated with the severity of heart failure and the LV dysfunction as detected by 2D speckle tracking echocardiography.

  17. Evaluation of myocardial disorders in patients with dilated cardiomyopathy and left ventricular eccentric hypertrophy; By sup 201 Tl myocardial SPECT

    Energy Technology Data Exchange (ETDEWEB)

    Yamazaki, Junichi; Ohsawa, Hidefumi; Uchi, Takashi (Toho Univ., Tokyo (Japan). School of Medicine) (and others)

    1992-03-01

    {sup 201}Tl myocardial SPECT was performed in cases of dilated cardiomyopathy and valvular heart disease with left ventricular eccentric hypertrophy, and the two groups were compared from the standpoint of the mechanism of onset of myocardial disorders. Significant coefficients of correlation were seen between the Tl score and LVDd (r=0.792, r=0.785) and Tl score and LVEF (r=-0.634, r=-0.555) in both dilated cardiomyopathy and valvular heart disease. In cases of valvular heart disease, significant correlation coefficients (r=-0.756, r=-0.720) between LVDd and r-WR (relative-washout rate), and Tl score and r-WR were observed, but no such correlation was seen in dilated cardiomyopathy. In valvular heart disease, a decrease in myocardial perfusion associated with enlargement of the left ventricle appeared, while in dilated cardiomyopathy, there was a marked decrease in LVEF in proportion to the thallium defect. Therefore, it was assumed that left ventricular wall disorders occur due to myocardial metabolic disorders and coronary microcirculation disorders. (author).

  18. Successful outcome in a patient with idiopathic dilated cardiomyopathy with bad obstetric history with no live issue

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    Renuka Malik

    2016-04-01

    Full Text Available Primary dilated cardiomyopathy is rare in women of childbearing age. Pregnancy in dilated cardiomyopathy carries high risk and can have adverse feto-maternal outcome even death. Traditionally patients of dilated cardiomyopathy with poor cardiac reserve are advised against pregnancy or termination of pregnancy in first trimester. A 26 year old patient with 5 abortions and no live issue diagnosed with Idiopathic dilated cardiomyopathy at 30 weeks at echocardiography (ECHO with left ventricular ejection fraction (LVEF of 25% (WHO class IV. She had no cardiac consultation earlier than in this pregnancy, though her earlier abortions were in hospital. Patient was kept with close monitoring. Dopplers parameters showed continued worsening. An emergency caesarean was done because of absent flow in ductus venosus at 34 weeks and a live child of 2.010 kg delivered. To date both mother and child are doing well. Awareness of such condition and a multidisciplinary approach is required in management of cardiomypathy in pregnancies, whose symptoms mimic that of normal pregnancy and can have adverse feto-maternal outcome. [Int J Reprod Contracept Obstet Gynecol 2016; 5(4.000: 1225-1227

  19. Recurrent and founder mutations in the Netherlands : mutation p.K217del in troponin T2, causing dilated cardiomyopathy

    NARCIS (Netherlands)

    Otten, E.; Deprez, R. H. Lekanne Dit; Weiss, M. M.; van Slegtenhorst, M.; Joosten, M.; van der Smagt, J. J.; Kerstjens-Frederikse, W. S.; Roofthooft, M. T. R.; Balk, A. H. M. M.; van den Berg, M. P.; van Tintelen, J. P.; Ruiter, J.S.; de Jonge, N.

    2010-01-01

    Background. About 30% of dilated cardiomyopathy (DCM) cases are familial. Mutations are mostly found in the genes encoding lanain A/C, beta-myosin heavy chain and the sarcomeric protein cardiac troponin-T (TNNT2). Mutations in TNNT2 are reported in approximately 3% of DCM patients. The overall pheno

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

    Science.gov (United States)

    Ponniah, U; Overholt, E

    2014-01-01

    We report 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.

  1. 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.

  2. Dilated cardiomyopathy and hypothyroidism associated with pegylated interferon and ribavirin treatment for chronic hepatitis C: case report and literature review

    Directory of Open Access Journals (Sweden)

    Wenxue Zhao

    2014-01-01

    Full Text Available Pegylated interferon alpha (Peg IFN-α in combination with ribavirin is the backbone of treatment in chronic hepatitis C (CHC. Cardiotoxicity due to interferon therapy is rare. The most frequent cardiovascular complications are arrhythmias and ischemic manifestations. Cardiomyopathy is extremely rare but can be life threatening. We present the case of a 41-year-old female patient with CHC in whom Peg IFN-α induced dilated cardiomyopathy and hypothyroidism. Chest radiography showed an enlarged and globular cardiac silhouette and pulmonary congestion. Echocardiography showed decreased left ventricular systolic function with an ejection fraction of 32% and fractional shortening of 15%. Cardiomyopathy had a complete remission after cessation of antiviral therapy with short-term heart failure medications and supportive care. Then we review the current literature about interferon induced cardiomyopathy in patients with HCV infection, as well as share our clinical experience in diagnosing and managing this rare complication.

  3. Persistent scarring and dilated cardiomyopathy suggest incomplete regeneration of the apex resected neonatal mouse myocardium

    DEFF Research Database (Denmark)

    Andersen, Ditte Caroline; Jensen, Charlotte Harken; Baun, Christina

    2016-01-01

    Heart damage in mammals is generally considered to result in scar formation, whereas zebrafish completely regenerate their hearts following an intermediate and reversible state of fibrosis after apex resection (AR). Recently, using the AR procedure, one-day-old mice were suggested to have full...... capacity for cardiac regeneration as well. In contrast, using the same mouse model others have shown that the regeneration process is incomplete and that scarring still remains 21days after AR. The present study tested the hypothesis that like in zebrafish, fibrosis in neonatal mammals could...... resection in mice results in irreversible fibrosis and dilated cardiomyopathy suggesting that cardiac regeneration is limited in neonatal mammals and thus distinct from the regenerative capacity seen in zebrafish....

  4. Anaesthetic management of a patient with dilated cardiomyopathy posted for non-cardiac surgery

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    Madhusudan M

    2016-07-01

    Full Text Available Dilated cardiomyopathy (DCM is characterized by impaired ventricular contractility and causes concern in anaesthetic management as it may sometimes predispose to malignant arrhythmias. A 77-year-old woman diagnosed to have irreducible umbilical hernia, was posted for emergency laparotomy and hernia repair. On examination, she belonged to American Society of Anesthesiologists (ASA physical status grade III with a functional status of 4 metabolic equivalents (METs. She was also suffering from DCM with severe left ventricular (LV dysfunction (LV ejection fraction 25%. This patient was successfully managed by administering general anaesthesia with ProSeal laryngeal mask airway. We report the detailed anaesthetic management of this patient who underwent emergency laparotomy and hernia repair.

  5. RESIDENT PROGENITOR CARDIAC CELLS IN PATIENTS WITH DILATED CARDIOMYOPATHY AND CONGESTIVE HEART FAILURE

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    T. G. Kulikova

    2014-01-01

    Full Text Available Aim. To study content of resident progenitor cardiomyocytes in endomyocardial biopsy samples of patients with dilated cardiomyopathy (DCM and heart failure (HF at different disease stages and relate it to patient clinical characteristics.Material and methods. Resident progenitor cardiomyocytes were studied in endomyocardial biopsy samples from 14 patients (age from 26 to 52 years old with DCM and HF by immunofluorescence method. Results were analyzed individually for each patient.Results. Resident progenitor cardiomyocytes expressing simultaneously stem cell markers c-kit, MDR-1 and early cardiomyocyte differentiation markers GATA-4 and Nkx2.5 were found in endomyocardial biopsy samples from patients with DCM and HF. Resident progenitor cardiomyocytes detected by these cell markers were found in all patients at all disease stages.Conclusion. Results show that the myocardial regenerative processes exist at all stages of the disease progression.

  6. RESIDENT PROGENITOR CARDIAC CELLS IN PATIENTS WITH DILATED CARDIOMYOPATHY AND CONGESTIVE HEART FAILURE

    Directory of Open Access Journals (Sweden)

    T. G. Kulikova

    2015-09-01

    Full Text Available Aim. To study content of resident progenitor cardiomyocytes in endomyocardial biopsy samples of patients with dilated cardiomyopathy (DCM and heart failure (HF at different disease stages and relate it to patient clinical characteristics.Material and methods. Resident progenitor cardiomyocytes were studied in endomyocardial biopsy samples from 14 patients (age from 26 to 52 years old with DCM and HF by immunofluorescence method. Results were analyzed individually for each patient.Results. Resident progenitor cardiomyocytes expressing simultaneously stem cell markers c-kit, MDR-1 and early cardiomyocyte differentiation markers GATA-4 and Nkx2.5 were found in endomyocardial biopsy samples from patients with DCM and HF. Resident progenitor cardiomyocytes detected by these cell markers were found in all patients at all disease stages.Conclusion. Results show that the myocardial regenerative processes exist at all stages of the disease progression.

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

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    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.

  8. The protective effects of ivabradine in preventing progression from viral myocarditis to dilated cardiomyopathy

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    Li Yue-Chun

    2016-11-01

    Full Text Available To study the beneficial effects of ivabradine in dilated cardiomyopathy mice, which evolved from coxsackievirus B3-induced chronic viral myocarditis. Four-to-five-week-old male balb/c mice were inoculated intraperitoneally with coxsackievirus B3 (Strain Nancy on day 1, day 14 and day 28. The day of the first virus inoculation was defined as day 1. Thirty-five days later, the surviving chronic viral myocarditis mice were divided randomly into two groups, a treatment group and an untreated group. Ivabradine was administered by gavage for 30 consecutive days in the treatment group, and the untreated group was administered normal saline. Masson’s trichrome stain was used to evaluate the fibrosis degree in myocardial tissue. The expression levels of tumor necrosis factor-α (TNF-α, interleukin-1β (IL-1β, interleukin-6 (IL-6, Collagen I, Collagen III and p38-MAPK signaling pathway proteins were detected by western blot. Electrocardiogram was used to investigate the heart rate and rhythm. The thickness of the ventricular septum and left ventricular posterior wall, left ventricular end diastolic dimension, left ventricular end systolic dimension, left ventricular ejection fractions and fractional shortening were studied by echocardiography. Compared with the untreated chronic viral myocarditis mice, ivabradine significantly increased the survival rate, attenuated the myocardial lesions and fibrosis, improved the impairment of the left ventricular function, diminished the heart dimension, decreased the production of collagen I and collagen III, reduced the expression of the proinflammatory cytokines TNF-α, IL-1β, and IL-6, and lowered the production of phospho-p38MAPK. The findings indicate the therapeutic effect of ivabradine in preventing the progression from viral myocarditis to dilated cardiomyopathy in mice with chronic viral myocarditis induced by coxsackievirus B3, is associated with inhibition of the p38MAPK pathway, downregulated

  9. 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.

  10. 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.

  11. Mutations in TAX1BP3 cause dilated cardiomyopathy with septo-optic dysplasia.

    Science.gov (United States)

    Reinstein, Eyal; Orvin, Katia; Tayeb-Fligelman, Einav; Stiebel-Kalish, Hadas; Tzur, Shay; Pimienta, Allen L; Bazak, Lily; Bengal, Tuvia; Cohen, Lior; Gaton, Dan D; Bormans, Concetta; Landau, Meytal; Kornowski, Ran; Shohat, Mordechai; Behar, Doron M

    2015-04-01

    We describe a Bedouin family with a novel autosomal recessive syndrome characterized by dilated cardiomyopathy and septo-optic dysplasia. Genetic analysis revealed a homozygous missense mutation in TAX1BP3, which encodes a small PDZ domain containing protein implicated in regulation of the Wnt/β-catenin signaling pathway, as the causative mutation. The mutation affects a conserved residue located at the core of TAX1BP3 binding pocket and is predicted to impair the nature of a crucial hydrophobic patch, thereby interrupting the structure and stability of the protein, and its ability to interact with other proteins. TAX1BP3 is highly expressed in heart and brain and consistent with the clinical findings observed in our patients; a knockdown of TAX1BP3 causes elongation defects, enlarged pericard, and enlarged head structures in zebrafish embryos. Thus, we describe a new genetic disorder that expands the monogenic cardiomyopathy disease spectrum and suggests that TAX1BP3 is essential for heart and brain development.

  12. Arginylation regulates myofibrils to maintain heart function and prevent dilated cardiomyopathy

    Science.gov (United States)

    Kurosaka, Satoshi; Leu, N. Adrian; Pavlov, Ivan; Han, Xuemei; Ribeiro, Paula Aver Bretanha; Xu, Tao; Bunte, Ralph; Saha, Sougata; Wang, Junling; Cornachione, Anabelle; Mai, Wilfried; Yates, John R; Rassier, Dilson E.; Kashina, Anna

    2012-01-01

    Protein arginylation mediated by arginyltransferase (ATE1) is essential for heart formation during embryogenesis, however its cell-autonomous role in cardiomyocytes and the differentiated heart muscle has never been investigated. To address this question, we generated cardiac muscle-specific Ate1 knockout mice, in which Ate1 deletion was driven by α-myosin heavy chain promoter (αMHC-Ate1 mouse). These mice were initially viable, but developed severe cardiac contractility defects, dilated cardiomyopathy, and thrombosis over time, resulting in high rates of lethality after 6 months of age. These symptoms were accompanied by severe ultrastructural defects in cardiac myofibrils, seen in the newborns and far preceding the onset of cardiomyopathy, suggesting that these defects were primary and likely underlay the development of the future heart defects. Several major sarcomeric proteins were arginylated in vivo. Moreover, Ate1 deletion in the hearts resulted in a significant reduction of active and passive myofibril forces, suggesting that arginylation is critical for both myofibril structural integrity and contractility. Thus, arginylation is essential for maintaining the heart function by regulation of the major myofibril proteins and myofibril forces, and its absence in the heart muscle leads to progressive heart failure through cardiomyocyte-specific defects. PMID:22626847

  13. A Heterozygous ZMPSTE24 Mutation Associated with Severe Metabolic Syndrome, Ectopic Fat Accumulation, and Dilated Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Damien Galant

    2016-04-01

    Full Text Available ZMPSTE24 encodes the only metalloprotease, which transforms prelamin into mature lamin A. Up to now, mutations in ZMPSTE24 have been linked to Restrictive Dermopathy (RD, Progeria or Mandibulo-Acral Dysplasia (MAD. We report here the phenotype of a patient referred for severe metabolic syndrome and cardiomyopathy, carrying a mutation in ZMPSTE24. The patient presented with a partial lipodystrophic syndrome associating hypertriglyceridemia, early onset type 2 diabetes, and android obesity with truncal and abdominal fat accumulation but without subcutaneous lipoatrophy. Other clinical features included acanthosis nigricans, liver steatosis, dilated cardiomyopathy, and high myocardial and hepatic triglycerides content. Mutated fibroblasts from the patient showed increased nuclear shape abnormalities and premature senescence as demonstrated by a decreased Population Doubling Level, an increased beta-galactosidase activity and a decreased BrdU incorporation rate. Reduced prelamin A expression by siRNA targeted toward LMNA transcripts resulted in decreased nuclear anomalies. We show here that a central obesity without subcutaneous lipoatrophy is associated with a laminopathy due to a heterozygous missense mutation in ZMPSTE24. Given the high prevalence of metabolic syndrome and android obesity in the general population, and in the absence of familial study, the causative link between mutation and phenotype cannot be formally established. Nevertheless, altered lamina architecture observed in mutated fibroblasts are responsible for premature cellular senescence and could contribute to the phenotype observed in this patient.

  14. 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

  15. 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.

  16. Monocyte Chemotactic Protein-1 Promotes the Myocardial Homing of Mesenchymal Stem Cells in Dilated Cardiomyopathy

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    Yunzeng Zou

    2013-04-01

    Full Text Available Dilated cardiomyopathy (DCM is the most common form of non-ischemic cardiomyopathy that leads to heart failure. Mesenchymal stem cells (MSCs are under active investigation currently as a potential therapy for DCM. However, little information is available about the therapeutic potential of intravenous administration of MSCs for DCM. Moreover, how MSCs home to the myocardium in DCM is also unclear. DCM was induced by intraperitoneally administering Doxorubicin and MSCs or vehicles were infused through the internal jugular vein. Cardiac functions including the percentage of fractional shortening, left ventricular diastolic dimension, left ventricular end-diastolic pressure, and left ventricular maximum dp/dt were evaluated by echocardiographic and hemodynamic studies. Fibrosis was determined by Masson’s trichrome staining. The mRNA expression levels of monocyte chemotactic protein-1 (MCP-1, stromal cell-derived factor-1 (SDF-1, macrophage inflammatory protein-1α (MIP-1α, and monocyte chemotactic protein-3 (MCP-3 were determined using real time polymerase chain reactions and the protein expression level of MCP-1 was detected with Western blot. The MSCs expression of C-C chemokine receptor type 2 (CCR2, a MCP-1 receptor, was confirmed by Western blot and flow cytometry analysis. The chemotactic effects of MCP-1/CCR2 were checked by assessing the migration in vitro and in vivo. MSCs transplantation improved the cardiac function and decreased the myocardial fibrosis of mice with DCM. MCP-1 was up-regulated in dilated myocardial tissue both at the mRNA and protein level while SDF-1, MIP-1α and MCP-3 remain unchanged. CCR2 was present in MSCs. MCP-1 promoted MSCs migration in vitro while CCR2 inhibition decreased the migration of MCP-1 to the dilated heart. This study provides direct evidences that peripheral intravenous infusion of MSCs can support the functional recovery of DCM. In addition, novel insights into the myocardial homing factor of MSCs

  17. 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.

  18. Different effects of bisoprolol on heart rate in patients with ischemic or idiopathic dilated cardiomyopathy (A 24-hour Holter substudy of the Cardiac Insufficiency Bisoprolol Study [CIBIS])

    NARCIS (Netherlands)

    Anthonio, RL; Brouwer, J; Lechat, P; Haaksma, J; van der Ven, L; van Veldhuisen, DJ; Crijns, HJGM; van Gilst, WH

    1999-01-01

    The effect of beta blockade on heart rate in patients with either idiopathic or ischemic cardiomyopathy was studied. It was found that beta blockade reduced the early morning increase in heart rate to a greater extent in patients with idiopathic dilated cardiomyopathy than in those with ischemic dil

  19. 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.

  20. Modulation of serotonin transporter function during fetal development causes dilated heart cardiomyopathy and lifelong behavioral abnormalities.

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    Cornelle W Noorlander

    Full Text Available BACKGROUND: Women are at great risk for mood and anxiety disorders during their childbearing years and may become pregnant while taking antidepressant drugs. In the treatment of depression and anxiety disorders, selective serotonin reuptake inhibitors (SSRIs are the most frequently prescribed drugs, while it is largely unknown whether this medication affects the development of the central nervous system of the fetus. The possible effects are the product of placental transfer efficiency, time of administration and dose of the respective SSRI. METHODOLOGY/PRINCIPAL FINDINGS: In order to attain this information we have setup a study in which these parameters were measured and the consequences in terms of physiology and behavior are mapped. The placental transfer of fluoxetine and fluvoxamine, two commonly used SSRIs, was similar between mouse and human, indicating that the fetal exposure of these SSRIs in mice is comparable with the human situation. Fluvoxamine displayed a relatively low placental transfer, while fluoxetine showed a relatively high placental transfer. Using clinical doses of fluoxetine the mortality of the offspring increased dramatically, whereas the mortality was unaffected after fluvoxamine exposure. The majority of the fluoxetine-exposed offspring died postnatally of severe heart failure caused by dilated cardiomyopathy. Molecular analysis of fluoxetine-exposed offspring showed long-term alterations in serotonin transporter levels in the raphe nucleus. Furthermore, prenatal fluoxetine exposure resulted in depressive- and anxiety-related behavior in adult mice. In contrast, fluvoxamine-exposed mice did not show alterations in behavior and serotonin transporter levels. Decreasing the dose of fluoxetine resulted in higher survival rates and less dramatic effects on the long-term behavior in the offspring. CONCLUSIONS: These results indicate that prenatal fluoxetine exposure affects fetal development, resulting in cardiomyopathy

  1. Cardiac-specific deletion of the microtubule-binding protein CENP-F causes dilated cardiomyopathy

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    Ellen Dees

    2012-07-01

    CENP-F is a large multifunctional protein with demonstrated regulatory roles in cell proliferation, vesicular transport and cell shape through its association with the microtubule (MT network. Until now, analysis of CENP-F has been limited to in vitro analysis. Here, using a Cre-loxP system, we report the in vivo disruption of CENP-F gene function in murine cardiomyocytes, a cell type displaying high levels of CENP-F expression. Loss of CENP-F function in developing myocytes leads to decreased cell division, blunting of trabeculation and an initially smaller, thin-walled heart. Still, embryos are born at predicted mendelian ratios on an outbred background. After birth, hearts lacking CENP-F display disruption of their intercalated discs and loss of MT integrity particularly at the costamere; these two structures are essential for cell coupling/electrical conduction and force transduction in the heart. Inhibition of myocyte proliferation and cell coupling as well as loss of MT maintenance is consistent with previous reports of generalized CENP-F function in isolated cells. One hundred percent of these animals develop progressive dilated cardiomyopathy with heart block and scarring, and there is a 20% mortality rate. Importantly, although it has long been postulated that the MT cytoskeleton plays a role in the development of heart disease, this study is the first to reveal a direct genetic link between disruption of this network and cardiomyopathy. Finally, this study has broad implications for development and disease because CENP-F loss of function affects a diverse array of cell-type-specific activities in other organs.

  2. Role of coxsackievirus and adenovirus receptor in the pathogenesis of dilated cardiomyopathy and its influencing factor

    Institute of Scientific and Technical Information of China (English)

    ZHANG Shuo; JIA Hai-bo; GONG Bin-sheng; ZHANG Shao-jun; LI Xia; YU Bo

    2008-01-01

    Background Although clinical treatment for heart failure and sudden death has been improved over the last few decades, the morbidity and mortality of dilated cardiomyopathy (DCM) have increased. So a better understanding of the underlying molecular events leading to DCM is urgent. Persistent viral infection (especially coxsackievirus group B) of the myocardium in viral myocarditis and DCM has never been neglected by experts. Recent data indicate that the up-regulation of coxsackievirus and adenovirus receptor (CAR) in viral cardiomyopathy contributes to viral infection as a key factor in the pathogenesis of this disease. This study aimed to investigate the role and regulatory mechanism of CAR in DCM by the bioinformatic method.Methods We identified the clusters of genes co-expressed with CAR by clustering algorithm based on the public available microarray dataset of DCM (Kittleson, et al. 2005), and mapped these genes into the protein-protein interaction networks to investigate the interaction relationship to each other at the protein level after confirming that the samples are characterized by the cluster of genes in correctly partitioning.Results The gene cluster GENESET 11 containing 33 genes including CAR with similar expression pattem was identified by cluster algorithm, of which 19 genes were found to have interaction information of the protein encoded by them in the current human protein interaction database. Especially, 12 genes present as critical nodes (called HUB node) at the protein level are involved in energy metabolism, signal transduction, viral infection, immuno-response, cell apoptosis, cell proliferation, tissue repair, etc.Conclusions The genes in GENESET 11 together with CAR may play a pathogenic role in the development of DCM, mainly involved in the mechanism of energy metabolism, signal transduction, viral infection, immuno-response, cell apoptosis and tissue repair.

  3. 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

  4. Different microcirculatory and interstitial matrix patterns in idiopathic dilated cardiomyopathy and Chagas' disease: a three dimensional confocal microscopy study

    OpenAIRE

    Higuchi, M.; Fukasawa, S; De Brito, T.; Parzianello, L; G. Bellotti; Ramires, J

    1999-01-01

    OBJECTIVE—To analyse the morphological aspects of the extracellular matrix and microcirculation to clarify whether chronic Chagas' cardiopathy (CCC) is an accurate model to study the pathogenesis of idiopathic dilated cardiomyopathy (IDCM).
DESIGN—Thick histological myocardial sections were prepared to analyse collagen, and microcirculation was examined during confocal laser and light microscopy.
SETTING—The specimens were prepared at the pathology service of the Heart Institute of São Paulo,...

  5. 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

  6. Anaesthetic management of laparoscopic surgery for rectal cancer in patients of dilated cardiomyopathy with poor ejection fraction: a case report

    Science.gov (United States)

    Wu, Yao-Hua; Hu, Liang; Xia, Jin; Hao, Quan-Shui; Feng, Li; Xiang, Hong-Bing

    2015-01-01

    A patient with dilated cardiomyopathy with poor ejection fraction posted for laparoscopic surgery for rectal cancer which was successfully performed under general anesthesia with endotracheal intubation and mechanical ventilation was reported. Our observations strongly indicate that detailed preoperative assessment, watchful intraoperative monitoring, and skillful optimization of fluid status and hemodynamic play important role in the high risk patient under general anesthesia with endotracheal intubation and mechanical ventilation. PMID:26309623

  7. 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.

  8. Serum lipidomics meets cardiac magnetic resonance imaging: profiling of subjects at risk of dilated cardiomyopathy.

    Science.gov (United States)

    Sysi-Aho, Marko; Koikkalainen, Juha; Seppänen-Laakso, Tuulikki; Kaartinen, Maija; Kuusisto, Johanna; Peuhkurinen, Keijo; Kärkkäinen, Satu; Antila, Margareta; Lauerma, Kirsi; Reissell, Eeva; Jurkko, Raija; Lötjönen, Jyrki; Heliö, Tiina; Orešič, Matej

    2011-01-20

    Dilated cardiomyopathy (DCM), characterized by left ventricular dilatation and systolic dysfunction, constitutes a significant cause for heart failure, sudden cardiac death or need for heart transplantation. Lamin A/C gene (LMNA) on chromosome 1p12 is the most significant disease gene causing DCM and has been reported to cause 7-9% of DCM leading to cardiac transplantation. We have previously performed cardiac magnetic resonance imaging (MRI) to LMNA carriers to describe the early phenotype. Clinically, early recognition of subjects at risk of developing DCM would be important but is often difficult. Thus we have earlier used the MRI findings of these LMNA carriers for creating a model by which LMNA carriers could be identified from the controls at an asymptomatic stage. Some LMNA mutations may cause lipodystrophy. To characterize possible effects of LMNA mutations on lipid profile, we set out to apply global serum lipidomics using Ultra Performance Liquid Chromatography coupled to mass spectrometry in the same LMNA carriers, DCM patients without LMNA mutation and controls. All DCM patients, with or without LMNA mutation, differed from controls in regard to distinct serum lipidomic profile dominated by diminished odd-chain triglycerides and lipid ratios related to desaturation. Furthermore, we introduce a novel approach to identify associations between the molecular lipids from serum and the MR images from the LMNA carriers. The association analysis using dependency network and regression approaches also helped us to obtain novel insights into how the affected lipids might relate to cardiac shape and volume changes. Our study provides a framework for linking serum derived molecular markers not only with clinical endpoints, but also with the more subtle intermediate phenotypes, as derived from medical imaging, of potential pathophysiological relevance.

  9. Serum lipidomics meets cardiac magnetic resonance imaging: profiling of subjects at risk of dilated cardiomyopathy.

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    Marko Sysi-Aho

    Full Text Available Dilated cardiomyopathy (DCM, characterized by left ventricular dilatation and systolic dysfunction, constitutes a significant cause for heart failure, sudden cardiac death or need for heart transplantation. Lamin A/C gene (LMNA on chromosome 1p12 is the most significant disease gene causing DCM and has been reported to cause 7-9% of DCM leading to cardiac transplantation. We have previously performed cardiac magnetic resonance imaging (MRI to LMNA carriers to describe the early phenotype. Clinically, early recognition of subjects at risk of developing DCM would be important but is often difficult. Thus we have earlier used the MRI findings of these LMNA carriers for creating a model by which LMNA carriers could be identified from the controls at an asymptomatic stage. Some LMNA mutations may cause lipodystrophy. To characterize possible effects of LMNA mutations on lipid profile, we set out to apply global serum lipidomics using Ultra Performance Liquid Chromatography coupled to mass spectrometry in the same LMNA carriers, DCM patients without LMNA mutation and controls. All DCM patients, with or without LMNA mutation, differed from controls in regard to distinct serum lipidomic profile dominated by diminished odd-chain triglycerides and lipid ratios related to desaturation. Furthermore, we introduce a novel approach to identify associations between the molecular lipids from serum and the MR images from the LMNA carriers. The association analysis using dependency network and regression approaches also helped us to obtain novel insights into how the affected lipids might relate to cardiac shape and volume changes. Our study provides a framework for linking serum derived molecular markers not only with clinical endpoints, but also with the more subtle intermediate phenotypes, as derived from medical imaging, of potential pathophysiological relevance.

  10. QT Dispersion Level and Its Clinical Significance.in Dilated Cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    2000-01-01

    Objective To evaluate the clinical significance of QT dis persion (QTd, QTcd) in dilated cardiomyopathy (DCM). Methods QTd and QTcd were measured on simultaneously recording 12-lead electrocardiograms ( ECGs) in 60 DCM patients and cormpared with 60 healthy subjects. Results The values of QTd and QTcd in DCM were significantly higher than those in control group ( P < 0. 01 ). With subgroup analysis, QTd and QTcd in patients uith cardiac sudden death (CSD) were longer than those in survivors and those died of progressive heart failure ( P < 0. 05), patients with ventricular tachycardia (VT) or with severe heart failure than those without (compared uith patients with ventricular premature beats [V PB], P<0.05, compared with patients without ventricular arrhythmia [VA], P<0. 01) or with mild heart failure (P<0. 01). The values of QTd and QTcd in patients with VPB were greater than those in patients without VA( P< 0. 05). There were significant differences in the rates of VT, CSD and heart failure between the groups of QTd> 110 ms and QTd≤110 ms(P<0. 01 or P<0.05), in contrast to ejection fraction (EF) and fractional shortening (FS)( P>0.05). Conclusion The values of QTd and QTcd increased in DCM patients were susceptive index for monitoring maligant VA in DCM, also important prognostic markers of CSD. QTd was correlated with NY HA functional class but not with EF and FS.

  11. Candidate gene expression analysis of toxin-induced dilated cardiomyopathy in the turkey (Meleagris gallopavo).

    Science.gov (United States)

    Lin, K-C; Gyenai, K; Pyle, R L; Geng, T; Xu, J; Smith, E J

    2006-12-01

    Dilated cardiomyopathy (DCM), a heart disease, affects many vertebrates including humans and poultry. The disease can be either idiopathic (IDCM) or toxin-induced (TIDCM). Although genetic and other studies of IDCM are extensive, the specific etiology of TIDCM is still unknown. In this study, we compared mRNA levels of cardiac troponin T (cTnT) and phospholamban (PLN) in turkeys affected and unaffected by TIDCM. Cardiac TnT and PLN were chosen because their altered expression has been observed in IDCM-affected birds. A total of 72 birds, 44 affected and 28 unaffected with TIDCM, were used. Differences in the mRNA levels of cTnT and PLN between affected and unaffected turkeys were significant only for cTnT. The sequence of the turkey PLN showed significant similarity at the nucleotide level to the reference chicken sequence and to those of other species. In addition to implicating cTnT in TIDCM, the present work describes a partial turkey PLN coding sequence that could be useful for future studies.

  12. Preliminary Survey on the Prevalence Rate of Hypertension in Patients with Dilated Cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    2008-01-01

    To investigate the prevalence of hypertension and its primary risk factors in patients with dilated cardiomyopathy (DCM). Methods Three hundred and sixty-two patients with DCM (DCM group)and 401 age-matched residents (control group) were enrolled randomly in the study, the hypertensive prevalence rate were calculated respectively in the two groups and were compared with each other; the patients in the DCM group were divided into two subgroups (hypertension subgroup and non-hypertension subgroup) according to whether the patients have hypertension;the clinical data related to blood pressure was compared between the two subgroups. Results The prevalence of hypertension in DCM group was significantly higher than that in the control group ( 32. 8% vs. 20. 1%, P< 0.01 ) ; There were no significant differences on the age, gender, occupation and left ventricular ejection fraction (LVEF) between the two subgroups, but the mean heart rate and the percentage of patients who had family history of hypertension were significantly higher in the hypertension subgroup than that in the non-hypertension subgroup ( P<0.05 and P<0. 01 ).Conclusions The prevalence of hypertension in patients with DCM was high; The increased activity of sympathetic nervous system and the hypertensive genetic factor may be the main risk factors of hypertension in patients with DCM.

  13. Induction of Ankrd1 in Dilated Cardiomyopathy Correlates with the Heart Failure Progression

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    Julius Bogomolovas

    2015-01-01

    Full Text Available Progression of idiopathic dilated cardiomyopathy (IDCM is marked with extensive left ventricular remodeling whose clinical manifestations and molecular basis are poorly understood. We aimed to evaluate the clinical potential of titin ligands in monitoring progression of cardiac remodeling associated with end-stage IDCM. Expression patterns of 8 mechanoptotic machinery-associated titin ligands (ANKRD1, ANKRD2, TRIM63, TRIM55, NBR1, MLP, FHL2, and TCAP were quantitated in endomyocardial biopsies from 25 patients with advanced IDCM. When comparing NYHA disease stages, elevated ANKRD1 expression levels marked transition from NYHA < IV to NYHA IV. ANKRD1 expression levels closely correlated with systolic strain depression and short E wave deceleration time, as determined by echocardiography. On molecular level, myocardial ANKRD1 and serum adiponectin correlated with low BAX/BCL-2 ratios, indicative of antiapoptotic tissue propensity observed during the worsening of heart failure. ANKRD1 is a potential marker for cardiac remodeling and disease progression in IDCM. ANKRD1 expression correlated with reduced cardiac contractility and compliance. The association of ANKRD1 with antiapoptotic response suggests its role as myocyte survival factor during late stage heart disease, warranting further studies on ANKRD1 during end-stage heart failure.

  14. Functional Class in Children with Idiopathic Dilated Cardiomyopathy. A pilot Study

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    Aline Cristina Tavares

    2016-01-01

    Full Text Available Abstract Background: Idiopathic dilated cardiomyopathy (IDCM, most common cardiac cause of pediatric deaths, mortality descriptor: a low left ventricular ejection fraction (LVEF and low functional capacity (FC. FC is never self reported by children. Objective: The aims of this study were (i To evaluate whether functional classifications according to the children, parents and medical staff were associated. (iv To evaluate whether there was correlation between VO2 max and Weber's classification. Method: Prepubertal children with IDCM and HF (by previous IDCM and preserved LVEF were selected, evaluated and compared. All children were assessed by testing, CPET and functional class classification. Results: Chi-square test showed association between a CFm and CFp (1, n = 31 = 20.6; p = 0.002. There was no significant association between CFp and CFc (1, n = 31 = 6.7; p = 0.4. CFm and CFc were not associated as well (1, n = 31 = 1.7; p = 0.8. Weber's classification was associated to CFm (1, n = 19 = 11.8; p = 0.003, to CFp (1, n = 19 = 20.4; p = 0.0001and CFc (1, n = 19 = 6.4; p = 0.04. Conclusion: Drawing were helpful for children's self NYHA classification, which were associated to Weber's stratification.

  15. Traditional Chinese Medicine Tongxinluo Improves Cardiac Function of Rats with Dilated Cardiomyopathy

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    Fang-Fang Shen

    2014-01-01

    Full Text Available The study aimed at testing the hypothesis that tongxinluo capsule might exert its cardioprotective effect by preventing ventricular remodeling and improving coronary microvascular function in a rat model of doxorubicin-induced dilated cardiomyopathy (DCM. Rats that survived DCM induction were randomly divided into three groups to be given 1.5 g·kg−1·day−1 (TXL-H, n=9 or 0.15 g·kg−1·day−1 (TXL-L, n=10 of tongxinluo, or normal saline at the same volume (DCM-C, n=10 intragastrically. Age matched normal rats treated with normal saline were used as normal controls (NOR-C, n=9. After four weeks of treatment, the DCM-C, TXL-H, and TXL-L groups exhibited significant cardiac dysfunction, left ventricular remodeling, and coronary microvascular dysfunction, compared with the NOR-C rats. However, myocardial functional parameters were significantly improved and microvascular density (MVD increased in the TXL-H group compared with the DCM-C group (all P<0.01. Left ventricular remodeling was prevented. There were close linear relationships between CVF and LVEF (r=-0.683, P<0.05, MVD and LVEF (r=0.895, P<0.05, and MVD and CVF (r=-0.798, P<0.05. It was indicated that high-dose tongxinluo effectively improved cardiac function in rat model of DCM.

  16. Effect of arotinolol on right ventricular function in patients with dilated cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    Objective Dilated cardiomyopathy (DCM) is generally considered to be accompanied by both left and right ventricular dysfunction,but most studies only analyze the left ventricular function. In this study, we evaluated the effect of arotinolol on right ventricular function in patients with DCM. Methods Right ventricular ejection fraction (RVEF) and right ventricular diameter (RVD) were measured by two-dimensional echocardiography (2-DE) in 33 DCM patients; RVEF measured by first-pass radionuclide angiography (FPRA) was compared with that by 2-DE. Results The treatment with arotinolol for one year resulted in a reduction in the right ventricular diameter (baseline, 23.0 ± 8.3 mm vs after one-year treatment, 20.7 ± 5.4 mm; P=0.004 ) and an associated increase in ejection fraction (baseline, 36.9 ± 10.3% vs after one-year treatment, 45.8 ± 9.6%; P < 0.001 ); there is a high correlation between the 2-DE method and radionuclide ventriculographic method. The correlation coefficient is 0.933 (P<0.001). Conclusion Arotinolol therapy could not only improve left ventricular function, but also improve right ventricular function in DCM patients.

  17. EFFECT OF AROTINOLOL ON LEFT VENTRICULAR FUNCTION IN PATIENTS WITH IDIOPATHIC DILATED CARDIOMYOPATHY

    Institute of Scientific and Technical Information of China (English)

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

    2007-01-01

    To evaluate the efficacy and safety of long-term treatment with arotinolol in patients with idiopathic dilated cardiomyopathy (IDCM).Methods 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 β-blocker. Left ventricular function was assessed with the New York Heart Association functional class and two-dimensional echocardiography.Results 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 <0.001). Left ventricular ejection fraction significantly increased from 27.39% ±7.94% to 41.13% ±9.45% (P <0.001). Left ventricular mass index decreased from 150. 47 ± 42. 42 g/m2 to 141.58 ± 34.36 g/m2 ( P<0.01). No adverse events leading to premature discontinuation of study drug occurred.Conclusion In this preliminary study, 12-month arotinolol treatment has a favorable effect on left ventricular function in patients with IDCM, and it is safe and well tolerated.

  18. 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.

  19. Global phosphoproteomic profiling reveals perturbed signaling in a mouse model of dilated cardiomyopathy

    Science.gov (United States)

    Kuzmanov, Uros; Guo, Hongbo; Buchsbaum, Diana; Cosme, Jake; Abbasi, Cynthia; Isserlin, Ruth; Sharma, Parveen; Gramolini, Anthony O.; Emili, Andrew

    2016-01-01

    Phospholamban (PLN) plays a central role in Ca2+ homeostasis in cardiac myocytes through regulation of the sarco(endo)plasmic reticulum Ca2+-ATPase 2A (SERCA2A) Ca2+ pump. An inherited mutation converting arginine residue 9 in PLN to cysteine (R9C) results in dilated cardiomyopathy (DCM) in humans and transgenic mice, but the downstream signaling defects leading to decompensation and heart failure are poorly understood. Here we used precision mass spectrometry to study the global phosphorylation dynamics of 1,887 cardiac phosphoproteins in early affected heart tissue in a transgenic R9C mouse model of DCM compared with wild-type littermates. Dysregulated phosphorylation sites were quantified after affinity capture and identification of 3,908 phosphopeptides from fractionated whole-heart homogenates. Global statistical enrichment analysis of the differential phosphoprotein patterns revealed selective perturbation of signaling pathways regulating cardiovascular activity in early stages of DCM. Strikingly, dysregulated signaling through the Notch-1 receptor, recently linked to cardiomyogenesis and embryonic cardiac stem cell development and differentiation but never directly implicated in DCM before, was a prominently perturbed pathway. We verified alterations in Notch-1 downstream components in early symptomatic R9C transgenic mouse cardiomyocytes compared with wild type by immunoblot analysis and confocal immunofluorescence microscopy. These data reveal unexpected connections between stress-regulated cell signaling networks, specific protein kinases, and downstream effectors essential for proper cardiac function. PMID:27742792

  20. Abnormal Glucose Tolerance Is Associated with a Reduced Myocardial Metabolic Flexibility in Patients with Dilated Cardiomyopathy.

    Science.gov (United States)

    Tricò, Domenico; Baldi, Simona; Frascerra, Silvia; Venturi, Elena; Marraccini, Paolo; Neglia, Danilo; Natali, Andrea

    2016-01-01

    Dilated cardiomyopathy (DCM) is characterized by a metabolic shift from fat to carbohydrates and failure to increase myocardial glucose uptake in response to workload increments. We verified whether this pattern is influenced by an abnormal glucose tolerance (AGT). In 10 patients with DCM, 5 with normal glucose tolerance (DCM-NGT) and 5 with AGT (DCM-AGT), and 5 non-DCM subjects with AGT (N-AGT), we measured coronary blood flow and arteriovenous differences of oxygen and metabolites during Rest, Pacing (at 130 b/min), and Recovery. Myocardial lactate exchange and oleate oxidation were also measured. At Rest, DCM patients showed a reduced nonesterified fatty acids (NEFA) myocardial uptake, while glucose utilization increased only in DCM-AGT. In response to Pacing, glucose uptake promptly rose in N-AGT (from 72 ± 21 to 234 ± 73 nmol/min/g, p equivalents, p metabolism and the reduced myocardial metabolic flexibility in response to an increased workload. This trial is registered with ClinicalTrial.gov NCT02440217.

  1. Timing of left heart base descent in dogs with dilated cardiomyopathy and normal dogs.

    Science.gov (United States)

    Simpson, Kerry E; Devine, Bryan C; Woolley, Richard; Corcoran, Brendan M; French, Anne T

    2008-01-01

    The identification and assessment of myocardial failure in canine idiopathic dilated cardiomyopathy (DCM) is achieved using a variety of two-dimensional and Doppler echocardiographic techniques. More recently, the availability of tissue Doppler imaging (TDI) has raised the potential for development of new ways of more accurately identifying a disease phenotype. Nevertheless, TDI has not been universally adapted to veterinary clinical cardiology primarily because of the lack of information on its utility in diagnosis. We assessed the application of timing of left heart base descent using TDI in the identification of differences between DCM and normal dogs. The times from the onset of the QRS complex on a simultaneously recorded electrocardiograph to the onset (Q--S'), peak (Q--peak S'), and end (Q--end S') of the systolic velocity peak were measured in the interventricular septum (IVS) and the left ventricular free wall. The duration of S' was also calculated. The Q--S' (FW), Q--end S' (FW), and duration S' (FW) were correlated with ejection fraction in the diseased group (P dogs at both the free wall and in the IVS (P dog.

  2. Functional Class in Children with Idiopathic Dilated Cardiomyopathy. A pilot Study

    Science.gov (United States)

    Tavares, Aline Cristina; Bocchi, Edimar Alcides; Guimarães, Guilherme Veiga

    2016-01-01

    Background Idiopathic dilated cardiomyopathy (IDCM), most common cardiac cause of pediatric deaths, mortality descriptor: a low left ventricular ejection fraction (LVEF) and low functional capacity (FC). FC is never self reported by children. Objective The aims of this study were (i) To evaluate whether functional classifications according to the children, parents and medical staff were associated. (iv) To evaluate whether there was correlation between VO2 max and Weber's classification. Method Prepubertal children with IDCM and HF (by previous IDCM and preserved LVEF) were selected, evaluated and compared. All children were assessed by testing, CPET and functional class classification. Results Chi-square test showed association between a CFm and CFp (1, n = 31) = 20.6; p = 0.002. There was no significant association between CFp and CFc (1, n = 31) = 6.7; p = 0.4. CFm and CFc were not associated as well (1, n = 31) = 1.7; p = 0.8. Weber's classification was associated to CFm (1, n = 19) = 11.8; p = 0.003, to CFp (1, n = 19) = 20.4; p = 0.0001and CFc (1, n = 19) = 6.4; p = 0.04). Conclusion Drawing were helpful for children's self NYHA classification, which were associated to Weber's stratification. PMID:27168472

  3. Risk factor of sudden death in dilated cardiomyopathy patients: A retrospective follow-up study

    Institute of Scientific and Technical Information of China (English)

    LIU Ping; MA Ai-qun; LIU Yu; ZHANG Yan-hui

    2005-01-01

    Objective: To discuss the related risk factors of sudden death in dilated cardiomyopathy(DCM)patients. Methods: A retrospective survey of DCM patients was conducted, ail patients were chosen at random from Xi'an city and 8 adjacent counties. One hundred and fifty patients were reinvestigated after 3. 1 ± 1.5 years. Binary multivariate logistic regression analyses and one way analysis of variance(ANOVA) were used to identify risk factors of the sudden death in DCM patients. Results: Risk factors of sudden death in 150 DCM patients were frequently ventricular premature beats (OR=11. 617), paroxysmalventricular tachycardia (OR=6.305), hypertension (OR= 5.689), EF (OR=0.977). The serum sodium concentration (P= 0. 023) and left ventricular diastolic dimension (LVDD)(P= 0. 039) were significant difference between the sudden death group and the survival group in one way ANOVA, LVDD was not a risk factor in multivariate analysis controlling for possible confounding. Conclusion: The present study identified some risk factors of sudden death in DCM patients, including frequently ventricular premature beats, paroxysmal ventricular tachycardia, hypertension and low EF value.

  4. Genetic analysis of dilated cardiomyopathy--HLA and immunoglobulin genes may confer susceptibility.

    Science.gov (United States)

    Nishi, H; Kimura, A; Fukuta, S; Kusukawa, R; Kawamura, K; Nimura, Y; Nagano, M; Yasuda, H; Kawai, C; Sugimoto, T

    1992-10-01

    To identify genetic factors in the immune system which control the susceptibility to dilated cardiomyopathy (DCM), HLA class II DNA typing was performed in 61 Japanese patients, using PCR/SSO probe analyses. The frequencies of HLA-DQB1*0503 (15% vs 5%; RR = 3.06, chi 2 = 7.19) and DQB1*0604 (21% vs 10%; RR = 2.41, chi 2 = 6.20) were significantly increased and that of HLA-DQB1*0502 (RR = 1.74) was slightly increased in the DCM patients. The frequency of DQB1*0303 (16% vs 31%; RR = 0.44, chi 2 = 5.16) was significantly decreased in the patients. The increased HLA-DQB1 alleles have a histidine residue in common at the 30th codon for the HLA-DQ beta chain. Among the genetic markers studied by Southern blot analyses, IGLV (immunoglobulin lambda light chain, pV3.3) showed a strong association with DCM, i.e. A2/A2 genotype was found in 37.7% of patients whereas it was observed in only 18.9% of the control subjects (RR = 2.6, chi 2 = 7.77). The frequency of this genotype was higher in patients under age 45 years at the time of diagnosis (45.5%, RR = 3.6, chi 2 = 10.02). These results suggest that HLA and immunoglobulin genes are closely linked to susceptibility to DCM.

  5. Intra-cardiac distribution of late gadolinium enhancement in cardiac sarcoidosis and dilated cardiomyopathy

    Science.gov (United States)

    Sano, Makoto; Satoh, Hiroshi; Suwa, Kenichiro; Saotome, Masao; Urushida, Tsuyoshi; Katoh, Hideki; Hayashi, Hideharu; Saitoh, Takeji

    2016-01-01

    Cardiac involvement of sarcoid lesions is diagnosed by myocardial biopsy which is frequently false-negative, and patients with cardiac sarcoidosis (CS) who have impaired left ventricular (LV) systolic function are sometimes diagnosed with dilated cardiomyopathy (DCM). Late gadolinium enhancement (LE) in magnetic resonance imaging is now a critical finding in diagnosing CS, and the novel Japanese guideline considers myocardial LE to be a major criterion of CS. This article describes the value of LE in patients with CS who have impaired LV systolic function, particularly the diagnostic and clinical significance of LE distribution in comparison with DCM. LE existed at all LV segments and myocardial layers in patients with CS, whereas it was localized predominantly in the midwall of basal to mid septum in those with DCM. Transmural (nodular), circumferential, and subepicardial and subendocardial LE distribution were highly specific in patients with CS, whereas the prevalence of striated midwall LE were high both in patients with CS and with DCM. Since sarcoidosis patients with LE have higher incidences of heart failure symptoms, ventricular tachyarrhythmia and sudden cardiac death, the analyses of extent and distribution of LE are crucial in early diagnosis and therapeutic approach for patients with CS. PMID:27721933

  6. Bmi1 limits dilated cardiomyopathy and heart failure by inhibiting cardiac senescence.

    Science.gov (United States)

    Gonzalez-Valdes, I; Hidalgo, I; Bujarrabal, A; Lara-Pezzi, E; Padron-Barthe, L; Garcia-Pavia, P; Gómez-del Arco, P; Gomez, P; Redondo, J M; Ruiz-Cabello, J M; Jimenez-Borreguero, L J; Enriquez, J A; de la Pompa, J L; Hidalgo, A; Gonzalez, S

    2015-03-09

    Dilated cardiomyopathy (DCM) is the most frequent cause of heart failure and the leading indication for heart transplantation. Here we show that epigenetic regulator and central transcriptional instructor in adult stem cells, Bmi1, protects against DCM by repressing cardiac senescence. Cardiac-specific Bmi1 deletion induces the development of DCM, which progresses to lung congestion and heart failure. In contrast, Bmi1 overexpression in the heart protects from hypertrophic stimuli. Transcriptome analysis of mouse and human DCM samples indicates that p16(INK4a) derepression, accompanied by a senescence-associated secretory phenotype (SASP), is linked to severely impaired ventricular dimensions and contractility. Genetic reduction of p16(INK4a) levels reverses the pathology of Bmi1-deficient hearts. In parabiosis assays, the paracrine senescence response underlying the DCM phenotype does not transmit to healthy mice. As senescence is implicated in tissue repair and the loss of regenerative potential in aging tissues, these findings suggest a source for cardiac rejuvenation.

  7. Diagnosis of Dilated Cardiomyopathy: Patient Reaction and Adaptation—Case Study and Review of the Literature

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    Solomis Solomou

    2016-01-01

    Full Text Available Objective. Heart failure remains a major cause of morbidity and mortality. Given that heart failure generally has a chronic course, it is important to appreciate the impact it can have on the quality of life of patients and also their partners or family carers. Method. Questionnaires were given to a patient newly diagnosed with dilated cardiomyopathy, during his hospital admission, as well as after discharge. The responses are summarised and explored in the discussion section, where we used review of the literature to discuss the implications of a new diagnosis of heart failure. Results. The patient’s responses to the questionnaires suggest certain anxieties that are part of his adaptation to the diagnosis of heart failure. Conclusion. Depression is a common comorbid condition in patients with heart failure. Various tools can be used to screen for depression in patients with heart failure. Both pharmacological and nonpharmacological options are available. Rapid evaluation of ongoing problems and active participation by a psychiatrist can ensure that the patient receives the best possible clinical care.

  8. Cardiac magnetic resonance imaging in dilated cardiomyopathy in adults - towards identification of myocardial inflammation

    Energy Technology Data Exchange (ETDEWEB)

    Voigt, Antje; Beling, Mark; Stangl, Karl [Charite-Universitaetsmedizin Berlin, Department of Cardiology, Campus Mitte, Berlin (Germany); Elgeti, Thomas; Durmus, Tahir; Idiz, Merve Ece; Schilling, Rene; Taupitz, Matthias; Wagner, Moritz [Charite-Universitaetsmedizin Berlin, Department of Radiology, Campus Mitte, Berlin (Germany); Butler, Craig [University of Alberta, Mazankowski Alberta Heart Institute, Edmonton (Canada); Klingel, Karin; Kandolf, Reinhard [University Hospital, Department of Molecular Pathology, Tuebingen (Germany); Kivelitz, Dietmar [Asklepios Klinik St. Georg, Department of Radiology, Hamburg (Germany)

    2011-05-15

    To assess active myocardial inflammation by cardiovascular magnetic resonance (CMR) and endomyocardial biopsy (EMB) amongst adult patients with dilated cardiomyopathy (DCM). We evaluated 23 adults with chronic DCM, who had successfully undergone both CMR and EMB within 3.5 {+-} 2.6 days. EMB was considered the gold standard. CMR assessment of myocardial inflammation used the following parameters as recommended by the recently published ''Lake Louise Criteria'': global myocardial oedema, global relative enhancement (RE), and late gadolinium enhancement (LGE). According to ''Lake Louise Criteria'', myocardial inflammation was diagnosed if two or more of the three above-mentioned parameters were positive. Myocardial inflammation was confirmed by immunohistology in 12 patients (52.2%). Sensitivity, specificity, and diagnostic accuracy of CMR to detect immunohistologically confirmed myocardial inflammation were 75.0%, 72.7%, and 73.9%, respectively. Sensitivity, specificity, and diagnostic accuracy of the individual CMR parameters to detect myocardial inflammation were as follows: global myocardial oedema, 91.7%, 81.8%, and 87.0%, respectively; global RE, 58.3%, 63.6%, and 60.9%, respectively; LGE, 58.3%, 45.4%, and 52.2%, respectively. Global myocardial oedema was identified as a promising CMR parameter for assessment of myocardial inflammation in patients with DCM. In these patients, global myocardial oedema yielded superior diagnostic performance compared to ''Lake Louise Criteria''. (orig.)

  9. Thiamin, selenium, and copper levels in patients with idiopathic dilated cardiomyopathy taking diuretics

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    Sérgio da Cunha

    2002-11-01

    Full Text Available OBJECTIVE: To analyze the association of thiamin, selenium, and copper serum levels with cardiac function in patients with idiopathic dilated cardiomyopathy using diuretics, and also to compare them with levels in control patients with no evidence of disease. METHODS: The study comprised 30 patients with heart disease and 30 healthy control individuals. Thiamin was analyzed by measuring the activity of erythrocytic transketolase and the effect of thiamin pyrophosphate. Selenium and copper serum levels were measured by hydride generation and flame atomic absorption spectrophotometry, respectively. RESULTS: Thiamin deficiency was observed in 10% of the control individuals and in 33% of the patients with heart disease (p=0.02. The mean selenium and copper serum levels in control individuals and patients with heart disease were, respectively, 73.2±9.9 µg/L (56.5 to 94.5 µg/L and 72.3±14.3 µg/L (35.5 to 94 µg/L (p=0.77; 1.1±0.4mg/L (0.6 to 1.8mg/L and 1.2± 0.4mg/L (0.6 to 2.2mg/L (p=0.27. No association between the levels of these nutrients and cardiac function was observed. CONCLUSION: Thiamin deficiency was significantly more frequent in patients with heart disease. No significant difference was observed between the mean selenium and copper serum levels in control individuals and in patients with heart disease. The results suggest possible benefits with thiamin replacement in patients taking diuretics.

  10. AN ASSESSMENT OF ECHOCARDIOGRAPHY AS A DIAGNOSTIC TOOL FOR DILATED CARDIOMYOPATHY IN TURKEY (MELEAGRIS GALLOPAVO

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    Kwaku Gyenai

    2012-01-01

    Full Text Available Our understanding of the etiology of Dilated Cardiomyopathy (DCM, which affects about 5% of turkeys, is limited. This limitation may be due to the lack of an easy-to-use diagnostic tool with well-defined parameters and does not involve necropsy. This lack of a widely tested non-necropsy method makes it difficult for a large-scale study of the genetic factors that underlie DCM. Here, we Evaluated Echocardiography (ECHO for its ease and reliability for identifying DCM-affected turkeys from hatch to four weeks-of-age. The parameters evaluated included Left Ventricular Internal-Diastolic (LVIDd, Internal-Systolic Dimension (LVISd, Interventricular Septum End-Diastolic (IVSEd, Interventricular Septum End-Systolic (IVSEs, Left Ventricular Wall End-Systolic (LVWEs and Left Ventricular Wall End-Diastolic (LVWEd. To induce DCM, feed containing 700 ppm of Furazolidone (Fz was fed to turkey poults from one to 28 days-of-age. The LVIDd and LVISd were the most consistent indicators of DCM. Both parameters revealed differences between control and treatment poults of between 25 and 326% at the 4 ages at which ECHO measurements were taken. The average difference in LVIDd between control and poults fed Fz-containing diets ranged from 25% in one week-old to 80% in 4-week-old poults. At similar ages, average differences between control and poults fed Fz-containing diets in LVISd were 74 and 326% respectively. Necropsy of poults that survived to the end of the 4-week Fz-treatment confirmed these ECHO measurements in treatment and normal poults. Our data suggest that using LVIDd and LVISd as parameters make ECHO a reliable tool for identifying DCM in turkeys. "

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

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    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.

  12. Abnormal Glucose Tolerance Is Associated with a Reduced Myocardial Metabolic Flexibility in Patients with Dilated Cardiomyopathy

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    Domenico Tricò

    2016-01-01

    Full Text Available Dilated cardiomyopathy (DCM is characterized by a metabolic shift from fat to carbohydrates and failure to increase myocardial glucose uptake in response to workload increments. We verified whether this pattern is influenced by an abnormal glucose tolerance (AGT. In 10 patients with DCM, 5 with normal glucose tolerance (DCM-NGT and 5 with AGT (DCM-AGT, and 5 non-DCM subjects with AGT (N-AGT, we measured coronary blood flow and arteriovenous differences of oxygen and metabolites during Rest, Pacing (at 130 b/min, and Recovery. Myocardial lactate exchange and oleate oxidation were also measured. At Rest, DCM patients showed a reduced nonesterified fatty acids (NEFA myocardial uptake, while glucose utilization increased only in DCM-AGT. In response to Pacing, glucose uptake promptly rose in N-AGT (from 72 ± 21 to 234 ± 73 nmol/min/g, p<0.05, did not change in DCM-AGT, and slowly increased in DCM-NGT. DCM-AGT sustained the extra workload by increasing NEFA oxidation (from 1.3 ± 0.2 to 2.9 ± 0.1 μmol/min/gO2 equivalents, p<0.05, while DCM-NGT showed a delayed increase in glucose uptake. Substrate oxidation rates paralleled the metabolites data. The presence of AGT in patients with DCM exacerbates both the shift from fat to carbohydrates in resting myocardial metabolism and the reduced myocardial metabolic flexibility in response to an increased workload. This trial is registered with ClinicalTrial.gov NCT02440217.

  13. Clinical significance and pathogenic role of anti-cardiac myosin autoantibody in dilated cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    2003-01-01

    Objective In order to explore the possible roles played by the autoimmune mechanism in the progression of myocarditis into dilated cardiomyopathy (DCM) using an animal model, we investigated whether autoimmune myocarditis might develop into DCM. Methods Experimental Balb/C mice (n=20) were immunized with cardiac myosin with Freund's complete adjuvant at days 0, 7 and 30. The control Balb/C mice (n=10) were immunized with Freund's complete adjuvant in the same mannere. Serum and myocardium samples were collected after the first immunization at days 15, 21 and 120. The anti-myosin antibody was examined by enzyme-linked immunosorbent assay and immunoblotting.Results Pathological findings demonstrated that there was myocardial necrosis or inflammatory infiltration during acute stages and fibrosis mainly in the late phase of experimental group, but the myocardial lesions were not found in the control group. Autoimmunity could induce myocarditis and DCM in the absence of viral infection. High titer anti-myosin IgG antibodies were found in the experimental group, but not in the control group. Furthermore, the anti-myosin heavy chain (200 KD) antibody was positive in 21 of 48 patients with DCM and viral myocarditis, but only 4 of 20 patients with coronary heart disease, including 1 case and 3 cases that reacted with heavy and light chains (27.5 KD), respectively. The antibodies were not detected in healthy donors.Conclusion Cardiac myosin might be an autoantigen that provokes autoimmunity and leads to the transformation of myocarditis into DCM. Detection of anti-myosin heavy chain antibody might contribute to diagnosis for DCM and viral myocarditis.

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

    Energy Technology Data Exchange (ETDEWEB)

    Manrique, Alain; Hitzel, Anne; Vera, Pierre [Rouen University Hospital - Henri Becquerel Center, Nuclear Medicine, Rouen (France); Bernard, Mathieu; Bauer, Fabrice [Rouen University Hospital, Cardiology, Rouen (France); Menard, Jean-Francois [Rouen University Hospital, Biostatistics, Rouen (France); Sabatier, Remi [Caen University Hospital, Cardiology, Caen (France); Jacobson, Arnold [GE Healthcare, Princeton, NJ (United States); Agostini, Denis [Caen University Hospital, Nuclear Medicine, Caen (France)

    2008-11-15

    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.)

  15. Analysis of regional LV function using radionuclide ventriculography in patients with dilated cardiomyopathy

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    Morozumi, Takakazu; Ishida, Yoshio; Sato, Hideyuki; Hori, Masatsugu; Kamada, Takenobu; Yamagami, Hidetoshi; Kozuka, Takahiro; Nishimura, Tsunehiko (Osaka Univ. (Japan). Faculty of Medicine)

    1993-02-01

    The study evaluated the clinical significance of RI ventriculography in determining left ventricular regional wall motion abnormality (WMA), i.e., asynergy or asynchrony, in dilated cardiomyopathy (DCM). The subjects were 22 DCM patients and 10 normal persons. RI multi-gated cardiac blood pool scans were performed; and the whole ventricular area was divided into 8 segments to determine regional WMA and coefficients of variation of regional ejection fraction (CV-rEF) and regional ejection time (CV-rET). According to Tl-201 myocardial SPECT images at rest, DCM patients were classified as having no regional myocardial fibrotic lesions (DCM-A, n=17) or as having fibrotic lesions (DCM-B, n=5). CV-rEF and CV-rET were, on the average, increased by +2 SD or more in 14 (82%) and 11 (65%) patients, respectively, in Group DCM-A and respective 4 patients (80%) in Group DCM-B. CV-rEF and CV-rET were 24.7[+-]5.7% and 7.5[+-]2.3%, respectively, in control group; and corresponding figures were 41.1[+-]14.3% and 23.2[+-]13.5% in Group DCM-A and 59.1[+-]19.8% and 19.0[+-]7.2% in Group DCM-B, respectively. Because regional contraction abnormality also existed even in Group DCM-A with no definitive evidence of myocardial fibrosis, regional contraction abnormality associated with DCM may be attributed to other causes than fibrosis. In 5 patients in whom left ventricular EF was increased by giving beta blockers, both CV-rEF and CV-rET improved. This suggested that beta blockers may homogeneously affect contractile function, resulting in the improvement of left ventricular function. (N.K.).

  16. Computer-based assessment of left ventricular wall stiffness in patients with ischemic dilated cardiomyopathy

    Science.gov (United States)

    Su, Y.; Teo, S. K.; Tan, R. S.; Lim, C. W.; Zhong, L.

    2013-02-01

    Ischemic dilated cardiomyopathy (IDCM) is a degenerative disease of the myocardial tissue accompanied by left ventricular (LV) structural changes such as interstitial fibrosis. This can induce increased passive stiffness of the LV wall. However, quantification of LV passive wall stiffness in vivo is extremely difficult, particularly in ventricles with complex geometry. Therefore, we sought to (i) develop a computer-based assessment of LV passive wall stiffness from cardiac magnetic resonance (CMR) imaging in terms of a nominal stiffness index (E*); and (ii) investigate whether E* can offer an insight into cardiac mechanics in IDCM. CMR scans were performed in 5 normal subjects and 5 patients with IDCM. For each data sample, an in-house software was used to generate a 1-to-1 corresponding mesh pair of the LV from the ED and ES phases. The E* values are then computed as a function of local ventricular wall strain. We found that E* in the IDCM group (40.66 - 215.12) was at least one order of magnitude larger than the normal control group (1.00 - 6.14). In addition, the IDCM group revealed much higher inhomogeneity of E* values manifested by a greater spread of E* values throughout the LV. In conclusion, there is a substantial elevated ventricular stiffness index in IDCM. This would suggest that E* could be used as discriminator for early detection of disease state. The computational performance per data sample took approximately 25 seconds, which demonstrates its clinical potential as a real-time cardiac assessment tool.

  17. Systolic-diastolic functional coupling in healthy children and in those with dilated cardiomyopathy.

    Science.gov (United States)

    Friedberg, Mark K; Margossian, Renee; Lu, Minmin; Mercer-Rosa, Laura; Henderson, Heather T; Nutting, Arni; Friedman, Kevin; Molina, Kimberly M; Altmann, Karen; Canter, Charles; Sleeper, Lynn A; Colan, Steven D

    2016-06-01

    Systolic and diastolic function affect dilated cardiomyopathy (DCM) outcomes. However, systolic-diastolic coupling, as a distinct characteristic, may itself affect function but is poorly characterized. We hypothesized that echocardiographic left ventricular (LV) longitudinal systolic tissue velocities (S') correlate with diastolic longitudinal velocities (E') and that their relationship is associated with ventricular function and that this relationship is impaired in pediatric DCM. We analyzed data from the Pediatric Heart Network Ventricular Volume Variability study, using linear regression and generalized additive modeling to assess relationships between S' and E' at the lateral and septal mitral annulus. We explored relationships between the systolic:diastolic (S:D) coupling ratio (S':E' relative to age) and ventricular function. Up to 4 echocardiograms from 130 DCM patients (mean age: 9.3 ± 6.1 yr) and 1 echocardiogram from each of 591 healthy controls were analyzed. S' and E' were linearly related in controls (r = 0.64, P < 0.001) and DCM (r = 0.83, P < 0.001). In DCM, the magnitude of association between S' and E' was reduced with progressive ventricular remodeling. The S:D ratio was more strongly associated with LV function in controls vs. DCM. The septal S:D ratio was higher (presumed worse) in DCM vs. controls (0.69 ± 0.13 vs. 0.62 ± 0.12, P = 0.001). A higher septal S:D ratio was associated with worse LV dimensions (parameter estimate: 0.0061, P = 0.004), mass (parameter estimate: 0.0074, P = 0.002), ejection fraction (parameter estimate: -0.0303, P = 0.024), and inflow propagation (parameter estimate: -0.3538, P < .001). S:D coupling becomes weaker in DCM with LV remodeling and dysfunction. The S:D coupling ratio may be useful to assess coupling, warranting study in relation to patient outcomes.

  18. Changes of Left Ventricular Geometry Shape and Left Ventricular Regional Function in Patients With Dilated Cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    Liang-yu WANG; Ming-xing XIE; Qing-bo LI; Ping CHEN; Zhi-xiong CAI; Zhi-dan ZHU

    2009-01-01

    Objectives To assess the left ventricle regional systolic and diastolic function, left ventricle geometry and left venti-tie sphericity indexes in patients with dilated cardiomyopathy (DCM) by quantitative tissue velocity imaging (QTVI). Methods Thirty normal subjects and 52 DCM patients underwent QTVI and colour Doppler flow imaging study in or-der to measure the left ventricular regional function along left ventricle apical long-axis view and the left ventricle geom-etry. Peak tissue velocities of left venticle regional muscular tissue during systole (Vs), systolic acceleration (a), ear-ly diastole(Ve) and left atrium contraction(Va) along left venticle apical long axis view were measured. The indexes of left ventdcular regional systolic and diastolic function were mearsured at the same time. The left ventricle geometry shape was reflected from the systolic and diastolic sphericity index (Sis and Sid), the left ventricular ejection fraction (LVEF) and D wave/A wave (PVd/Pva) of pulmonary veins flowing spectrum reflected the global left ventricular systolic and diastolic function. The Vs, Ve, Va, a, PVd/Pva ratio, LVEF, Sis, Sid and their correlations between normal subjects and patients with DCM were compared and analyzed. Results Vs, Ve, Va, a, PVd/Pva, Sis and Sid in patients with DCM were lower than those in normal persons. There were significant relations between Sis and a (r=0.6142, P<0.05), Ve/Va and Sid (r=0.6271, P<0.05). Conclusions QTVI offer a newer method which has a higher sensitivity and accuracy in evaluating the left venticle regional systolic and diastolic function in DCM patients. There was significant relation between regional cardiac function and left venticle sphericity.

  19. Increased stromal-cell-derived factor 1 enhances the homing of bone marrow derived mesenchymal stem cells in dilated cardiomyopathy in rats

    Institute of Scientific and Technical Information of China (English)

    ZHOU Yan-li; Michael Fu; ZHANG Hai-feng; LI Xin-li; DI Ruo-min; YAO Wen-ming; LI Dian-fu; FENG Jian-lin; HUANG Jun; CAO Ke-jiang

    2010-01-01

    Background Stem cell transplantation has been shown to have beneficial effects on dilated cardiomyopathy. However,mechanism for stem cell homing to cardiac tissue in dilated cardiomyopathy has not yet been elucidated.Methods Mesenchymal stem cells were obtained from rat bone marrow, expanded in vitro, and labeled with 99mTc.Cardiomyopathy model was induced by doxorubicin in rats. 99mTc labeled cells were infused into the left ventricles in cardiomyopathy and control rats. Sixteen hours after injection, animals were sacrificed and different tissues were harvested to measure specific radioactivity. By use of real-time polymerase chain reaction and immunohistochemistry,Mrna and protein expressions for stromal-cell-derived factor 1 in cardiac tissue were measured.Results Labeling efficiency of mesenchymal stem cells was (70.0±11.2)%. Sixteen hours after mesenchymal stem cell transplantation, the heart-to-muscle radioactivity ratio was increased significantly in cardiomyopathy hearts as compared to control hearts. Both Mrna and rotein expressions of stromal-cell-derived factor 1 were up-regulated in cardiomyopathy hearts as compared with control hearts.Conclusion In dilated cardiomyopathy induced by doxorubicin up-regulated expression of stromal-cell-derived factor 1in heart may induce mesenchymal stem cells home to the heart.

  20. 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

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    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)

  1. An Indian family with an Emery-Dreifuss myopathy and familial dilated cardiomyopathy due to a novel LMNA mutation

    Directory of Open Access Journals (Sweden)

    Khushal B Jadhav

    2012-01-01

    Full Text Available Emery-Dreifuss myopathy can be associated with a cardiomyopathy and cardiac dysrhythmias. The inheritance pattern of Emery-Dreifuss muscular dystrophy (EDMD is X linked, whereas EDMD2 is autosomal dominant. EDMD2 is caused by lamin A/C gene (LMNA mutations that produce alterations in the lamin proteins that are integral to nuclear and cell integrity. A 53-year-old man was brought to us with a right internal carotid artery dissection. Detailed work-up of the patient and family members revealed some unusual features, and genetic sequencing of the LMNA gene was undertaken. A novel mutation was identified in two of the samples sent for analysis. We present the first Indian family of EDMD2 with familial dilated cardiomyopathy and cardiac dysrhythmias in whom LMNA gene sequencing was performed. A novel mutation was identified and additional unusual clinical features were described.

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

    Directory of Open Access Journals (Sweden)

    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.

  3. 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.

  4. Comparison of mortality rates and progression of left ventricular dysfunction in patients with idiopathic dilated cardiomyopathy and dilated versus nondilated right ventricular cavities.

    Science.gov (United States)

    Sun, J P; James, K B; Yang, X S; Solankhi, N; Shah, M S; Arheart, K L; Thomas, J D; Stewart, W J

    1997-12-15

    This study assesses the influence of right ventricular (RV) dilation on the progression of left ventricular (LV) dysfunction and survival in patients with idiopathic dilated cardiomyopathy (IDC). Using transthoracic echocardiography, we studied 100 patients with IDC aged 20 to 80 years (mean 55 +/- 14); 67% were men. In the apical 4-chamber view, diastolic LV and RV chamber area measurements classified patients into 2 groups: group RV enlargement+ (RV area/LV area > 0.5) included 54 patients; group RV enlargement- (no RV enlargement) had RV area/LV area < or = 0.5. Echocardiographic studies were repeated in all patients after a mean of 33 +/- 16 months. At the time of the initial study, the 2 groups did not differ in age, gender, incidence of atrial fibrillation and diabetes, left ventricular mass, and LV ejection fraction, but the RV enlargement+ group had more severe tricuspid regurgitation and less LV enlargement. After 47 +/- 22 months (range 12 to 96), patients in group RV enlargement+ had lower LV ejection fraction (29% vs 34%, p = 0.006) than patients with initial RV enlargement-. At clinical follow-up, mortality was higher (43%) in patients with initial RV enlargement+ than the RV enlargement- patients (15%), p = 0.002. For survivors, the mitral deceleration time averaged 157 +/- 36 ms; for nonsurvivors or patients who required transplant, the mitral deceleration time averaged 97 +/- 12 ms (p < 0.0001). With use of a multivariate Cox model adjusting for LV ejection fraction, LV size, and age, the relative risk ratio of mortality from initial RV enlargement+ was 4.4 (95% confidence limits 1.7 to 11.1) (p = 0.002). Thus, patients with significant RV dilation had nearly triple the mortality over 4 years and more rapidly deteriorating LV function than patients with less initial RV dilation. In IDC, RV enlargement is a strong marker for adverse prognosis that may represent a different morphologic subset.

  5. Use of I-123 MIBG cardiac scintigraphy to assess the impact of carvedilol on cardiac adrenergic neuronal function in childhood dilated cardiomyopathy; Interet de la scintigraphie cardiaque a l'I-123 MIBG pour evaluer l'impact du carvedilol sur la fonction neuronale adrenergique cardiaque dans les myocardiopathies dilatees de l'enfant

    Energy Technology Data Exchange (ETDEWEB)

    Maunoury, C. [Hopital Europeen Georges Pompidou (HEGP), Dept. de Physiologie et Radio-Isotopes, 75 - Paris (France); Acar, P. [Centre Hospitalier Universitaire, Service de Cardiologie Pediatrique, Hopital des Enfants, 31 - Toulouse (France); Sidi, D. [Centre Hospitalier Universitaire Necker-Enfants-Malades, 75 - Paris (France)

    2006-04-15

    I-123 MIBG cardiac scintigraphy is a useful tool to assess cardiac adrenergic neuronal function, which is impaired in children with dilated cardiomyopathy (DCM). In adults with DCM, long-term treatment with carvedilol improves both cardiac adrenergic neuronal function and left ventricular function. The aim of this prospective study was to evaluate the impact of carvedilol on cardiac adrenergic neuronal function and on left ventricular function in seventeen patients (11 female, 6 male, mean age 39 {+-} 57 months, range 1 - 168 months) with DCM. All patients underwent I-123 MIBG cardiac scintigraphy and equilibrium radio-nuclide angiography before and after a 6 month period of carvedilol therapy. A static anterior view of the chest was acquired 4 hours after intravenous injection of 20 to 75 MBq of I-123 MIBG. Cardiac neuronal uptake of I-123 MIBG was measured using the heart to mediastinum count ratio (HMR). Radionuclide left ventricular ejection fraction (LVEF) was assessed following a standard protocol. There was no major cardiac events (death or transplantation) during the follow-up period. I-123 MIBG cardiac uptake and left ventricular function respectively increased by 38% and 65% after 6 months of treatment with carvedilol (HMR 223 {+-} 49% vs 162 {+-} 26%, p < 0.0001 and LVEF = 43 {+-} 17% vs 26 {+-} 11%, p < 0.0001). Carvedilol can improve cardiac adrenergic neuronal function and left ventricular function in children with DCM. Further studies are needed to assess the relationship between improvement in I-123 MIBG cardiac uptake and the beneficial effects of carvedilol on morbidity and mortality. (authors)

  6. E2F6 Impairs Glycolysis and Activates BDH1 Expression Prior to Dilated Cardiomyopathy

    Science.gov (United States)

    Major, Jennifer L.; Dewan, Aaraf; Salih, Maysoon; Leddy, John J.; Tuana, Balwant S.

    2017-01-01

    Rationale The E2F pathway plays a critical role in cardiac growth and development, yet its role in cardiac metabolism remains to be defined. Metabolic changes play important roles in human heart failure and studies imply the ketogenic enzyme β-hydroxybutyrate dehydrogenase I (BDH1) is a potential biomarker. Objective To define the role of the E2F pathway in cardiac metabolism and dilated cardiomyopathy (DCM) with a focus on BDH1. Methods and Results We previously developed transgenic (Tg) mice expressing the transcriptional repressor, E2F6, to interfere with the E2F/Rb pathway in post-natal myocardium. These Tg mice present with an E2F6 dose dependent DCM and deregulated connexin-43 (CX-43) levels in myocardium. Using the Seahorse platform, a 22% decrease in glycolysis was noted in neonatal cardiomyocytes isolated from E2F6-Tg hearts. This was associated with a 39% reduction in the glucose transporter GLUT4 and 50% less activation of the regulator of glucose metabolism AKT2. The specific reduction of cyclin B1 (70%) in Tg myocardium implicates its importance in supporting glycolysis in the postnatal heart. No changes in cyclin D expression (known to regulate mitochondrial activity) were noted and lipid metabolism remained unchanged in neonatal cardiomyocytes from Tg hearts. However, E2F6 induced a 40-fold increase of the Bdh1 transcript and 890% increase in its protein levels in hearts from Tg pups implying a potential impact on ketolysis. By contrast, BDH1 expression is not activated until adulthood in normal myocardium. Neonatal cardiomyocytes from Wt hearts incubated with the ketone β-hydroxybutyrate (β-OHB) showed a 100% increase in CX-43 protein levels, implying a role for ketone signaling in gap junction biology. Neonatal cardiomyocyte cultures from Tg hearts exhibited enhanced levels of BDH1 and CX-43 and were not responsive to β-OHB. Conclusions The data reveal a novel role for the E2F pathway in regulating glycolysis in the developing myocardium

  7. Changes in myocardial collagen content before and after left ventricular assist device application in dilated cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    LIANG Hong 梁红; Roland Hetzer; Johannes Müller; WENG Yu-guo 翁渝国; Gerd Wallukat; FU Ping 付平; LIN Han-sheng 林汉生; Sabina Bartel; Christoph Knosalla; Reinhard Pregla

    2004-01-01

    Background The purposes of this study were to confirm the changes in myocardial collagen level after left ventricular assist device (LVAD) support in dilated cardiomyopathy (DCM), find the relation between these changes and prognosis, and test a practical method to assess the level of myocardial collagen.Methods Left ventricular samples were collected from DCM patients with different prognosis (transplanted group n=8, weaning group n=10) at the time when the LVADs were implanted and again during cardiac transplantation (n=8). The level of neutral salt soluble collagen (NSC) and acid soluble collagen (ASC) was measured by Sircol collagen assay, and that of total collagen and insoluble collagen (ISC) by quantification of hydroxyproline (Hyp). Serum samples were collected from a portion of these patients (transplanted group, n=6; weaning group n=7) at the time the LVADs were implanted, 1 month after implantation and on explantation. Circulating concentration of carboxy-terminal propeptide of type Ⅰ procollagen (PⅠCP), amino-terminal propeptide of type Ⅰ procollagen (PⅠNP), amino-terminal propeptide of type Ⅲ procollagen (PⅢNP) and type Ⅰ collagen telopeptide (ⅠCTP) were measured by the equilibrium type radioimmunoassay. Results Before LVAD implantation the level of NSC and ISC in the weaning group was higher but ASC in the transplanted group was lower than in the controls (P<0.05). After LVAD support, the level of total collagen was higher, but ASC was also lower in the transplanted group than in the controls (P<0.05). In comparison of the pre- and post-LVAD subgroups of the transplanted and weaning groups, all collagen fraction levels before LVAD implantation were lower in the transplanted group than in the weaning group (P<0.05); but this difference disappeared after LVAD support. Comparison of the pre- and post-LVAD subgroups of the transplanted group showed increased level of NSC and total collagen after LVAD support. The changes of serum peptide

  8. 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.

  9. Multicenter study on hepatitis C virus infection in patients with dilated cardiomyopathy. North Italy Transplant Program (NITP).

    Science.gov (United States)

    Prati, D; Poli, F; Farma, E; Picone, A; Porta, E; De Mattei, C; Zanella, A; Scalamogna, M; Gamba, A; Gronda, E; Faggian, G; Livi, U; Puricelli, C; Viganò, M; Sirchia, G

    1999-06-01

    Preliminary epidemiological and histological studies from Japan suggested that hepatitis C virus (HCV) infection has a role in the development of dilated cardiomyopathy (DCM). This multicenter study was conducted to verify this hypothesis on a large cohort of Italian patients with end-stage heart failure. Antibodies to HCV were determined in the 752 consecutive patients (608 males and 144 females; age, 53 +/- 13 years) who entered the waiting list for cardiac transplantation from 1995 to 1997 at the six cardiac surgery centers participating in the North Italy Transplant program. Three hundred and nine patients (41%) had dilated, 9 (1%) restrictive, and 4 (0.5%) hypertrophic cardiomyopathy; 284 patients (38%) had ischemic, 65 (9%) valvular, and 22 (3%) congenital heart disease; 5 patients (0.5%) had primary pulmonary hypertension; 54 patients (7%) had other or nonspecified heart disease. Overall, 41 of 752 patients (5.4%) resulted anti-HCV-reactive. Serological evidence of HCV infection was found in 12 of 309 patients with DCM (3.9%; 95% CI, 1.7-6.0), and in 29 of 443 without DCM (6.5%; 95% CI, 4.2-8.8), without statistical difference (difference of prevalence rate: 2.6%; 95% CI, -4.9 to 5.8). In conclusion, HCV does not seem to have a primary role in the pathogenesis of DCM. However, since our findings are in disagreement with those obtained in smaller series of patients of other ethnicity, large studies from different countries should be conducted.

  10. 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.

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

    Science.gov (United States)

    Bengi Bakal, Ruken; Hatipoglu, Suzan; Sahin, Muslum; Emiroglu, Mehmet Yunus; Bulut, Mustafa; Ozdemir, Nihal

    2014-01-01

    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. PMID:25610556

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

    Directory of Open Access Journals (Sweden)

    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.

  13. 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.

  14. 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.

  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. Early combined treatment with sildenafil and adipose-derived mesenchymal stem cells preserves heart function in rat dilated cardiomyopathy

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    Fu Morgan

    2010-09-01

    Full Text Available Abstract Background We investigated whether early combined autologous adipose-derived mesenchymal stem cell (ADMSC and sildenafil therapy offers an additive benefit in preserving heart function in rat dilated cardiomyopathy (DCM. Methods Adult Lewis rats (n = 8 per group were divided into group 1 (normal control, group 2 (saline-treated DCM rats, group 3 [2.0 × 106 ADMSC implanted into left ventricular (LV myocardium of DCM rats], group 4 (DCM rats with sildenafil 30 mg/kg/day, orally, and group 5 (DCM rats with combined ADMSC-sildenafil. Treatment was started 1 week after DCM induction and the rats were sacrificed on day 90. Results The results showed that mitochondrial protein expressions of connexin43 and cytochrome-C were lowest in group 2, and lower in groups 3 and 4 than in group 5 (p Conclusion Early combined ADMSC/sildenafil is superior to either treatment alone in preserving LV function.

  17. Anaesthetic Management of Renal Transplant Surgery in Patients of Dilated Cardiomyopathy with Ejection Fraction Less Than 40%

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    Divya Srivastava

    2014-01-01

    Full Text Available Cardiovascular disease (CVD is an important comorbidity of chronic kidney disease, and reducing cardiovascular events in this population is an important goal for the clinicians who care for chronic kidney disease patients. The high risk for CVD in transplant recipients is in part explained by the high prevalence of conventional CVD risk factors (e.g., diabetes, hypertension, and dyslipidemia in this patient population. Current transplant success allows recipients with previous contraindications to transplant to have access to this procedure with more frequency and safety. Herein we provide a series of eight patients with dilated cardiomyopathy with poor ejection fraction posted for live donor renal transplantation which was successfully performed under regional anesthesia with sedation.

  18. Influence of Mechanical Circulatory Support on Endothelin Receptor Expression in Human Left Ventricular Myocardium from Patients with Dilated Cardiomyopathy (DCM)

    Science.gov (United States)

    Gärtner, Florian; Abraham, Getu; Kassner, Astrid; Baurichter, Daniela; Milting, Hendrik

    2017-01-01

    Background In terminal failing hearts ventricular assist devices (VAD) are implanted as a bridge to transplantation. Endothelin receptor (ETR) antagonists are used for treatment of secondary pulmonary hypertension in VAD patients. However, the cardiac ETR regulation in human heart failure and during VAD support is incompletely understood. Methods In paired left ventricular samples of 12 dilated cardiomyopathy patients we investigated the density of endothelin A (ETA) and B (ETB) receptors before VAD implantation and after device removal. Left ventricular samples of 12 non-failing donor hearts served as control. Receptor quantification was performed by binding of [125I]-ET-1 in the presence of nonselective and ETA selective ETR ligands as competitors. Additionally, the ETR mRNA expression was analyzed using quantitative real-time-PCR. Results The mRNA of ETA but not ETB receptors was significantly elevated in heart failure, whereas total ETR density analyzed by radioligand binding was significantly reduced due to ETB receptor down regulation. ETA and ETB receptor density showed poor correlation to mRNA data (spearman correlation factor: 0.43 and 0.31, respectively). VAD support had no significant impact on the density of both receptors and on mRNA expression of ETA whereas ETB mRNA increased during VAD. A meta-analysis reveals that the ETA receptor regulation in human heart failure appears to depend on non-failing hearts. Conclusions In deteriorating hearts of patients suffering from dilated cardiomyopathy the ETA receptor density is not changed whereas the ETB receptor is down regulated. The mRNA and the proteins of ETA and ETB show a weak correlation. Non-failing hearts might influence the interpretation of ETA receptor regulation. Mechanical unloading of the failing hearts has no impact on the myocardial ETR density. PMID:28095452

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

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    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.

  20. Evaluation of dilated cardiomyopathy by /sup 201/Tl myocardial single photon emission computed tomography. Morphological and quantitative analysis

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    Futagami, Yasuo; Makino, Katsutoshi; Ichikawa, Takehiko

    1984-08-01

    To estimate dilated cardiomyopathy (DCM)morphologically and quantitatively, /sup 201/Tl myocardial single photon emission computed tomography (SPECT) was performed in 14 DCM and 5 normal cases. Using a rotating dual-gamma camera system, resting SPECT data were collected for 6 minutes. Quantitative analysis of clinical cases was based on phantom studies. Marked spherical left ventricular (LV) dilatation (14/14), localized-diffuse low uptake or defect (12/14), and right ventricular visualization (6/14) were characteristic features in DCM. Differentiation of DCM from ischemic heart disease by SPECT was possible through the feature indicating disproportionately large LV cavity to defect size or degree. Quantitative analysis When DCM was compared with normal control (n-5), following 3 features were impressive: DCM was significantly higher in LV myocardial /sup 201/Tl uptake ratio and LV volume than normal control; DCM was significantly lower in LV myocardial /sup 201/Tl uptake ratio of unit volume (1 ml) than normal control; DCM was significantly lower in mean myocardial count/mean lung count.ratio than normal control.

  1. Cardiac-specific VLCAD deficiency induces dilated cardiomyopathy and cold intolerance

    Science.gov (United States)

    Xiong, Dingding; He, Huamei; James, Jeanne; Tokunaga, Chonan; Powers, Corey; Huang, Yan; Osinska, Hanna; Towbin, Jeffrey A.; Purevjav, Enkhsaikhan; Balschi, James A.; Javadov, Sabzali; McGowan, Francis X.; Strauss, Arnold W.

    2013-01-01

    The very long-chain acyl-CoA dehydrogenase (VLCAD) enzyme catalyzes the first step of mitochondrial β-oxidation. Patients with VLCAD deficiency present with hypoketotic hypoglycemia and cardiomyopathy, which can be exacerbated by fasting and/or cold stress. Global VLCAD knockout mice recapitulate these phenotypes: mice develop cardiomyopathy, and cold exposure leads to rapid hypothermia and death. However, the contribution of different tissues to development of these phenotypes has not been studied. We generated cardiac-specific VLCAD-deficient (cVLCAD−/−) mice by Cre-mediated ablation of the VLCAD in cardiomyocytes. By 6 mo of age, cVLCAD−/− mice demonstrated increased end-diastolic and end-systolic left ventricular dimensions and decreased fractional shortening. Surprisingly, selective VLCAD gene ablation in cardiomyocytes was sufficient to evoke severe cold intolerance in mice who rapidly developed severe hypothermia, bradycardia, and markedly depressed cardiac function in response to fasting and cold exposure (+5°C). We conclude that cardiac-specific VLCAD deficiency is sufficient to induce cold intolerance and cardiomyopathy and is associated with reduced ATP production. These results provide strong evidence that fatty acid oxidation in myocardium is essential for maintaining normal cardiac function under these stress conditions. PMID:24285112

  2. Exercise training in childhood cancer survivors with subclinical cardiomyopathy who were treated with anthracyclines.

    Science.gov (United States)

    Smith, Webb A; Ness, Kirsten K; Joshi, Vijaya; Hudson, Melissa M; Robison, Leslie L; Green, Daniel M

    2013-11-06

    Childhood cancer survivors (CCS) treated with anthracyclines are at risk for cardiomyopathy. This case series evaluated the response of anthracycline exposed CCS with subclinical cardiomyopathy to aerobic and strength training. Body composition, strength and cardiopulmonary fitness were evaluated before and after the 12-week intervention. All equipment and materials were provided to five 10+ year CCS (3 males, mean age 38.0 ± 3.3 years) for a guideline-based home exercise program. All five completed the study with no adverse events. Compliance with exercise was 86%. These results suggest that exercise training may improve exercise capacity of CCS with subclinical cardiomyopathy. Pediatr Blood Cancer. © 2013 Wiley Periodicals, Inc.

  3. Dilated cardiomyopathy in homozygous myosin-binding protein-C mutant mice

    OpenAIRE

    1999-01-01

    To elucidate the role of cardiac myosin-binding protein-C (MyBP-C) in myocardial structure and function, we have produced mice expressing altered forms of this sarcomere protein. The engineered mutations encode truncated forms of MyBP-C in which the cardiac myosin heavy chain-binding and titin-binding domain has been replaced with novel amino acid residues. Analogous heterozygous defects in humans cause hypertrophic cardiomyopathy. Mice that are homozygous for the mutated MyBP-C alleles expre...

  4. A new surgical approach for treating dilated cardiomyopathy with mitral regurgitation

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    Buffolo Enio

    2000-01-01

    Full Text Available OBJECTIVE: To evaluate the early outcome of mitral valve prostheses implantation and left ventricular remodeling in 23 patients with end-stage cardiomyopathy and secondary mitral regurgitation (NYHA class III and IV. METHODS: Mitral valvular prosthesis implantation with preservation of papillary muscles and chordae tendinae, and plasty of anteriun cuspid for remodeling of the left ventricle. RESULTS: The surgery was performed in 23 patients, preoperative ejection fraction (echocardiography varied from 13% to 44% (median: 30%. In 13 patients associated procedures were performed: myocardial revascularization (9, left ventricle plicature repair (3 and aortic prosthese implantation (1. Early deaths (2 occurred on the 4th PO day (cardiogenic shock and on the 20th PO day (upper gastrointestinal bleeding, and a late death in the second month PO (ventricular arrhythmia. Improvement occurred in NYHA class in 82.6% of the patients (P<0.0001, with a survival rate of 86.9% (mean of 8.9 months of follow-up. CONCLUSION: This technique offers a promising therapeutic alternative for the treatment of patients in refractory heart failure with cardiomyopathy and secondary mitral regurgitation.

  5. A case of juvenile acromegaly that was initially diagnosed as severe congestive heart failure from acromegaly-induced dilated cardiomyopathy.

    Science.gov (United States)

    Sue, Mariko; Yoshihara, Aya; Okubo, Yoichiro; Ishikawa, Mayumi; Ando, Yasuyo; Hiroi, Naoki; Shibuya, Kazutoshi; Yoshino, Gen

    2010-01-01

    Acromegaly is characterized by chronic hypersecretion of growth hormone (GH) and is associated with increased mortality rate because of the potential complications such as cardiovascular disease, respiratory disease, or malignancy, which are probably caused by the long-term exposure of tissues to excess GH, for at least 10 years, before diagnosis and treatment. A 22-year-old man with a 2-month history of fatigue was admitted to our hospital because of chest discomfort, dyspnea, and pitting edema of the lower limbs experienced over a 1-month period. On admission, his height and body weight were 186 cm and 138.5 kg, respectively, with a BMI of 39.8 kg/m(2). He showed acromegalic features and elevated serum GH and IGF-1 levels, which were 11.5 ng/mL and 960 ng/mL, respectively. There was no GH suppression in the 75-g oral glucose tolerance test. Pituitary magnetic resonance imaging (MRI) revealed microadenoma. Chest X-ray revealed cardiomegaly, and echocardiogram showed dilated left ventricular (LV) cavity and diffuse hypokinesis with extremely decreased ejection fraction (EF). He was diagnosed as having acromegaly with congestive heart failure from diastolic cardiomyopathy. After the successful transsphenoidal resection of the pituitary adenoma, the level of GH was normalized. However, the cardiac dysfunction did not show any improvement even after the administration of β-blockers, angiotensin-converting enzyme inhibitor (ACE-I), or diuretics. The patient was re-hospitalized, and he died of cardiac failure at the age of 25 years. Patients with acromegaly have been reported to have about 30% higher mortality rate, and cardiovascular disease accounts for 60% of the deaths. We report a case of a patient with juvenile acromegaly who was diagnosed with severe cardiac failure at the time of diagnosis and failed to recover cardiac function even after the successful resection of the pituitary adenoma. Immediate diagnosis and treatment are required for better control of

  6. [Ultrastructural changes of neutrophilic granulocytes in dilated cardiomyopathy and their dynamics after blood irradiation with Helium-Neon laser in vitro].

    Science.gov (United States)

    Khomeriki, S G; Morozov, I A

    1998-01-01

    Venous blood from 10 patients with dilated cardiomyopathy was irradiated with a laser in vitro. The control group consisted of 20 healthy donors. The neutrophil granulocytes were separated at gradient centrifugation. Alterations of neutrophils manifested with an increase of specific cytoplasmic granules number, thickening of submembrane actin, cell configuration changes with a relative increase of their surface. Laser irradiation of the blood resulted in destruction of the altered (less resistant) cells while morphometric parameters of the remaining cells approaches those of donor cells. Thus, low-intensity laser irradiation results in the renewal of the neutrophil population in patients with dilated cardiomyopathy and normalization of structural-functional changes in the circulating neutrophil population.

  7. Dilated Cardiomyopathy Mutation (R134W) in Mouse Cardiac Troponin T Induces Greater Contractile Deficits against α-Myosin Heavy Chain than against β-Myosin Heavy Chain

    OpenAIRE

    Gollapudi, Sampath K.; Murali Chandra

    2016-01-01

    Many studies have demonstrated that depressed myofilament Ca2+ sensitivity is common to dilated cardiomyopathy (DCM) in humans. However, it remains unclear whether a single determinant — such as myofilament Ca2+ sensitivity — is sufficient to characterize all cases of DCM because the severity of disease varies widely with a given mutation. Because dynamic features dominate in the heart muscle, alterations in dynamic contractile parameters may offer better insight on the molecular mechanisms...

  8. DNA sequence and haplotype variation in two candidate genes for dilated cardiomyopathy in the turkey Meleagris gallopavo.

    Science.gov (United States)

    Lin, Kuan-chin; Xu, Jun; Kamara, Davida; Geng, Tuoyu; Gyenai, Kwaku; Reed, Kent M; Smith, Edward J

    2007-05-01

    Determining variation in genes is fundamental to understanding their function in the disease state. Cardiac troponin T (cTnT) and phospholamban (PLN) genes have been implicated in dilated cardiomyopathy (DCM) in human and model species. To investigate the role of these 2 candidate genes in DCM in the turkey Meleagris gallopavo, understanding sequence variants and map position distribution is necessary. To this end, a total of 1854 and 1771 bp of cTnT and PLN gene sequences, respectively, were scanned for single nucleotide polymorphisms (SNPs) in a randomly bred population. A total of 15 SNPs was identified in the cTnT and PLN genomic sequences. Nine haplotypes, 5 in cTnT and 4 in PLN, were identified. Observed heterozygosities (0.02-0.39) in the turkey population were low for both genes. Within each gene, 1 SNP corresponding to a restriction enzyme site was identified and used to develop a PCR-restriction fragment length polymorphism (RFLP) genotyping assay. The PLN gene was genetically mapped to turkey chromosome 2, equivalent to Gallus gallus chromosome 3, and cTnT mapped to a turkey microchromosome. Although limited because of the relatively small sample size of 55 birds, the data from this SNP analysis of PLN and cTnT provide a foundation from which to evaluate the function of cTnT and PLN in the turkey. Information about the distribution of the SNPs and haplotypes will facilitate future association and linkage studies.

  9. Antioxidants Improve the Phenotypes of Dilated Cardiomyopathy and Muscle Fatigue in Mitochondrial Superoxide Dismutase-Deficient Mice

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    Takahiko Shimizu

    2013-01-01

    Full Text Available Redox imbalance elevates the reactive oxygen species (ROS level in cells and promotes age-related diseases. Superoxide dismutases (SODs are antioxidative enzymes that catalyze the degradation of ROS. There are three SOD isoforms: SOD1/CuZn-SOD, SOD2/Mn-SOD, and SOD3/EC-SOD. SOD2, which is localized in the mitochondria, is an essential enzyme required for mouse survival, and systemic knockout causes neonatal lethality in mice. To investigate the physiological function of SOD2 in adult mice, we generated a conditional Sod2 knockout mouse using a Cre-loxP system. When Sod2 was specifically deleted in the heart and muscle, all mice exhibited dilated cardiomyopathy (DCM and died by six months of age. On the other hand, when Sod2 was specifically deleted in the skeletal muscle, mice showed severe exercise disturbance without morphological abnormalities. These provide useful model of DCM and muscle fatigue. In this review, we summarize the impact of antioxidants, which were able to regulate mitochondrial superoxide generation and improve the phenotypes of the DCM and the muscle fatigue in mice.

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

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    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.

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

    Science.gov (United States)

    Panikkath, Ragesh; Panikkath, Deepa; Sanchez-Iglesias, S.; Araujo-Vilar, D; Lado-Abeal, Joaquin

    2016-01-01

    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. PMID:27504462

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

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    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

  13. Randomized Comparison of Allogeneic Vs. Autologous Mesenchymal Stem Cells for Non-lschemic Dilated Cardiomyopathy: POSEIDON-DCM Trial

    Science.gov (United States)

    Hare, Joshua M.; DiFede, Darcy L; Castellanos, Angela M; Florea, Victoria; Landin, Ana M; El-Khorazaty, Jill; Khan, Aisha; Mushtaq, Muzammil; Lowery, Maureen H; Byrnes, John J; Hendel, Robert C; Cohen, Mauricio G; Alfonso, Carlos E; Valasaki, Krystalenia; Pujol, Marietsy V; Golpanian, Samuel; Ghersin, Eduard; Fishman, Joel E; Pattany, Pradip; Gomes, Samirah A; Delgado, Cindy; Miki, Roberto; Abuzeid, Fouad; Vidro-Casiano, Mayra; Premer, Courtney; Medina, Audrey; Porras, Valeria; Hatzistergos, Konstantinos E.; Anderson, Erica; Mendizabal, Adam; Mitrani, Raul; Heldman, Alan W.

    2016-01-01

    Background While human mesenchymal stem cells (hMSCs) have been tested in ischemic cardiomyopathy, few studies exist in chronic non-ischemic dilated cardiomyopathy (NIDCM). Objectives The POSEIDON-DCM trial is a randomized comparison of safety and efficacy of autologous (auto) vs. allogeneic (allo) bone marrow-derived hMSCs in NIDCM. Methods Thirty-seven patients were randomized to either allo- or auto-hMSCs in a 1:1 ratio. Patients were recruited between December 2011 and July 2015 at the University of Miami Hospital. Patients (age: 55.8 ± 11.2; 32% female) received hMSCs (100 million) by transendocardial stem cell injection (TESI) in ten left ventricular sites by NOGA Catheter. Treated patients were evaluated at baseline, 30 days, 3-, 6-, and 12-months for safety: serious adverse events (SAE), and efficacy endpoints: Ejection Fraction (EF), Minnesota Living with Heart Failure Questionnaire (MLHFQ), Six Minute Walk Test (6MWT), MACE, and immune-biomarkers. This trial is registered with ClinicalTrials.gov, #NCT01392625. Results There were no 30-day treatment-emergent (TE)-SAEs. 12-month SAE incidence was 28.2% (95% CI: 12.8, 55.1) in allo, and 63.5% (95% CI: 40.8, 85.7; p=0.1004) in auto. One allo-group patient developed an elevated donor specific cPRA. EF increased in allo by 8.0 units (95% Cl: 2.8, 13.2; p=0.004), and in auto: 5.4 units (95% Cl: −1.4, 12.1; p=0.116, allo vs. auto p=0.4887). 6MWT increased for allo: 37.0 meters (95% Cl: 2.0 to 72.0; p=0.04), but not auto: 7.3 meters (95% Cl: −47.8, 33.3; p=0.71, auto vs. allo p=0.0168). MLHFQ score decreased in allo (p=0.0022), and auto (p=0.463; p=0.172). The MACE rate was lower in allo vs. auto (p=0.0186). Tumor necrosis factor alpha (TNF-α) decreased (p=0.0001 for each), to a greater extent in allo vs. auto at six-months (p=0.05). Conclusion These findings demonstrate safety and support greater, clinically meaningful efficacy of allo-hMSC vs. auto-hMSC in NIDCM patients. Pivotal trials of allo-hMSCs are

  14. 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

  15. Preliminary clinical study of left ventricular myocardial strain in patients with non-ischemic dilated cardiomyopathy by three-dimensional speckle tracking imaging

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    Duan Fengxia

    2012-03-01

    Full Text Available Abstract Background Non-ischemic dilated cardiomyopathy (DCM is the most common cardiomyopathy worldwide, with significant mortality. Correct evaluation of the patient's myocardial function has important clinical significance in the diagnosis, therapeutic effect assessment and prognosis in non-ischemic DCM patients. This study evaluated the feasibility of three-dimensional speckle tracking imaging (3D-STE for assessment of the left ventricular myocardial strain in patients with non-ischemic dilated cardiomyopathy (DCM. Methods Apical full-volume images were acquired from 65 patients with non-ischemic DCM (DCM group and 59 age-matched normal controls (NC group, respectively. The following parameters were measured by 3D-STE: the peak systolic radial strain (RS, circumferential strain (CS, longitudinal strain (LS of each segment. Then all the parameters were compared between the two groups. Results The peak systolic strain in different planes had certain regularities in normal groups, radial strain (RS was the largest in the mid region, the smallest in the apical region, while circumferential strain (CS and longitudinal strain (LS increased from the basal to the apical region. In contrast, the regularity could not be applied to the DCM group. RS, CS, LS were significantly decreased in DCM group as compared with NC group (P Conclusions 3D-STE is a reliable tool for evaluation of left ventricular myocardial strain in patients with non-ischemic DCM, with huge advantage in clinical application.

  16. HEART REMODELING AT DIFFERENT STAGES OF CHRONIC HEART FAILURE IN PATIENTS WITH POSTINFARCTION CARDIOSCLEROSIS AND DILATED CARDIOMYOPATHY

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    V. V. Mazur

    2010-01-01

    Full Text Available Aim. To study the features of cardiac remodeling in patients with dilated cardiomyopathy (DCM and postinfarction cardiosclerosis (PICS, that can be used for differential diagnosis of these diseases.Material and methods. Patients with DCM (27 men and 5 women; aged 43.1±2.3 and patients with PICS (62 men; aged 56.4±1.1 and chronic heart failure (CHF were included in the study. The diagnosis of DCM was based on clinical investigation, which also includes coronary angiography. The diagnosis of DCM in 19 patients was proven by the results of postmortem investigation. The diagnosis of PICS was based on documented history of myocardial infarction, ECG and echocardiographic sings. Echocardiography was performed in all patients and 14 healthy volunteers.Results. End-systolic size (ESS of left ventricular (LV in patients with DCM and PICS at I (respectively 7.60±0.17 and 7.94±0.18 cm, IIA (7.66±0.28 and 8.64±0.30 cm and IIB stages of CHF (8.26±0.28 and 8.94±0.15 cm was significantly more than this in healthy volunteers (6.36±0.16, all p<0.01. ESS of right ventricular (RV in DCM patients of the same CHF stages (respectively 7.21±0.22, 7.40±0.27 and 8.23±0.27 cm is also more than this in healthy volunteers (5.95±0.17, all p<0.01. ESS RV in PICS patients at I (5.40±0.11 cm and IIA (5.80±0.26 cm CHF stages did not differ from healthy volunteers, and this index risen to IIB stage (6.62±0.21 cm, but was lower than in DCM patients.The ESS LV/ESS RV ratio at any CHF stage in PICS patients was significantly higher than this in DCM patients (1.48±0.04 and 1.06±0.02, 1.50±0.05 and 1.04±0.02, 1.37±0,06 and 1.00±0.01, respectively.Conclusion. The ESS LV/ESS RV ratio can be used for differential diagnosis of dilatation in DCM and PICS patients.

  17. Knock-in Mice Harboring a Ca2+ Desensitizing Mutation in Cardiac Troponin C Develop Early Onset Dilated Cardiomyopathy

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    Bradley K McConnell

    2015-08-01

    Full Text Available The physiological consequences of aberrant Ca2+ binding and exchange with cardiac myofilaments are not clearly understood. In order to examine the effect of decreasing Ca2+ sensitivity of cTnC on cardiac function, we generated knock-in mice carrying a D73N mutation (not known to be associated with heart disease in human patients in cTnC. The D73N mutation was engineered into the regulatory N-domain of cTnC in order to reduce Ca2+ sensitivity of reconstituted thin filaments by increasing the rate of Ca2+ dissociation. In addition, the D73N mutation drastically blunted the extent of Ca2+ desensitization of reconstituted thin filaments induced by cTnI pseudo-phosphorylation. Compared to wild-type mice, heterozygous knock-in mice carrying the D73N mutation exhibited a substantially decreased Ca2+ sensitivity of force development in skinned ventricular trabeculae. Kaplan-Meier survival analysis revealed that median survival time for knock-in mice was twelve weeks. Echocardiographic analysis revealed that knock-in mice exhibited increased left ventricular dimensions with thinner walls. Echocardiographic analysis also revealed that measures of systolic function, such as ejection fraction and fractional shortening, were dramatically reduced in knock-in mice. In addition, knock-in mice displayed electrophysiological abnormalities, namely prolonged QRS and QT intervals. Furthermore, ventricular myocytes isolated from knock-in mice did not respond to β-adrenergic stimulation. Thus, knock-in mice developed pathological features similar to those observed in human patients with dilated cardiomyopathy (DCM. In conclusion, our results suggest that decreasing Ca2+ sensitivity of the regulatory N-domain of cTnC is sufficient to trigger the development of DCM.

  18. Heart mitochondrial proteome study elucidates changes in cardiac energy metabolism and antioxidant PRDX3 in human dilated cardiomyopathy.

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    Esther Roselló-Lletí

    Full Text Available Dilated cardiomyopathy (DCM is a public health problem with no available curative treatment, and mitochondrial dysfunction plays a critical role in its development. The present study is the first to analyze the mitochondrial proteome in cardiac tissue of patients with DCM to identify potential molecular targets for its therapeutic intervention.16 left ventricular (LV samples obtained from explanted human hearts with DCM (n = 8 and control donors (n = 8 were extracted to perform a proteomic approach to investigate the variations in mitochondrial protein expression. The proteome of the samples was analyzed by quantitative differential electrophoresis and Mass Spectrometry. These changes were validated by classical techniques and by novel and precise selected reaction monitoring analysis and RNA sequencing approach increasing the total heart samples up to 25. We found significant alterations in energy metabolism, especially in molecules involved in substrate utilization (ODPA, ETFD, DLDH, energy production (ATPA, other metabolic pathways (AL4A1 and protein synthesis (EFTU, obtaining considerable and specific relationships between the alterations detected in these processes. Importantly, we observed that the antioxidant PRDX3 overexpression is associated with impaired ventricular function. PRDX3 is significantly related to LV end systolic and diastolic diameter (r = 0.73, p value<0.01; r = 0.71, p value<0.01, fractional shortening, and ejection fraction (r = -0.61, p value<0.05; and r = -0.62, p value<0.05, respectively.This work could be a pivotal study to gain more knowledge on the cellular mechanisms related to the pathophysiology of this disease and may lead to the development of etiology-specific heart failure therapies. We suggest new molecular targets for therapeutic interventions, something that up to now has been lacking.

  19. 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.

  20. EFFECTS OF EPCs OR b-FGF INTRAMYOCARDIAL INFUSION ON CARDIAC FUNCTION AND NEOVASCULARIZATION FOR DILATED CARDIOMYOPATHY RATS

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    ZHANG Xin; WEI Meng; YAN Xiao-yu

    2008-01-01

    Objective To compare the different effects of endothelia progenitor cells (EPCs) or basic fibroblast growth factor (b-FGF) intromyocardial infusion on cardiac function and neovascularization for dilated cardiomyopathy(DCM)rats.Methods Fifty adult female rats received inguinal subcutaneous injections of isoproterenol (ISO, 250 mg/kg) for induction of DCM. Four weeks later, the model rats were randomly divided into EPCs group, b-FGF group and control group. The 2×106 EPCs (resolved in 100 μL PBS), 100 μL b-FGF (100 μg/mL) and 100 μL PBS were evenly transplanted into the myocardium of EPCs group, b-FGF group and control group, respectively. Three months later, echocardiographic examination and regional myocardial blood flow (RMBF)measurement were performed. EPCs were traced by fluorescence in situ hybridization (FISH). The protein and mRNA expression of b-FGF in each group was measured by ELISA assay and reverse transcription-polymerase chain reaction (RT-PCR).Results Three months after transplantation, sry positive cells were detected only in EPCs group. The cardiac function as well as RMBF was significantly improved in EPCs group compared with b-FGF group or control group. There was higher capillary density in EPCs group. The protein and mRNA expression of b-FGF was stronger than b-FGF group and control group.Conclusion Transplantation of EPCs can improve cardiac function, induce neovascularization and increase RMBF for DCM rats. The treatment with EPCs has better effect than administration of b-FGF alone.

  1. Can Serum Tenascin-C Be Used as a Marker of Inflammation in Patients with Dilated Cardiomyopathy?

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    Alyaa A. Kotby

    2013-01-01

    Full Text Available Background. Tenascin-C (TN-C is an extracellular matrix glycoprotein that appears at sites of inflammation in cardiac pathologies. Aim of the Work. To evaluate the role of TN-C as a marker for active inflammation in children with dilated cardiomyopathy (DCM. Subjects and Methods. 24 consecutive patients with primary nonfamilial DCM aged 6–72 months (mean 45.19±11.03 were divided into group I, twelve patients with acute onset DCM (6 months duration, and compared to 20 healthy age- and sex-matched controls. Investigations included estimation of serum TN-C and echocardiographic evaluation using M-mode and 2D speckle tracking echocardiography (STE. Results. Serum TN-C showed a higher significant statistical elevation among patients than controls (P<0.001 and in group I than group II (P<0.001. EF was significantly decreased, and LVEDD and EDV increased in patients than controls and in GI than GII. STE showed a statistically significant difference in global peak strain longitudinal (GPSL average in patients than controls (P<0.05 and between GI and GII (P<0.001. STE wall motion scoring showed normokinesia (33.5%, hypokinesia (8.33%, and akinesia (50% in GI and hypokinesia (100% in GII. There was a statistically significant positive correlation between serum TN-C and GPSL average. Conclusions. Increased serum TN-C can be used as a marker of inflammation in DCM and is associated with the severity of heart failure and LV dysfunction as detected by STE.

  2. A proton leak current through the cardiac sodium channel is linked to mixed arrhythmia and the dilated cardiomyopathy phenotype.

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    Pascal Gosselin-Badaroudine

    Full Text Available Cardiac Na(+ channels encoded by the SCN5A gene are essential for initiating heart beats and maintaining a regular heart rhythm. Mutations in these channels have recently been associated with atrial fibrillation, ventricular arrhythmias, conduction disorders, and dilated cardiomyopathy (DCM.We investigated a young male patient with a mixed phenotype composed of documented conduction disorder, atrial flutter, and ventricular tachycardia associated with DCM. Further family screening revealed DCM in the patient's mother and sister and in three of the mother's sisters. Because of the complex clinical phenotypes, we screened SCN5A and identified a novel mutation, R219H, which is located on a highly conserved region on the fourth helix of the voltage sensor domain of Na(v1.5. Three family members with DCM carried the R219H mutation.The wild-type (WT and mutant Na(+ channels were expressed in a heterologous expression system, and intracellular pH (pHi was measured using a pH-sensitive electrode. The biophysical characterization of the mutant channel revealed an unexpected selective proton leak with no effect on its biophysical properties. The H(+ leak through the mutated Na(v1.5 channel was not related to the Na(+ permeation pathway but occurred through an alternative pore, most probably a proton wire on the voltage sensor domain.We propose that acidification of cardiac myocytes and/or downstream events may cause the DCM phenotype and other electrical problems in affected family members. The identification of this clinically significant H(+ leak may lead to the development of more targeted treatments.

  3. A Novel Murine Model of Parvovirus Associated Dilated Cardiomyopathy Induced by Immunization with VP1-Unique Region of Parvovirus B19

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    Šimoliūnas, Egidijus; Rinkūnaitė, Ieva; Smalinskaitė, Luka; Podkopajev, Andrej; Bironaitė, Daiva; Weis, Cleo-Aron; Marx, Alexander; Bukelskienė, Virginija; Gretz, Norbert; Grabauskienė, Virginija; Labeit, Dittmar; Labeit, Siegfried

    2016-01-01

    Background. Parvovirus B19 (B19V) is a common finding in endomyocardial biopsy specimens from myocarditis and dilated cardiomyopathy patients. However, current understanding of how B19V is contributing to cardiac damage is rather limited due to the lack of appropriate mice models. In this work we demonstrate that immunization of BALB/c mice with the major immunogenic determinant of B19V located in the unique sequence of capsid protein VP1 (VP1u) is an adequate model to study B19V associated heart damage. Methods and Results. We immunized mice in the experimental group with recombinant VP1u; immunization with cardiac myosin derived peptide served as a positive reference and phosphate buffered saline served as negative control. Cardiac function and dimensions were followed echocardiographically 69 days after immunization. Progressive dilatation of left ventricle and decline of ejection fraction were observed in VP1u- and myosin-immunized mice. Histologically, severe cardiac fibrosis and accumulation of heart failure cells in lungs were observed 69 days after immunization. Transcriptomic profiling revealed ongoing cardiac remodeling and immune process in VP1u- and myosin-immunized mice. Conclusions. Immunization of BALB/c mice with VP1u induces dilated cardiomyopathy in BALB/c mice and it could be used as a model to study clinically relevant B19V associated cardiac damage. PMID:27812527

  4. 扩张型心肌病心房颤动预后研究%Impact of atrial fibrillation on the prognostic significance in patients with dilated cardiomyopathy

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    汪剑锋; 罗博哲; 李智; 卢喜烈

    2011-01-01

    Objective To assess the clinical and echocardiographic characteristics of atrial fibrillation in patients with dilated cardiomyopathy, and evaluate the prognostic significance. Methods A total of 173 patients with dilated cardiomyopathy were divided into atrial fibrillation group (w = 62) and non-atrial fibrillation group(n= 111). The clinical and echocardiographic characteristics, cardiac death rates and cerebral embolism events were evaluated. Results The incidence of atrial fibrillation was 35. 8% in atrial fibrillation group. The index of male proportion, the ventricular rate, hypertension incidences, QRS time limit, left ventricular end-systolic dimension, left ventricular end-systolic volume, left atrial diameter, the cerebral embolism rate and cardiac death rate were higher in atrial fibrillation group than those in non-atrial fibrillation group (P<0. 05, P<0. 01). Logistic regression analysis showed atrial fibrillation was an independent risk factor for cardiac death ( P < 0.01). Conclusion Atrial fibrillation increases the incidence of cerebral embolism events in patients with dilated cardiomyopathy, and is an independent risk factor for cardiac death.%目的 了解扩张型心肌病( dilated cardiomyopathy,DCM)合并心房颤动(atrial fibrillation,AF)的心脏超声和临床特征,评价AF对DCM患者的预后意义.方法 DCM患者173例,分为AF组和非AF组,比较2组心脏超声、临床特征及脑栓塞发生率和心源性病死率.结果 AF组62例,AF发生率35.8% ;AF组男性比例、心室率、高血压比例、QRS时限、左心室收缩末直径、左心室收缩末容量、左心房直径、脑栓塞发生率及心源性病死率均高于非AF组(P<0.05,P<0.01);Logistic回归分析显示AF是DCM患者心源性死亡的独立危险因素.结论 DCM患者并发AF时脑栓塞发生率增高,是心源性死亡的独立预测因素.

  5. Dilated cardiomyopathy and left bundle branch block associated with ingestion of colloidal gold and silver is reversed by British antiLewisite and vitamin E: the potential toxicity of metals used as health supplements.

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    Archer, Stephen Lawrence

    2008-05-01

    A case of left bundle branch block and a dilated, nonhypertrophic cardiomyopathy associated with ingestion of colloidal gold and silver as an 'energy tonic' is described. The cardiac disease was reversed within two months by a course of dimercaprol (Akorn Inc, USA) (British antiLewisite) and vitamin E. This is the first case of gold and silver cardiomyopathy in humans, and highlights the risks of these colloidal metal 'health supplements'.

  6. Virus-induced dilated cardiomyopathy is characterized by increased levels of fibrotic extracellular matrix proteins and reduced amounts of energy-producing enzymes.

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    Nishtala, Krishnatej; Phong, Truong Q; Steil, Leif; Sauter, Martina; Salazar, Manuela G; Kandolf, Reinhard; Kroemer, Heyo K; Felix, Stephan B; Völker, Uwe; Klingel, Karin; Hammer, Elke

    2011-11-01

    The most relevant clinical phenotype resulting from chronic enteroviral myocarditis is dilated cardiomyopathy (DCM). Mice of the susceptible mouse strain A.BY/SnJ mimick well human DCM since they develop as a consequence of persistent infection and chronic inflammation a dilation of the heart ventricle several weeks after coxsackievirus B3 (CVB3) infection. Therefore, this model is well suited for the analysis of changes in the heart proteome associated with DCM. Here, we present a proteomic survey of the dilated hearts based on differential fluorescence gel electrophoresis and liquid chromatography-mass spectrometric centered methods in comparison to age-matched non-infected hearts. In total, 101 distinct proteins, which belong to categories immunity and defense, cell structure and associated proteins, energy metabolism and protein metabolism/modification differed in their levels in both groups. Levels of proteins involved in fatty acid metabolism and electron transport chain were found to be significantly reduced in infected mice suggesting a decrease in energy production in CVB3-induced DCM. Furthermore, proteins associated with muscle contraction (MLRV, MLRc2, MYH6, MyBPC3), were present in significantly altered amounts in infected mice. A significant increase in the level of extracellular matrix proteins in the dilated hearts indicates cardiac remodeling due to fibrosis.

  7. 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

  8. Multistep ion channel remodeling and lethal arrhythmia precede heart failure in a mouse model of inherited dilated cardiomyopathy.

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    Takeshi Suzuki

    Full Text Available BACKGROUND: Patients with inherited dilated cardiomyopathy (DCM frequently die with severe heart failure (HF or die suddenly with arrhythmias, although these symptoms are not always observed at birth. It remains unclear how and when HF and arrhythmogenic changes develop in these DCM mutation carriers. In order to address this issue, properties of the myocardium and underlying gene expressions were studied using a knock-in mouse model of human inherited DCM caused by a deletion mutation ΔK210 in cardiac troponinT. METHODOLOGY/PRINCIPAL FINDINGS: By 1 month, DCM mice had already enlarged hearts, but showed no symptoms of HF and a much lower mortality than at 2 months or later. At around 2 months, some would die suddenly with no clear symptoms of HF, whereas at 3 months, many of the survivors showed evident symptoms of HF. In isolated left ventricular myocardium (LV from 2 month-mice, spontaneous activity frequently occurred and action potential duration (APD was prolonged. Transient outward (I(to and ultrarapid delayed rectifier K(+ (I(Kur currents were significantly reduced in DCM myocytes. Correspondingly, down-regulation of Kv4.2, Kv1.5 and KChIP2 was evident in mRNA and protein levels. In LVs at 3-months, more frequent spontaneous activity, greater prolongation of APD and further down-regulation in above K(+ channels were observed. At 1 month, in contrast, infrequent spontaneous activity and down-regulation of Kv4.2, but not Kv1.5 or KChIP2, were observed. CONCLUSIONS/SIGNIFICANCE: Our results suggest that at least three steps of electrical remodeling occur in the hearts of DCM model mice, and that the combined down-regulation of Kv4.2, Kv1.5 and KChIP2 prior to the onset of HF may play an important role in the premature sudden death in this DCM model. DCM mice at 1 month or before, on the contrary, are associated with low risk of death in spite of inborn disorder and enlarged heart.

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

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    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.

  10. Assessment of right ventricular oxidative metabolism by PET in patients with idiopathic dilated cardiomyopathy undergoing cardiac resynchronisation therapy

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    Knuuti, Juhani; Naum, Alexandru; Stolen, Kira Q.; Kalliokoski, Riikka [University of Turku, Turku PET Centre, P.O. Box 52, Turku (Finland); Sundell, Jan [University of Turku, Turku PET Centre, P.O. Box 52, Turku (Finland); University of Turku, Department of Medicine, Turku (Finland); Engblom, Erik; Koistinen, Juhani; Airaksinen, K.E. Juhani [University of Turku, Department of Medicine, Turku (Finland); Ylitalo, Antti [Satakunta Central Hospital, Department of Medicine, Pori (Finland); Nekolla, Stephan G. [Klinikum rechts der Isar der Technischen Universitaet Muenchen, Klinik und Poliklinik fuer Nuklearmedizin, Munich (Germany); Bax, K.E. Jeroen J. [Leiden University, Department of Cardiology, Leiden (Netherlands)

    2004-12-01

    Right ventricular (RV) performance is known to have prognostic value in patients with congestive heart failure (CHF). Cardiac resynchronisation therapy (CRT) has been found to enhance left ventricular (LV) energetics and metabolic reserve in patients with heart failure. The interplay between the LV and RV may play an important role in CRT response. The purpose of the study was to investigate RV oxidative metabolism, metabolic reserve and the effects of CRT in patients with CHF and left bundle brach block. In addition, the role of the RV in the response to CRT was evaluated. Ten patients with idiopathic dilated cardiomyopathy who had undergone implantation of a biventricular pacemaker 8{+-}5 months earlier were studied under two conditions: CRT ON and after CRT had been switched OFF for 24 h. Oxidative metabolism was measured using [{sup 11}C]acetate positron emission tomography (K{sub mono}). The measurements were performed at rest and during dobutamine-induced stress (5 {mu}g/kg per minute). LV performance and interventricular mechanical delay (interventricular asynchrony) were measured using echocardiography. CRT had no effect on RV K{sub mono} at rest (ON: 0.052{+-}0.014, OFF: 0.047{+-}0.018, NS). Dobutamine-induced stress increased RV K{sub mono} significantly under both conditions but oxidative metabolism was more enhanced when CRT was ON (0.076{+-}0.026 vs 0.065{+-}0.027, p=0.003). CRT shortened interventricular delay significantly (45{+-}33 vs 19{+-}35 ms, p=0.05). In five patients the response to CRT was striking (32% increase in mean LV stroke volume, range 18-36%), while in the other five patients no response was observed (mean change +2%, range -6% to +4%). RV K{sub mono} and LV stroke volume response to CRT correlated inversely (r=-0.66, p=0.034). None of the other measured parameters, including all LV parameters and electromechanical parameters, were associated with the response to CRT. In responders, RV K{sub mono} with CRT OFF was significantly lower

  11. Modeling structural and functional deficiencies of RBM20 familial dilated cardiomyopathy using human induced pluripotent stem cells.

    Science.gov (United States)

    Wyles, Saranya P; Li, Xing; Hrstka, Sybil C; Reyes, Santiago; Oommen, Saji; Beraldi, Rosanna; Edwards, Jessica; Terzic, Andre; Olson, Timothy M; Nelson, Timothy J

    2016-01-15

    Dilated cardiomyopathy (DCM) is a leading cause of heart failure. In families with autosomal-dominant DCM, heterozygous missense mutations were identified in RNA-binding motif protein 20 (RBM20), a spliceosome protein induced during early cardiogenesis. Dermal fibroblasts from two unrelated patients harboring an RBM20 R636S missense mutation were reprogrammed to human induced pluripotent stem cells (hiPSCs) and differentiated to beating cardiomyocytes (CMs). Stage-specific transcriptome profiling identified differentially expressed genes ranging from angiogenesis regulator to embryonic heart transcription factor as initial molecular aberrations. Furthermore, gene expression analysis for RBM20-dependent splice variants affected sarcomeric (TTN and LDB3) and calcium (Ca(2+)) handling (CAMK2D and CACNA1C) genes. Indeed, RBM20 hiPSC-CMs exhibited increased sarcomeric length (RBM20: 1.747 ± 0.238 µm versus control: 1.404 ± 0.194 µm; P < 0.0001) and decreased sarcomeric width (RBM20: 0.791 ± 0.609 µm versus control: 0.943 ± 0.166 µm; P < 0.0001). Additionally, CMs showed defective Ca(2+) handling machinery with prolonged Ca(2+) levels in the cytoplasm as measured by greater area under the curve (RBM20: 814.718 ± 94.343 AU versus control: 206.941 ± 22.417 AU; P < 0.05) and higher Ca(2+) spike amplitude (RBM20: 35.281 ± 4.060 AU versus control:18.484 ± 1.518 AU; P < 0.05). β-adrenergic stress induced with 10 µm norepinephrine demonstrated increased susceptibility to sarcomeric disorganization (RBM20: 86 ± 10.5% versus control: 40 ± 7%; P < 0.001). This study features the first hiPSC model of RBM20 familial DCM. By monitoring human cardiac disease according to stage-specific cardiogenesis, this study demonstrates RBM20 familial DCM is a developmental disorder initiated by molecular defects that pattern maladaptive cellular mechanisms of pathological cardiac remodeling. Indeed, hiPSC-CMs recapitulate RBM20 familial DCM phenotype in a dish and establish a tool

  12. FTY720 (Gilenya) treatment prevents spontaneous autoimmune myocarditis and dilated cardiomyopathy in transgenic HLA-DQ8-BALB/c mice.

    Science.gov (United States)

    Boldizsar, Ferenc; Tarjanyi, Oktavia; Olasz, Katalin; Hegyi, Akos; Mikecz, Katalin; Glant, Tibor T; Rauch, Tibor A

    2016-01-01

    Although dilated cardiomyopathy (DCM) is often caused by viral infections, it frequently involves autoimmune mechanisms associated with particular HLA-DR and DQ alleles. Our homozygous HLA-DQ8Ab(0) transgenic mice in the BALB/c background (HLA-DQ8(BALB/c)-Tg) developed early and progressive fatal heart failure from 4 to 5 weeks of age. Clinical signs of the disease included cyanotic eyes, tachycardia with dyspnea (from pale to cyanotic limbs), and terminal whole body edema. Sick mice had extremely dilated hearts, enlarged liver and spleen, and pleural/peritoneal effusion. Histology of the heart showed extensive heart muscle destruction with signs of fibrosis. The autoimmune nature of the disease was shown by high titers of antimyosin antibodies in the sera and IgG deposits in sick heart muscles, as well as focal neutrophil, T cell, and macrophage infiltration of the heart muscle. The sera of the sick mice showed a granular staining pattern on sections of healthy heart muscle. Quantitative analyses of DCM-specific gene expression studies revealed that sets of genes are involved in inflammation, hypoxia, and fibrosis. Treatment with FTY720 (Fingolimod/Gilenya) protected animals from the development of cardiomyopathy. HLA-DQ8(BALB/c)-Tg mice represent a spontaneous autoimmune myocarditis model that may provide a useful tool for studying the autoimmune mechanism of DCM and testing immunosuppressive drugs.

  13. Systematic review of pregnancy in women with inherited cardiomyopathies

    NARCIS (Netherlands)

    Krul, Sebastien P. J.; van der Smagt, Jasper J.; van den Berg, Maarten P.; Sollie, Krystyna M.; Pieper, Petronella G.; van Spaendonck-Zwarts, Karin Y.

    2011-01-01

    Pregnancy exposes women with inherited cardiomyopathies to increased risk for heart failure and arrhythmias. In this paper, we review the clinical course and management of pregnant women with the following inherited cardiomyopathies: hypertrophic cardiomyopathy, dilated cardiomyopathy, arrhythmogeni

  14. Pregnancy, cardiomyopathies, and genetics

    NARCIS (Netherlands)

    Van Tintelen, J. Peter; Pieper, Petronella G.; Van Spaendonck-Zwarts, Karin Y.; Van den Berg, Maarten P.

    2014-01-01

    Although familial forms of cardiomyopathy such as hypertrophic or dilated cardiomyopathy have been recognized for decades, it is only recently that much of the genetic basis of these inherited cardiomyopathies has been elucidated. This has provided important insights into the pathophysiological mech

  15. Dasatinib and Prednisolone Induction Therapy for a Case of Philadelphia Chromosome-Positive Acute Lymphoblastic Leukemia with Dilated Cardiomyopathy Accompanied by Life-Threatening Ventricular Tachycardia

    Directory of Open Access Journals (Sweden)

    Mitsutaka Nishimoto

    2017-01-01

    Full Text Available A 56-year-old man being treated for dilated cardiomyopathy presented with epigastralgia. He was diagnosed with ventricular tachycardia and Philadelphia chromosome-positive acute lymphoblastic leukemia. After treating incessant ventricular tachycardia, we commenced induction therapy for leukemia with dasatinib and prednisolone to minimize toxicity towards cardiomyocytes and the cardiac conduction system. Although dasatinib was temporarily withheld because of a recurrence of ventricular tachycardia, we rechallenged dasatinib while using bisoprolol and amiodarone and achieved a complete hematological response three weeks later. Although drug interactions between dasatinib and amiodarone were of concern, the blood concentration of each drug remained within the safe range after concomitant use, and there were no adverse cardiac effects such as QT prolongation after rechallenging dasatinib. Induction therapy with dasatinib and prednisolone may be an acceptable therapeutic option for Philadelphia chromosome-positive acute lymphoblastic leukemia with severe cardiac complications.

  16. 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.

  17. CLINICAL AND MORPHOLOGICAL APPROACH TO DIAGNOSIS OF "IDIOPATHIC" ARRHYTHMIAS AND DILATED CARDIOMYOPATHY SYNDROME AS A BASIS FOR DIFFERENTIATED THERAPY. Part I (Diagnostics

    Directory of Open Access Journals (Sweden)

    O. V. Blagova

    2014-01-01

    Full Text Available PAim. 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% of IA and 66.7% of DCM-patients, 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, while in the main group 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 patients.Conclusion. Nosological cause of IA and DCM syndrome can be diagnosed in most patients by using an integrated clinical and morphological approach.

  18. CLINICAL AND MORPHOLOGICAL APPROACH TO DIAGNOSIS OF "IDIOPATHIC" ARRHYTHMIAS AND DILATED CARDIOMYOPATHY SYNDROME AS A BASIS FOR DIFFERENTIATED THERAPY. Part I (Diagnostics

    Directory of Open Access Journals (Sweden)

    O. V. Blagova

    2015-09-01

    Full Text Available PAim. 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% of IA and 66.7% of DCM-patients, 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, while in the main group 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 patients.Conclusion. Nosological cause of IA and DCM syndrome can be diagnosed in most patients by using an integrated clinical and morphological approach.

  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. Sex differences in cardiomyopathies

    NARCIS (Netherlands)

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

    2014-01-01

    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 (RC

  3. Early-progressive dilated cardiomyopathy in a family with Becker muscular dystrophy related to a novel frameshift mutation in the dystrophin gene exon 27.

    Science.gov (United States)

    Tsuda, Takeshi; Fitzgerald, Kristi; Scavena, Mena; Gidding, Samuel; Cox, Mary O; Marks, Harold; Flanigan, Kevin M; Moore, Steven A

    2015-03-01

    We report a family in which two male siblings with Becker muscular dystrophy (BMD) developed severe dilated cardiomyopathy (DCM) and progressive heart failure (HF) at age 11 years; one died at age 14 years while awaiting heart transplant and the other underwent left ventricular assist device implantation at the same age. Genetic analysis of one sibling showed a novel frameshift mutation in exon 27 of Duchenne muscular dystrophy (DMD) gene (c.3779_3785delCTTTGGAinsGG), in which seven base pairs are deleted and two are inserted. Although this predicts an amino-acid substitution and premature termination (p.Thr1260Argfs*8), muscle biopsy dystrophin immunostaining instead indicates that the mutation is more likely to alter splicing. Despite relatively preserved skeletal muscular performance, both the siblings developed progressive HF secondary to early-onset DCM. In addition, their 7-year-old nephew with delayed gross motor development, mild proximal muscle weakness and markedly elevated serum creatine kinase level (>13 000 IU l(-1)) at 16 months was recently demonstrated to have the familial DMD mutation. Here, we report a novel genotype of BMD with early-onset DCM and progressive lethal HF during early adolescence.

  4. 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.

  5. 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.

  6. Prognostic value of repeated {sup 123}I-metaiodobenzylguanidine imaging in patients with dilated cardiomyopathy with congestive heart failure before and after optimized treatments. Comparison with neurohumoral factors

    Energy Technology Data Exchange (ETDEWEB)

    Matsui, Toshiki; Tsutamoto, Takayoshi; Maeda, Keiko; Kusukawa, Junya; Kinoshita, Masahiko [Shiga Univ. of Medical Science, Otsu (Japan)

    2002-06-01

    The present study was undertaken to assess whether repeated measurement of cardiac {sup 123}I-metaiodobenzylguanidine (MIBG) imaging parameters before and after optimized treatments is useful for predicting the prognosis of patients with congestive heart failure (CHF) resulting from dilated cardiomyopathy (DCM). The subjects were 85 consecutive patients with DCM who had a left ventricular ejection fraction (LVEF) of less than 45%. The MIBG and the concentrations of neurohumoral factors were measured at baseline and after 6 months of optimized treatments. Cox proportional hazards analysis was performed to assess the various parameters before and after treatment. Twenty-three patients had a cardiac event (12 died; 11 hospitalized) during a mean follow-up period of 2 years. Although there was no difference between the baseline heart to mediastinum (H/M) ratio measured by MIBG between survivors and nonsurvivors, the H/M ratio was significantly decreased in nonsurvivors after 6 months. Multivariate analysis revealed that a high plasma concentration of brain natriuretic peptide level after 6 months (p=0.0049) and absolute changes in the H/M ratio (p=0.0046) were independent predictors of mortality. Comparison of the H/M ratio on MIBG imaging before and after optimized additional treatment provided useful information for predicting mortality and was independent of clinical and neurohumoral factors previously shown to be associated with poor prognosis in patients with DCM. (author)

  7. R25G mutation in exon 1 of LMNA gene is associated with dilated cardiomyopathy and limb-girdle muscular dystrophy 1B

    Institute of Scientific and Technical Information of China (English)

    YUAN Wo-liang; HUANG Wei-jun; HUANG Chun-yan; WANG Jing-feng; XIE Shuang-lun; NIE Ru-qiong; LIU Ying-mei; LIU Pin-ming; ZHOU Shu-xian; CHEN Su-qin

    2009-01-01

    Background Mutations of the LMNA gene encoding lamin A and C are associated with dilated cardiomyopathy (DCM), conduction system defects and skeletal muscle dystrophy. Here we report a family with a mutation of the LMNA gene to identify the relationship between genotype and phenotype.Methods All 30 members of the family underwent clinical and genetic evaluation. A mutation analysis of the LMNA gene was performed. All of the 12 exons of LMNA gene were extended with polymerase chain reaction (PCR) and the PCR products were screened for gene mutation by direct sequencing.Results Ten members of the family had limb-girdle muscular dystrophy (LGMD) and 6 are still alive. Two patients suffered from DCM. Cardiac arrhythmias included atrioventricular block and atrial fibrillation; sudden death occurred in 2 patients. The pattern of inheritance was autosomal dominant. Mutation c.73C>G (R25G) in exon 1 encoding the globular domains was confirmed in all of the affected members, resulting in the conversion of arginine (Arg) to glycine (Gly). Conclusions The mutation R25G in exon 1 of LMNA gene we reported here in a Chinese family had a phenotype of malignant arrhythmia and mild LGMD, suggesting that patients with familial DCM, conduction system defects and skeletal muscle dystrophy should be screened by genetic testing for the LMNA gene.

  8. A Mitochondrial DNA A8701G Mutation Associated with Maternally Inherited Hypertension and Dilated Cardiomyopathy in a Chinese Pedigree of a Consanguineous Marriage

    Institute of Scientific and Technical Information of China (English)

    Ye Zhu; Xiang Gu; Chao Xu

    2016-01-01

    Background: Cardiovascular diseases, including dilated cardiomyopathy (DCM) and hypertension, are the leading cause of death worldwide.The role of mitochondrial DNA (mtDNA) in the pathogenesis of these diseases has not been completely clarified.In this study, we evaluate whether A8701G mutation is associated with maternally inherited hypertension and DCM in a Chinese pedigree of a consanguineous marriage.Methods: Fourteen subjects in a three-generation Han Chinese family with hypertension and DCM, in which consanguineous marriage was present in the parental generation, were interviewed.We divided all the family members into case (7 maternal members) and control group (7 nonmaternal members) for comparison.Clinical evaluations and sequence analysis ofmtDNA were obtained from all participants.Frequency differences between maternal and nonmaternal members were tested to locate the disease-associated mutations.Results: The majority of the family members presented with a maternal inheritance of hypertension and DCM.Sequence analysis of mtDNA in this pedigree identified eight mtDNA mutations.Among the mutations identified, there was only one significant mutation: A8701G (P =0.005), which is a homoplasmic mitochondrial missense mutation in all the matrilineal relatives.There was no clear evidence for any synergistic effects between A8701G and other mutations.Conclusions: A8701G mutation may act as an inherited risk factor for the matrilineal transmission of hypertension and DCM in conj unction with genetic disorders caused by consanguineous marriage.

  9. [Arrhythmic cardiomyopathy. Case report].

    Science.gov (United States)

    Streangă, Violeta; Dimitriu, A G; Iordache, C; Georgescu, G; Grecu, Mihaela

    2004-01-01

    An 11 year-old boy was admitted with incessant sinus node reentrant tachycardia and secondary dilated arrhythmic cardiomyopathy, treated by radiofrequency ablation. Two years later he was admitted with incessant automatic atrial tachycardia and arrhythmic cardiomyopathy; a second catheter ablation procedure failed, but the third one, performed four month later, was successfully and resulted in a restoration of a normal sinus rhythm and a complete regression of arrhythmic cardiomyopathy.

  10. Experimental Study ofa New Operative Procedure for Non-Ischemic Dilated Cardiomyopathy-Overlapping Cardiac Volume Reduction Operation

    Institute of Scientific and Technical Information of China (English)

    罗滨; 孟春营; 温定国; 松居喜朗; 安田庆秀

    2003-01-01

    Objectives To assess anewly devised procedure of cardiac volume reduction without resection of cardiac muscle and evaluated in experimental settings. Methods Ten beagle dogs underwent a rapid pacing leading to heart failure for 3 weeks and received the left ventricular reduction termed overlapping cardiac volume reduction operation (OLCVR) ,which consisted of a longitudinal incision in left ventricular (LV) free wall, sutures of the left marginal to the septal wall, and the right marginal to LV free wall.A slope of the linear preload recruitable stroke work relationship (Mw) , with a X - intercept (Vo) were calculated as the precise indicators of left ventricular systolic function. The constant of isovolumic pressure decay (Tau) and a peak filling rate (PFR) were also calculated as the indicators of LV diastolic function.Results LV end- diastolic dimensions was significantly reduced by OLCVR (43±2 to 25±1; mm).Fractional shortening was significantly improved by OLCVR (11±2 to 30±4;%). Mw (erg* cm-3* 103)was also significantly improved (21±2 to 33 ±3 (p<0. 001 ) ) , whereas Vo, Tau and PFR did not show significant changes. Conclusions The OLCVR significantly increased in the early LV systolic function without detrimental effects on diastolic function. This procedure may become a therapeutic option for end - stage cardiomyopathy.

  11. [Peripartum cardiomyopathy].

    Science.gov (United States)

    Mouquet, Frédéric; Bouabdallaoui, Nadia

    2015-01-01

    The peripartum cardiomyopathy is a rare form of dilated cardiomyopathy resulting from alteration of angiogenesis toward the end of pregnancy. The diagnosis is based on the association of clinical heart failure and systolic dysfunction assessed by echocardiography or magnetic resonance imaging. Diagnoses to rule out are myocardial infarction, amniotic liquid embolism, myocarditis, inherited cardiomyopathy, and history of treatment by anthracycline. Risk factors are advance maternal age (>30), multiparity, twin pregnancy, African origin, obesity, preeclampsia, gestational hypertension, and prolonged tocolytic therapy. Treatment of acute phase is identical to usual treatment of acute systolic heart failure. After delivery, VKA treatment should be discussed in case of systolic function <25% because of higher risk of thrombus. A specific treatment by bromocriptine can be initiated on a case-by-case basis. Complete recovery of systolic function is observed in 50% of cases. The mortality risk is low. Subsequent pregnancy should be discouraged, especially if systolic function did not recover.

  12. 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

  13. Impact of repeated intravenous bone marrow mesenchymal stem cells infusion on myocardial collagen network remodeling in a rat model of doxorubicin-induced dilated cardiomyopathy.

    Science.gov (United States)

    Yu, Qin; Li, Qianxiao; Na, Rongmei; Li, Xiaofei; Liu, Baiting; Meng, Lili; Liutong, Hanyu; Fang, Weiyi; Zhu, Ning; Zheng, Xiaoqun

    2014-02-01

    Bone marrow mesenchymal stem cells (MSCs) transplantation improved cardiac function and reduced myocardial fibrosis in both ischemic and non-ischemic cardiomyopathies. We evaluated the effects of repeated peripheral vein injection of MSCs on collagen network remodeling and myocardial TGF-β1, AT1, CYP11B2 (aldosterone synthase) gene expressions in a rat model of doxorubicin (DOX)-induced dilated cardiomyopathy (DCM). Thirty-eight out of 53 SD rats survived at 10 weeks post-DOX injection (2.5 mg/kg/week for 6 weeks, i.p.) were divided into DCM blank (without treatment, n = 12), DCM placebo (intravenous tail injection of 0.5 mL serum-free culture medium every other day for ten times, n = 13), and DCM plus MSCs group (intravenous tail injection of 5 × 10(6) MSCs dissolved in 0.5 mL serum-free culture medium every other day for 10 times, n = 13). Ten untreated rats served as normal controls. At 20 weeks after DOX injection, echocardiography, myocardial collagen content, myocardial expressions of types I and III collagen, TGF-β1, AT1, and CYP11B2 were compared among groups. At 20 weeks post-DOX injection, 8 rats (67%) survived in DCM blank group, 9 rats (69%) survived in DCM placebo group while 13 rats (100 %) survived in DCM plus MSCs group. Left ventricular end-diastolic diameter was significantly higher and ejection fraction was significantly lower in DCM blank and DCM placebo groups compared to normal control rats, which were significantly improved in DCM plus MSCs group (all p collagen volume fraction, types I and III collagen, myocardial mRNA expressions of TGF-β1, AT1, CYP11B2, and collagen I/III ratio were all significantly lower in DCM plus MSCs group compared to DCM blank and DCM placebo groups (all p collagen network remodeling possibly through downregulating renin-angiotensin-aldosterone system in DOX-induced DCM rats.

  14. Expression of Fas Protein of Myocardium in Dilated Cardiomyopathy%扩张型心肌病心肌Fas蛋白表达

    Institute of Scientific and Technical Information of China (English)

    魏淑荣; 陈新山; 陈煌峰; 孙许朋; 黄光照

    2012-01-01

    Objective To investigate Fas protein expression of the myocardium in dilated cardiomyopathy (DCM) and its relationship with occurrence of sudden death caused by DCM. Methods Nine autopsy cases of sudden death caused by DCM along with the heart samples were chosen from the archives in the Department of Forensic Medicine, Tongji Medical College, HUST from 1997 to 2007. Other 11 cases which died of violence and other diseases were selected as the control group. Expressions of myocardial Fas protein in the samples were quantitatively detected by immunohistochemistry and computerized imaging analysis. Results Myocardial Fas protein expression increased significantly in the DCM group. Positive color showed brown-yellow granulated or striped distribution in the longitudinal section of myocardial within the cell membrane and cytoplasm, and showed circular brown granules in the cross section of the cell membrane, while these changes were not observed in the control group though there was focal weak staining noted. Statistical significance was observed between the experimental and control groups (P=0.002), but no statistical significance was found for the average optical density value between these two groups (P=0.675). Conclusion The expression of Fas protein increased obviously in the DCM group. Such alteration in expression quantity and distribution of myocardial Fas protein may be related to arrhythmia and heart failure in the patients with DCM.%目的 探讨扩张型心肌病(dilated cardiomyopathy,DCM)心肌Fas蛋白的表达情况及其与DCM猝死的关系. 方法 用免疫组织化学法和图像分析技术定量检测本教研室1997-2007年间9例DCM猝死和11例暴力死或其他死者的心肌Fas蛋白表达情况.结果 DCM猝死组心肌Fas蛋白表达明显增多,阳性着色在心肌纵切面细胞膜上及胞浆内呈棕黄色颗粒状或条状分布,横断面细胞膜见环形棕黄色颗粒带;对照组未见明显变化或仅见小

  15. Comparison of cardiac magnetic resonance imaging features of isolated left ventricular non-compaction in adults versus dilated cardiomyopathy in adults

    Energy Technology Data Exchange (ETDEWEB)

    Cheng, H. [Department of Radiology, Cardiovascular Institute and Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100037 (China); Zhao, S., E-mail: cjrzhaoshihua2009@163.com [Department of Radiology, Cardiovascular Institute and Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100037 (China); Jiang, S.; Lu, M.; Yan, C.; Ling, J.; Zhang, Y.; Liu, Q.; Ma, N.; Yin, G.; Wan, J. [Department of Radiology, Cardiovascular Institute and Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100037 (China); Yang, Y. [Department of Cardiology, Cardiovascular Institute and Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100037 (China); Li, L. [Department of Pathology, Cardiovascular Institute and Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100037 (China); Jerecic, R. [MR Research and Development, Siemens Medical Solutions, Chicago, IL (United States); He, Z. [Department of Nuclear Medicine, Cardiovascular Institute and Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100037 (China)

    2011-09-15

    Aim: To compare cardiac magnetic resonance imaging (MRI) features between isolated left ventricular non-compaction (IVNC) and dilated cardiomyopathy (DCM) in adults. Materials and methods: A consecutive series of 50 patients with IVNC from a single institution were reviewed. During the same period, 50 patients with DCM who had prominent trabeculations, who were matched for age, gender, and body surface area, were prospectively included. Left ventricular (LV) morphology and function were assessed using cardiac MRI. Results: Compared with patients with DCM, patients with IVNC had a significantly lower LV sphericity index and end-diastolic volume index (LVEDVI) and a greater LV ejection fraction (LVEF), number of trabeculated segments, and ratio of non-compacted to compacted myocardium (NC/C ratio). There were no significant differences in stroke volume index, cardiac output, and cardiac index between the two patient groups. In patients with IVNC, the number of trabeculated segments and the NC/C ratio correlated positively with LVEDVI (r = 0.626 and r = 0.559, respectively) and negatively with LVEF (r = -0.647 and r = -0.521, respectively, p < 0.001 for all). In patients with DCM, the number of non-compacted segments and the NC/C ratio had no correlation with either the LVEDVI (r = -0.082 and r = -0.135, respectively) or the LVEF (r = 0.097 and r = 0.205, respectively). Conclusion: There are demonstrable morphological and functional differences between IVNC and DCM at LV assessment using cardiac MRI. The occurrence of trabeculated myocardium might be due to a different pathophysiological mechanism.

  16. Symbolic dynamics to discriminate healthy and ischaemic dilated cardiomyopathy populations: an application to the variability of heart period and QT interval

    Science.gov (United States)

    Valencia, José Fernando; Vallverdu, Montserrat; Rivero, Isidre; Voss, Andreas; de Luna, Antonio Bayes; Porta, Alberto; Caminal, Pere

    2015-01-01

    Myocardial ischaemia is hypothesized to stimulate the cardiac sympathetic excitatory afferents and, therefore, the spontaneous changes of heart period (approximated as the RR interval), and the QT interval in ischaemic dilated cardiomyopathy (IDC) patients might reflect this sympathetic activation. Symbolic analysis is a nonlinear and powerful tool for the extraction and classification of patterns in time-series analysis, which implies a transformation of the original series into symbols and the construction of patterns with the symbols. The aim of this work was to investigate whether symbolic transformations of RR and QT cardiac series can provide a better separation between IDC patients and healthy control (HC) subjects compared with traditional linear measures. The variability of these cardiac series was studied during daytime and night-time periods and also during the complete 24 h recording over windows of short data sequences of approximately 5 min. The IDC group was characterized by an increase in the occurrence rate of patterns without variations (0 V%) and a reduction in the occurrence rate of patterns with one variation (1 V%) and two variations (2 V%). Concerning the RR variability during the daytime, the highest number of patterns had 0 V%, whereas the rates of 1 V% and 2 V% were lower. During the night, 1 V% and 2 V% increased at the expense of diminishing 0 V%. Patterns with and without variations between consecutive symbols were able to increase the separation between the IDC and HC groups, allowing accuracies higher than 80%. With regard to entropy measures, an increase in RR regularity was associated with cardiac disease described by accuracy >70% in the RR series and by accuracy >60% in the QTc series. These results could be associated with an increase in the sympathetic tone in IDC patients. PMID:25548268

  17. 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.)

  18. Dilated cardiomyopathy mutation (R134W in mouse cardiac troponin T induces greater contractile deficits against α-myosin heavy chain than against β-myosin heavy chain

    Directory of Open Access Journals (Sweden)

    Sampath K Gollapudi

    2016-10-01

    Full Text Available Many studies have demonstrated that depressed myofilament Ca2+ sensitivity is common to dilated cardiomyopathy (DCM in humans. However, it remains unclear whether a single determinant — such as myofilament Ca2+ sensitivity — is sufficient to characterize all cases of DCM because the severity of disease varies widely with a given mutation. Because dynamic features dominate in the heart muscle, alterations in dynamic contractile parameters may offer better insight on the molecular mechanisms that underlie disparate effects of DCM mutations on cardiac phenotypes. Dynamic features are dominated by myofilament cooperativity that stem from different sources. One such source is the strong tropomyosin binding region in troponin T (TnT, which is known to modulate crossbridge (XB recruitment dynamics in a myosin heavy chain (MHC-dependent manner. Therefore, we hypothesized that the effects of DCM-linked mutations in TnT on contractile dynamics would be differently modulated by α- and β-MHC. After reconstitution with the mouse TnT equivalent (TnTR134W of the human DCM mutation (R131W, we measured dynamic contractile parameters in detergent-skinned cardiac muscle fiber bundles from normal (α-MHC and transgenic mice (β-MHC. TnTR134W significantly attenuated the rate constants of tension redevelopment, XB recruitment dynamics, XB distortion dynamics, and the magnitude of length-mediated XB recruitment only in α-MHC fiber bundles. TnTR134W decreased myofilament Ca2+ sensitivity to a greater extent in α-MHC (0.14 pCa units than in β-MHC fiber bundles (0.08 pCa units. Thus, our data demonstrate that TnTR134W induces a more severe DCM-like contractile phenotype against α-MHC than against β-MHC background.

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

    Energy Technology Data Exchange (ETDEWEB)

    He, Shu-Lan [Key Laboratory of Environment and Gene Related Diseases, Xi' an Jiaotong University, Ministry Education, No. 76 Yanta West Road, Xi' an, Shaanxi 710061 (China); Key Laboratory of Trace Elements and Endemic Diseases, Xi' an Jiaotong University, Ministry of Health, No. 76 Yanta West Road, Xi' an, Shaanxi 710061 (China); Tan, Wu-Hong, E-mail: tanwh@mail.xjtu.edu.cn [Key Laboratory of Environment and Gene Related Diseases, Xi' an Jiaotong University, Ministry Education, No. 76 Yanta West Road, Xi' an, Shaanxi 710061 (China); Key Laboratory of Trace Elements and Endemic Diseases, Xi' an Jiaotong University, Ministry of Health, No. 76 Yanta West Road, Xi' an, Shaanxi 710061 (China); Zhang, Zeng-Tie; Zhang, Feng [Key Laboratory of Environment and Gene Related Diseases, Xi' an Jiaotong University, Ministry Education, No. 76 Yanta West Road, Xi' an, Shaanxi 710061 (China); Key Laboratory of Trace Elements and Endemic Diseases, Xi' an Jiaotong University, Ministry of Health, No. 76 Yanta West Road, Xi' an, Shaanxi 710061 (China); Qu, Cheng-Juan [Institute of Biomedicine, University of Eastern Finland, Kuopio (Finland); Lei, Yan-Xia; Zhu, Yan-He [Key Laboratory of Environment and Gene Related Diseases, Xi' an Jiaotong University, Ministry Education, No. 76 Yanta West Road, Xi' an, Shaanxi 710061 (China); Key Laboratory of Trace Elements and Endemic Diseases, Xi' an Jiaotong University, Ministry of Health, No. 76 Yanta West Road, Xi' an, Shaanxi 710061 (China); Yu, Han-Jie [Department of Biotechnology, Northwest University, Xi' an, Shaanxi 710069 (China); Xiang, You-Zhang [Shandong Institute for prevention and Treatment of Endemic Disease, Jinan, Shandong 250014 (China); and others

    2013-10-15

    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.

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

    Energy Technology Data Exchange (ETDEWEB)

    Li, Shou-lin; Ikeda, Jun; Takita, Tamotsu; Sekiguchi, Yohei; Demachi, Jun; Chikama, Hisao; Goto, Atsushi; Shirato, Kunio [Tohoku Univ., Sendai (Japan). School of Medicine

    1998-11-01

    The relationship between the myocardial uptake of iodine-123 metaiodobenzylguanidine ({sup 123}I-MIBG) and heart rate variability parameters has not been determined. This study determined the relationship between the change in myocardial uptake of {sup 123}I-MIBG and improvement in left ventricular function after treatment, to determine the usefulness of {sup 123}I-MIBG imaging to assess the effect of therapy on heart failure due to dilated cardiomyopathy (DCM). {sup 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 {sup 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 {sup 123}I-MIBG imaging and heart rate variability parameters are useful to assess the autonomic tone in DCM with heart failure. (author)

  1. Relationship between cardiac {sup 123}I-Metaiodobenzylguanidine imaging and the transcardiac gradient of neurohumoral factors in patients with dilated cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Matsui, Toshiki; Tsutamoto, Takayoshi; Kinoshita, Masahiko [Shiga Univ. of Medical Science, Otsu (Japan)

    2001-12-01

    Cardiac sympathetic nervous function is altered in congestive heart failure (CHF) and the uptake and washout rate of cardiac {sup 123}I-metaiodobenzylguanidine (MIBG) are useful markers for evaluating the severity of it. To assess what parameters predict decreased uptake or increased washout rate of MIBG, the concentrations of neurohumoral factor in both the aorta (Ao) and coronary sinus (CS) were measured, as well as hemodynamic parameters by catheterization, in patients with dilated cardiomyopathy (DCM). MIBG imaging was performed within 1 week of cardiac catheterization. Regarding MIBG parameters, the correlation with the transcardiac gradient of norepinephrine (NE), brain natriuretic peptide (BNP) and hemodynamics was investigated. Stepwise multivariate regression analysis was used to determine which variables closely correlated with cardiac MIBG parameters. There was a significant increase in the NE level between the Ao (446 pg/ml) and the CS (727 pg/ml). According to stepwise multivariate regression analysis, the heart/mediastinum (H/M) ratio independently correlated with the transcardiac gradient of BNP (r=-0.480, p<0.01), and the washout rate independently correlated with the transcardiac gradient of NE (r=0.481, p<0.01). These findings indicate that the H/M ratio may reflect the transcardiac gradient of BNP, which implies the degree of left ventricular dysfunction and/or damage and the washout rate may reflect altered cardiac sympathetic nerve terminal in DCM patients with CHF, suggesting that both the H/M ratio and washout rate provide important information about the failing ventricle. (author)

  2. 正五聚蛋白3与扩张型心肌病的研究进展%Relationship between PTX3 and Idiopathic Dilated Cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    张艳春

    2013-01-01

    Myocarditis is a disease which causes inflammation of the cardiac muscle. There are two classifications of myocarditis梡rimary and secondary. Myocarditis is classed as secondary when there is a systemic disease at the time of diagnosis, otherwise it is classed as primary. Idiopathic dilated cardiomyopathy ( DCM ) accounts for 70 to 80 percent of primary myocarditis cases. Possible pathogenic factors of DCM are viral infection, autoimmune response and genetic factor. The inflammatory reaction is the critical factor in DCM. Pentranxin 3 ( PTX3 ) is a new inflammatory marker. This article examines the potential use of PTX3 in DCM.%原发性心肌病是一类原因不明、以心功能障碍为主要特征的心肌病变.扩张型心肌病约占原发性心肌病的70%~80%.目前扩张型心肌病发病机制分为三类:病毒感染、自身免疫炎症反应、遗传因素.炎症反应是介导扩张型心肌病发生的重要机制,正五聚蛋白3(PTX3)是一种新的炎症标志物,与C反应蛋白属于PTX家族.现主要针对穿透素3这种新的炎症标志物与扩张型心肌病关系的研究进展做一综述.

  3. Use of thallium-201 myocardial scintigraphy for the prediction of the response to {beta}-blocker therapy in patients with dilated cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Hara, Yuji; Hamada, Mareomi; Ohtsuka, Tomoaki; Ogimoto, Akiyoshi; Saeki, Hideyuki; Suzuki, Jun; Matsunaka, Tsuyoshi; Nakata, Shigeru; Shigematsu, Yuji [Ehime Univ., Shigenobu (Japan). School of Medicine

    2002-12-01

    This study was performed to evaluate whether thallium-201 myocardial scintigraphy (Tl-201) and iodine-123-metaiodobenzylguanidine (MIBG) myocardial scintigraphy could predit the usefulness of {beta}-blocker therapy in patients with dilated cardiomyopathy (DCM). Tl-201 and MIBG were performed in 47 patients before {beta}-blocker therapy. Patients were classified into group A, if their cardiac function improved, and group B, whose function remained unchanged Two types of extent score (ES) by Tl-201 were proposed to quantitate myocardial damage, mean-2SD (ES-2) and mean -3SD (ES-3). The ES difference between ES-2 and ES-3 was calculated, and according to ES and ES difference, DCM cases were classified into 3 groups: mild-defect type (mild-type), moderate-defect type (moderate-type) and severe-defect type (severe-type). The heart-to-mediastinum (H/M) MIBG uptake ratio was evaluated, and the percent washout ratio of myocardial MIBG was obtained from these data. Group A comprised 18 mild-type, 14 moderate-type and 1 severe-type cases, and group B comprised 5 mild-type, 4 moderate-type and 5 severe-type cases. A significant relation was observed between the defect type on Tl-201 and the response to {beta}-blocker therapy (p=0.0090). Both H/M MIBG uptake ratios and washout ratio were not significantly different in the 2 groups. Tl-201 may be useful for predicting the response to {beta}-blocker therapy in patients with DCM. (author)

  4. 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.

  5. Comparative study of peripartum cardiomyopathy and idiopathic dilated cardiomyopathy MRI%围产期心肌病与特发性扩张型心肌病的MRI比较研究

    Institute of Scientific and Technical Information of China (English)

    李小虎; 兰天; 杨新令; 万俊义; 崔辰; 陆敏杰; 余永强; 刘斌; 赵世华; 程怀兵; 尹刚; 张岩; 戴琳琳

    2015-01-01

    目的:研究围产期心肌病(PPCM)与特发性扩张型心肌病(IDCM)的心脏MR(CMR)特征,探讨MRI对PPCM的诊断价值。方法回顾性搜集临床明确诊断为PPCM(PPCM组)及IDCM (IDCM组)的患者各10例。所有患者均采用1.5 T MRI扫描仪对心脏形态(房室大小、小梁化程度、最薄心室壁厚度)、功能(室壁局部运动与整体运动功能)、心肌灌注与心肌纤维化等方面进行综合评价,主要评价指标有心输出量(CO)、舒张末期容积(EDV)、射血分数(EF)、收缩末期容积(ESV)、每搏输出量(SV)等。采用独立样本t检验及Fisher精确概率法进行统计学分析。结果 PPCM与IDCM组的房室大小、CO、EDV、EF、ESV、SV差异均无统计学意义(P值均>0.05)。CMR检查均提示心室壁变薄,4例PPCM及3例IDCM左室心尖部可见过度小梁化。7例PPCM及4例IDCM出现单纯性收缩运动减弱,3例PPCM及6例IDCM出现双室的收缩运动减弱。2例PPCM出现小灶状延迟强化,4例IDCM出现肌壁间延迟强化。1年后随访10例PPCM及4例IDCM心功能恢复正常。结论 MRI多序列成像是PPCM较为理想的影像检查方法,PPCM与IDCM患者在心脏形态结构、功能无明显差异,但PPCM的预后较IDCM好。%Objective To characterize the cardiac magnetic resonance (CMR) features of peripartum cardiomyopathy(PPCM) and idiopathic dilated cardiomyopathy(IDCM), and to explore the value of MRI in the diagnosis of PPCM. Methods Ten cases of PPCM and 10 cases of Idiopathic dilated cardiomyopathy (IDCM) were included in this study. With 1.5 T MRI scanner, the heart shape (atrioventricular size, hypertrabeculation, thickness of the thinnest ventricular wall), function (ventricular wall movement and the overall function), cardiomyopathy perfusion were comprehensively evaluated. Paired samples t⁃test and Fisher exact probability method were used for statistical analysis. Results Between PPCM

  6. Influence of co-existing atrial fibrillation on the efficacy of atorvastatin treatment in patients with dilated cardiomyopathy: a pilot study

    Directory of Open Access Journals (Sweden)

    Desai Ravi

    2010-02-01

    Full Text Available Abstract Introduction The aim of the study was to assess the influence of co-existing atrial fibrillation (AF on inflammatory condition factors, left ventricular function, clinical course and the efficacy of statin treatment of congestive heart failure in the course of dilated cardiomyopathy (DCM. Material and methods In a prospective, randomized, open-label study, 69 patients with DCM and left ventricular ejection fraction (LVEF ≤40% were divided into two groups, with and without AF, who were treated according to the recommended standards. 68% of patients from the group with AF and 59% of patients from the group without AF were administered atorvastatin 40 mg daily for 8 weeks and 10 mg for next 4 months. Clinical examination with the assessment of body mass index (BMI and waist size were followed by routine laboratory tests, measurement of concentration of tumor necrosis factor (TNF-α, interleukin-6 (IL-6, and IL-10 in blood plasma, N-terminal pro-brain natriuretic peptide (NT-proBNP concentration in blood serum, echocardiographic examination, and the assessment of exercise capacity in 6-minute walk test (6-MWT. After six months, morbidity rate and the number of heart failure hospitalizations were also observed. Results In the whole population of patients, a significantly higher concentration of NT-proBNP was observed in the AF group (2669 ± 2192 vs 1540 ± 1067, p = 0.02. After statin treatment, in patients with DCM and co-existing AF, higher values of NT-proBNP and IL-6 were observed compared to non-AF patients (1530 ± 1054 vs 1006 ± 1195, p = 0.04 and (14.16 ± 13.40 vs 6.74 ± 5.45, p = 0.02, respectively. Conclusion In patients with DCM and co-existing AF, a weaker effect of atorvastatin concerning the reduction of IL-6 and NT-proBNP concentration was observed than in patients without atrial fibrillation. Trials Registration (ClinialTrial.gov No.: NCT01015144

  7. 扩张型心肌病的离子通道发病机制研究进展%Current Status of Research in Dilated Cardiomyopathy Pathogenesis in Ion Channels

    Institute of Scientific and Technical Information of China (English)

    万伟; 周显顺; 蔡红专

    2012-01-01

    扩张型心肌病是一侧或双侧心腔扩大、心肌收缩功能障碍为主要特征的心肌疾病.其发病机制可能与病毒感染、免疫反应以及遗传因素有关.近年来研究发现心脏钠通道基因突变可导致扩张型心肌病.因此,现重点从心肌细胞离子通道的角度来论述扩张型心肌病的发病机制,探讨其治疗的新靶点.%Dilated cardiomyopathy ( DCM) is a myocardial disease in which the unilateral or bilateral chambers of the heart expand and myocardial contraction is weakened. The pathogenesis may be associated with viral infections, immune response and genetic factors. Recently, studies have found that the cardiac sodium channel gene mutations can cause DCM. This review discusses the pathogenesis of dilated cardiomyopathy from the perspective of myocardial cell ion channels in order to explore a new therapeutic target.

  8. Novel paradigms to measure variability of behavior in early childhood: Posture, gaze, and pupil dilation

    Directory of Open Access Journals (Sweden)

    Robert eHepach

    2015-07-01

    Full Text Available A central challenge of investigating the underlying mechanisms of and the individual differences in young children’s behavior is the measurement of the internal physiological mechanism and the involved expressive emotions. Here, we illustrate two paradigms that assess concurrent indicators of both children’s social perception as well as their emotional expression. In one set of studies, children view situations while their eye movements are mapped onto a live scene. In these studies, children’s internal arousal is measured via changes in their pupil dilation by using eye-tracking technology. In another set of studies, we measured children's emotional expression via changes in their upper-body posture by using depth sensor imaging technology. Together, these paradigms can provide new insights into the internal mechanism and outward emotional expression involved in young children’s behavior.

  9. Novel paradigms to measure variability of behavior in early childhood: posture, gaze, and pupil dilation.

    Science.gov (United States)

    Hepach, Robert; Vaish, Amrisha; Tomasello, Michael

    2015-01-01

    A central challenge of investigating the underlying mechanisms of and the individual differences in young children's behavior is the measurement of the internal physiological mechanism and the involved expressive emotions. Here, we illustrate two paradigms that assess concurrent indicators of both children's social perception as well as their emotional expression. In one set of studies, children view situations while their eye movements are mapped onto a live scene. In these studies, children's internal arousal is measured via changes in their pupil dilation by using eye tracking technology. In another set of studies, we measured children's emotional expression via changes in their upper-body posture by using depth sensor imaging technology. Together, these paradigms can provide new insights into the internal mechanism and outward emotional expression involved in young children's behavior.

  10. Alcoholic cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    Gonzalo; Guzzo-Merello; Marta; Cobo-Marcos; Maria; Gallego-Delgado; Pablo; Garcia-Pavia

    2014-01-01

    Alcohol is the most frequently consumed toxic substance in the world. Low to moderate daily intake of alcohol has been shown to have beneficial effects on the cardiovascular system. In contrast, exposure to high levels of alcohol for a long period could lead to progressive cardiac dysfunction and heart failure. Cardiac dysfunction associated with chronic and excessive alcohol intake is a specific cardiac disease known as alcoholic cardiomyopathy(ACM). In spite of its clinical importance, data on ACM and how alcohol damages the heart are limited. In this review, we evaluate available evidence linking excessive alcohol consumption with heart failure and dilated cardiomyopathy. Additionally, we discuss the clinical presentation, prognosis and treatment of ACM.

  11. Infiltrative Cardiomyopathies

    Science.gov (United States)

    Bejar, David; Colombo, Paolo C; Latif, Farhana; Yuzefpolskaya, Melana

    2015-01-01

    Infiltrative cardiomyopathies can result from a wide spectrum of both inherited and acquired conditions with varying systemic manifestations. They portend an adverse prognosis, with only a few exceptions (ie, glycogen storage disease), where early diagnosis can result in potentially curative treatment. The extent of cardiac abnormalities varies based on the degree of infiltration and results in increased ventricular wall thickness, chamber dilatation, and disruption of the conduction system. These changes often lead to the development of heart failure, atrioventricular (AV) block, and ventricular arrhythmia. Because these diseases are relatively rare, a high degree of clinical suspicion is important for diagnosis. Electrocardiography and echocardiography are helpful, but advanced techniques including cardiac magnetic resonance (CMR) and nuclear imaging are increasingly preferred. Treatment is dependent on the etiology and extent of the disease and involves medications, device therapy, and, in some cases, organ transplantation. Cardiac amyloid is the archetype of the infiltrative cardiomyopathies and is discussed in great detail in this review. PMID:26244036

  12. 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.

  13. Deformação miocárdica pelo speckle tracking na cardiomiopatia dilatada grave Myocardial deformation by speckle tracking in severe dilated cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Maria Cristina Donadio Abduch

    2012-09-01

    Full Text Available FUNDAMENTO: A alta e crescente prevalência de Cardiomiopatia Dilatada (CMD representa sério problema de saúde pública. Novas tecnologias vêm sendo utilizadas objetivando diagnósticos mais sofisticados, que melhorem a abordagem terapêutica. Nesse cenário, o Speckle Tracking (STE utiliza marcadores miocárdicos naturais para analisar a deformação sistólica do Ventrículo Esquerdo (VE. OBJETIVO: Mensurar o strain transmural longitudinal global (SG do VE através do STE em pacientes com CMD grave, comparando os resultados com indivíduos normais e com parâmetros ecocardiográficos consagrados para análise da função sistólica do VE, validando o método nessa população. MÉTODOS: Foram estudados 71 pacientes com CMD grave, (53 ± 12a, 72% homens e 20 controles (30 ± 8a, 45% homens. Foram obtidos os volumes e a FEVE pela ecocardiografia bi e tridimensional, parâmetros do Doppler, Doppler tecidual e o SG pelo STE. RESULTADOS: Comparados ao grupo controle, os volumes do VE foram maiores no grupo CMD; entretanto, a FEVE e velocidade de pico da onda E foram menores neste último. O índice de performance miocárdica foi maior entre os pacientes. As velocidades do miocárdio pelo Doppler tecidual (S', e', a' foram consideravelmente menores e a relação E/e' foi maior no grupo CMD. O SG apresentou-se diminuído no grupo CMD (-5,5% ± 2,3%, em relação aos controles (-14,0% ± 1,8%. CONCLUSÃO: No presente estudo, o SG foi significativamente menor nos pacientes com CMD grave, abrindo novas perspectivas para abordagens terapêuticas nessa população específica.BACKGROUND: The high and increasing prevalence of Dilated Cardiomyopathy (DCM represents a serious public health problem. New technologies are being used aiming at more accurate diagnoses in order to improve therapeutic approach. In this scenario, speckle tracking echocardiography (STE uses natural myocardial markers to analyze the systolic deformation of the left ventricle (LV

  14. 扩张型心肌病患者肌钙蛋白I和心功能的关系%The relationship between cardiac troponin I and cardiac function in patients with dilated cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    王一伟

    2014-01-01

    Objective:To investigate the relationship between cardiac troponin I and cardiac function in patients with dilated cardiomyopathy.Methods:65 patients who were diagnosed with dilated cardiomyopathy were selected from 2011 to 2012.They were classified according to different function,and determined the level of serum cardiac troponin I,and analyzed the relationship between cardiac troponin I and cardiac function.Results:When we measured the serum troponin I in 65 patients,we found that the levels of serum cardiac troponin I had obviously differences in different cardiac function groups,the higher of the grade,the higher of the level of troponin I.Conclusion:The worse of the cardiac function in patients with dilated cardiomyopathy,the higher of the level of troponin I,so we can concluded that cardiac troponin I is an important index that can react cardiac function decreased.%目的:探讨扩张型心肌病患者肌钙蛋白 I 和心功能的关系。方法:2011-2012年收治扩张型心肌病患者65例,对患者按照不同心功能进行分级后血清肌钙蛋白I的水平情况进行测定,并分析肌钙蛋白I与心功能的关系。结果:对65例患者的血清肌钙蛋白 I 进行测定后发现,在不同的心功能组的肌钙蛋白 I 的水平有明显的差异,分级越高,肌钙蛋白I水平越高。结论:扩张型心肌病患者的心功能越差,肌钙蛋白I水平就越高,肌钙蛋白I是反应患者心功能下降的重要指标。

  15. 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

  16. DNA analysis in inherited cardiomyopathies : Current status and clinical relevance

    NARCIS (Netherlands)

    Van Spaendonck-Zwarts, Karin Y.; Van den Berg, Maarten P.; Van Tintelen, J. Peter

    2008-01-01

    Most hypertrophic cardiomyopathies and a subset of dilated and arrhythmogenic right ventricular cardiomyopathies are familial diseases. They generally show an autosomal dominant pattern of inheritance and have underlying mutations in genes encoding sarcomeric, cytoskeletal, nuclear envelope, and des

  17. 多普勒超声心动图评价小儿扩张型心肌病%Evaluation of Doppler echocardiograms on dilated cardiomyopathy in children English Column

    Institute of Scientific and Technical Information of China (English)

    梁光明

    2002-01-01

    Objective To evaluate the appraisal of Doppler echocardiograms on dilated cardiomyopathy(DCM) in children. Methods 26 patients with DCM were tested by Doppler echocardiograms. The left ventricular diastolic function, systolic function, and pulmonary artery systolic pressure (PASP)were tested before and after treatment. Results After treatment , the systolic function still changed, but situation got well obviously. Diastolic function was mostly false normal type (52.6% ), 3 patients returned to normal. Each index parameter (except E/A) of diastolie function was much different from the normal value(P<0.001). Serious continuous pulmonary hypertension is a prognosticate index for the children with DCM .Conclusions Doppler echocardiograms can be used to assess myocardium rehabilitation for the children with DCM.

  18. Hunting for the genetic cause in a case with familial Peripartum/Dilated cardiomyopathy using haplotype sharing analysis and exome sequencing

    NARCIS (Netherlands)

    Van Spaendonck-Zwarts, K.Y.; Jongbloed, J.D.H.; Van Der Zwaag, P.A.; Posafalvi, A.; Koetsier, W.; Van Langen, I.M.; Van Veldhuisen, D.J.; Sinke, R.J.; Van Den Berg, M.P.; Van Tintelen, J.P.

    2011-01-01

    Background/purpose: Peripartum cardiomyopathy (PPCM) is a cause of pregnancyassociated heart failure. It typically develops during the last month of pregnancy and up to six months after delivery in women without known cardiovascular disease. Recently, we showed that some cases of PPCM are part of th

  19. 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)

  20. MRPL44 mutations cause a slowly progressive multisystem disease with childhood-onset hypertrophic cardiomyopathy

    NARCIS (Netherlands)

    Distelmaier, F.; Haack, T.B.; Catarino, C.B.; Gallenmuller, C.; Rodenburg, R.J.T.; Strom, T.M.; Baertling, F.; Meitinger, T.; Mayatepek, E.; Prokisch, H.; Klopstock, T.

    2015-01-01

    Defects in mitochondrial translation may lead to combined respiratory chain deficiency and typically cause childhood-onset multisystem disease. Only recently, a homozygous missense mutation (c.467T > G, p.Leu156Arg) in MRPL44, encoding a protein of the large subunit of the mitochondrial ribosome,

  1. Peripartum cardiomyopathy: a review.

    Science.gov (United States)

    Bhattacharyya, Anirban; Basra, Sukhdeep Singh; Sen, Priyanka; Kar, Biswajit

    2012-01-01

    Peripartum cardiomyopathy is idiopathic heart failure occurring in the absence of any determinable heart disease during the last month of pregnancy or the first 5 months postpartum. The incidence varies worldwide but is high in developing nations; the cause of the disease might be a combination of environmental and genetic factors. Diagnostic echocardiographic criteria include left ventricular ejection fraction 2.7 cm/m(2). Electrocardiography, magnetic resonance imaging, endomyocardial biopsy, and cardiac catheterization aid in the diagnosis and management of peripartum cardiomyopathy. Cardiac protein assays can also be useful, as suggested by reports of high levels of NT-proBNP, cardiac troponin, tumor necrosis factor-α, interleukin-6, interferon-γ, and C-reactive protein in peripartum cardiomyopathy. The prevalence of mutations associated with familial dilated-cardiomyopathy genes in patients with peripartum cardiomyopathy suggests an overlap in the clinical spectrum of these 2 diseases.Treatment for peripartum cardiomyopathy includes conventional pharmacologic heart-failure therapies-principally diuretics, angiotensin-converting enzyme inhibitors, vasodilators, digoxin, β-blockers, anticoagulants, and peripartum cardiomyopathy-targeted therapies. Therapeutic decisions are influenced by drug-safety profiles during pregnancy and lactation. Mechanical support and transplantation might be necessary in severe cases. Targeted therapies (such as intravenous immunoglobulin, pentoxifylline, and bromocriptine) have shown promise in small trials but require further evaluation. Fortunately, despite a mortality rate of up to 10% and a high risk of relapse in subsequent pregnancies, many patients with peripartum cardiomyopathy recover within 3 to 6 months of disease onset.

  2. Tissue Doppler Imaging Combined with Advanced 12-Lead ECG Analysis Might Improve Early Diagnosis of Hypertrophic Cardiomyopathy in Childhood

    Science.gov (United States)

    Femlund, E.; Schlegel, T.; Liuba, P.

    2011-01-01

    Optimization of early diagnosis of childhood hypertrophic cardiomyopathy (HCM) is essential in lowering the risk of HCM complications. Standard echocardiography (ECHO) has shown to be less sensitive in this regard. In this study, we sought to assess whether spatial QRS-T angle deviation, which has shown to predict HCM in adults with high sensitivity, and myocardial Tissue Doppler Imaging (TDI) could be additional tools in early diagnosis of HCM in childhood. Methods: Children and adolescents with familial HCM (n=10, median age 16, range 5-27 years), and without obvious hypertrophy but with heredity for HCM (n=12, median age 16, range 4-25 years, HCM or sudden death with autopsy-verified HCM in greater than or equal to 1 first-degree relative, HCM-risk) were additionally investigated with TDI and advanced 12-lead ECG analysis using Cardiax(Registered trademark) (IMED Co Ltd, Budapest, Hungary and Houston). Spatial QRS-T angle (SA) was derived from Kors regression-related transformation. Healthy age-matched controls (n=21) were also studied. All participants underwent thorough clinical examination. Results: Spatial QRS-T angle (Figure/ Panel A) and septal E/Ea ratio (Figure/Panel B) were most increased in HCM group as compared to the HCM-risk and control groups (p less than 0.05). Of note, these 2 variables showed a trend toward higher levels in HCM-risk group than in control group (p=0.05 for E/Ea and 0.06 for QRS/T by ANOVA). In a logistic regression model, increased SA and septal E/Ea ratio appeared to significantly predict both the disease (Chi-square in HCM group: 9 and 5, respectively, p less than 0.05 for both) and the risk for HCM (Chi-square in HCM-risk group: 5 and 4 respectively, p less than 0.05 for both), with further increased predictability level when these 2 variables were combined (Chi-square 10 in HCM group, and 7 in HCM-risk group, p less than 0.01 for both). Conclusions: In this small material, Tissue Doppler Imaging and spatial mean QRS-T angle

  3. Peripartum cardiomyopathy: a review

    Directory of Open Access Journals (Sweden)

    Capriola M

    2012-12-01

    Full Text Available Michael CapriolaThomasville Medical Center, Department of Emergency Medicine, Thomasville Medical Center, Thomasville, NC, USAAbstract: Peripartum cardiomyopathy (PPCM is a form of dilated cardiomyopathy of unclear etiology affecting women without preexisting heart disease during the last month of pregnancy or during the first 5 months postpartum. Its incidence shows marked geographic and ethnic variation, being most common in Africa and among women of African descent. Most women present in the first month postpartum with typical heart failure symptoms such as dyspnea, lower extremity edema, and fatigue. These symptoms are often initially erroneously diagnosed as part of the normal puerperal process. Diagnosis can be aided by the finding of a significantly elevated serum brain natriuretic peptide. The etiology of PPCM is unclear; however, recent research suggests abnormal prolactin metabolism is seminal in its development, and prolactin antagonism with bromocriptine shows promise as a novel treatment for PPCM.Keywords: pregnancy, pregnancy complications, cardiovascular, cardiomyopathy, dilated

  4. Polymorphism of the second exon of human leukocyte antigen-DQA1, -DQB1 gene and genetic susceptibility to idiopathic dilated cardiomyopathy in people of the Han nationality in northern China

    Institute of Scientific and Technical Information of China (English)

    LIU Wei; LI Wei-min; SUN Ning-ling

    2005-01-01

    @@ Idiopathic dilated cardiomyopathy (IDC) is characterized by dilation and impaired contraction of the left ventricle or both, and it is a relevant cause of heart failure and a common indication for heart transplantation. The major pathogenetic hypothesis in IDC involves autoimmune mediated damage to myocytes. The development of autoimmune inflammatory damage occurs only in patients with a predisposing genetic background. Changes in the immune system concerning cell-mediated and humoral immunity have been detected. The immune system is strictly related to human leukocyte antigen (HLA), which is located on the surface of antigen presenting cells. Its primary function is to restrict T-cell receptors in the process of recognizing auto- or exterior antigen, and thus participates in or mediates immunological recognition, immunological response and immune regulation at various levels. HLA is a genetic marker of susceptibility to autoimmune myocardial damage.1 In the present study, the HLA-DQA1 and -DQB1 alleles in IDC patients were detected with the techniques of polymerase chain reaction-sequence specific primers (PCR-SSP) to explore the immunogenetic mechanisms involved in the pathogenesis of IDC.

  5. 左卡尼汀对扩张型心肌病患者体内卡尼汀群的代谢影响%Effect of Levocarnitine on Carnitine Set of Patients with Dilated Cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    李盛楠; 荆凡波; 夏蕴秋; 郭琳琳; 孙振龙; 韩志武; 隋忠国; 王春波

    2013-01-01

    OBJECTIVE: To study the metabolize of Levocarnitine (LC) injection on heart function of patients with dilated cardiomyopathy. METHODS: 100 cases of dilated cardiomyopathy were randomly divided into treatment group and control group. Both groups were given conventional treatment, and treatment group were additionally given intravenous push of LC injection 2.0 g added into 0.9% sodium chloride 20 ml bid for 14 days. The systolic volume (SV), cardiac output (CO) and left ventricular ejection fraction (LVEF) were determined by Siemens ACUSON X300 color Doppler before and after treatment, and the content of car-nitines was determined by pre-column HPLC. RESULTS: After treatment for 14 days, SV, CO and LVEF were (62.30 ± 4.80) ml/ beat, (5.9 ± 0.5)L/min and (59.9 ± 5.2)% in treatment groups, which were higher than control group (P<0.05); after treatment for 14 days, the plasma levels of LC, acetyl-LC and propionyl-LC in treatment group were (146.64 ± 6.71), (21.40 ± 3.11) and (9.72 ± 2.05)μmol/L, which were all higher than in control group (P<0.05). There was no obvious ADR in 2 groups. CONCLUSION : LC injection can improve the LVEF, CO and SV, and can increase the plasma concentration of LC, ALC and PLC in patients with dilated cardiomyopathy.%目的:探讨左卡尼汀注射液在扩张型心肌病(DCM)患者体内的代谢情况.方法:将100例DCM患者随机均分为治疗组和对照组,2组患者均给予常规治疗.治疗组在常规治疗基础上将左卡尼汀注射液2.0 g加入0.9%氯化钠注射液20 ml中稀释后静脉推注,bid,疗程为14d.采用彩色多普勒超声仪检测治疗前后2组患者的心搏出量(SV)、心输出量(CO)和射血分数(LVEF),并采用柱前高效液相色谱法检测患者体内卡尼汀群的含量.结果:治疗组给药14d后的LVEF为(59.9±5.2)%、CO为(5.9±0.5)L/min、SV为(62.30±4.80)ml/beat,均显著高于对照组同期(P<0.05);给药14d后治疗组的血浆左卡尼汀、乙酰左卡尼汀、丙

  6. Charcot-Marie-Tooth disease and dilated cardiomyopathy. A rare combination. Enfermedad de Charcot-Marie-Tooth y miocardiopatía dilatada. Una rara asociación.

    Directory of Open Access Journals (Sweden)

    Rafael Pila Pérez

    2011-07-01

    Full Text Available

    Se presenta el caso de un paciente de 50 años de edad, con 14 años de evolución de manifestaciones clínicas, destacándose las alteraciones musculoesqueléticas de los cuatro miembros con atrofia de las prominencias tenar e hipotenar y de la musculatura de ambas piernas. Se destacó la presencia de alteraciones sensitivas en miembros inferiores con distribución en calcetín, atrofia, atonía, arreflexia y marcha equina. Desde el punto de vista cardiaco, el paciente presentaba un fibriloaleteo. La radiografía de tórax mostró un aumento marcado del área cardiaca y la ecocardiografía puso de manifiesto una miocardiopatía dilatada. El estudio histopatológico confirmó la presencia de la enfermedad de Charcot-Marie-Tooth asociada a miocardiopatía dilatada. El diagnóstico se basó en las características clínicas, la velocidad de conducción motora, y el estudio histopatológico, que demostró desmielinización con lesiones en “cebolla”, si bien faltaron los estudios genéticos. La enfermedad de Charcot-Marie-Tooth es una enfermedad rara; aproximadamente un 60 % de los pacientes que la padecen, son portadores de una duplicación del cromosoma 17. Por ello, se consideró oportuno transmitir la experiencia de este caso.

    The case of a 50 years old male patient is presented. Along 14 years of clinical evolution, four limbs musculoskeletal disorders with atrophy of the thenar and hypothenar prominences and muscles of both legs had been emphasized. The presence of sensory impairment in lower limbs with stocking distribution, atrophy, weakness, areflexia and equine gait were very peculiar in this case. From the cardiac point of view, the patient presented a fibrillation/flutter. Chest radiography showed a marked increase in the cardiac area and echocardiography revealed dilated cardiomyopathy. Histopathological examination confirmed the presence of Charcot-Marie-Tooth disease associated with dilated cardiomyopathy. While genetic

  7. O uso da L-carnitina como adjuvante no tratamento da miocardiopatia dilatada em criança com Aids Usage of L-carnitine as adjuvant in the treatment of dilated cardiomyopathy in a child with Aids

    Directory of Open Access Journals (Sweden)

    Lourdes Zélia Zanoni

    2011-06-01

    Full Text Available OBJETIVO: Apresentar a resposta cardiovascular à L-carnitina de um paciente com insuficiência cardíaca congestiva decorrente de miocardiopatia dilatada pelo vírus da imunodeficiência humana. DESCRIÇÃO DO CASO: Criança com quadro clínico de insuficiência cardíaca congestiva grave devido à miocardiopatia dilatada pela síndrome de imunodeficiência adquirida. O tratamento para as manifestações clínicas foi instituído, com pouca resposta clínica. Com objetivo de melhorar o desempenho energético/metabólico dos cardiomiócitos, foi instituída terapia com L-carnitina. Observou-se significativa melhora clínica do paciente, em relação ao desempenho cardíaco, mesmo antes do início do tratamento com os fármacos antirretrovirais. COMENTÁRIOS: A L-carnitina é um composto que facilita o transporte dos ácidos graxos de cadeia longa para dentro da mitocôndria. Nesse caso, o uso da L-carnitina parece ser clinica e bioquimicamente justificado.OBJECTIVE: To present the cardiovascular response to L-carnitine of a patient with congestive heart failure caused by dilated cardiomyopathy and human immunodeficiency virus. CASE DESCRIPTION: Child with a clinical history of severe congestive heart failure due to dilated cardiomyopathy caused by acquired immunodeficiency syndrome. The treatment for the symptoms resulted in a poor clinical response. In order to improve the energetic performance/metabolism of cardiomyocytes, therapy with L-carnitine was established. There was significant clinical improvement of the cardiac performance of the patient, even before starting the treatment with antiretroviral drugs. COMMENTS: L-carnitine is a compound that facilitates the transport of long-chain fatty acids into the mitochondria. In this case the administration of L-carnitine appears to be clinically and biochemical justified.

  8. Arrhythmias in peripartum cardiomyopathy.

    Science.gov (United States)

    Honigberg, Michael C; Givertz, Michael M

    2015-06-01

    Peripartum cardiomyopathy (PPCM) is a complication of late pregnancy and the early postpartum period characterized by dilated cardiomyopathy and heart failure with reduced ejection fraction. Approximately half of women fail to recover left ventricular function. Standard management of heart failure is indicated, with some exceptions for women who are predelivery or breastfeeding. Atrial and ventricular arrhythmias are reported in PPCM, but the frequency of arrhythmias in this condition is not well characterized. Management of PPCM-associated arrhythmias may include antiarrhythmic drugs, catheter ablation, and wearable or implantable cardioverter-defibrillators. Further research is needed on the prevalence, natural history, and optimal management of arrhythmias in PPCM.

  9. Loss of αT-catenin alters the hybrid adhering junctions in the heart and leads to dilated cardiomyopathy and ventricular arrhythmia following acute ischemia.

    Science.gov (United States)

    Li, Jifen; Goossens, Steven; van Hengel, Jolanda; Gao, Erhe; Cheng, Lan; Tyberghein, Koen; Shang, Xiying; De Rycke, Riet; van Roy, Frans; Radice, Glenn L

    2012-02-15

    It is generally accepted that the intercalated disc (ICD) required for mechano-electrical coupling in the heart consists of three distinct junctional complexes: adherens junctions, desmosomes and gap junctions. However, recent morphological and molecular data indicate a mixing of adherens junctional and desmosomal components, resulting in a 'hybrid adhering junction' or 'area composita'. The α-catenin family member αT-catenin, part of the N-cadherin-catenin adhesion complex in the heart, is the only α-catenin that interacts with the desmosomal protein plakophilin-2 (PKP2). Thus, it has been postulated that αT-catenin might serve as a molecular integrator of the two adhesion complexes in the area composita. To investigate the role of αT-catenin in the heart, gene targeting technology was used to delete the Ctnna3 gene, encoding αT-catenin, in the mouse. The αT-catenin-null mice are viable and fertile; however, the animals exhibit progressive cardiomyopathy. Adherens junctional and desmosomal proteins were unaffected by loss of αT-catenin, with the exception of the desmosomal protein PKP2. Immunogold labeling at the ICD demonstrated in the αT-catenin-null heart a preferential reduction of PKP2 at the area composita compared with the desmosome. Furthermore, gap junction protein Cx43 was reduced at the ICD, including its colocalization with N-cadherin. Gap junction remodeling in αT-catenin-knockout hearts was associated with an increased incidence of ventricular arrhythmias after acute ischemia. This novel animal model demonstrates for the first time how perturbation in αT-catenin can affect both PKP2 and Cx43 and thereby highlights the importance of understanding the crosstalk between the junctional proteins of the ICD and its implications for arrhythmogenic cardiomyopathy.

  10. Transient dilated cardiomyopathy in a newborn exposed to idarubicin and all-trans-retinoic acid (ATRA) early in the second trimester of pregnancy.

    Science.gov (United States)

    Siu, B L; Alonzo, M R; Vargo, T A; Fenrich, A L

    2002-01-01

    Acute promyelocytic leukemia was diagnosed in a 28-year-old pregnant woman at 13 gestational weeks. She was immediately started on idarubicin and all-trans-retinoic acid (ATRA) and achieved remission after her fourth cycle of treatment. Serial fetal ultrasonograms throughout pregnancy did not reveal any intrauterine growth retardation or other obvious malformations. The mother delivered a term (36.7 gestational weeks), 2720-gram female neonate. The infant was admitted to the intermediate care nursery for observation due to transient mild respiratory distress during the peripartum period. Because of right ventricular hypertrophy on an electrocardiogram, an echocardiogram was performed on the first day of life which showed moderate dilation of the right atrium and right ventricle with mildly depressed function, two small secundum atrial septal defects, and a small patent ductus arteriosus. The neonate remained hemodynamically stable and no arrhythmias were detected. The remainder of the hospital course was uneventful. When reassessed 1-1/2 months later, she was doing well and did not show any signs of congestive heart failure. A repeat echocardiogram at that time demonstrated complete resolution of the right heart enlargement and closure of the ductus arteriosus with persistence of the small and hemodynamically insignificant secundum atrial septal defects.

  11. 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.

  12. Relationship between BNP level and PRC of patients with stable dilated cardiomyopathy%稳定扩张性心肌病患者中血浆B型利钠肽和肾素浓度的相互关系

    Institute of Scientific and Technical Information of China (English)

    张丽娜; 李雅楠; 曹艳菲

    2012-01-01

    Objective To interpret B-type natriurenic peptide(BNP)level in patients with stable chronic heart failure (CHF) and to learn whether the change in BNP represents disease progression. Methods To compare change in BNP and biological variation in factors of the renin angiotensin aldosterone system(RAS) in stable CHF patients with dilated cardiomyopathy(DCM) ,the level of BNP and RAS factors were measured in 80 stable DCM patients. Results According to stepwise multivariate analysis,serum BNP at baseline(P = 0. 005) .presence of atrial fibrilla-tion(P=0. 014) ,a high biological variation in plasma renin concentration (PRC,P = 0. 002) were significant inde-pendent dominant factors related to a high biological variation in BNP. Although there was no change in body weight or blood pressure during the 2 months following up study period,the change in hematocritf %) was negatively corre-lated with the change in BNP( % ) (r= - 0. 325 ,P=0. 000 7) and positively correlated with the change in PRC( r= 0. 680, P=0. 001). Conclusions There is significant relationship between biological variation in BNP and biologicao variation in PRC,suggesting that the physiological interaction between natriuretic peptide system and RAS may con-tribute to the biological variation in BNP in stable DCM patients.%目的 研究B型利纳肽(B-type natriuretic peptide,BNP)生物学变异是否体现稳定慢性心力衰竭(chronic heart failure,CHF)患者疾病进程.方法 比较存在扩张性心肌病(dilated cardiomyopathy,DCM)的稳定CHF患者中BNP和肾素-血管紧张素醛固酮系统(renin-angiotensin-aldosterone system,RAS)因子生物学变量.存在DCM稳定CHF患者80例,测定BNP和RAS因子.结果 经梯式多变量分析,血清BNP在基线水平(P=0.005)、存在房颤(P=0.014)、高血浆肾素浓度(plasma renin concentration,PRC)(PRC,P=0.002)是显著性独立,优势因子,与高BNP相关.随访2个月,无体重和血压的改变,血细胞比容

  13. 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.

  14. Usefulness of {sup 123}I-metaiodobenzylguanidine myocardial scintigraphy for predicting the effectiveness of {beta}-blockers in patients with dilated cardiomyopathy from the standpoint of long-term prognosis

    Energy Technology Data Exchange (ETDEWEB)

    Fujimoto, Shinichiro; Inoue, Aritomo; Hisatake, Shinji; Yamashina, Shohei; Yamashina, Hisayo; Nakano, Hajime; Yamazaki, Junichi [Toho University School of Medicine, Division of Cardiovascular Medicine, Department of Internal Medicine, Ohmori Hospital, Tokyo (Japan)

    2004-10-01

    The usefulness of {sup 123}I-metaiodobenzylguanidine (MIBG) myocardial scintigraphy in predicting the effectiveness of {beta}-blocker therapy in dilated cardiomyopathy (DCM) was investigated from the standpoint of long-term prognosis. The subjects were 53 DCM patients in whom {beta}-blockers had been successfully introduced and used for 6 months or longer. When symptoms were stable before the introduction of {beta}-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 {beta}-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 {beta}-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 {beta}-blocker therapy are those with low early EXT and early SEV and high WR before {beta}-blocker introduction regardless of LVEF values. (orig.)

  15. Holter electrocardiography in dogs showing doxorubicin-induced dilated cardiomyopathy Eletrocardiografia Holter em cães com cardiomiopatia dilatada experimental induzida pela doxorrubicina

    Directory of Open Access Journals (Sweden)

    G.B. Pereira Neto

    2006-12-01

    Full Text Available Early identification of arrhythmias in dogs showing doxorubicin-induced cardiomyopathy was studied. Ten healthy dogs were assigned to groups A (n=5 and B (n=5. Dogs from group B were given doxorubicin 30mg/m² intravenously, every 21 days, until a cumulative dose of 180mg/m² or 240mg/m² was reached. Dogs from group A (used as control were administered saline intravenously at the same group B intervals. As soon as myocardium dysfunction was observed in dogs from group B, determined by a shortening fraction below 20%, increased E-point to septal separation above 0.7cm, and increased end-systolic left ventricular volume index (61.4ml/m², a 24-hour Holter was recorded in all dogs from both groups. There was an increase of minimum heart rate (44.6% and mean heart rate (41.7% in animals from group B in comparison with the control animals. Either supraventricular or ventricular arrhythmias were observed, despite group B dogs showed higher occurrence of supraventricular arrhytmias. Holter monitoring is efficient in early determination of heart rate and cardiac rhythm alterations in dogs showing doxorubicin-induced myocardial dysfunction.O estudo consistiu na identificação precoce da ocorrência de arritmias em cães com cardiomiopatia dilatada experimental induzida pela doxorrubicina (DOX. Utilizaram-se 10 cães adultos, sadios, distribuídos nos grupos A (n=5 e B (n=5. O grupo B recebeu 30mg/m² de DOX, via intravenosa, a cada 21 dias, até a dose cumulativa de 180 ou 240mg/m². No grupo A (controle, administrou-se solução salina 0,9%, via intravenosa, nos mesmos intervalos do grupo B. Ao se evidenciar o quadro de disfunção miocárdica nos cães do grupo B, caracterizado pela fração de encurtamento menor que 20%, aumento da separação septal do ponto E acima de 0,7cm e aumento do índice volumétrico do ventrículo esquerdo ao final da sístole (61,4ml/m², realizaram-se os eletrocardiogramas por 24 horas. Os resultados demonstraram

  16. Importance of genetic evaluation and testing in pediatric cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    Muhammad; Tariq; Stephanie; M; Ware

    2014-01-01

    Pediatric cardiomyopathies are clinically heterogeneous heart muscle disorders that are responsible for significant morbidity and mortality. Phenotypes include hypertrophic cardiomyopathy, dilated cardiomyopathy, restrictive cardiomyopathy, left ventricular noncompaction and arrhythmogenic right ventricular cardiomyopathy. There is substantial evidence for a genetic contribution to pediatric cardiomyopathy. To date, more than 100 genes have been implicated in cardiomyopathy, but comprehensive genetic diagnosis has been problematic because of the large number of genes, the private nature of mutations, and difficulties in interpreting novel rare variants. This review will focus on current knowledge on the genetic etiologies of pediatric cardiomyopathy and their diagnostic relevance in clinical settings. Recent developments in sequencing technologies are greatly impacting the pace of gene discovery and clinical diagnosis. Understanding the genetic basis for pediatric cardiomyopathy and establishing genotypephenotype correlations may help delineate the molecular and cellular events necessary to identify potential novel therapeutic targets for heart muscle dysfunction in children.

  17. 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

  18. 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.

  19. 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

  20. 阿霉素诱导扩张型心肌病大鼠心功能的变化%The Changes of Cardiac Function in Rats with Adriamycin-induced Dilated Cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    李倩晓; 那荣妹; 李晓菲; 刘百亭; 于勤

    2013-01-01

    Objective:To investigate the changes of cardiac function in rats with adriamycin-induced dilated cardiomyopathy.Method:85 male SD rats(SPF degree,weighing 240-290 g,8 weeks old)were divided into 2 groups:DCM group(n=65),normal control group(n=20). The rats of DCM group were given intraperitoneal injection of adriamycin(2.5 mg/kg every time,one time every week for 6 weeks,total dose:15 mg/kg);the rats of normal control group were given the same injection volume of normal saline instead of adriamycin intraperitoneal injection. In the 10th week after intraperitoneal injection,the two groups of rats underwent echocardiography for left ventricular end-diastolic diameter,left ventricular end-systolic diameter,left ventricular ejection fraction and left ventricular fractional shortening. Result:Cardiac ultrasound examination showed that expanded heart chamber and diffuse weakening of the wall activity of DCM group of rats. Their left ventricular end-diastolic diameter and left ventricular end-systolic diameter were significantly larger than normal control group(P<0.05),while left ventricular ejection fraction and left ventricular fractional shortening were significantly lower than normal control group(P<0.05). Conclusion:Intraperitoneal injection of adriamycin induced DCM rats cardiac function decline.%目的:通过腹腔注射阿霉素诱导制备扩张型心肌病(dilated cardiomyopathy,DCM)大鼠模型,探讨其心功能的变化。方法:选取体质量为240~290 g的8周龄、SPF级近交系雄性SD大鼠85只,分为2组:DCM组(n=65)、正常对照组(n=20)。DCM组采用腹腔注射阿霉素的方法构建DCM模型;正常对照组则注射等容积的生理盐水。给药第10周对两组大鼠行心脏超声检查,观察左室舒张末期内径、左室收缩末期内径、左室射血分数、左室短轴缩短率。结果:心脏超声检查显示DCM组大鼠心腔扩大,室壁活动度弥漫性减弱,左室舒

  1. 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.

  2. Insights into restrictive cardiomyopathy from clinical and animal studies

    Institute of Scientific and Technical Information of China (English)

    Pierre-Yves Jean-Charles; Yue-Jin Li; Chang-Long Nan; Xu-Pei Huang

    2011-01-01

    Catdiomyopathies are diseases that primarily affect the myocardium,leading to serious cardiac dysfimction and heart failure.Out of the three major categories of candiomyopathies(hypertrophic,dilated and restrictive),restrictive cardiomyopathy(RCM)is less common and also the least studied However,the prognosis for RCM is poor as some patients dying in their childhood The molecular mechanisms behind the disease development and progression are not very clear and the treatment of RCM is very difficult and often ineffective.In this article,we reviewed the recent progress in RCM research from the clinical studies and the translational studies done on diseased transgenic animal models.This will help for a better understanding of tare mechanisms underlying the etiology and development of RCM and for the design of better treatments for the disease.

  3. Indium-111 antimyosin monoclonal antibody uptake in patients with cardiomyopathy and myocarditis

    Energy Technology Data Exchange (ETDEWEB)

    Matsumori, Akira; Yamada, Takehiko; Morishima, Shigeru (Kyoto Univ. (Japan). Faculty of Medicine) (and others)

    1990-10-01

    Prognostic significance of myocardial uptake of indium-111 antimyosin antibody was evaluated in 17 patients with idiopathic cardiomyopathy; 10 patients with dilated cardiomyopathy and 7 patients with hypertrophic cardiomyopathy. Seven of 10 patients with dilated cardiomyopathy showed positive images. Three of these 7 patients with strongly positive scans died after scintigraphic examination. Six of 7 patients with hypertrophic cardiomyopathy showed positive images. Three of the patients with dilated left ventricle had prominent positive scans and higher heart to lung ratio. The heart to lung ratio of antimyosin uptake in total patients was correlated with left ventricular end-diastolic dimension and ejection fraction measured by echocardiography. In patients with myocarditis, all three patients showed positive scintigrams within 4 weeks after the onset of the disease and 1 of 6 patients was positive thereafter, who had dilated ventricle and decreased cardiac function. Thus, indium-111 antimyosin antibody imaging may be useful to evaluate prognosis of patients with cardiomyopathy and myocarditis. (author).

  4. 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.

  5. 扩张型心肌病患者心外膜脂肪厚度与左室重构的相关性研究%Association between epicardial adipose tissue thickness and left ventricular remodeling in dilated cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    马晶

    2014-01-01

    Objective To explore the association between epicardial adipose tissue (EAT) thickness and left ventricular remodeling,left ventricular dysfunction in dilated cardiomyopathy (DCM).Methods One hundred and sixteen patients with DCM (DCM group) and 76 healthy subjects (control group) were examined by ultrasoundcardiogram.Left ventricular end-systolic diameter (LVESD),left ventricular end-diastolic diameter (LVEDD),left ventricular end-systolic volume (LVESV),left ventricular end-diastolic volume (LVEDV),left ventricular end-systolic volume index (LVESVI),left ventficular end-diastolic volume index (LVEDVI),left ventricular ejection fraction (LVEF) and EAT thickness were measured or calculated,and the relationship was evaluated.Results EAT thickness in DCM group was significantly lower than that in control group [(4.7 ± 1.2) mm vs.(7.6 ± 2.1) mm],and there was statistical difference (P < 0.05).The linear correlation analysis results showed there was positive correlation between EAT thickness and LVESD,LVEDD,LVESV,LVEDV,LVEDVI,LVESVI (r =0.236,0.220,0.245,0.256,0.282,0.279,P < 0.05),and there was no relationship between EAT thickness and LVEF (r =0.134,P >0.05).Conclusions In patients with DCM,there is a correlation between EAT thickness and ventricular remodeling.There is no correlation between EAT thickness and left ventricular dysfunction.%目的 探讨扩张型心肌病(DCM)患者的心外膜脂肪(EAT)厚度与左室重构及左室功能不全的关系.方法 116例DCM患者(DCM组)和76例健康体检者(对照组)均行超声心动图检查,测量或计算左室收缩末期内径(LVESD)、左室舒张末期内径(LVEDD)、左室收缩末期容积(LVESV)、左室舒张末期容积(LVEDV)、左室收缩末期容积指数(LVESVI)、左室舒张末期容积指数(LVEDVI)、左室射血分数(LVEF)、EAT厚度等,并进行比较分析.结果 DCM组EAT厚度为(4.7±1.2) mm,显著低于对照组的(7.6±2.1) mm,差异有统计学意义(P<0.05).经线性相

  6. 扩张型心肌病患儿血清periostin蛋白的检测及意义%Expression of serum periostin in children with dilated cardiomyopathy and its clinical significance

    Institute of Scientific and Technical Information of China (English)

    吴岚; 孙景辉; 张春艳; 王朝霞

    2014-01-01

    目的 探讨血清periostin蛋白水平与扩张型心肌病(DCM)患儿病情严重程度的相关性.方法 选取2009年1月至2013年6月于吉林大学第一医院儿科诊断扩张型心肌病患儿32例为DCM组,根据ROSS评分标准分为0~2分组(6例)、3~6分组(7例)、7~9分组(11例)及10~12分组(8例);选取于吉林大学第一医院同期体检的健康儿童20例为健康对照组.酶联免疫法测定各组儿童血清periostin蛋白水平,免疫抑制法测定肌酸激酶同工酶(CK-MB)水平,心脏彩超测定左心室射血分数(LVEF)及左心室舒张末期内径(LVEDD),比较各组间上述指标的差异,采用直线相关分析法分析periostin与ROSS评分及LVEF的相关性.结果 1.与健康对照组比较,DCM组血清periostin蛋白水平显著增高,差异有统计学意义(P=0.00);且DCM患儿随着ROSS评分的增高血清periostin蛋白水平逐渐增高,不同ROSS评分组间差异有统计学意义(P均<0.05).2.与健康对照组比较,DCM组CK-MB水平显著增高,差异有统计学意义(P=0.00);不同ROSS评分组间CK-MB水平比较差异无统计学意义(P均>0.05).3.与健康对照组比较,DCM组LVEF显著降低,差异有统计学意义(P=0.00);且DCM患儿随着ROSS评分的增高LVEF逐渐降低,不同ROSS评分组间差异有统计学意义(P均<0.05).4.与健康对照组比较,DCM组LVEDD显著增大,差异有统计学意义(P=0.00);不同ROSS评分组间LVEDD比较差异无统计学意义(P均>0.05).5.血清periostin蛋白水平与ROSS评分呈正相关(r=0.742,P< 0.001),与CK-MB水平无相关性(r=0.247,P>0.05),与LVEF呈负相关(r=-0.424,P<0.01).结论 血清periostin蛋白水平在DCM患儿中显著增高,其水平与ROSS评分呈正相关,与LVEF呈负相关,可作为DCM患儿病情评估的新指标之一.%Objective To investigate the correlation of serum periostin and severe degree of dilated cardiomyopathy(DCM) in children.Methods Thirty-two children with DCM from Jan.2009 to Jun

  7. 自体骨髓单个核细胞移植治疗心肌病疗效及安全性评估%Effect and safety of autologous intracoronary bone marrow mononuclear cells transplantation in dilated cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    王悦喜; 阿荣; 张迎军; 董莉; 李婧; 杨振华; 任保军

    2014-01-01

    Objective To investigate the effect and safety of autologous bone marrow mononuclear cells(BMMNCs) transplantation in dilated cardiomyopathy.Methods 20 patients aged 18-67 years with dilated cardiomyopathy,who suffered from New York Heart Association class Ⅲ to Ⅳ heart failure,left ventricular ejection fraction(LVEF) <35%,were randomized to transplantation group (intracoronary injection of autologous BMMNCs 1.80 × 109~ 5.90 × 109/L,combined with drug treatment,n=8) or the control group(ordinary drugs treatment,n=12).The left ventricular end diastolic diameter(LVEDD),ejection fraction(EF),6-minute walk test and myocardial metabolism detected by emission computed tomography(ECT) were observed after 6 months of treatment.Results There were significant differences in LVEDD,EF,6-minute walk test between the transplantation group and the control group after 6 months of treatment [(50.3 ± 4.2) mm vs.(55.4 ±3.7) mm,(45.4±5.2)% vs.(39.2±6.3)%,(76.6±5.8) m vs.(69.7±8.6) m,t=2.93,3.21,2.96,respectively,all P<0.05].After 6 months of treatment,LVEDD was shorted from(57.2± 6.5) mm to(50.3±4.2) mm(t=5.60,P<0.01) and EF was increased from(30.4±6.7) % to(45.4 ±5.2) %(t=6.30,P<0.01) in the transplantation group,and EF was increased from(31.1±5.9) % to(39.2±6.3) %(t=3.60,P<0.05) in the control group.Compared with pre-treatment,the 6-minute walk distance were increased in the two group after 6 months of treatment [transplantation group:(76.6±5.8) m vs.(54.0±6.2) m,P<0.05; control group:(69.7±8.6) m vs.(55.0±5.7) m,P<0.05].Myocardialmetabolism density of radioactive 18-fluorodeoxyglucose(18-FDG) in the same segment was significantly increased,and the metabolic density of radioactive FDG in sparse segment was significantly reduced in transplantation group after 6 months of treatment as compared with pre-treatment.No serious complications associated with BMMNCs injection,including ventricular arrhythmia and death,were observed in

  8. 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

  9. 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.

  10. Stem Cell-Based Therapies in Chagasic Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Antonio Carlos Campos de Carvalho

    2015-01-01

    Full Text Available 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.

  11. 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.)

  12. 卡维地洛对扩张型心肌病心力衰竭患者心功能及运动耐量的改善作用%Improving effect of carvedilol on cardiac function and exercise tolerance in patients with congestive heart failure of dilated cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    吴大庆; 杨永健

    2002-01-01

    Objective To observe the improving effect of carvedilol on cardiac function in patients with congestive heart failure(CHF) of dilated cardiomyopathy(DCM).Methods Total 60 patients with chronic heart failure secondary to DCM were divided into two groups randomly, namely carvedilol group additionally treated with carvedilol (the test group), conventional group receiving placebo (the controls). The left ventricular fraction shortening (FS), ejection fraction (EF), stroke volume (SV), left ventricular diastolic dimension (LVDD) were measured with echocardiography, were measured before and after 4 month treatment. Results After treated for 4 months , the cardiac function improved greatly in both groups.In the test group, LVDD, EF and 6 minute walking distance improved more signicantly compared to the controls. Conclusion Carvedilol can improve cardiac function and exercise tolerance in the patients with CHF of DCM obviously.

  13. Psychological disorders in adults with inherited cardiomyopathies and Takotsubo syndrome.

    Science.gov (United States)

    Suárez Bagnasco, Mariana; Núñez-Gil, Iván J

    2016-06-03

    We performed a narrative review about psychological disorders in adults with Takotsubo syndrome and inherited cardiomyopathies. Through the electronic database PubMed and PsycINFO we searched all relevant related manuscripts published between 2000 and 2015. We found twelve studies that explore psychological disorders in Takotsubo syndrome and eight about inherited cardiomyopathies: five enrolled patients with hypertrophic cardiomyopathy, two dilated cardiomyopathy, and one arrhythmogenic right ventricular cardiomyopathy. All papers reported the presence of psychological disorders. In Takotsubo syndrome, depression fluctuates between 20.5 and 48% and anxiety was present among 26 and 56%. A study reported that anxiety increases the probability of developing Takotsubo syndrome. In dilated cardiomyopathy, anxiety was present in 50% and depression in 22%. In arrhythmogenic right ventricular cardiomyopathy, younger age, poorer functional capacity and having experienced at least one implantable cardioverter defibrillator shock, were significant independent predictors of both device-specific and generalized anxiety. In hypertrophic cardiomyopathy, anxiety and depression were present in 45.2% and 17.9%, respectively. Thirty seven percent met diagnostic criteria for anxiety disorders and 21% for mood disorders. Nearby half hypertrophic cardiomyopathy patients report triggering of chest pain, dyspnea, and dizziness by emotional stress. Due to the small number of studies, conclusions are limited. However, we discuss some results.

  14. Bone marrow mesenchymal stem cells transplantation for treatment of dilated cardiomyopathy in rats%骨髓间充质干细胞移植治疗大鼠扩张型心肌病

    Institute of Scientific and Technical Information of China (English)

    徐燕; 张瑶; 李丽丽

    2012-01-01

    BACKGROUND: Myocardial fibrosis induced by dilated cardiomyopathy (DCM) is the pathological basis of heart failure. At present, drug treatment, interventionaI therapy and surgical intervention cannot ameliorate necrotic myocardium and completely improve cardiac function.OBJECTIVE: To investigate the effect of allogenic bone marrow mesenchymal stem cells (BMSCs) transplantation on cardiac function and myocardial fibrosis in rats with DCM.METHODS: Forty Wistar rats were randomly divided into three groups: cell transplantation group (n=15), control group (n=15) and blank group (n=10). DCM models were established in cell transplantation group and control group. Four weeks after the models were established successfully, rats in cell transplantation group were injected with allogeneic BMSCs 150 μL (containing 3×106 cells). Rats in control group and blank group were also implanted with the culture medium in the same amount. RESULTS AND CONCLUSION: Compared with the blank group, echocardiography showed that the left ventricular end-systolic inside diameter was increased before transplantation, while the ejection fraction and fractional shortening was decreased significantly (P < 0.01) in cell transplantation group and control group. Four weeks after transplantation, the echocardiography showed that the left ventricular end-systolic inner diameter was decreased, while the ejection fraction and fractional shortening was increased obviously (P < 0.01) in cell transplantation group compared with before transplantation. Expression of cardiac collagen in cell transplantation group was lower than that in control group (P < 0.05). Compared with the control group, the expression of matrix metalloproteinase 2 and matrix metalloproteinase 9 was significantly increased in the other two groups (P < 0.05). In conclusion, bone marrow mesenchymal stem cells (BMSCs) transplantation can improve cardiac function and ameliorate myocardial fibrosis in rats with DCM.%背景:扩张型心肌

  15. 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

  16. 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.

  17. Predictive value of QT interval dynamicity for sudden death in patients with idiopathic dilated cardiomyopathy%QT间期动态性在扩张型心肌病猝死风险预测中的价值

    Institute of Scientific and Technical Information of China (English)

    包明威; 谭团团; 于胜波; 陈葵; 黄从新

    2010-01-01

    目的 研究QT间期频率依赖性在原发性扩张型心肌病(扩心病)患者猝死风险预测中的作用.方法 选取55例原发性扩心病患者和27例健康志愿者(对照组).询问病史并行心脏超声、心电图和动态心电图检查.检测左室舒张末期内径(LVEDD)、左室射血分数(LVEF)、QT间期离散度(QTd)、心率变异性(SDNN)、QT/RR相关直线的斜率、24 h室性早搏(VPB)和非持续性室性心动过速(NSVT)的次数.随访扩心病患者,随访终点为全因死亡.结果 扩心病组的LVEDD、QTd、VPB、NSVT、QTe/RR(QTe为Q波起始至T波终点的间期)和QTp/RR(QTp为Q波起始至T波顶点的间期)斜率显著高于对照组;LVEF和SDNN显著低于对照组.扩心病猝死组、非猝死组和对照组相比,LVEDD、LVEF、QTd、SDNN、QTe/RR和QTp/RR斜率的差异有统计学意义.扩心病猝死组和非猝死组比较,LVEF、SDNN、QTe/RR和QTp/RR斜率的差异有统计学意义.扩心病NSVT阳性组和NSVT阴性组比较,LVEF、QTd、VPB、QTe/RR和QTp/RR斜率的差异有统计学意义.扩心病患者的猝死率,QTe/RR斜率≥0.210者显著高于<0.210者(54.5%与21.1%,P<0.05);QTp/RR斜率≥0.190者显著高于<0.190者(52.2%与21.9%,P<0.05);在LVEF≤35%和NSVT阳性的基础上结合应用QTe/RR≥0.210或QTp/RR≥0.190,猝死率显著提高.结论 扩心病猝死组QT/RR斜率显著高于非猝死组和对照组,QT频率依赖性对扩心病患者猝死有较高的预测价值,并可进一步提高NSVT和LVEF的预测价值.%Objective To explore the predictive value of QT interval dynamicity for sudden death in patients with idiopathic dilated cardiomyopathy ( DCM ). Methods Fifty-five patients with DCM ( DCM group) and 27 healthy subjects (Control group, Con) were enrolled. Investigations included history collection, clinical examination, echocardiography, electrocardiogram and 24 h ambulatory electrocardiogram. Following indexes were determined: left ventricle end diastolic

  18. 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

  19. [Peripartum cardiomyopathy--a case report].

    Science.gov (United States)

    Banaczek, Zbigniew; Rak, Grzegorz; Gołyska-Rączkiewicz, Danuta

    2015-01-01

    Peripartum cardiomyopathy, a type of dilated cardiomyopathy of unknown origin, occurs in previously healthy women in the final month of pregnancy and up to 5 months after delivery. Although the incidence is low--less than 0.1% of pregnancies--morbidity and mortality rates are high at 5% to 32%. The etiology of left ventricular dysfunction is unknown. Diagnosis of peripartum cardiomyopathy requires heightened awareness among multidisciplinary patient care teams and a high degree of suspicion. Confirmation involves the echocardiography reveals severe left ventricular failure. The outcome of peripartum cardiomyopathy is also highly variable. For some women, the clinical and echocardiographic status improves and sometimes returns to normal, whereas for others, the disease progresses to severe cardiac failure and even sudden cardiac death. Management of peripartum cardiomyopathy should aim first at improving heart-failure symptoms through conventional therapies, and then at administering targeted therapies.The prognosis is best when peripartum cardiomyopathy is diagnosed and treated early. Fortunately, despite a high risk of recurrence in subsequent pregnancies, many patients with peripartum cardiomyopathy recover within 3 to 6 months of disease onset. Future pregnancy is not recommended especially in patients with persistent left ventricular dysfunction because of the risk of dangerous complications.

  20. 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.

    Science.gov (United States)

    Zaragoza, Michael V; Fung, Lianna; Jensen, Ember; Oh, Frances; Cung, Katherine; McCarthy, Linda A; Tran, Christine K; Hoang, Van; Hakim, Simin A; Grosberg, Anna

    2016-01-01

    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.

  1. 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.

  2. Fusiform dilatation of the internal carotid artery in childhood-onset craniopharyngioma : multicenter study on incidence and long-term outcome

    NARCIS (Netherlands)

    Hoffmann, Anika; Warmuth-Metz, Monika; Lohle, Kristin; Reichel, Julia; Daubenbuchel, Anna M. M.; Sterkenburg, Anthe S.; Mueller, Hermann L.

    2016-01-01

    Fusiform dilatations of the internal carotid artery (FDCA) represent a vascular complication following surgery for suprasellar tumors in children. Incidence rate and long-term prognosis of FDCA in terms of survival rates, vascular complications, and quality of survival are unknown for patients with

  3. Restrictive cardiomyopathy. Report of seven cases

    Directory of Open Access Journals (Sweden)

    Fonseca Sánchez Luis Alfonso

    2014-07-01

    Full Text Available Restrictive cardiomyopathy is a disease characterized by ventricular diastolic failure with elevation of end-dyastolic pressure and preserved systolic function. Materials and methods: retrospective study of patients with a diagnosis of restrictive cardiomyopathy. We carry out an analysis of demographic data, clinical presentation, and studies of patients diagnosed in the last 15 years at Instituto Nacional de Pediatría. Results: all included patients had clinical data of heart failure manifested mainly by medium-sized efforts dyspnea on schoolchildren and dyspnea by feeding in infants, as well as polypnea and diaphoresis. The most important signs were hepatomegaly, ascites, and gallop rhythm. Cardiomegaly by right atrial dilatation was the most frequent radiological data. The most frequent electrocardiographic data were dilatation of both atria, ST-segment depression and negative T waves. Echocardiogram showed in all cases binaural dilation and restrictive pattern. Conclusions: our patients were similar to those described in the specialized literature. Echocardiogram is still the best study for the diagnosis and the use of functional measurements as Doppler imaging can help to reveal early diastolic failure. In our country the heart transplant is just feasible; mortality remains 100%. Keywords: Restrictive cardiomyopathy, Heart failure, Cardiomyopathy.

  4. [Prevalence of positive serology to Trypanosoma cruzi in patients with clinical diagnosis of dilated myocardiopathy in the state of Campeche].

    Science.gov (United States)

    Alducin-Téllez, César; Rueda-Villegas, Enrique; Medina-Yerbes, Isaí; Hernández, Oscar; López, Ruth; Peña-Hernández, Virginia; Monteón, Víctor

    2011-01-01

    The prevalence of chronic Chagas' heart disease as a cause of dilated cardiomyopathy is unknown in the State of Campeche, Mexico. A study was conducted to determine the prevalence of positive serology for Trypanosoma cruzi in patients with clinical diagnosis of dilated cardiomyopathy. Of a total of 127 patients diagnosed with dilated cardiomyopathy, we studied 91 with two positive serological tests for T. cruzi. We identified 14 positive cases for a prevalence of 15 % of chronic Chagas' heart disease. This prevalence is similar to that reported for the rest of the Yucatan Peninsula.

  5. Evaluation of left ventricular systolic function in patients with dilated cardiomyopathy by real-time three-dimensional speckle tracking echocardiography%实时三维超声心动图斑点追踪技术评价扩张型心肌病患者左室收缩功能

    Institute of Scientific and Technical Information of China (English)

    李阳; 邓又斌; 黄润青; 孙杰; 刘琨; 汤乔颖

    2013-01-01

    目的 应用实时三维超声心动图斑点追踪技术评价扩张型心肌病(DCM)患者左室收缩功能.方法 应用实时三维超声斑点追踪技术分别测量24例DCM患者(DCM组)和19例健康成人志愿者(对照组)左室收缩期纵向、圆周向、径向以及面积应变峰值,比较两组左室心肌基底部、中间部及心尖部局部应变和总体应变的差异,并分析总体应变与左室射血分数的相关性.结果 DCM组左室心肌纵向、圆周向、径向及面积的基底部、中间部、心尖部局部应变和心肌总体应变均明显小于对照组对应节段,差异均有统计学意义(均P<0.01).左室心肌纵向、圆周向、径向以及面积总体应变均与左室射血分数有良好的相关性(r=0.873、0.862、0.885及0.894,均P<0.01).结论 实时三维超声心动图斑点追踪技术可以为DCM的诊断、疗效评估以及预后判断提供较好的检测手段,具有较大的临床价值.%Objective To evaluate the systolic function of the left ventricle in patients with dilated cardiomyopathy by real-time three-dimensional speckle tracking echocardiography.Methods The peak systolic longitudinal train,circumferential stain,radial strain and area strain of left ventricle were measued by real time three-dimensional speckle tracking echocardiography technology in 24 patients with dilated cardiomyopathy (DCM group) and 19 healthy volunteers (control group).The difference of regional myocardial strain of basal,middle,apical level and global myocardial strain were compared between the two groups.The correlation between global myocardial strain in all directions and left ventricular ejection fraction was analyzed.Results The global and each level longitudinal strain,circumferential strain,radial strain and area strain in DCM group were significantly lower than those in control group(P<0.01).The global myocardial longitudinal train,circumferential stain,radial strain and area strain were

  6. Nursing of the dilated cardiomyopathy complicated with malignant arrhythmia patients treated with cardiac resynchronization pacing defibrillator%经心脏再同步化起搏除颤器治疗扩张型心肌病伴恶性心律失常患者的护理研究

    Institute of Scientific and Technical Information of China (English)

    吴晓英

    2015-01-01

    Objective:To investigate the nursing method and effects in the dilated cardiomyopathy complicated with malignant arrhythmia patients treated with cardiac resynchronization pacing defibrillator. Methods:Ninety patients with the dilated cardiomyopathy complicated with malignant arrhythmia treated with cardiac resynchronization pacing defibrillator were randomly divided into the control group(treatment with routine care) and observation group(treatment with nursing intervention)(45 cases each group). The quality of life in two groups was evaluated by WHO quality of life scale(WHOQOL-BREF) before and after treatment. The changes of clinical indicators,treatment effect, heart function classification, postoperative adverse events and satisfaction were compared between two groups. Results:The LVEF,6 min walking distance,WHOQOL-BREF score(including physical field,psychological field,social field and environment field) in two groups after treatment were significantly higher than those in before treatment(P0. 05). Conclusions:When the patients with dilated cardiomyopathy complicated with malignant arrhythmia are treated with cardiac resynchronization pacing defibrillator,the effective nursing intervention can significantly improve heart function and quality of life.%目的::探讨心脏再同步化起搏除颤器治疗扩张型心肌病伴恶性心律失常的护理方法及其效果。方法:将行心脏再同步化起搏除颤器治疗的扩张型心肌病伴恶性心律失常患者90例,随机分为对照组(常规护理)和观察组(护理干预)各45例。采用世界卫生组织生存质量测定量表( WHOQOL-BREF)评价2组患者治疗前后的生存质量,比较2组患者临床指标改变情况、疗效、心功能分级、术后不良事件发生情况和护理满意度。结果:2组患者治疗后左心室射血分数、6 min 步行距离、WHOQOL-BREF评分(生理领域、心理领域、社会领域、环境领域)均显著增加(P0.05)。结论:扩

  7. PERIPARTUM CARDIOMYOPATHY: A MANAGEMENT DILEMMA

    Directory of Open Access Journals (Sweden)

    Niranjan Kumar

    2014-07-01

    Full Text Available Peripartum cardiomyopathy (PPCM is a form of dilated cardiomyopathy of unclear etiology, affecting women without pre-existing cardiac diseases, during the last month of pregnancy or up to five postpartum months1 As with other form of dilated cardiomyopathies, PPCM involves the systolic dysfunction of the heart with a decrease in Left Ventricular Ejection Fraction (LVEF <40, associated with congestive heart failure and with an increased risk of supra ventricular or ventricular arrhythmias, thromboembolism and even sudden cardiac death. PPCM is diagnosed by exclusion, where the patient has no history of previous heart disease, coincides with the pregnancy period (one month pre-operative to five months post- operative, and where no other possible cause of heart failure present. Diagnosis of Peripartum cardiomyopathy is a dilemma for the obstetricians, physicians, cardiologists, and the general physicians, as most of these patients are hurriedly diagnosed and treated first as a case of Left Ventricular Failure (LVF of some cardiac origin. Specific treatment is started late or not at all. So, relapses are very common with the treatment line of Left Ventricular Failure of cardiac origin only without any specific treatment and no precautions taken, thereafter. Although the exact cause of PPCM is unknown, yet (a some cardio tropic virus (b immune system dysfunction8, (c genetic factors 1, (d deficiency of micro-nutrients or trace elements (e some cardio-toxins may serve as a trigger to malfunction of immune system that may be responsible in the development of PPCM. Recently two cases of PPCM were transferred to our ICU from the obstetrics and gynecology department immediately after deliveries for acute left ventricular failure in quick succession and were diagnosed as PPCM and successfully managed with Diuretic, ACE inhibitor, Beta blocker and Nitrates with mechanical ventilation. After discharge from the hospital they are being followed up and are put

  8. Takotsubo cardiomyopathy or broken heart syndrome: A review article

    Directory of Open Access Journals (Sweden)

    Allahyar Golabchi

    2011-01-01

    Full Text Available Stress-induced cardiomyopathy or Takotsubo cardiomyopathy is a recently increasing diagnosed disease showed by transient apical or mid left ventricular dilation and dysfunction. This sign is similar to acute myocardial infarction but without significant coronary artery stenosis and intra coronary clots. On the other hand there are important and essential differences in their management. Consequently, our physicians should know about its pathophysiology, diagnosis and treatment.

  9. Takotsubo cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Sénior, Juan Manuel

    2015-04-01

    Full Text Available Takotsubo cardiomyopathy or stress-induced cardiomyopathy is often diagnosed as an acute coronary syndrome in postmenopausal women, because its clinical presentation may mimic an acute myocardial infarction: anginal chest pain, changes in the ST segment and T wave in precordial leads and elevated cardiac biomarkers of necrosis. It is characterized by systolic dysfunction with transient ballooning of the apical and middle portions of the left ventricle in the absence of significant coronary disease. Prognosis is good and complete recovery occurs in days to weeks. We report three cases of postmenopausal women with initial diagnosis of acute myocardial infarction; no significant coronary lesions were found in the coronary angiography; apical ballooning, characteristic of this syndrome, was observed on left ventriculography. On follow-up, the three patients had complete recovery of systolic function at six weeks.

  10. Cirrhotic cardiomyopathy

    DEFF Research Database (Denmark)

    Møller, Søren; Henriksen, Jens H

    2010-01-01

    , nitric oxide overproduction, and cannabinoid receptor activation. Systolic incompetence in patients can be revealed by pharmacological or physical strain and during stressful procedures, such as transjugular intrahepatic portosystemic shunt insertion and liver transplantation. Systolic dysfunction has...... and electrophysiological abnormalities. This syndrome is termed cirrhotic cardiomyopathy. Results of experimental studies indicate the involvement of several mechanisms in the pathophysiology, such as reduced beta-adrenergic receptor signal transduction, altered transmembrane currents and electromechanical coupling...

  11. Cirrhotic cardiomyopathy

    DEFF Research Database (Denmark)

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

    2014-01-01

    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...... in relation to invasive procedures such as shunt insertion and liver transplantation. Current pharmacological treatment is nonspecific and directed towards left ventricular failure, and liver transplantation is currently the only proven treatment with specific effect on cirrhotic cardiomyopathy....

  12. Takotsubo Cardiomyopathy Occurring in the Postoperative Period.

    Science.gov (United States)

    Deniz, Süleyman; Bakal, Ömer; İnangil, Gökhan; Şen, Hüseyin; Özkan, Sezai

    2015-02-01

    Takotsubo cardiomyopathy simulates acute myocardial infarction, and it is characterised by reversible left ventricular failure. A case of Takotsubo cardiomyopathy diagnosed after emergency angiography performed in a patient with evidence of acute myocardial infarction in the postoperative period will be described in this report. Transurethral resection of a bladder tumour (TUR-BT) was performed in a 92-year-old male patient by the urology clinic. The patient was transferred to the post-anaesthesia care unit after the operation. An echocardiography was performed because of the sudden onset of dyspnoea, tachycardia (140-150 beats per minute, rhythm-atrial fibrillation) and ST-segment elevation on electrocardiography (ECG) at the first postoperative hour, and midapical dyskinesia was detected at the patient. An immediate angiography was performed due to suspicion of acute coronary syndrome. Patent coronary arteries and temporary aneurysmatic dilatation of the apex of the heart were revealed by angiography. As a result of these findings, the patient was diagnosed with Takotsubo cardiomyopathy by the cardiology service. The patient was discharged uneventfully following 10 days in the intensive care unit. Aneurysm of the apex of the left ventricle and normal anatomy of the coronary arteries in the angiography have diagnostic value for Takotsubo cardiomyopathy. Diuretics (furosemide) and beta-blockers (metoprolol) are commonly used for the treatment of Takotsubo cardiomyopathy. Even though Takotsubo cardiomyopathy is a rare and benign disease, it should be kept in mind in patients suspected for acute myocardial infarction in the postoperative period.

  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. Two cases of apical ballooning syndrome masking apical hypertrophic cardiomyopathy.

    Science.gov (United States)

    Roy, Ranjini Raina; Hakim, Fayaz A; Hurst, R Todd; Simper, David; Appleton, Christopher P

    2014-04-01

    Apical akinesis and dilation in the absence of obstructive coronary artery disease is a typical feature of stress-induced (takotsubo) cardiomyopathy, whereas apical hypertrophy is seen in apical-variant hypertrophic cardiomyopathy. We report the cases of 2 patients who presented with takotsubo cardiomyopathy and were subsequently found to have apical-variant hypertrophic cardiomyopathy, after the apical ballooning from the takotsubo cardiomyopathy had resolved. The first patient, a 43-year-old woman with a history of alcohol abuse, presented with shortness of breath, electrocardiographic and echocardiographic features consistent with takotsubo cardiomyopathy, and no significant coronary artery disease. An echocardiogram 2 weeks later revealed a normal left ventricular ejection fraction and newly apparent apical hypertrophy. The 2nd patient, a 70-year-old woman with pancreatitis, presented with chest pain, apical akinesis, and a left ventricular ejection fraction of 0.39, consistent with takotsubo cardiomyopathy. One month later, her left ventricular ejection fraction was normal; however, hypertrophy of the left ventricular apex was newly noted. To our knowledge, these are the first reported cases in which apical-variant hypertrophic cardiomyopathy was masked by apical ballooning from stress-induced cardiomyopathy.

  15. 磁共振成像对扩张型心肌病转基因模型小鼠左右心室对比分析%Comparative analysis of left and right ventricular dilated cardiomyopathy in transgenic mice by magnetic resonance imaging

    Institute of Scientific and Technical Information of China (English)

    朱皓; 吕丹; 高凯; 张连峰

    2013-01-01

    目的 对cTnTR141W扩张型心肌病转基因模型小鼠左、右心室进行对比分析,研究cTnTR141W转基因小鼠作为右心室心肌病的动物模型的可行性.方法 利用7.0T高场强磁共振成像(MRI)技术,定量分析了2、4、6和8月龄对照组及cTnTR141W转基因模型小鼠左、右心室的舒张末容积(EDV)、收缩末容积(ESV)和射血分数(EF)的变化情况,同时对6月龄对照组cTnTR141W转基因模型小鼠心肌组织进行组织学分析.结果 转基因阴性对照小鼠相比,cTnTR141W转基因小鼠左、右心室的容积在2月龄时已有增大趋势,而射血分数有减小趋势.右心室射血分数减小出现最早也最显著(P<0.05).随年龄增加,cTnTR141W转基因小鼠与转基因阴性对照小鼠相比,右心室的结构和功能的病理生理变化与左心室同时趋于严重.该小鼠左、右心室在4月龄后表现典型的扩张型心肌病表型.结论 cTnTR141W转基因模型小鼠左心室和右心室的扩张性心肌病表型同时出现,该小鼠可作为右室性心肌病等右心室功能下降相关疾病研究的动物模型.%Objective To compare the left and right ventricular function of cTnTR141wtransgenic mice with dilated cardiomyopathy, and to assess whether cTnT transgenic mice can be used as an animal model of right ventricular cardiomyopathy. Methods The structural and functional changes of left and right ventricular myocardium in non-transgenic mice ( NTG) and cTnTR141w transgenic mice at 2, 4 , 6 and 8 months of age were assessed and analyzed by 7. 0 T high-field magnetic resonance imaging technology. Changes of the left and right ventricular end-diastolic volume ( EDV) , end-systolic volume (ESV) , and ejection fraction (EF) were quantitatively analyzed with ages. Pathologic changes of myocardial tissue from NTG and cTnTR141w transgenic mice at 6 month of age were analyzed. Results Compared with the NTG mice, the left and right ventricular volume of cTnTR141w transgenic

  16. 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.

  17. 二维斑点追踪超声心动图评价扩张型心肌病左心功能的研究进展%Progress of Two-dimensional Speckle Tracking Echocardiography in Evaluating Left Heart Function of Dilated Cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    王银荣

    2012-01-01

    二维斑点追踪超声心动图(STE)是近年发展起来的一种超声新技术,是应变/应变率成像的一种方法.这种非侵入性诊断方法能够区分节段心肌的主动和被动运动,量化心肌内的收缩不同步,评估局部心肌功能,具有广阔的应用前景.现简要阐述STE的基本概念及其在定量评价扩张型心肌病的临床应用,并讨论该技术的局限性和发展前景.%Two-dimentional speckle tracking echocardiography( STE )is a new ultrasound method developed in recent years,which is a method of strain/strain rate imaging. This noninvasive diagnostic method can distinguish active myocardial movement from passive movement, quantify myocardial dyssynchrony, and assess partial myocardial function,which has promising application field. Here is to elaborate the basic concept of STE and its clinical application in evaluating dilated cardiomyopathy quantitatively, and discuss the limitations and development prospects of the technology.

  18. Study on Left Ventricular Systolic Function in Patients with Dilated Cardiomyopathy Using Doppler Tissue Imaging%多普勒组织成像对扩张型心肌病患者左室收缩功能的研究

    Institute of Scientific and Technical Information of China (English)

    白文伟; 章克信; 陆映珠; 汪丽琼; 冯震霞

    2004-01-01

    目的探讨多普勒组织成像(Doppler tissue imaging,DTI)评价扩张型心肌病(dilated cardiomyopathy,DCM)患者左室收缩功能的应用价值.方法脉冲DTI测定左室长轴切面室间隔(IVS)左室面、右室面及左室后壁(LVPW)内膜下心肌、外膜下心肌的收缩期运动峰值速度(Peak-S).计算左室面与右室面,内膜下心肌与外膜下心肌间的速度差(△V=peakS1-peakS2);心尖四腔切面测定二尖瓣环外侧收缩期运动峰值速度(Sa峰值速度).结果 DCM患者室壁收缩期运动峰值速度(peak-S)、内外膜间峰值速度差(△V)及Sa峰值速度较正常人明显降低,(P<0.01),二尖瓣侧环Sa峰值速度与LVE F高度相关(r=0.79,P<0.01).结论 DTI可作为评价DCM患者左室局部及整体收缩功能的新方法.

  19. 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

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    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

  20. Emergency management of decompensated peripartum cardiomyopathy

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    Lata Indu

    2009-01-01

    Full Text Available Peripartum cardiomyopathy (PPCM is a rare life-threatening cardiomyopathy of unknown cause that occurs in the peripartum period in previously healthy women. [1] the symptomatic patients should receive standard therapy for heart failure, managed by a multidisciplinary team. The diagnosis of PPCM rests on the echocardiographic identification of new left ventricular systolic dysfunction during a limited period surrounding parturition. Diagnostic criteria include an ejection fraction of less than 45%, fractional shortening of less than 30%, or both, and end-diastolic dimension of greater than 2.7 cm/m 2 body surface-area. This entity presents a diagnostic challenge because many women in the last month of a normal pregnancy experience dyspnea, fatigue, and pedal edema, symptoms identical to early congestive heart failure. There are no specific criteria for differentiating subtle symptoms of heart failure from normal late pregnancy. Therefore, it is important that a high index of suspicion be maintained to identify the rare case of PPCM as general examination showing symptoms of heart failure with pulmonary edema. PPCM remains a diagnosis of exclusion. No additional specific criteria have been identified to allow distinction between a peripartum patient with new onset heart failure and left ventricular systolic dysfunction as PPCM and another form of dilated cardiomyopathy. Therefore, all other causes of dilated cardiomyopathy with heart failure must be systematically excluded before accepting the designation of PPCM. Recent observations from Haiti [2] suggest that a latent form of PPCM without clinical symptoms might exist. The investigators identified four clinically normal postpartum women with asymptomatic systolic dysfunction on echocardiography, who subsequently either developed clinically detectable dilated cardiomyopathy or improved and completely recovered heart function.

  1. A fatal case of peripartum cardiomyopathy.

    Science.gov (United States)

    Cohen, Ronny; Mallet, Thierry; Mirrer, Brooks; Loarte, Pablo; Gale, Michael; Kastell, Paul

    2014-06-01

    Peripartum cardiomyopathy is a life-threatening cardiac condition affecting pregnant women either late in pregnancy or early in the post-partum period. The latest studies show a dramatic improvement in the mortality rates of women affected with this disorder, which has been correlated with advances in medical therapy for heart failure. However, patients continue to die of this condition. The following case report describes a typical patient with peripartum cardiomyopathy diagnosed on clinical grounds, along with echocardiogram findings of severe systolic dysfunction and global hypokinesis consistent with dilated cardiomyopathy. Emergency cesarean delivery had to be performed for fetal distress. There was significant improvement of the patient's condition with standard pharmacological management for heart failure at the time of discharge. However, five weeks after discharge, fatal cardiac arrest occurred. It is hoped that this article will raise awareness about this rare but potentially fatal condition and promote understanding of its main clinical features, diagnostic criteria, and conventional pharmacological management.

  2. Evaluation of dilated cardiomyopathy myocardial ’s motion function using two-dimensional speckle tracking displacement ima-ging%二维斑点追踪位移成像评价扩张型心肌病心肌运动时效性的研究

    Institute of Scientific and Technical Information of China (English)

    张文军; 郭智宇

    2014-01-01

    Objective To investigate clinical diagnosis value of dilated cardiomyopathy myocardial motion functions using two‐dimensional speckle tracking displacement imaging .Methods We quantitated longitudinal displacement and time to peak longitudinal displacement of left ventricular 17 segment through 45 normal persons and 32 patients with dilated car‐diomyopathy myocardial using GE Vivid E9 ultrasound unit and Echopac advanced workstation .Results LV longitudinal displacement was significantly decreasing from basal to apical segments in the normal group .The left ventricular segment displacement was greater in the normal group than DCM group with statistically significant difference .LV time to peak longitudinal displacement was located before AVC (aortic vavle colsed)in the normal group ,and after AVC in the DCM group;LV time to peak longitudinal displacement was more significantly delayed in the normal group than DCM group and there was a statistically significant difference between groups .Conclusion Two‐dimensional speckle tracking displacement imaging can provide new parameter for clinical evaluation of dilated cardiomyopathy myocardial motion functions .%目的:探讨二维斑点追踪位移成像在临床诊断扩张型心肌病心肌运动时效性中的价值。方法应用GE Vivid E9超声诊断仪及Echopac高级心血管分析工作站对45例正常人及32例扩张型心肌病患者的左心室心肌17节段长轴位移及位移达峰时间进行定量分析。结果正常组左心室长轴基底段位移最大,心尖段最小,差异有统计学意义;左心室长轴各节段位移达峰时间均位于AVC(主动脉瓣膜关闭)之前。扩心病组左心室长轴各节段位移均减低,心尖段位移小于基底段差异有统计学意义;左心室长轴大部分节段位移达峰时间均位于AVC之后。正常组左心室长轴位移均大于扩心病组并有统计学差异;两组长轴节段位移达峰时间均有统计学差

  3. Primary prophylaxis of sudden death in hypertrophic cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, and dilated cardiomyopathy.

    Science.gov (United States)

    Klein, George J; Krahn, Andrew D; Skanes, Allan C; Yee, Raymond; Gula, Lorne J

    2005-09-01

    We present an evidence-based overview of primary prevention of sudden cardiac death. Several recent studies have provided important data regarding pharmacologic and device-based therapy for patients with conditions that confer high risk for sudden death. A rational approach to these therapies, with emphasis on implanted cardiovertor defibrillators, is discussed.

  4. 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.

  5. Inherited cardiomyopathies caused by troponin mutations

    Institute of Scientific and Technical Information of China (English)

    Qun-Wei Lu; Xiao-Yan Wu; Sachio Morimoto

    2013-01-01

    Genetic investigations of cardiomyopathy in the recent two decades have revealed a large number of mutations in the genes encoding sarcomeric proteins as a cause of inherited hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), or restrictive cardiomyopathy (RCM). Most functional analyses of the effects of mutations on cardiac muscle contraction have revealed significant changes in the Ca2+-regulatory mechanism, in which cardiac troponin (cTn) plays important structural and functional roles as a key regulatory protein. Over a hundred mutations have been identified in all three subunits of cTn, i.e., cardiac troponins T, I, and C. Recent studies on cTn mutations have provided plenty of evidence that HCM- and RCM-linked mutations increase cardiac myofilament Ca2+ sensitivity, while DCM-linked mutations decrease it. This review focuses on the functional consequences of mutations found in cTn in terms of cardiac myofilament Ca2+ sensitivity, ATPase activity, force generation, and cardiac troponin I phosphorylation, to understand potential molecular and cellular pathogenic mechanisms of the three types of inherited cardiomyopathy.

  6. Role of hepatitis C virus in myocarditis and cardiomyopathies

    Institute of Scientific and Technical Information of China (English)

    Akira Matsumori

    2004-01-01

    Recent nationwide clinico-epidemiological surveys in Japan showed that the occurrence of cardiomyopathies was most frequently seen in the age of sixties, and that cardiomyopathies are important causes of heart failure in the elderly. Viral infection was conventionally considered to cause myocarditis, which resulted in the development of dilated cardiomyopathy. Recent studies suggest that hepatitis C virus (HCV) is involved in the development of dilated cardiomyopathy, hypertrophic cardiomyopathy and arrhythmogenic right ventricular cardiomyopathy in addition to myocarditis. Furthermore, left ventricular aneurysm represents the same morbid state not only after myocardial infarction but also after myocarditis. There were wide variations in the frequency of detection of HCV genomes in cardiomyopathy in different regions and in different populations. Major histocompatibility complex class Ⅱ genes may play a role in the susceptibility to HCV infection, and may influence the development of different phenotypes of cardiomyopathy. If in fact the myocardial damage is caused by HCV, it might be expected that interferon (IFN) administration would be useful for its treatment. Hepatitis patients receiving IFN treatment for hepatitis were screened by thallium myocardial scintigraphy, and an abnormality was discovered in half of the patients. Treatment with IFN resulted in a disappearance of the image abnormality. It has thus been suggested that mild myocarditis and myocardial damage may be cured with IFN. We have recently found that high concentrations of circulating cardiac troponin T are a specific marker of cardiac involvement in HCV infection. By measuring cardiac troponin T in patients with HCV infection, the prevalence of cardiac involvement in HCV infection will be clarified. We are proposing a collaborative work on a global network on myocarditis/cardiomyopathies due to HCV infection. (J Geriatr Cardiol 2004;1(2):83-89. )

  7. Dilating Eye Drops

    Science.gov (United States)

    ... Corneal Abrasions Dilating Eye Drops Lazy eye (defined) Pink eye (defined) Retinopathy of Prematurity Strabismus Stye (defined) Vision ... Corneal Abrasions Dilating Eye Drops Lazy eye (defined) Pink eye (defined) Retinopathy of Prematurity Strabismus Stye (defined) Vision ...

  8. A Treatable Cause of Cardiomyopathy: Vitamin D Deficiency

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    Erdal Eren

    2015-08-01

    Full Text Available Dilated cardiomyopathy is an important cause of heart failure in children. Medical therapy rarely results in complete improvement of the disease, treatment of which usually requires transplantation. Herein, we present a patient with cardiomyopathy and rickets. Case report: A 3-month-old boy was referred to Pediatric Endocrinology Clinic due to low calcium level. On his physical examination, enlarged wrists and large anterior fontanel were remarkable. Results of laboratory analyses revealed a calcium level of 6.8 mg/dL, phosphorus level of 4.9 mg/dL, alkaline phosphatase level of 1637 U/L, parathyroid hormone level of 191.2 pg/ mL, and 25-hydroxyvitamin D level of 5.7 ng/mL. Hand-wrist radiograph revealed signs consistent with rickets. Echocardiogram revealed dilated left ventricle, hypokinetic myocardium, an ejection fraction of 42%, and fractional shortening by 20%. Oral calcium lactate was started and then vitamin D treatment was added. At the 3rd month of the therapy, laboratory tests completely returned to normal and signs of rickets disappeared. Echocardiogram findings returned to normal. Since cardiac functions began to improve after the therapy, dilated cardiomyopathy associated with vitamin D deficiency was considered. Vitamin D deficiency should be considered while evaluating dilated cardiomyopathy in the regions that are endemic for nutritional rickets and it should be kept in mind that the therapy may provide dramatic improvement

  9. Cardiomyopathies in children

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    Young Mi Hong

    2013-02-01

    Full Text Available Cardiomyopathy (CMP is a heterogeneous disease caused by a functional abnormality of the cardiac muscle. CMP is of 2 major types, dilated and hypertrophic, and is further classified as either primary or secondary. Secondary CMP is caused by extrinsic factors, including infection, ischemia, hypertension, and metabolic disorders. Primary CMP is diagnosed when the extrinsic factors of secondary CMP are absent. Furthermore, the World Health Organization, American Heart Association, and European Cardiology Association have different systems for clinically classifying primary CMP. Primary CMP is rare and associated with a family history of the disease, implying that genetic factors might affect its incidence. In addition, the incidence of CMP varies widely according to patient ethnicity. Genetic testing plays an important role in the care of patients with CMP and their families because it confirms diagnosis, determines the appropriate care for the patient, and possibly affects patient prognosis. The diagnosis and genetic identification of CMP in patients’ families allow the possibility to identify novel genes that may lead to new treatments. This review focuses on the epidemiology, pathophysiology, diagnosis, and treatment of CMP, with the aim of providing pediatricians with insights that may be helpful in the early identification and management of idiopathic CMP in children.

  10. Discoveries in peripartum cardiomyopathy.

    Science.gov (United States)

    Fett, James D; Markham, David W

    2015-07-01

    The past decade has seen remarkable gains for outcomes in peripartum cardiomyopathy (PPCM), one of the leading causes of maternal mortality and morbidity in the USA and many other countries, including the high-incidence areas of Haiti and South Africa. This review article emphasizes the importance of continuing the process of increasing awareness of PPCM and presents details of this evolving picture, including important discoveries that point the way to full recovery for almost all PPCM subjects. In addition, new interventions will be highlighted, which may facilitate recovery. Numerous studies have demonstrated that when the diagnosis of PPCM is made with LVEF > 0.30, the probability is that recovery to LVEF ≥ 0.50 will occur in the overwhelming majority of subjects. PPCM patients diagnosed with severely depressed systolic function (LVEF < 0.30) and a remodeled left ventricle with greater dilatation (LVEDd ≥ 60mm) are least likely to reach the outcome recovery goals. These are the patients with the greatest need for newer interventional strategies.

  11. Assessment of radial movement of left ventricle with velocity vector imaging in patients with dilated cardiomyopathy%速度向量成像技术评价扩张型心肌病患者径向室壁运动

    Institute of Scientific and Technical Information of China (English)

    王玮; 施仲伟; 胡厚达; 许燕; 张凤如

    2010-01-01

    Objective To assess the radial systolic function of left ventricle(LV) in patients with dilated cardiomyopathy(DCM) by velocity vector imaging(VVI).Methods Sixteen patients with DCM and twenty control subjects were detected by VVI.VVI data were collected from the six basal segments and six mid segments in parastenal LV short axis views.The radial systolic velocity(V) ,strain(ε) ,strain rate(SR),the time to peak systolic velocity(PTV) and the time to maximum strain(PTε) were measured with special software.The differene of the earliest and the latest time to peak velocity(T-max) and the standard deviation of time to peak velocity(T-SD) of 12 segments were calculated.Results Compared to the controlled group,patients with DCM had significantly lower radial V,ε and SR (P <0.01) in all the 12 segments,significantly longer PTV and PTε (P < 0.05) in most segments, and significantly larger T-max and T-SD (P <0.05).Conclusions VVI is useful to assess the abnormalities in LV radial movement in patients with DCM and could provide more information about regional cardiac function.%目的 应用速度向量成像技术(velocity vector imaging,VVI)评价扩张型心肌病(dilated cardiomyopathy,DCM)径向局部心肌收缩功能和同步性.方法 16例DCM患者和20例对照者进行超声心动图检查,脱机分析左室短轴观中6个基底节段和6个中间节段共12个节段的径向收缩期峰值速度(V)、应变(ε)、应变率(SR)、径向速度达峰时间(PTV)、应变达峰时间(PTε),计算12节段的最早与最晚速度达峰时间差值(T-max)及速度达峰时间标准差(T-SD).结果 ①DCM组各节段的V、ε、SR的平均值均显著低于对照组相应节段(P<0.01);②DCM组的PTV除乳头肌水平前间隔及后间隔外,其余节段均大于对照组(P<0.05),PTε除前间隔二尖瓣水平、乳头肌水平和后间隔二尖瓣水平、乳头肌水平外,其余节段均显著延长(P<0.05);③DCM组的T-max

  12. Genetic heterogeneity of left-ventricular noncompaction cardiomyopathy.

    Science.gov (United States)

    Moric-Janiszewska, Ewa; Markiewicz-Łoskot, Grazyna

    2008-05-01

    Isolated noncompaction of the ventricular myocardium (INVM) sometimes referred to as spongy myocardium is a rare, congenital and also acquired cardiomyopathy. It appears to divide the presentation into neonatal, childhood and adult forms of which spongy myocardium and systolic dysfunction is the commonality. The disorder is characterized by a left ventricular hypertrophy with deep trabeculations, and with diminished systolic function, with or without associated left ventricular dilation. In half or more of the cases, the right ventricle is also affected. The sporadic type, however, in some patients, may be due to chromosomal abnormalities and the occurrence of familial incidence. Isolated noncompaction of the left ventricular myocardium in the majority of adult patients is an autosomal dominant disorder. The familial and X-linked disorders have been described by various authors. We here describe the genetic background of this disorder: some of the most mutated genes that are responsible for the disease are (G4.5 (tafazzin gene): alpha-dystrobrevin gene (DTNA); FKBP-12 gene; lamin A/C gene; Cypher/ZASP (LIM, LDB3) gene); and some genotype-phenotype correlations (Becker muscular dystrophy, Emery-Dreifuss muscular dystrophy or Barth syndrome) based on the literature review.

  13. Cardiac function of young and middle-aged patients with dilated cardiomyopathy complica-ting heart failure%中青年扩张型心肌病伴心力衰竭患者心功能的临床研究

    Institute of Scientific and Technical Information of China (English)

    席延琴

    2014-01-01

    Objective To explore the relationship between cardiac function and dilated cardio-myopathy with or without heart failure among young and middle-aged patients and discuss its clinical significance.Methods Examinations of echocardiogram and ECG were carried out on 123 patients with dilated cardiomyopathy in our hospital,among whom were 65 cases complicating heart failure (heart-failure group)and 58 cases not accompanied with the disease(non-heart-failure group).Be-tween the 2 groups,the sizes of heart,cardiac function and the occurrence of arrhythmia were com-pared.Results Compared with non-heart-failure group,the differences in left ventricular end sys-tolic diameter (LVESd ),left ventricular end diastolic diameter (LVEDd ),left atrial diameter (LAD),left ventricular ejection fraction(LVEF)and left ventricular fractional shortening(LVFS) of heart-failure group were statistically significant(P0.05).Compared with non-heart-failure group,there were statistically significant differences (P<0.01 )in the incidences of atrial arrhythmia,ventricular arrhythmia and conduction block in heart-failure group.③ On admission,cardiac function of patients in heart-failure group was classi-fied into level Ⅲ and Ⅳ,accounting for 58.4%and 21.5%,respectively.After treatment in hos-pital,cardiac function of the 2 groups both improved significantly compared with that on admission (P<0.05).Conclusion Dilated cardiomyopathy is followed by obvious increase of left ventricular end diastolic diameter and significant decrease of left ventricular ejection fraction(LVEF)if accompanied with heart failure and arrhythmia while the phenomena are more or less associated with cardiac func-tion.By getting rid of inducing factors of heart failure,early diagnosis and treatment can improve cardiac function,prognosis and life quality of patients.%目的:探讨青年人扩张型心肌病伴或不伴心力衰竭与心脏功能关系及临床意义。方法对我院扩张型心

  14. Assessment of the Left Ventricular Systolic Function in Dilated Cardiomyopathy Patients by Two -dimensional Speckle Tracking Imaging( 2D - STI)%二维斑点追踪成像技术评价扩张型心肌病患者左心室心肌纵向收缩功能

    Institute of Scientific and Technical Information of China (English)

    黄俊; 胡元平; 宋樟伟; 杨炜宇; 徐瑞; 倪显达

    2012-01-01

    Objective To assess the left ventricular systolic function in patients with dilated cardiomyopathy ( DCM ) . Methods 35 healthy subjects and 39 dilated cardiomyopathy patients underwent conventional echocardiography examination. Left atrial ( LA) diameter were measured by M - mode echocardiography, left ventricular( LV) end - systolic volume, end - diastolic volume and left ventricular ejection fraction (LVEF) were calculated by bi -plane Simpson's method. The peak velocity during early diastole(Ve) and late diastole (Va) of anterior mitral valve were measured by pulse -waved doppler, and the ratio Ve/Va was calculated. We acquired the apical four - chamber, two - chamber and the long - axis views of the left ventricular images in these patients with GE - Vivid7 - dimension. Then the peak longitudinal velocity, strain and strain rate in systolic period were measured and recorded. Results The values of LAD, LVESV and LVEDV in DCM patients were significantly higher than those of healthy subjects (P 0. 05 ) . The peak velocity in systolic period of the base and middle LV segments in DCM patients were lower than those of the healthy subjects (P < 0. 05). The peak longitudinal strain and strain rate were significantly lower than healthy subjects (P < 0. 01). The peak velocity of the healthy subjects and the DCM patients were descent from the base to the apex. Conclusion The peak velocity, stain and strain rate of regional myocardial function in long - axis of left ventricular can be analyzed by 2D - STI, and it is a feasible technique for the assessment of cardiac longitudinal systolic function in DCM patients, and it can be widely used in the cardiac examination.%目的 评价二维斑点追踪成像技术(2D - STI)在扩张型心肌病(dilated cardiomyopathy,DCM)患者的左心室心肌纵向收缩功能应用价值.方法 对39例DCM患者和35例正常对照组行常规超声心动图检查得到左心房内径(LAD)、左心室射血分数(LVEF)、过二尖瓣口

  15. O papel da L-carnitina no estado nutricional e na evolução ecocardiográfica da cardiomiopatia dilatada idiopática da infância The role of L-carnitine in nutritional status and echocardiographic parameters in idiopathic dilated cardiomyopathy in children

    Directory of Open Access Journals (Sweden)

    Vitor M. P. Azevedo

    2005-10-01

    Full Text Available OBJETIVO: A desnutrição é marcadora independente de óbito na cardiomiopatia dilatada idiopática. Foi analisada a repercussão da introdução da L-carnitina nos parâmetros nutricionais e ecocardiográficos em crianças com cardiomiopatia dilatada idiopática. MÉTODOS: Estudo prospectivo aberto de 11 crianças, comparadas com 40 controles, pareados para sexo e idade. Foi administrada L-carnitina oral (100 mg/kg/dia, além do tratamento padrão. Foram realizadas 118 pesagens no grupo L-carnitina e 264 nos controles, além de 65 ecocardiogramas no grupo L-carnitina e 144 nos controles. Análise estatística: qui-quadrado, teste t de Student, ANOVA e correlação de Pearson. Foi utilizado alfa = 0,05. RESULTADOS: Grupo L-carnitina: idade = 3,82 anos, 72,7% (p = 0,033 menores de 2 anos e do sexo feminino, e 90,9% (p = 0,001 em classe funcional III e IV. Não ocorreram óbitos no período. Não houve diferença no percentil de peso inicial (31,2±8,74 vs. 19,6±21,2 (p = 0,29 nem no índice z (-0,68±1,05 vs. -1,16±0,89 (p = 0,24. Ocorreu aumento do percentil (p = 0,026 e do índice z (p = 0,033 após a L-carnitina. Não houve diferença na fração de ejeção na apresentação (54,9%±3,8 vs. 49,3%±6,6 (p = 0,19, porém a massa VE/SC foi superior no grupo L-carnitina (169,12 g/m²±26,24 vs. 110,67 g/m²±15,62 (p = 0,0005. Após a L-carnitina, a ANOVA demonstrou aumento da fração de ejeção (48,3±7 para 67,2±7 (p = 0,044, e a massa do VE/SC foi reduzida (164,29g/m²±28,14 para 110,88g/m²±28,88, porém sem significância estatística (p = 0,089. CONCLUSÃO: Na cardiomiopatia dilatada idiopática na infância, a suplementação com L-carnitina pode auxiliar na recuperação nutricional e na melhora da fração de ejeção, facilitando a reversão do quadro de caquexia e da insuficiência cardíaca.OBJECTIVES: Malnutrition is an independent predictor of death in idiopathic dilated cardiomyopathy. An analysis was performed of the

  16. Evolução clínica e capacidade funcional de pacientes com cardiomiopatia dilatada após quatro anos do transplante Clinical and functional capacity of patients with dilated cardiomyopathy after four years of transplantation

    Directory of Open Access Journals (Sweden)

    Daniela Gardano Bucharles Mont'Alverne

    2012-12-01

    Full Text Available OBJETIVO: Avaliar a evolução do paciente miocardiopata após transplante (Tx cardíaco, analisando sua sobrevida, complicações trans e pós-operatórias e respostas cardiovasculares após cerca de quatro anos do procedimento cirúrgico. MÉTODOS: A pesquisa foi realizada no período de fevereiro a maio de 2011, com pacientes submetidos a Tx cardíaco no Hospital Dr. Carlos Alberto Studart Gomes - Hospital de Messejana (HDM. A amostra foi composta de todos os pacientes transplantados no ano de 2007 no referido hospital. Inicialmente, foi aplicada uma ficha de avaliação, coletando dados dos prontuários, sobre a evolução do paciente no período trans e pós-operatório até a alta hospitalar. Após a coleta dessas informações, os pacientes foram submetidos ao teste da caminhada dos seis minutos (TC6. Os valores encontrados na distância percorrida foram comparados aos valores de referência esperados para a população utilizando a equação de Enright e Sherrill. RESULTADOS: Do total de 24 pacientes que realizaram Tx cardíaco no HDM no ano de 2007, 14 foram avaliados e 10 excluídos do estudo. Com relação às complicações, no período transoperatório, a mais evidenciada foi a disfunção do ventrículo direito (64,3% e, no pós-operatório, quadro de taquicardia (64,3%. Analisando o TC6 observou-se diminuição de 11,6% na distância percorrida quando comparada à distância estimada (486 ± 55 m, 550 ± 59 m, respectivamente. CONCLUSÃO: Os resultados obtidos neste estudo perante o TC6 evidenciam que as respostas cardiovasculares dos pacientes avaliados estão abaixo do estimado, contudo dentro da faixa de normalidade estabelecida.OBJECTIVE: To evaluate patient with cardiomyopathy's progress after cardiac transplant, by analyzing his survival, complications and cardiovascular responses after nearly four years of surgery. METHODS: The survey was conducted from February to May 2011, with patients undergoing cardiac transplantation

  17. Smooth muscle LDL receptor-related protein-1 deletion induces aortic insufficiency and promotes vascular cardiomyopathy in mice.

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    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.

  18. Peripartum cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Rodolfo Citro

    2011-07-01

    Full Text Available Peripartum cardiomyopathy is an uncommon form of congestive heart failure associated with systolic dysfunction of left ventricle. The onset is characterised by symptoms of heart failure occurring between the last month of pregnancy and 5-6 months postpartum. The early diagnosis and the institution of medical treatment for this disease are essential because the inadequate management may affect the patient’s long-term prognosis and can lead to severe complications, including death.Currently its aetiology is not completely understood. Many aetiopathogenetic hypotheses have been formulated: inflammation, viral agents, autoimmune processes. In the last years, evidences aroused for a role of prolactin and its 16 kDa metabolite in reducing cardiomyocite metabolic activity and contraction. In this article we have reviewed the current literature with special emphasis on the role of prolactin and the related current treatment strategies. In particular, bromocriptine appears promising, even if women need to be informed that the drug stops the production of breastmilk. Further researchers, such as large multicenter trials, are needed to decide the best treatment for the women suffering of this disease.

  19. Mutation Analysis of Mitochondrial tRNA Gene in Patients with Primary Dilated Cardiomyopathy%原发性扩张型心肌病的线粒体tRNA基因突变分析

    Institute of Scientific and Technical Information of China (English)

    李红超; 舒红英; 李晓杰; 倪斌; 谢海龙; 周海燕; 倪崖

    2015-01-01

    To identify the potential pathogenic mutations of mitochondrial tRNA in patients with primary dilated cardio-myopathy(DCM) and the possible association of the mutations with DCM.Paraffin-embedded myocardial tissues from two patients with DCM and 10 healthy controls,which were discarded after forensic examination,were used for the study.PCR amplification wasperformed for the mitochondrial tRNA genes and direct sequencing was conducted.Sequencing re-sults showed no variation for mitochondrial tRNA genes in the normal myocardial tissues.The tRNAVal G1664A variation and tRNAMet T4454C variation were identified in patients with DCM.These two variations were previously reported as polymorphism in MitoMap.There was no pathogenic mutation detected in mitochondrial tRNA genes of the two patients with DCM.A patient pool of large sample size is expected for analysis of the pathogenic mutations,polymorphism loci and haplogroup that might be associated with DCM.%为寻找原发性扩张型心肌病病例是否存在已知以及未知的线粒体tRNA致病性突变,以探讨扩张型心肌病可能的发病原因.收集2例原发性扩张型心肌病患者和10例正常对照尸检心肌组织石蜡标本,针对22种线粒体tRNA基因分别设计一对引物,PCR扩增后并测序分析线粒体tRNA基因突变情况.结果在对照样本中未检测到线粒体tRNA变异位点,在1例患者中检测到了tRNAVal基因G1664A变异,Mitomap已有报道为多态性位点;于另1例患者中检测到tRNAMetT4454C变异,有文章报道该位点与线粒体功能障碍有关,Mitomap报道为多态性位点.本研究中2例病例中未检测到线粒体tRNA致病性突变位点,可能与病例个体的心衰程度有关,有必要扩大样本量深入研究线粒体tRNA以及mtDNA其他基因突变与原发性扩张型心肌病之间的关系,以寻找可能的致病突变位点、易感的多态性位点或者单倍体群,为认识原发性扩张型心肌病的发病机制进一步提供理论基础和依据.

  20. Effect of high epidural anesthesia on interleukin-6 and soluble interleukin-2 receptor in patients with dilated cardiomyopathy%高位硬膜外阻滞对扩张型心肌病患者白细胞介素6及可溶性白细胞介素2受体的影响

    Institute of Scientific and Technical Information of China (English)

    李秀玉; 辛晓敏; 刘凤岐

    2005-01-01

    治疗组治疗后可溶性白细胞介素2受体水平[(1 086.68±1.34)ng/L]低于治疗前[(1 328.01±1.51)ng/L,(t=2.145,P<0.05)].常规治疗组治疗前后可溶性白细胞介素2受体水平相似[(1473.33±1.66)ng/L(1 331.07±1.52)ng/L,t=-1.06,P>0.05].结论:高位硬膜外阻滞治疗后细胞因子白细胞介素6及可溶性白细胞介素2受体水平都明显下降,而常规治疗组未见此效果,表明高位硬膜外阻滞治疗对细胞因子有良好的调节作用,优于常规治疗.高位硬膜外阻滞对细胞因子的调节作用与其阻滞效应中的全面抑制心脏交感神经,抑制交感神经对体液免疫系统的激活及阻断其恶性循环有关.%BACKGROUND: Serum cytokines in patients with dilated cardiomyopathy are increased obviously, and the expression of interleukin-6mRNA is also observed in myocardial tissues. High epidural anesthesia can block the vicious cycle involving cytokines and improve cardiac function. OBJECTIVE: To observe the changes of interleukin-6 and soluble interleukin-2 receptor in patients with dilated cardiomyopathy after high epidural anesthesia treatment. DESIGN: A case-controlled analysis. PARTICIPANTS: Thirty-five inpatients with dilated cardiomyopathy were selected from the Department of Cardiology, First Hospital Affiliated to Harbin Medical University, from October 2001 to May 2002. All the patients were randomly divided into high epidural anesthesia group and conventianal treatment group. High epidural anesthesia group consisted of 22patients, 15 males and 7 females, whose cardiac function was classified into grade Ⅱ in 4 patients, grade Ⅲ in 9 and grade Ⅳ in 9. Conventional treatment group consisted of 13 patients, 11 males and 2 females, whose cardiac function was grade Ⅱ in 1 patient, grade Ⅲ in 5 and grade Ⅳ in 7. Healthy control group comprised 21 people, 13 males and 8 females,who received physical examination at the same period. INTERVENTIONS: Patients with dilated

  1. Magnetic Resonance Imaging of Non-ischemic Cardiomyopathies: A Pictorial Essay

    Directory of Open Access Journals (Sweden)

    Cristina I Olivas-Chacon

    2015-01-01

    Full Text Available Non-ischemic cardiomyopathies are defined as either primary or secondary diseases of the myocardium resulting in cardiac dysfunction. While primary cardiomyopathies are confined to the heart and can be genetic or acquired, secondary cardiomyopathies show involvement of the heart as a manifestation of an underlying systemic disease including metabolic, inflammatory, granulomatous, infectious, or autoimmune entities. Non-ischemic cardiomyopathies are currently classified as hypertrophic, dilated, restrictive, or unclassifiable, including left ventricular non-compaction. Cardiovascular Magnetic Resonance Imaging (CMRI not only has the capability to assess cardiac morphology and function, but also the ability to detect edema, hemorrhage, fibrosis, and intramyocardial deposits, providing a valuable imaging tool in the characterization of non-ischemic cardiomyopathies. This pictorial essay shows some of the most important non-ischemic cardiomyopathies with an emphasis on magnetic resonance imaging features.

  2. Magnetic Resonance Imaging of Non-ischemic Cardiomyopathies: A Pictorial Essay.

    Science.gov (United States)

    Olivas-Chacon, Cristina I; Mullins, Carola; Stewart, Kevan; Akle, Nassim; Calleros, Jesus E; Ramos-Duran, Luis R

    2015-01-01

    Non-ischemic cardiomyopathies are defined as either primary or secondary diseases of the myocardium resulting in cardiac dysfunction. While primary cardiomyopathies are confined to the heart and can be genetic or acquired, secondary cardiomyopathies show involvement of the heart as a manifestation of an underlying systemic disease including metabolic, inflammatory, granulomatous, infectious, or autoimmune entities. Non-ischemic cardiomyopathies are currently classified as hypertrophic, dilated, restrictive, or unclassifiable, including left ventricular non-compaction. Cardiovascular Magnetic Resonance Imaging (CMRI) not only has the capability to assess cardiac morphology and function, but also the ability to detect edema, hemorrhage, fibrosis, and intramyocardial deposits, providing a valuable imaging tool in the characterization of non-ischemic cardiomyopathies. This pictorial essay shows some of the most important non-ischemic cardiomyopathies with an emphasis on magnetic resonance imaging features.

  3. Cardiomyopathy in congenital and acquired generalized lipodystrophy: a clinical assessment.

    Science.gov (United States)

    Lupsa, Beatrice C; Sachdev, Vandana; Lungu, Andreea O; Rosing, Douglas R; Gorden, Phillip

    2010-07-01

    Lipodystrophy is a rare disorder characterized by loss of adipose tissue and low leptin levels. This condition is characterized by severe dyslipidemia, insulin resistance, diabetes mellitus, and steatohepatitis. Another phenotypic feature that occurs with considerable frequency in generalized lipodystrophy is cardiomyopathy. We report here the cardiac findings in a cohort of patients with generalized congenital and acquired lipodystrophy, and present a literature review of the cardiac findings in patients with generalized lipodystrophy. We studied 44 patients with generalized congenital and acquired lipodystrophy, most of them enrolled in a clinical trial of leptin therapy. Patients underwent electrocardiograms and transthoracic echocardiograms to evaluate their cardiac status. We followed these patients for an extended time period, some of them up to 8 years. Evaluation of our cohort of patients with generalized lipodystrophy shows that cardiomyopathy is a frequent finding in this population. Most of our patients had hypertrophic cardiomyopathy, and only a small number had features of dilated cardiomyopathy. Hypertrophic cardiomyopathy was more frequent in patients with seipin mutation, a finding consistent with the literature. The underlying mechanism for cardiomyopathy in lipodystrophy is not clear. Extreme insulin resistance and the possibility of a "lipotoxic cardiomyopathy" should be entertained as possible explanations.

  4. Cardiomyopathy in Africa: heredity versus environment.

    Science.gov (United States)

    Mayosi, Bongani M; Somers, Krishna

    2007-01-01

    Unlike other parts of the world in which cardiomyopathy is rare, heart muscle disease is endemic in Africa. The major forms of cardiomyopathy in Africa are dilated cardiomyopathy (DCM) and endomyocardial fibrosis (EMF). Whereas DCM is a major cause of heart failure throughout the continent, EMF is restricted to the tropical regions of East, Central, and West Africa. Although epidemiological studies are lacking, hypertrophic cardiomyopathy and arrhythmogenic right ventricular cardiomyopathy seem to have characteristics similar to those of other populations elsewhere in the world. Recent advances in the genetic analysis of DCM in other parts of the world indicate that it is a genetically heterogeneous disorder in which some cases have a Mendelian cause and others have a non-genetic or multifactorial cause. This heterogeneous pattern of inheritance has been confirmed in small studies that have been conducted so far in Africa. The advent of human immunodeficiency virus infection and its association with cardiomyopathy has emphasised the role of inflammatory agents in the pathogenesis of DCM. By contrast with DCM in which some cases have major genetic contributions, there is scanty evidence for the role of genetic factors in the aetiology of EMF. Although the pathogenesis of EMF is not fully understood, it appears that the conditioning factor may be geography (in its widest sense, to include climate and socio-economic status), the triggering factor may be an as yet unidentified infective agent, and the perpetuating factor may be eosinophilia. There is a need for renewed effort to identify genetic and non-genetic factors in EMF and other forms of heart muscle disease that are prevalent on the continent of Africa.

  5. Molecular pathogenetic mechanisms and new therapeutic perspectives in anthracycline-induced cardiomyopathy

    OpenAIRE

    Distefano Giuseppe

    2009-01-01

    Abstract Anthracyclines are among the most powerful drugs for the treatment of oncologic diseases both in childhood and in adulthood. Nevertheless, their major antineoplastic efficacy can be seriously impaired by collateral toxic cardiac effects causing cardiomyopathy with chronic heart failure that is refractory to conventional medical therapy. This article reports possible subcellular molecular alterations of anthracycline-induced cardiomyopathy (reactive oxygen species formation, apoptosis...

  6. 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.

  7. 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)

  8. Clinical Characteristics and Treatment of Cardiomyopathies in Children.

    Science.gov (United States)

    Price, Jack F; Jeewa, Aamir; Denfield, Susan W

    2016-01-01

    Cardiomyopathies are diseases of the heart muscle, a term introduced in 1957 to identify a group of myocardial diseases not attributable to coronary artery disease. The definition has since been modified to refer to structural and or functional abnormalities of the myocardium where other known causes of myocardial dysfunction, such as systemic hypertension, valvular disease and ischemic heart disease, have been excluded. In this review, we discuss the pathophysiology, clinical assessment and therapeutic strategies for hypertrophic, dilated and hypertrophic cardiomyopathies, with a particular focus on aspects unique to children.

  9. Cardiomyopathy induced by pulmonary sequestration in a 50-year-old man.

    Science.gov (United States)

    Chatelain, Shaun; Comp, Robert A; Grace, R Randal; Sabbath, Adam M

    2015-02-01

    A 50-year-old black man presented at the emergency department with midsternal, nonradiating chest pressure and chronic dyspnea on exertion. Four years before the current admission, he had been diagnosed with nonischemic cardiomyopathy at another facility. After our complete evaluation, we suspected that his symptoms arose from left-to-left shunting in association with pulmonary sequestration, a congenital malformation. Our preliminary diagnosis of secondary dilated cardiomyopathy was confirmed by normalization of the patient's ventricular size and function after lobectomy. To our knowledge, this patient is the oldest on record to present with cardiomyopathy consequent to pulmonary sequestration. His case is highly unusual because of his age and the rapid resolution of his symptoms after lobectomy. We believe that pulmonary sequestration should be included in the differential diagnosis of dilated cardiomyopathy.

  10. Comparison of the effects of carvedilol and astragalus on the cardiac function in rats with dilated cardiomyopathy%卡维地洛和黄芪对扩张型心肌病大鼠心功能影响的对比观察

    Institute of Scientific and Technical Information of China (English)

    袁勇华; 何学华; 方亦兵

    2011-01-01

    目的 探讨卡维地洛和黄芪对阿霉素诱导的扩张型心肌病(dilated cardiomyopathy,DCM)大鼠心功能的影响.方法 将Wistar大鼠应用阿霉素腹腔注射,于第8周末建立Wistar DCM大鼠模型.将造模成功的大鼠分为DCM模型对照组、卡维地洛治疗组和黄芪治疗组,并设立健康对照组.自第9周至12周末,健康对照组与DCM模型对照组予双蒸水灌胃4周,卡维地洛治疗组和黄芪治疗组分别予以卡维地洛和黄芪加双蒸水灌胃治疗4周.应用超声检测左室舒张期末内径(LVDD)、左室收缩期末内径(LVSD)、左室射血分数(LVEF)、左室缩短分数(LVFS).结果 至12周末,与DCM模型对照组比较,卡维地洛治疗组和黄芪治疗组的LVSD和LVDD均明显减小,LVEF和LVFS均明显增高(P均< 0.01);卡维地洛治疗组和黄芪治疗组的LVSD高于健康对照组,LVEF、LVFS低于健康对照组(P均< 0.01);卡维地洛治疗组与黄芪治疗组比较,LVDD、LVSD、LVEF和LVFS差异无统计学意义(P均> 0.05);与第8周末时比较,DCM模型对照组在12周末时LVSD明显增加,LVEF和LVFS明显降低(P均< 0.05);卡维地洛治疗组和黄芪治疗组与第8周末的DCM模型对照组比较,两组的LVSD明显减小,LVEF和LVFS均明显增高(P均< 0.05).结论 卡维地洛和黄芪均能有效改善阿霉素诱导的DCM大鼠心功能,两者在改善心功能方面无明显差异.%Objective To explore the effects of carvedilol and astragalus on cardiac functions in rats with adriamycin-induced dilated cardiomyopathy ( DCM) . Methods The Wistar rats were randomly divided into normal group and model group. The rats in model group were injected with adriamycin (ADR) to establish the rat model of DCM. At the end of the eighth week. the successful modeling rats were divide into DCM control group (n = 17) ,carvedilol group (n = 16) and astragalus group (n = 16). Then the interventions started and lasted for four weeks. The rats were gavaged by double

  11. Expressions and Significance of Th1/Th2/Th17 Cytokines and Antimyocardial Antibodies in Mouse Dilated ;Cardiomyopathy%Th1/Th2/Th17细胞因子及抗心肌抗体在小鼠扩张型心肌病中的表达及其意义

    Institute of Scientific and Technical Information of China (English)

    李丽萍; 孔清; 赖文盈; 潘晓芬; 伍伟锋

    2015-01-01

    Objective:To observe the expressions of Th1/Th2/Th1 7 cytokines and antimyocardial antibodies in mouse dilated cardiomyopathy (DCM),and explore their roles and significance in DCM pathogenesis.Methods:BALB/c mouse model of DCM was built with coxsackievirus(CVB3).Mice in control group were treated with phosphate-buffered saline (PBS)i.p. Mice were killed at the 24th week,and the fibrosis degree of myocardial tissue was observed by myocardial Masson’s staining. Expressions of Th1/Th2/Th1 7 cytokines in plasma were detected by using protein microarray.The mouse plasma levels of an-timyocardial antibodies such as adenine nucleotide translocator(ANT)antibody,β-myosin heavy chain(β-MHC)antibody,car-diac L-type calcium channel(CACH2),and anti-β1-adrenergic receptor(β1 AR)were measured by enzyme-linked immunosorbent assay (ELISA).Results:The result of myocardial pathological examination in DCM group fit the features of DCM 24 weeks af-ter virus infection.Compared with those in control group,expression levels of interleukin(IL)-2,IL-10,IL-13,IL-28 and TNF-αin DCM group decreased(P <0.05),however,the expression levels of IL-6,IL-1 7,IL-21 ,IL-22,IL-23 and TGF-βin-creased significantly(P <0.05).Meanwhile,compared with those in control group,the plasma levels of ANT andβ-MHC anti-bodies in DCM group increased significantly (P <0.05).Furthermore,positive correlation was found between level of IL-22 and level of anti-ANT according to correlative analysis(P <0.05 ).Conclusions:Immunologic derangement induced by imbal-ance of antimyocardial antibodies such as ANT and β-MHC,and Th1/Th2/Th1 7 cytokines such as IL-2、IL-10、IL-13、IL-28、TNF-α、IL-6、IL-1 7、IL-21 、IL-22、IL-23 and TGF-βmay play important roles in the pathogenesis of DCM.Furthermore,IL-22 may boost the production of ANT antibody.%目的::观察 Th1/Th2/Th17细胞因子及抗心肌抗体在小鼠扩张型心肌病(dilated cardiomyopathy,DCM)中的表达,并探讨其在 DCM 发病机制中

  12. Bloqueio simpático esquerdo por videotoracoscopia no tratamento da cardiomiopatia dilatada Bloqueo simpático izquierdo por videotoracoscopia en el tratamiento de la cardiomiopatía dilatada Endoscopic left sympathetic blockade in the treatment for dilated cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Paulo M. Pêgo-Fernandes

    2010-12-01

    effects. METHODS: Fifteen patients with dilated cardiomyopathy and left ventricular ejection fraction (LVEF 65 bpm, despite either adequate betablocker use or intolerant to it, were enrolled. Ten patients underwent left infra-stellate ganglion plus T3-T4 interspinal space clipping through videothoracoscopy, while the other five patients were randomized to a control group. RESULTS: None of the treated patients had any procedure-related adverse cardiovascular events at the perioperative period. Two patients from the surgical group died due to pulmonary thromboembolism or myocardial infarction within 6 months of the initial follow-up, while three patients from the control group had heart failure progression and died or developed cardiogenic shock during the same period. Treated patients presented improvement in quality of life, level of physical activity and LVEF (from 25 ± 9% to 32 ± 8%, p=0.024 at 6 months of follow-up, whereas these parameters did not change in control patients. CONCLUSION: Endoscopic left thoracic sympathetic blockade is feasible and appears to be safe in severe heart failure patients. This initial study suggests that this procedure might be an effective alternative approach to sympathetic blockade in the treatment of dilated cardiomyopathies.

  13. Atlas of the clinical genetics of human dilated cardiomyopathy

    DEFF Research Database (Denmark)

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

    2015-01-01

    : 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...... a sound database of the genetic causes of DCM....

  14. Dilated pore of winer

    Directory of Open Access Journals (Sweden)

    Mittal R

    2002-01-01

    Full Text Available Two cases of dilated pore of Winer were observed. First case had single defined black papule with well defined margin, central pore and discharge of black powdery material from nose since 3 years. The second case had one 9mm, black well-defined papule with central pore discharging black powdery material on right forearm since 9 months and 9 similar smaller papules were seen on forearm and lower abdomen. Histopathologically both revealed greatly dilated infundibulum lined by acanthotic epidermis and atrophic subinfundibular hair structures thus confirming diagnosis of dilated pore of Winer

  15. Penetrance of Hypertrophic Cardiomyopathy in Children and Adolescents

    DEFF Research Database (Denmark)

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

    2013-01-01

    The penetrance of hypertrophic cardiomyopathy (HCM) during childhood and adolescence has been only sparsely described. We studied the penetrance of HCM and the short- and long-term outcomes of clinical screening and predictive genetic testing of child relatives of patients with HCM....

  16. [Peripartum cardiomyopathy: A multiple entity].

    Science.gov (United States)

    Vanzetto, Gérald; Martin, Alix; Bouvaist, Hélène; Marlière, Stéphanie; Durand, Michel; Chavanon, Olivier

    2012-06-01

    Peripartum cardiomyopathy (PPCMP) is a dilated and hypokinetic cardiomyopathy occurring during pregnancy or after delivery, with an estimated incidence between 1/1000 and 1/4000 births. It has been defined as a new onset of heart failure in the month preceding or following delivery, without demonstrated aetiology nor previously known heart disease, and with echocardiographic evidences of left ventricular (LV) dysfunction (LV ejection fraction<0.45). It's a multifactorial disease, immunologic, hormonal, and possibly viral mechanisms playing a determinant pathophysiological role. The classical clinical presentation is a rapid and unexpected onset of heart failure in a previously healthy woman, echocardiography being the key examination for positive and differential diagnosis, prognostication, therapeutic decision-making, and follow-up. The potential severity of PPCMP, and its unpredictable evolution in the first days following diagnosis, require that patients be referred to a tertiary care centre with a high skill in intensive cardiology care. Therapeutic management of PPCMP does not offer any specificity when compared to other causes of acute or chronic heart failure (from diuretics to extracorporeal life support), except for ACE-inhibitors, that are contraindicated before delivery. The high incidence of thrombo-embolic complications observed in the disease requires however rapid and curative anticoagulation, and immuno-suppressive treatment has been proposed in fulminant and highly inflammatory presentation, but its efficacy remains controversial. Very recently, promising results have been reported with bromocriptin-a prolactin secretion inhibitor-for reducing 6-month morbidity and mortality, but these findings have to be confirmed in larger scale randomised trials. As for the long-term evolution, approximately half of the patients will heal, while half of the women will keep some degree of LV dysfunction, 25% of them developing moderate to severe chronic heart

  17. Hypertrophic cardiomyopathy in owl monkeys (Aotus spp.).

    Science.gov (United States)

    Knowlen, Grant G; Weller, Richard E; Perry, Ruby L; Baer, Janet F; Gozalo, Alfonso S

    2013-06-01

    Cardiac hypertrophy is a common postmortem finding in owl monkeys. In most cases the animals do not exhibit clinical signs until the disease is advanced, making antemortem diagnosis of subclinical disease difficult and treatment unrewarding. We obtained echocardiograms, electrocardiograms, and thoracic radiographs from members of a colony of owl monkeys that previously was identified as showing a 40% incidence of gross myocardial hypertrophy at necropsy, to assess the usefulness of these modalities for antemortem diagnosis. No single modality was sufficiently sensitive and specific to detect all monkeys with cardiac hypertrophy. Electrocardiography was the least sensitive method for detecting owl monkeys with hypertrophic cardiomyopathy. Thoracic radiographs were more sensitive than was electrocardiography in this context but cannot detect animals with concentric hypertrophy without an enlarged cardiac silhouette. Echocardiography was the most sensitive method for identifying cardiac hypertrophy in owl monkeys. The most useful parameters suggestive of left ventricular hypertrophy in our owl monkeys were an increased average left ventricular wall thickness to chamber radius ratio and an increased calculated left ventricular myocardial mass. Parameters suggestive of dilative cardiomyopathy were an increased average left ventricular myocardial mass and a decreased average ratio of left ventricular free wall thickness to left ventricular chamber radius. When all 4 noninvasive diagnostic modalities (physical examination, echocardiography, electrocardiography, and thoracic radiography) were used concurrently, the probability of detecting hypertrophic cardiomyopathy in owl monkeys was increased greatly.

  18. Peripartum Cardiomyopathy From a Genetic Perspective.

    Science.gov (United States)

    Kamiya, Chizuko A; Yoshimatsu, Jun; Ikeda, Tomoaki

    2016-07-25

    Peripartum cardiomyopathy (PPCM) is a rare, but life-threatening condition that occurs during the peripartum period in previously healthy women. Although its etiology remains unknown, potential risk factors include hypertensive disorders during pregnancy, such as preeclampsia, advanced maternal age, multiparity, multiple gestation, and African descent. Several cohort studies of PPCM revealed that the prevalence of these risk factors was quite similar. Clinically, approximately 40% of PPCM patients are complicated with hypertensive disorders during pregnancy. Because PPCM is a diagnosis of exclusion, heterogeneity is a common element in its pathogenesis. Recent genetic research has given us new aspects of the disease. PPCM and dilated cardiomyopathy (DCM) share genetic predisposition: 15% of PPCM patients were found to have genetic mutations that have been associated with DCM, and they showed a lower recovery rate. Other basic research using PPCM model mice suggests that predisposition genes related to both hypertensive and cardiac disorders via angiogenic imbalance may explain common elements of hypertensive disorders and PPCM. Furthermore, hypertensive disorders during pregnancy are now found to be a risk factor of not only PPCM, but also cardiomyopathy in the future. Understanding genetic variations allows us to stratify PPCM patients and to guide therapy. (Circ J 2016; 80: 1684-1688).

  19. 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.

  20. Diurnal variation features of PR intervals in patients with dilated cardiomyopathy and heart failure%原发性扩张型心肌病心衰患者心电图PR间期昼夜变化特性的研究

    Institute of Scientific and Technical Information of China (English)

    汪丽; 蔡尚郎

    2012-01-01

    Objective: To study diurnal variation features of PR interval in dilated cardiomyopathy (DCM) patients with sinus rhythm and heart failure to provide evidence for reasonable regulating A - V interval and optimizing cardiac resynchronization therapy (CRT). Methods: A total of 58 DCM patients were enrolled as DCM group, including 20 cases with NYHA cardiac function class II , 19 cases with class IE and 19 cases with class W ; Another 20 normal subjects undergoing physical examination were regard as normal control group. Indexes of 24h dynamic electrocardiogram were measured in two groups. Results: Compared with normal control group, there were significant increase in daytime PR interval [ (142 ± 40. 33) ms vs. (163 ± 48. 9) ms] and nighttime PR interval [ (186 ± 49. 91) ms vs. (195 ± 51. 72) ms], and significant decrease in difference of PR interval between day and night [(44± 15. 37) ms vs. (32 ± 15. 72) ms] in DCM group (NYHA class I group as example), P<0. 05 all; As cardiac function aggravated, there were significant increase in daytime and nighttime PR interval, and significant decrease in difference of PR interval between day and night (P<0. 05 or <0. 01). Conclusion: In DCM patients with sinus rhythm and heart failure, there are significant increase in daytime and nighttime PR interval, significant decrease in difference of PR interval between day and night and they were more significant as cardiac function aggravates, indicating that in this time vagus nerve damage is more severe, optimizing CRT may significantly improve curative effects in these patients with NYHA class Ⅳ.%目的:探讨扩张型心肌病(DCM)窦性心律心衰患者PR间期昼夜变化规律及其意义,为A-V间期合理调整及心脏再同步化治疗(CRT)的优化方案提供依据.方法:入选原发性DCM患者58例(DCM组),其中NY-HAⅡ级20例、Ⅲ级19例、Ⅳ19例;另选体检正常者20例(正常对照组),测量各组24h动态心电图指标.结果:与正常对照

  1. V-plane显像与组织多普勒技术评价扩张型心肌病患者左心室收缩同步性的一致性研究%Evaluation of consistency between V-plane imaging and tissue Doppler imaging for systolic synchronicity in patients with dilated cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    宋宏宁; 周青; 秦真英; 陈金玲; 郭瑞强

    2014-01-01

    目的 应用V-plane技术观察扩张型心肌病(DCM)患者左心室收缩不同步显像,并与临床常用的组织多普勒显像(TDI)技术进行对比,评价其相关性与一致性.方法 对20例DCM患者及20例健康对照者进行超声检查,获取二维图像、V-plane双平面图像和TDI图像,测量左室12个节段TDI脉冲频谱达峰时间,并计算其标准差(TDI_SD),测量V-plane显像中12节段收缩位移达峰时间,并计算其标准差(V-plane_SD).结果 与对照组相比,DCM组的TDI SD和V-plane_SD增大,TDI达峰时间延迟,V-plane达峰时间缩短,差异有统计学意义(P<0.01);DCM组与对照组12节段中各节段V-plane收缩达峰时间均显著高于TDI收缩达峰时间,差异有统计学意义(P<0.01);V-plane_SD与TDI_SD具有显著相关性(r=0.925,P<0.001),Bland-Ahman分析显示TDI SD与V-plane_SD比值的95%数据点在一致性界限内,其比值的一致性界限为(0.50,1.36).结论 在评价DCM患者左室收缩同步性时V-plane技术与TDI技术具有较高的相关性及一致性,V-plane能克服TDI不能在同一心动周期显示12节段的局限性.%Objective To evaluate left ventricular systolic synchronicity in patients with dilated cardiomyopathy by V-plane imaging and compare with clinical commonly used tissue Doppler imaging (TDI),evaluate the relevance and consistency between these two parameters.Methods 20 patients diagnosed with DCM and 20 healthy controls were enrolled,2D images,V-plane imaging and TDI waveform were acquired.Time to peak velocity of left ventricular 12 segments were measured by TDI and the standard deviation (TDI_SD) were calculated.Displacement time to peak were measured by V-plane and the standard deviation (V-plane_SD) were calculated.Results Compared with control group,TDI_SD and V-plane_SD increased significantly (P < 0.01),TDI time to peak increased and V-plane time to peak decreased significantly(P <0.01).In the two group,12 segment time to peak measured by V

  2. Children's Cardiomyopathy Foundation

    Science.gov (United States)

    ... families living with cardiomyopathy through CCF’s new online Coffee & Chat event. On Wednesday, November 16, CCF will be hosting "Navigating Disability Benefits," a webinar to provide an overview of disability ...

  3. Dilations of matricies

    OpenAIRE

    Cohen, David

    2015-01-01

    We explore aspects of dilation theory in the finite dimensional case and show that for a commuting $n$-tuple of operators $T=(T_1,...,T_n) $ acting on some finite dimensional Hilbert space $H$ and a compact set $X\\subset \\mathbb{C}^n$ the following are equivalent: 1. $T$ has a normal $ X$-dilation. 2. For any $m\\in \\mathbb{N}$ there exists some finite dimensional Hilbert space $K$ containing $H$ and a tuple of commuting normal operators $N=(N_1,...,N_n)$ acting on $K$ such that $$ q(T)=P_Hq(N...

  4. Animal Models of Congenital Cardiomyopathies Associated With Mutations in Z-Line Proteins.

    Science.gov (United States)

    Bang, Marie-Louise

    2017-01-01

    The cardiac Z-line at the boundary between sarcomeres is a multiprotein complex connecting the contractile apparatus with the cytoskeleton and the extracellular matrix. The Z-line is important for efficient force generation and transmission as well as the maintenance of structural stability and integrity. Furthermore, it is a nodal point for intracellular signaling, in particular mechanosensing and mechanotransduction. Mutations in various genes encoding Z-line proteins have been associated with different cardiomyopathies, including dilated cardiomyopathy, hypertrophic cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, restrictive cardiomyopathy, and left ventricular noncompaction, and mutations even within the same gene can cause widely different pathologies. Animal models have contributed to a great advancement in the understanding of the physiological function of Z-line proteins and the pathways leading from mutations in Z-line proteins to cardiomyopathy, although genotype-phenotype prediction remains a great challenge. This review presents an overview of the currently available animal models for Z-line and Z-line associated proteins involved in human cardiomyopathies with special emphasis on knock-in and transgenic mouse models recapitulating the clinical phenotypes of human cardiomyopathy patients carrying mutations in Z-line proteins. Pros and cons of mouse models will be discussed and a future outlook will be given. J. Cell. Physiol. 232: 38-52, 2017. © 2016 Wiley Periodicals, Inc.

  5. Biventricular Takotsubo Cardiomyopathy

    Science.gov (United States)

    Daoko, Joseph; Rajachandran, Manu; Savarese, Ronald; Orme, Joseph

    2013-01-01

    Biventricular takotsubo cardiomyopathy is associated with more hemodynamic instability than is isolated left ventricular takotsubo cardiomyopathy; medical management is more invasive and the course of hospitalization is longer. In March 2011, a 62-year-old woman presented at our emergency department with abdominal pain, nausea, and vomiting. On hospital day 2, she experienced chest pain. An electrocardiogram and cardiac enzyme levels suggested an acute myocardial infarction. She underwent cardiac angiography and was found to have severe left ventricular systolic dysfunction involving the mid and apical segments, which resulted in a left ventricular ejection fraction of 0.10 to 0.15 in the absence of obstructive coronary artery disease. Her hospital course was complicated by cardiogenic shock that required hemodynamic support with an intra-aortic balloon pump and dobutamine. A transthoracic echocardiogram revealed akinesis of the mid-to-distal segments of the left ventricle and mid-to-apical dyskinesis of the right ventricular free wall characteristic of biventricular takotsubo cardiomyopathy. After several days of medical management, the patient was discharged from the hospital in stable condition. To the best of our knowledge, this is the first review of the literature on biventricular takotsubo cardiomyopathy that compares its hemodynamic instability and medical management requirements with those of isolated left ventricular takotsubo cardiomyopathy. Herein, we discuss the case of our patient, review the pertinent medical literature, and convey the prevalence and importance of right ventricular involvement in patients with takotsubo cardiomyopathy. PMID:23914028

  6. 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

  7. Cardiac MRI in a Patient with Coincident Left Ventricular Non-Compaction and Hypertrophic Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Zahra Alizadeh-Sani

    2011-12-01

    Full Text Available Left ventricular non-compaction cardiomyopathy is a rare congenital cardiomyopathy that affects both children and adults. Since the clinical manifestations are not sufficient to establish diagnosis, echocardiography is the diagnostic tool that makes it possible to document ventricular non-compaction and establish prognostic factors. We report a 47-year-old woman with a history of dilated cardiomyopathy with unknown etiology. Echocardiography showed mild left ventricular enlargement with severe systolic dysfunction (EF = 20-25%. According to cardiac magnetic resonance imaging findings non-compaction left ventricle with hypertrophic cardiomyopathy was considered, and right ventricular septal biopsy was recommended. Right ventricular endomyocardial biopsy showed moderate hypertrophy of cardiac myocytes with foci of myocytolysis and moderate interstitial fibrosis. No evidence of infiltrative deposition was seen.

  8. Fine mapping of the 1p36 deletion syndrome identifies mutation of PRDM16 as a cause of cardiomyopathy

    DEFF Research Database (Denmark)

    Arndt, Anne-Karin; Schafer, Sebastian; Drenckhahn, Jorg-Detlef

    2013-01-01

    Deletion 1p36 syndrome is recognized as the most common terminal deletion syndrome. Here, we describe the loss of a gene within the deletion that is responsible for the cardiomyopathy associated with monosomy 1p36, and we confirm its role in nonsyndromic left ventricular noncompaction...... cardiomyopathy (LVNC) and dilated cardiomyopathy (DCM). With our own data and publically available data from array comparative genomic hybridization (aCGH), we identified a minimal deletion for the cardiomyopathy associated with 1p36del syndrome that included only the terminal 14 exons of the transcription...... of cardiomyocytes and also revealed evidence of impaired cardiomyocyte proliferative capacity. In conclusion, mutation of PRDM16 causes the cardiomyopathy in 1p36 deletion syndrome as well as a proportion of nonsyndromic LVNC and DCM....

  9. Evaluation of left ventricular function in rat adriamycin-induced dilated cardiomyopathy model by using echocardiography%超声心动图对阿霉素心肌病模型大鼠左心收缩功能的评价

    Institute of Scientific and Technical Information of China (English)

    郭文清; 赵子牛; 袁建军; 刘洪智

    2013-01-01

    Objective To evaluate the left ventricular (LV) function in the rat adriamycin-induced dilated cardiomyopathy model (ADR-DCM).Methods Thirty-five adult male Wistar rats were randomly divided into 2 groups.The ADR-DCM group (n =25) was injected with adriamycin at a dose of 2.5 mg/kg intravenously once a week for 10 weeks,and the control group (n =10) received an equivalent volume of 0.9% saline alone intravenously.Echocardiographic measurements were obtained at 12th week after treatment.The pathological changes of LV were examined by hematoxylin-eosin staining.Results The cumulative mortality in ADR-DCM group was 40%.As compared with control group,LV end-diastolic diameter [LVEDD,(0.67 ±0.07) cm],LV end systolic diameter [LVESD,(0.44 ±0.06) cm],and the hydroxyproline and collagen contents were significantly increased (all P < 0.01),short-axis fractional shortening (FS,33.94 ± 3.56)% and the systolic blood flow velocity of aorta (0.71 ± 0.10) m/s were significantly reduced (all P < 0.01) in ADR-DCM group.The pathological changes in ADR-DCM group were consistent with those of cardiomypathy.Conclusion (1) Chronic administration of adriamycin at a regular dose intravenously to rats could result in left ventricular myocardial fibrosis and heart falure;(2) Echocardiography can sensitively,rapidly and non-invasively evaluate the left ventricular function of the rat ADR-DCM.%目的 探讨超声心动图技术在评估阿霉素心肌病模型大鼠心功能的优势及应用价值.方法 将35只雄性Wistar大鼠按体质量随机分2组:一组制备阿霉素心肌病模型.正常对照组注射等量生理盐水.于实验第12周应用VIVID7彩色超声显像仪行二维、多普勒及M型超声检测评价其左心收缩功能,并作苏木素-伊红(HE)染色观察心肌组织学变化.结果 (1)死亡率:阿霉素心肌病组大鼠12周累计死亡10只,死亡率为40%.死亡原因为充血性心力衰竭;正常对照组无死亡.(2)大鼠心脏超声

  10. Over-expression of D5F173L in heart tissue causes dilated cardiomyopathy in the transgenic mice%多巴胺D5受体突变基因F173L在心脏过表达引起转基因小鼠扩张型心肌病

    Institute of Scientific and Technical Information of China (English)

    胡永艳; 董伟; 姜晓亮; 刘星; 杨志伟

    2011-01-01

    Objective To establish heart-specific D5F173L transgenic mice and to investigate the mechanism of dopamine D5 receptor in cardiac hypertrophy. Methods The transgenic plasmid was constructed by inserting the human D5F173L gene into the down-stream of α-MHC promoter. The transgenic mice were generated by microinjection. The genotype of transgenic lines was identified by PCR, and the expression levels of the D5 receptor were detected by Western Blotting. The functional and pathologic changes of the heart were analyzed by echocardiography and observed by microscopy. Results The heart-specific D5F173L transgenic mice with high expression levels of D5 receptor were established. Compared with the negative controls, the systolic and diastolic volume and inner diameter of left ventricle of the transgenic mice were greater [volume (44.97± 14.54) vs (24.66±5. 34)tL, (83.99±18.42) vs (64.83±9.90)μL; inner diameter: (3.26±0.42) vs (2.58±0.23), (4.28±0.39) vs (3.86±0.25)mm; all P<0.05] and the ejection fraction and the fraction shortening decreased [(48.01 ± 8.73 )% vs (62.18±4.84)%, (24.23±5.15)% vs (33.15±3.52)%, both P<0.05]. Conclusion The heart-specific D5F173L transgenic mice suffer from dilated cardiomyopathy.%目的 建立心脏特异表达人多巴胺D5受体突变基因F173L(D5F173L)的转基因小鼠,利用该转基因动物模型来研究多巴胺D5受体在心脏肥大发生中的作用机制.方法 利用心脏特异启动子α-MHC构建转基因表达载体,显微注射法建立心脏特异表达人多巴胺D5 F173L(α-MHC-D5F173L)的转基因小鼠,PCR鉴定转基因小鼠的基因型,Western blot检测多巴胺D5受体在心脏组织中的表达,心脏超声检测转基因小鼠及野生小鼠的心脏结构和功能.光学显微镜检查α-MHC-D5F173L转基因小鼠心脏的病理改变.结果 建立了α-MHC-D5F173L转基因小鼠.在3月龄,与野生型小鼠比较,α-MHC-D5F173L转基因小鼠心脏收缩期和舒张期左

  11. 实时三维超声心动图容积-时间曲线评价心脏再同步化术后左心室舒张早期同步性%Assessment of the left ventricular early diastolic synchrony of cardiac resynchronization therapy by real time three-dimensional echocardiographic volume-time curves in patients with dilated cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    刘海兰; 叶雪存; 崔亮; 王卫真; 袁高乐

    2014-01-01

    Objective To evaluate the diastolic function and relationship between diastolic function and early diastolic synchrony in patients with dilated cardiomyopathy (DCM) by real-time three-dimensional echocardiography (RT-3DE) volume-time curves (VTC) after cardiac resynchronization therapy (CRT).Methods Thirty-nine patients with DCM were enrolled by RT-3DE VTC before and 1 week,6 months,12 months after CRT,draw the left ventricular (LV) 16,12,6 segments LV diastolic early volume standard deviation of the time (Tedv-SD),and with the R-R interval normalized as early diastolic unsynchronized index (DDI) ;draw end-systolic volume (LVESV),LV end-diastolic volume (LVEDV),LV ejection fraction (LVEF) ;and calculate the diastolic peak filling rate(PFR),the ratio of early diastolic volume and enddiastolic volume(EDVearly/EDV).Results LVEDV,LVESV had no significant improvement.Compared with the before and 1 week after CRT,but the improvement was statistically significant after 6 months and 12 months (P <0.05,P <0.01); LVEF after 1 week,6 months and 12 months were statistically significant (P <0.05) ;Compared with before,PFR after 6 months was significantly increased (P <0.05),EDVearly/EDV at 12 months after CRT was significantly reduced (P < 0.01); There was a significantly shortened in each segment (Tedv-SD)/R-R 1 week after CRT (P <0.01),but the parameters had no obvious improvement later.Correlation analysis:△ DDI and △ EDVearly/EDV reduction was significant positive correlation (r =0.52,P <0.01),△DDI and PFR has negative correlation (r =-0.40,P < 0.05),△ EDVearly/EDV and △PFR also had a good relationship (r =-0.56,P <0.01).Conclusions The LV synchrony and diastolic function were improved after CRT in patients with DCM; PFR,EDVearly/EDV can be used as evaluation of left ventricular diastolic function effectively targets.%目的 应用实时三维超声心动图(RT-3DE)容积-时间曲线评价扩张型心肌病(DCM)患者心脏同步化(CRT)术后

  12. Relationship among heart rate turbulence, QT dispersion and heart function in patients with dilated cardiomyopathy%扩张型心肌病窦性心率震荡及QT离散度与心功能变化的相关性

    Institute of Scientific and Technical Information of China (English)

    郭海鹏; 唐其柱; 邓伟; 周恒; 丘天翼; 严玲; 沈涤非

    2010-01-01

    Objective To explore the relationship among heart rate turbulence (HRT), QT dispersion (QTd) and heart function in patients with dilated cardiomyopathy (DCM) and assess its clinical value. Methods A total of 81 DCM patients with ventricular premature contraction (VPC) were divided into two groups according to heart function: Group A (NYHA class Ⅰ -Ⅱ , n = 34) and Group B (NYHA class Ⅲ-V, n =47). Thirty out-patient control cases were chosen from those undergoing regular physical examination. 24hour holter was performed to monitor turbulence onset ( TO ) and turbulence slope (TS).Electrocardiogram (ECG) was used to assess QTd. Meanwhile left ventricular ejection fraction ( LVEF),left ventricular end-diastolic dimension ( LVEDD), E and A-wave peak velocities, E/A were measured by echocardiogram. After a comparison of all indicators in each group, an investigation was conducted to discern the relationship among HRT, QTd and heart function. Results Compared with normal group, TO significantly increased in DCM A and B group: [0. 38 ( - 0.99 ~ 1.85 ) % vs 1.82 ( 0. 02 ~ 3.92 ) % vs ( -4. 03 ± 3.48 )%, P < 0. 01]. TS significantly decreased while QTd increased. The trend of QTd addition was apparent along with heart failure. TO was negatively correlated with LVEF ( r = -0. 701, P <0. 05 ) but positively correlated with LVEDD ( r =0. 621, P <0. 05 ). There was no correlation with E and A-wave peak velocities. TS and QTd also had an obvious correlation with LVEF and LVEDD ( all P < 0. 05 ) .Conclusions HRT is dramatically blunted in DCM patients and has a certain correlation with cardiac dysfunction. A combined test of HRT and QTd is a sensitive and indirect index in assessing autonomic nerve functions. It has a high clinical value of predicting the prognosis.%目的 探讨扩张型心肌病(DCM)患者窦性心率震荡(HRT)及QT离散度(QTa)与心功能变化的相关性及临床价值.方法 81例临床诊断DCM伴室性早搏患者,按心功能分级

  13. Dilation and Curettage (D&C)

    Science.gov (United States)

    ... For Patients About ACOG Dilation and Curettage (D&C) Home For Patients Search FAQs Dilation and Curettage ( ... February 2016 PDF Format Dilation and Curettage (D&C) Special Procedures What is dilation and curettage (D& ...

  14. Treatment of Chagas Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Fernando A. Botoni

    2013-01-01

    Full Text Available 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.

  15. Tako-tsubo cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    Yan Zhuang; Di Xu

    2009-01-01

    Tako-tsubo cardiomyopathy(TC) is a recently described acute cardiac syndrome, which the latest cardiomyopathy classification of the European Society of Cardiology describes as an unclassified cardiomyopathy. TC mimics acute myocardial infarction(AMI) and is characterised by ischaemic chest symptoms, an elevated electrocardiogram ST-segment, and moderately increased levels of cardiac disease markers. However, patients with TC have no coronary angiogram-detectable or non-obstructive coronary arterial disease(CAD), and left ventriculography documents transient left apical and middle ventricular wall dysfunction. In this review, we describe TC and evaluate epidemiological, clinical and instrumental features, pathophysiological mechanisms, therapy and prognosis of this syndrome, with a view to raising awareness of the disease.

  16. Childhood acquired heart diseases in Jos, north central Nigeria

    Directory of Open Access Journals (Sweden)

    Fidelia Bode-Thomas

    2013-01-01

    Full Text Available Background: The patterns of childhood acquired heart diseases (AHD vary in different parts of the world and may evolve over time. We aimed to compare the pattern of childhood AHD in our institution to the historical and contemporary patterns in other parts of the country, and to highlight possible regional differences and changes in trend. Materials and Methods: Pediatric echocardiography records spanning a period of 10 years were reviewed. Echocardiography records of children with echocardiographic or irrefutable clinical diagnoses of AHD were identified and relevant data extracted from their records. Results: One hundred and seventy five children were diagnosed with AHD during the period, including seven that had coexisting congenital heart disease (CHD. They were aged 4 weeks to 18 years (mean 9.84΁4.5 years and comprised 80 (45.7% males and 95 (54.3% females. Rheumatic heart disease (RHD was the cause of the AHD in 101 (58.0% children, followed by dilated cardiomyopathy (33 cases, 18.9% which was the most frequent AHD in younger (under 5 years children. Other AHD encountered were cor pulmonale in 16 (9.1%, pericardial disease in 15 (8.6%, infective endocarditis in 8 (4.6% and aortic aneurysms in 2 (1.1% children. Only one case each of endomyocardial fibrosis (EMF and Kawasaki Disease were seen during the period. Conclusions: The majority of childhood acquired heart diseases in our environment are still of infectious aeitology, with RHD remaining the most frequent, particularly in older children. Community-based screening and multicenter collaborative studies will help to better describe the pattern of AHD in our country. More vigorous pursuit of the Millennium development goals will contribute to reducing the burden of childhood acquired heart diseases in the country.

  17. Pathophysiology and epidemiology of peripartum cardiomyopathy.

    Science.gov (United States)

    Hilfiker-Kleiner, Denise; Sliwa, Karen

    2014-06-01

    Cardiovascular diseases are a major cause of complications in pregnancy worldwide, and the number of patients who develop cardiac problems during pregnancy is increasing. Peripartum cardiomyopathy (PPCM) is a potentially life-threatening heart disease that emerges towards the end of pregnancy or in the first months postpartum, in previously healthy women. Symptoms and signs of PPCM are similar to those in patients with idiopathic dilated cardiomyopathy. The incidence varies geographically, most likely because of socioeconomic and genetic factors. The syndrome is associated with a high morbidity and mortality, and diagnosis is often delayed. Various mechanisms have been investigated, including the hypothesis that unbalanced peripartum or postpartum oxidative stress triggers the proteolytic cleavage of the nursing hormone prolactin into a potent antiangiogenic, proapoptotic, and proinflammatory 16 kDa fragment. This theory provides the basis for the discovery of disease-specific biomarkers and promising novel therapeutic targets. In this Review, we describe the latest understanding of the epidemiology, pathophysiology, and novel treatment strategies for patients with PPCM.

  18. Phidippides cardiomyopathy: a review and case illustration.

    Science.gov (United States)

    Trivax, Justin E; McCullough, Peter A

    2012-02-01

    Phidippides was a Greek messenger who experienced sudden death after running more than 175 miles in two days. In today's world, marathon running and other endurance sports are becoming more popular and raising concern about sudden deaths at these events. Once etiologies such has hypertrophic cardiomyopathy, anomalous coronary arteries, and coronary atherosclerosis have been excluded, there is now an additional consideration termed Phidippides cardiomyopathy. Because endurance sports call for a sustained increase in cardiac output for several hours, the heart is put into a state of volume overload. It has been shown that approximately one-third of marathon runners experience dilation of the right atrium and ventricle, have elevations of cardiac troponin and natriuretic peptides, and in a smaller fraction later develop small patches of cardiac fibrosis that are the likely substrate for ventricular tachyarrhythmias and sudden death. Cardiac magnetic resonance imaging is emerging as the diagnostic test of choice for this condition. This review and case report summarizes the key features of this newly appreciated disorder.

  19. Barth Syndrome: Connecting Cardiolipin to Cardiomyopathy.

    Science.gov (United States)

    Ikon, Nikita; Ryan, Robert O

    2017-02-01

    The Barth syndrome (BTHS) is caused by an inborn error of metabolism that manifests characteristic phenotypic features including altered mitochondrial membrane phospholipids, lactic acidosis, organic acid-uria, skeletal muscle weakness and cardiomyopathy. The underlying cause of BTHS has been definitively traced to mutations in the tafazzin (TAZ) gene locus on chromosome X. TAZ encodes a phospholipid transacylase that promotes cardiolipin acyl chain remodeling. Absence of tafazzin activity results in cardiolipin molecular species heterogeneity, increased levels of monolysocardiolipin and lower cardiolipin abundance. In skeletal muscle and cardiac tissue mitochondria these alterations in cardiolipin perturb the inner membrane, compromising electron transport chain function and aerobic respiration. Decreased electron flow from fuel metabolism via NADH ubiquinone oxidoreductase activity leads to a buildup of NADH in the matrix space and product inhibition of key TCA cycle enzymes. As TCA cycle activity slows pyruvate generated by glycolysis is diverted to lactic acid. In turn, Cori cycle activity increases to supply muscle with glucose for continued ATP production. Acetyl CoA that is unable to enter the TCA cycle is diverted to organic acid waste products that are excreted in urine. Overall, reduced ATP production efficiency in BTHS is exacerbated under conditions of increased energy demand. Prolonged deficiency in ATP production capacity underlies cell and tissue pathology that ultimately is manifest as dilated cardiomyopathy.

  20. 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.

  1. Myocarditis and inflammatory cardiomyopathy: from diagnosis to treatment.

    Science.gov (United States)

    Escher, Felicitas; Tschöepe, Carsten; Lassner, Dirk; Schultheiss, Heinz-Peter

    2015-12-01

    Based on the definition in the European Society of Cardiology statement, myocarditis is an inflammatory disease of the myocardium diagnosed by established histological, immunological, and immunohistochemical criteria, whereas inflammatory cardiomyopathy is myocarditis in association with cardiac dysfunction. Actual incidences of myocarditis and CMi are difficult to determine. Studies addressing the issue of sudden cardiac death in young people report a highly variable autopsy prevalence of myocarditis, ranging from 2-42% of cases. Similarly, biopsy-proven myocarditis has been reported in 9-16% of adult patients with unexplained nonischemic dilated cardiomyopathy (DCM). In up to 30% of cases, biopsy-proven myocarditis can progress to DCM and is associated with a poor prognosis. Prognosis in myocarditis patients also varies according to underlying etiology.

  2. Anesthetic management of peripartum cardiomyopathy using "epidural volume extension" technique: A case series

    Directory of Open Access Journals (Sweden)

    Akhilesh Kumar Tiwari

    2012-01-01

    Full Text Available Peripartum cardiomyopathy is a rare cause of dilated cardiomyopathy in parturients, occurring in approximately one in 1000 deliveries, manifesting during the last few months or the first 5 months of the postpartum period. It can result in severe ventricular dysfunction during late puerperium. The major concern while managing these patients is to optimize fluid administration and avoid myocardial depression, while maintaining stable intraoperative hemodynamics. We present a case series of five parturients that were posted for elective cesarean section and managed successfully by the epidural volume extension technique.

  3. Correction of human phospholamban R14del mutation associated with cardiomyopathy using targeted nucleases and combination therapy

    NARCIS (Netherlands)

    Karakikes, Ioannis; Stillitano, Francesca; Nonnenmacher, Mathieu; Tzimas, Christos; Sanoudou, Despina; Termglinchan, Vittavat; Kong, Chi-Wing; Rushing, Stephanie; Hansen, Jens; Ceholski, Delaine; Kolokathis, Fotis; Kremastinos, Dimitrios; Katoulis, Alexandros; Ren, Lihuan; Cohen, Ninette; Gho, Johannes M. I. H.; Tsiapras, Dimitrios; Vink, Aryan; Wu, Joseph C.; Asselbergs, Folkert W.; Li, Ronald A.; Hulot, Jean-Sebastien; Kranias, Evangelia G.; Hajjar, Roger J.

    2015-01-01

    A number of genetic mutations is associated with cardiomyopathies. A mutation in the coding region of the phospholamban (PLN) gene (R14del) is identified in families with hereditary heart failure. Heterozygous patients exhibit left ventricular dilation and ventricular arrhythmias. Here we generate i

  4. The mitochondrial DNA T16189C polymorphism and HIV-associated cardiomyopathy: a genotype-phenotype association study

    Directory of Open Access Journals (Sweden)

    Poulton Joanna

    2009-04-01

    Full Text Available Abstract Background The mitochondrial DNA (mtDNA T16189C polymorphism, with a homopolymeric C-tract of 10–12 cytosines, is a putative genetic risk factor for idiopathic dilated cardiomyopathy in the African and British populations. We hypothesized that this variant may predispose to dilated cardiomyopathy in people who are infected with the human immunodeficiency virus (HIV. Methods A case-control study of 30 HIV-positive cases with dilated cardiomyopathy and 37 HIV-positive controls without dilated cardiomyopathy was conducted. The study was confined to persons of black African ancestry to minimize confounding of results by population admixture. HIV-positive patients with an echocardiographically confirmed diagnosis of dilated cardiomyopathy and HIV-positive controls with echocardiographically normal hearts were studied. Patients with secondary causes of cardiomyopathy (such as hypertension, diabetes, pregnancy, alcoholism, valvular heart disease, and opportunistic infection were excluded from the study. DNA samples were sequenced for the mtDNA T16189C polymorphism with a homopolymeric C-tract in the forward and reverse directions on an ABI3100 sequencer. Results The cases and controls were well matched for age (median 35 years versus 34 years, P = 0.93, gender (males 60% vs 53%, P = 0.54, and stage of HIV disease (mean CD4 T cell count 260.7/μL vs. 176/μL, P = 0.21. The mtDNA T16189C variant with a homopolymeric C-tract was detected at a frequency of 26.7% (8/30 in the HIV-associated cardiomyopathy cases and 13.5% (5/37 in the HIV-positive controls. There was no significant difference between cases and controls (Odds Ratio 2.33, 95% Confidence Interval 0.67–8.06, p = 0.11. Conclusion The mtDNA T16189C variant with a homopolymeric C-tract is not associated with dilated cardiomyopathy in black African people infected with HIV.

  5. [The clinical practice guidelines of the Sociedad Española de Cardiología on cardiomyopathies and myocarditis].

    Science.gov (United States)

    Galve Basilio, E; Alfonso Manterola, F; Ballester Rodés, M; Castro Beiras, A; Fernández de Soria Pantoja, R; Penas Lado, M; Sánchez Domínguez, J

    2000-03-01

    Myocardial diseases are a extraordinarily heterogeneous group of processes that only have in common the fact that they involve heart muscle and that they cause a wide spectrum of myocardial dysfunction. The approach of the management and treatment of the cardiomyopathies is a continuous matter of discussion because the vast majority of alternatives in this field have not been based on the best scientific possible evidence and, since except for the case of heart failure associated with dilated cardiomyopathy. The majority of different options have not been studied by means of large (or even small) randomized trials. Nevertheless, this chapter has tried to provide the reader with different approaches on how to deal with important clinical problems in dilated, hypertrophic and restrictive cardiomyopathies, and in myocarditis as well. For this, we have utilized the most relevant information found coupled with our best clinical judgment, although we admit that many of the clinical recommendations can be controversial.

  6. Saw-tooth cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Karatza Ageliki A

    2009-12-01

    Full Text Available Abstract We present an unusual case of cardiomyopathy in a two month old male infant with a grade-I systolic murmur. Echocardiographic examination disclosed left ventricular (LV, dysplasia with saw-tooth like inwards myocardial projections extending from the lateral walls towards the LV cavity. There was mild LV systolic dysfunction with apical hypokinesia. Cardiovascular magnetic resonance demonstrated in detail these cross bridging muscular projections originating from the inferior interventricular septum and lateral LV wall, along with areas of hypokinesis at the LV septum and apex in a noncoronary distribution, without any late gadolinium enhancement. We have termed this condition saw-tooth cardiomyopathy because of the very characteristic appearance.

  7. Effects of mesenchymal stem cell transplantatiOn on cardiac function and structure and eIectrophysiology in rabbits with dilated cardiomyopathy%同种异体骨髓间充质干细胞移植对扩张型心肌病模型兔心功能与结构及电生理的影响

    Institute of Scientific and Technical Information of China (English)

    余更生; 沈兴; 田杰; 白永虹; 朱静; 刘官信; 陈沅

    2008-01-01

    :细胞移植组实验兔左室收缩末期内径较模型组减小,左室射血分数,左室短轴缩短率均高于模型组,差异有统计学意义(P<0.05).②心电生理检测结果:细胞移植组实验兔MAPDS0、MAPD90短于模型组,差异有统计学意义(P<0.05).③心肌组织学观察:模型组可见心肌细胞旱广泛的水肿和显著的空泡变性改变,电镜示心肌细胞线粒体肿胀,增生,肌节长短不一,出现异常收缩带,可见局部心肌溶解,细胞移植组移植区组织与之相比,各种受损表现较轻,且移植细胞有肌钙蛋白T和缝隙连接蛋白43的表达.结论:同种异体体内移植骨髓间充质干细胞后在一定程度上可改善扩张型心肌病模型兔心功能,减轻病理损害,并可能抑制心电紊乱的进一步发展.%BACKGROUND: The study of cell transplantation to repair injured cardiac muscle and improve cardiac function of dilated cardiomyopathy (DCM) has become a hotspot in recent years. However, the effect of cardiac electrophysiology following transplantation is still unknown.OBJECTIVE: To explore the influence of ailogenic implanted mesenchymal stem cells (MSCs) on cardiac function, structure and electrophysiology of rabbits with DCM.DESIGN, TIME AND SETTING: Randomized controlled animal trial was performed at Electrophysiology Laboratory of Institute of Pediatrics, Chongqing Medical University between January 2004 and May 2006.MATERIALS: Forty-three New Zealand whiterabbits, weighing 3.0-3.5 kg, irrespective of gender, were selected. Adriamycin was produced by Haizheng Medical Products Company of Zhejiang Province, China, No. 050307. The Ultrasonograph SSD-5000 came from Aloka, Japan, and RM6240 multiplying channel electrophysiolograph of Chengdu Instrument Company was applied.METHODS: All animals were randomized into normal group (n=12), cell transplantation group (n=13), and DCM model group (n=13). Except the normal group, adriamycin was applied to create rabbit DCM model, I mg/kg, twice a week for

  8. Role of left ventricular twist mechanics in cardiomyopathies, dance of the helices

    Institute of Scientific and Technical Information of China (English)

    Floris; Kauer; Marcel; Leonard; Geleijnse; Bastiaan; Martijn; van; Dalen

    2015-01-01

    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. 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.)

  10. VIP Gene Deletion in Mice Causes Cardiomyopathy Associated with Upregulation of Heart Failure Genes

    Energy Technology Data Exchange (ETDEWEB)

    Szema, Anthony M.; Hamidi, Sayyed A.; Smith, S. David; Benveniste, Helene; Katare, Rajesh Gopalrao

    2013-05-20

    Vasoactive Intestinal Peptide (VIP), a pulmonary vasodilator and inhibitor of vascular smooth muscle proliferation, is absent in pulmonary arteries of patients with idiopathic pulmonary arterial hypertension (PAH). We previously determined that targeted deletion of the VIP gene in mice leads to PAH with pulmonary vascular remodeling and right ventricular (RV) dilatation. Whether the left ventricle is also affected by VIP gene deletion is unknown. In the current study, we examined if VIP knockout mice (VIP-/-) develop both right (RV) and left ventricular (LV) cardiomyopathy, manifested by LV dilatation and systolic dysfunction, as well as overexpression of genes conducive to heart failure.

  11. Role of neuropeptides in cardiomyopathies.

    Science.gov (United States)

    Dvorakova, Magdalena Chottova; Kruzliak, Peter; Rabkin, Simon W

    2014-11-01

    The role of neuropeptides in cardiomyopathy-associated heart failure has been garnering more attention. Several neuropeptides--Neuropeptide Y (NPY), vasoactive intestinal peptide (VIP), calcitonin gene related peptide (CGRP), substance P (SP) and their receptors have been studied in the various types of cardiomyopathies. The data indicate associations with the strength of the association varying depending on the kind of neuropeptide and the nature of the cardiomyopathy--diabetic, ischemic, inflammatory, stress-induced or restrictive cardiomyopathy. Several neuropeptides appear to alter regulation of genes involved in heart failure. Demonstration of an association is an essential first step in proving causality or establishing a role for a factor in a disease. Understanding the complexity of neuropeptide function should be helpful in establishing new or optimal therapeutic strategies for the treatment of heart failure in cardiomyopathies.

  12. Bootstrapping Time Dilation Decoherence

    CERN Document Server

    Gooding, Cisco

    2015-01-01

    We present a general relativistic model of a spherical shell of matter with a perfect fluid on its surface coupled to an internal oscillator, which generalizes a model recently introduced by the authors to construct a self-gravitating interferometer [1]. The internal oscillator evolution is defined with respect to the local proper time of the shell, allowing the oscillator to serve as a local clock that ticks differently depending on the shell's position and momentum. A Hamiltonian reduction is performed on the system, and an approximate quantum description is given to the reduced phase space. If we focus only on the external dynamics, we must trace out the clock degree of freedom, and this results in a form of intrinsic decoherence that shares some features with a proposed "universal" decoherence mechanism attributed to gravitational time dilation [2]. We show that the proposed decoherence remains present in the (gravity-free) limit of flat spacetime, indicating that the effect can be attributed entirely to ...

  13. Indium-111 antimyosin monoclonal antibody imaging; Imaging of myocardial infarction, myocarditis and cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Matsumori, Akira; Yamada, Takehiko; Morishima, Shigeru (Kyoto Univ. (Japan). Faculty of Medicine) (and others)

    1991-02-01

    A retrospective review was made on In-111 antimyosin (AM) monoclonal antibody scintigrams of a total of 75 patients, consisting of 49 with myocardial infarction, 9 with myocarditis, and 17 with cardiomyopathy. Planar and SPECT images were obtained 48 hours after iv injection of 74 MBq (2 mCi) of In-labeled AM monoclonal antibody. In 35 myocardial infarction patients within 16 days after the onset, 33 (94%) had positive In-111 AM scintigraphic findings, although CPK and ECG findings returned to normal and Tc-99m pyrophosphate scintigraphic findings were negative. In-111 AM scintigraphy was negative when coronary reperfusion was successful early after the onset of myocardial infarction and CPK was not increased. It was still positive in 80% one to 2 months after the onset, and in 58% within one year. For myocarditis, In-111 AM scintigraphy was positive in 100% within one month after the onset, and in 14% at one year or later. A positive rate of In-111 AM scintigraphy was 70% for dilated cardiomyopathy and 86% for hypertrophic cardiomyopathy. Three patients with dilated cardiomyopathy who showed marked accumulation of In-111 AM died. Strong accumulation of AM was associated with both dilation of the left ventricular lumen and decreased cardiac function. The heart/lung ratio was positively correlated with left ventricular enddiastolic dimension and negatively correlated with left ventricular ejection fraction. In-111 AM monoclonal antibody scintigraphy may be promising in the evaluation of the presence of myocardial cell damage, clinical process, and prognosis in myocardial infarction, myocarditis, and cardiomyopathy. (N.K.).

  14. 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)

  15. Coronary artery ectasia and systolic flow cessation in a patient with hypertrophic cardiomyopathy: a case report.

    Science.gov (United States)

    Zografos, Theodoros; Kokladi, Maria; Katritsis, Demosthenes

    2010-12-01

    Coronary artery ectasia (CAE) is characterized by diffuse or localized inappropriate dilation of coronary arteries and is often associated with slow coronary blood flow. Although CAE has been described to coexist with several clinical entities there are only three reports of CAE in the presence of hypertrophic cardiomyopathy (HCM). We report a case of CAE and slow coronary flow with systolic flow cessation in a 61-year old male with coronary artery disease and HCM.

  16. Nesprin-1 mutations in human and murine cardiomyopathy

    Science.gov (United States)

    Puckelwartz, Megan J.; Kessler, Eric J.; Kim, Gene; DeWitt, Megan M.; Zhang, Yuan; Earley, Judy U.; Depreux, Frederic F.S.; Holaska, James; Mewborn, Stephanie K.; Pytel, Peter; McNally, Elizabeth M.

    2009-01-01

    Mutations in LMNA, the gene encoding the nuclear membrane proteins, lamins A and C, produce cardiac and muscle disease. In the heart, these autosomal dominant LMNA mutations lead to cardiomyopathy frequently associated with cardiac conduction system disease. Herein, we describe a patient with the R374H missense variant in nesprin-1α, a protein that binds lamin A/C. This individual developed dilated cardiomyopathy requiring cardiac transplantation. Fibroblasts from this individual had increased expression of nesprin-1α and lamins A and C, indicating changes in the nuclear membrane complex. We characterized mice lacking the carboxy-terminus of nesprin-1 since this model expresses nesprin-1 without its carboxy-terminal KASH domain. These Δ/Δ KASH mice have a normally assembled but dysfunctional nuclear membrane complex and provide a model for nesprin-1 mutations. We found that Δ/Δ KASH mice develop cardiomyopathy with associated cardiac conduction system disease. Older mutant animals were found to have elongated P wave duration, elevated atrial and ventricular effective refractory periods indicating conduction defects in the myocardium, and reduced fractional shortening. Cardiomyocyte nuclei were found to be elongated with reduced heterochromatin in the Δ/Δ KASH hearts. These findings mirror what has been described from lamin A/C gene mutations and reinforce the importance of an intact nuclear membrane complex for a normally functioning heart. PMID:19944109

  17. 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.

  18. Neonatal cardiomyopathies and metabolic crises due to oxidative phosphorylation defects.

    Science.gov (United States)

    Schiff, Manuel; Ogier de Baulny, Hélène; Lombès, Anne

    2011-08-01

    Neonatal cardiomyopathies due to mitochondrial oxidative phosphorylation (OXPHOS) defects are extremely severe conditions which can be either isolated or included in a multi-organ disease, with or without metabolic crises, of which profound lactic acidosis is the prominent feature. Cardiomyopathy is more often hypertrophic than dilated. Antenatal manifestations such as fetal cardiomyopathy, arrhythmia and/or hydrops have been reported. Pathophysiological mechanisms are complex, going beyond ATP deficiency of the high-energy-consuming neonatal myocardium. Birth is a key metabolic period when the myocardium switches ATP production from anaerobic glycolysis to mitochondrial fatty acid oxidation and OXPHOS. Heart-specificity of the defect may be related to the specific localization of the defect, to the high myocardium dependency on OXPHOS, and/or to interaction between the primary genetic alteration and other factors such as modifier genes. Therapeutic options are limited but standardized diagnostic procedures are mandatory to confirm the OXPHOS defect and to identify its causal mutation, allowing genetic counseling and potential prenatal diagnosis.

  19. Takotsubo cardiomyopathy (Broken heart syndrome).

    Science.gov (United States)

    Javed, Aqib; Chitkara, Kamal; Mahmood, Arslan; Kainat, Aleesha

    2015-11-01

    Takotsubo cardiomyopathy is an acute reversible cardiomyopathy characterised by transient regional left ventricular (LV) motion abnormalities. It is diagnosed on a coronary angiography and left ventriculography. We report the case of a 50-year-old lady who presented with sudden onset of chest pain, with no history of cardiac disease and no risk factors. Remarkably though, she had lost her husband the previous night. Coronary and LV angiography was done which revealed findings typical of takotsubo cardiomyopathy. We report this case for its rarity. Informed consent was taken from the patient before undertaking and reporting this study.

  20. Heart Failure with Multi-organ Thrombosis: A Case of Antiphospholipid Syndrome Co-existing with Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Xueqi Li and Shipeng Wei

    2012-09-01

    Full Text Available Antiphospholipid Syndrome (APS is an autoimmune disease featured by venous or arterial thrombosis, fetal losses and thrombocytopenia in the presence of antiphospholipid antibodies. Here we reported one case of antiphospholipid syndrome together with dilated cardiomyopathy. A 46-year-old female patient complaining short of breath was found enlargement of atrial and ventricular compartments. The ecletrocardiogram and blood test revealed anteroseptal myocardial infarction, while no pulmonary thrombosis was present and therefore diagnosis of dilated cardiomyopathy was made. There were also thrombi formed in the cardial chambers and deep venous. During hospitalization, there was an onset of ischemic brain stroke and head MRI showed newly developed small infarctions. An elevation of anticardiolipin immunoglobulin A (ACAIGA was detected from the blood sample. The patient was discharged after being treated with anticoagulant, corticosteroid and other medicines for improving heart function. In our case, APS is the basic cause leading to multi-organ thrombosis and heart failure is mainly due to dilated cardiomyopathy, thus independent of APS. So this is the first time that cardiomyopathy co-existing with APS was reported.

  1. 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.)

  2. 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 reduce...

  3. Takotsubo cardiomyopathy following subarachnoid haemorrhage.

    Science.gov (United States)

    Maekawa, Hidetsugu; Hadeishi, Hiromu

    2014-08-01

    A 67-year-old woman was admitted with aneurysmal subarachnoid haemorrhage and a 12-lead ECG showed ST segment elevation. Transthoracic echocardiography confirmed akinesis of the left ventricular mid-apical segment, with an ejection fraction of 26%, features characteristic of takotsubo cardiomyopathy. Five days later, we identified thrombus in the apex of the left ventricle. Sixteen days after onset, the thrombus had disappeared and wall motion improved (ejection fraction 58%) without evidence of cardioembolism. Takotsubo cardiomyopathy is a cause of cardiac dysfunction after stroke, including SAH. It is characterised by transiently depressed contractile function of the left mid and apical ventricle, without obstructive coronary artery disease. Clinicians should suspect takotsubo cardiomyopathy in patients with subarachnoid haemorrhage who have an ECG abnormality. Echocardiography is needed to detect the distinctive regional wall motion abnormality. Despite its severity in the acute phase, takotsubo cardiomyopathy is self-limiting and its management is conservative.

  4. Electrocardiographic abnormalities and uremic cardiomyopathy

    NARCIS (Netherlands)

    Stewart, GA; Gansevoort, RT; Mark, PB; Rooney, E; McDonagh, TA; Dargie, HJ; Stuart, R; Rodger, C; Jardine, AG

    2005-01-01

    Background. Progressive renal disease is associated with an increased risk of cardiovascular death, specifically sudden death. We investigated the link between uremic cardiomyopathy, QT interval and dispersal, and arrhythmias (by ambulatory ECG monitoring) in patients at different stages of progress

  5. MELAS syndrome and cardiomyopathy: linking mitochondrial function to heart failure pathogenesis.

    Science.gov (United States)

    Hsu, Ying-Han R; Yogasundaram, Haran; Parajuli, Nirmal; Valtuille, Lucas; Sergi, Consolato; Oudit, Gavin Y

    2016-01-01

    Heart failure remains an important clinical burden, and mitochondrial dysfunction plays a key role in its pathogenesis. The heart has a high metabolic demand, and mitochondrial function is a key determinant of myocardial performance. In mitochondrial disorders, hypertrophic remodeling is the early pattern of cardiomyopathy with progression to dilated cardiomyopathy, conduction defects and ventricular pre-excitation occurring in a significant proportion of patients. Cardiac dysfunction occurs in approximately a third of patients with mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) syndrome, a stereotypical example of a mitochondrial disorder leading to a cardiomyopathy. We performed unique comparative ultrastructural and gene expression in a MELAS heart compared with non-failing controls. Our results showed a remarkable increase in mitochondrial inclusions and increased abnormal mitochondria in MELAS cardiomyopathy coupled with variable sarcomere thickening, heterogeneous distribution of affected cardiomyocytes and a greater elevation in the expression of disease markers. Investigation and management of patients with mitochondrial cardiomyopathy should follow the well-described contemporary heart failure clinical practice guidelines and include an important role of medical and device therapies. Directed metabolic therapy is lacking, but current research strategies are dedicated toward improving mitochondrial function in patients with mitochondrial disorders.

  6. 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.

  7. Takotsubo or Stress Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    J. P. Bounhoure

    2012-01-01

    Full Text Available Many case reports have been published of reversible left ventricular dysfunction precipitated by sudden emotional stress. We have evaluated 10 women hospitalized for acute chest pain and dyspnea, mimicking an acute coronary syndrome, after a severe emotional trigger. Those patients, postmenopausal women, presented ST segment alterations on the EKG, minor elevations of cardiac enzymes, and biomarkers levels. At the coronarography there was not coronary thrombosis or severe stenosis, but the ventriculography showed wall motion abnormalities involving the left ventricular apex and midventricle, in the absence of significant obstructive coronary disease. The course was benign without complication, with a full recovery of left ventricular function in some weeks. These observations, like other reports, demonstrate the impact of emotional stress on left ventricular function and the risk of cardiovascular disease. The cause of this cardiomyopathy is still unknown, and several mechanisms have been proposed: catecholamine myocardial damage, microvascular spasm, or neural mediated myocardial stunning.

  8. PERIPARTUM CARDIOMYOPATHY: A REVIEW

    Directory of Open Access Journals (Sweden)

    Rajat

    2016-05-01

    Full Text Available Peripartum Cardiomyopathy is a pregnancy associated rare but severe myocardial disease. The incidence of PPCM is about 1 in 3186 live births in United States. Causes include viral infections, toxins, environmental and geographic factors, familial predisposition, Hormonal abnormalities, haemodynamic burden of pregnancy, malnutrition, inflammation, etc. NT-proBNP, Cathepsin D, tyrosine kinase SFlt1 may be elevated and can be used as biomarkers to diagnose PPCM. Restriction of dietary sodium, beta-blockers, diuretics-thiazide and furosemide, in lowest possible doses can be given for symptomatic management. Prognosis of PPCM is positively related to the recovery of ventricular failure. Only about 50% women with PPCM recover baseline ventricular function within 6 months of delivery. Failure of heart size to return to normal is associated with increased mortality and morbidity. Future pregnancies are not recommended in patient with PPCM, as there is an increased risk for recurrence of PPCM in the subsequent pregnancy

  9. Molecular pathogenetic mechanisms and new therapeutic perspectives in anthracycline-induced cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Distefano Giuseppe

    2009-11-01

    Full Text Available Abstract Anthracyclines are among the most powerful drugs for the treatment of oncologic diseases both in childhood and in adulthood. Nevertheless, their major antineoplastic efficacy can be seriously impaired by collateral toxic cardiac effects causing cardiomyopathy with chronic heart failure that is refractory to conventional medical therapy. This article reports possible subcellular molecular alterations of anthracycline-induced cardiomyopathy (reactive oxygen species formation, apoptosis, inflammatory signalling, altered expression of cardiomyocytes specific genes, etc and indicates some new therapeutic perspectives resulting from a better understanding of the molecular pathogenetic mechanisms.

  10. 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...... of the state variable, we demonstrate that the model can describe basic features of the dilatant fluid such as the stress-shear rate curve that represents discontinuous severe shear thickening, hysteresis upon changing shear rate, and instantaneous hardening upon external impact. An analysis of the model...... reveals that the shear thickening fluid shows an instability in a shear flow for some regime and exhibits the shear thickening oscillation (i.e., the oscillatory shear flow alternating between the thickened and the relaxed states). The results of numerical simulations are presented for one- and two...

  11. 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 of ...

  12. [Specific dilated myocardiopathy. Chronic chagasic cardiopathy at the National Institute of Cardiology Ignacio Chávez].

    Science.gov (United States)

    Monteón-Padilla, Víctor Manuel; Vargas-Alarcón, Gilberto; Vallejo-Allende, Maité; Reyes, Pedro A

    2002-01-01

    Cardiomyopathies are a heterogenous group of heart ailments. Some of them are primary myocardial diseases and are classified as dilated, hypertrophic, restrictive and arryhithmogenic. Dilated cardiomyopathies (DCs) are the most common. Sometimes it is possible to identify an etiologic agent, in that case we talk about a specific dilated cardiomyopathy. Here in, we review one of these specific DCs, the so called Chronic Chagasic Cardiopathy (CCC) from the point of view of our personal experience at the Instituto Nacional de Cardiología "Ignacio Chávez". Chagas' disease is present in Mexico, therefore CCC is also present. We estimate that 5,000 people, suffer CCC with severe symptoms. In Mexico, Chagas' disease occurs below the Tropic of Cancer and between 2,000-2,500 m above sea level, in this area there is a real risk for vectorial infection, mainly in rural villages. Clinical diagnosis should be supported by epidemiological and seroepidemiological confirmatory data. There is not appropriate therapy yet for this condition.

  13. Hepatitis C virus from the hearts of patients with myocarditis and cardiomyopathy.

    Science.gov (United States)

    Matsumori, A; Yutani, C; Ikeda, Y; Kawai, S; Sasayama, S

    2000-07-01

    The myocardium may be the target of several types of viral infections. The importance of hepatitis C virus (HCV) infection has been recently noted in patients with myocarditis and in patients with dilated or hypertrophic cardiomyopathy. The present study sought to detect HCV genomes in formalin-fixed paraffin sections of autopsied hearts from patients with myocarditis and patients with dilated or hypertrophic cardiomyopathy. Paraffin sections were deparaffinized, RNA was extracted, and the positive and negative strands of HCV RNA were detected by performing reverse transcription and nested polymerase chain reaction. The polymerase chain reaction products were cloned and sequenced. beta-actin gene was used as a control for the successful amplification of a housekeeping gene. Among 106 hearts examined, beta-actin gene was amplified in 61 hearts (57.5%). Among the latter, HCV RNA was detected in 13 hearts (21.3%), and negative strands in 4 hearts (6.6%). HCV RNA was found in 4 hearts (33.3%) with myocarditis, in 3 hearts (11.5%) with dilated cardiomyopathy, and in 6 hearts (26.0%) with hypertrophic cardiomyopathy. The sequences recovered from nine patients were highly homologous to the standard strain of HCV. HCV genomes were not found in either 35 hearts from patients with myocardial infarction or 20 hearts from patients with noncardiac diseases. These HCV RNA positive samples were obtained from 1 heart in 1979, 7 hearts between 1980 and 1989, and 5 hearts since 1990, indicating that HCV RNA can be amplified from paraffin-embedded hearts preserved for many years. This method of detecting HCV genomes in formalin-fixed paraffin cardiac specimens has enabled us to widen our research into HCV infection and has been helpful in identifying the presence of HCV infection in cardiac myopathic disorders.

  14. Cerebral embolic stroke after disappearing takotsubo cardiomyopathy.

    Science.gov (United States)

    Matsuzono, Kosuke; Ikeda, Yoshio; Deguchi, Shoko; Yamashita, Toru; Kurata, Tomoko; Deguchi, Kentaro; Abe, Koji

    2013-11-01

    Takotsubo cardiomyopathy can induce cerebral embolic stroke because of intracardiac thrombosis, but the timing of cardiogenic embolism relating to takotsubo cardiomyopathy has not been well described. We evaluated a 71-year-old woman with takotsubo cardiomyopathy, who developed cardiogenic cerebral embolism after recovery of cardiac wall motion. Nevertheless, we treated her with anticoagulation therapy. The present clinical observation suggests that attention should be paid to the timing when takotsubo cardiomyopathy resolves against risk of cardiogenic cerebral embolism.

  15. [Levosimendan for septic shock with takotsubo cardiomyopathy].

    Science.gov (United States)

    Schlürmann, C-N; Reinöhl, J; Kalbhenn, J

    2016-01-01

    As a stress-induced disease, takotsubo cardiomyopathy can also occur in septic syndromes; however, the hemodynamic management is fundamentally different from the treatment approaches for classical septic cardiomyopathy, as beta mimetics can increase the heart failure symptoms in takotsubo cardiomyopathy. This article reports the case of an 82-year-old female patient who presented with acute abdomen due to adhesion ileus and takotsubo cardiomyopathy, developed severe septic shock with peritonitis and could be successfully hemodynamically stabilized with levosimendan.

  16. Endothelial function in pre-pubertal children at risk of developing cardiomyopathy: a new frontier

    Directory of Open Access Journals (Sweden)

    Aline Cristina Tavares

    2012-01-01

    Full Text Available Although it is known that obesity, diabetes, and Kawasaki's disease play important roles in systemic inflammation and in the development of both endothelial dysfunction and cardiomyopathy, there is a lack of data regarding the endothelial function of pre-pubertal children suffering from cardiomyopathy. In this study, we performed a systematic review of the literature on pre-pubertal children at risk of developing cardiomyopathy to assess the endothelial function of pre-pubertal children at risk of developing cardiomyopathy. We searched the published literature indexed in PubMed, Bireme and SciELO using the keywords 'endothelial', 'children', 'pediatric' and 'infant' and then compiled a systematic review. The end points were age, the pubertal stage, sex differences, the method used for the endothelial evaluation and the endothelial values themselves. No studies on children with cardiomyopathy were found. Only 11 papers were selected for our complete analysis, where these included reports on the flow-mediated percentage dilatation, the values of which were 9.80±1.80, 5.90±1.29, 4.50±0.70, and 7.10±1.27 for healthy, obese, diabetic and pre-pubertal children with Kawasaki's disease, respectively. There was no significant difference in the dilatation, independent of the endothelium, either among the groups or between the genders for both of the measurements in children; similar results have been found in adolescents and adults. The endothelial function in cardiomyopathic children remains unclear because of the lack of data; nevertheless, the known dysfunctions in children with obesity, type 1 diabetes and Kawasaki's disease may influence the severity of the cardiovascular symptoms, the prognosis, and the mortality rate. The results of this study encourage future research into the consequences of endothelial dysfunction in pre-pubertal children.

  17. 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...

  18. Post-stenotic aortic dilatation

    Directory of Open Access Journals (Sweden)

    Jahangiri Marjan

    2006-03-01

    Full Text Available Abstract Aortic stenosis is the most common valvular heart disease affecting up to 4% of the elderly population. It can be associated with dilatation of the ascending aorta and subsequent dissection. Post-stenotic dilatation is seen in patients with AS and/or aortic regurgitation, patients with a haemodynamically normal bicuspid aortic valve and following aortic valve replacement. Controversy exists as to whether to replace the aortic root and ascending aorta at the time of aortic valve replacement, an operation that potentially carries a higher morbidity and mortality. The aetiology of post-stenotic aortic dilatation remains controversial. It may be due to haemodynamic factors caused by a stenotic valve, involving high velocity and turbulent flow downstream of the stenosis, or due to intrinsic pathology of the aortic wall. This may involve an abnormality in the process of extracellular matrix remodelling in the aortic wall including inadequate synthesis, degradation and transport of extracellular matrix proteins. This article reviews the aetiology, pathology and management of patients with post-stenotic aortic dilatation.

  19. Takotsubo cardiomyopathy triggered by alcohol withdrawal.

    Science.gov (United States)

    Alexandre, Joakim; Benouda, Leila; Champ-Rigot, Laure; Labombarda, Fabien

    2011-07-01

    Takotsubo cardiomyopathy is a reversible cardiomyopathy frequently precipitated by a sudden emotional or physical stress. The exact physiopathology is still debated and may involve catecholamine-induced myocardial stunning. Alcohol withdrawal is associated with an hyperadrenergic state and may be a period at risk of cardiac events. We report a 56-year-old man with Takotsubo cardiomyopathy triggered by alcohol withdrawal.

  20. Biventricular Takotsubo cardiomyopathy in Graves hyperthyroidism.

    Science.gov (United States)

    Perkins, Matthew J; Schachter, David T

    2014-03-01

    Graves hyperthyroidism is commonly seen in clinical practice and Takotsubo stress cardiomyopathy is an increasingly recognized cardiac complication of physical or emotional stress. We report the rare case of a patient with Graves hyperthyroidism that was complicated by severe biventricular takotsubo cardiomyopathy, which was demonstrated on heart catheterization. After appropriate pharmacologic treatment of her hyperthyroidism, she had complete resolution of her cardiomyopathy.

  1. Mitochondrial Mechanisms in Septic Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    María Cecilia Cimolai

    2015-08-01

    Full Text Available Sepsis is the manifestation of the immune and inflammatory response to infection that may ultimately result in multi organ failure. Despite the therapeutic strategies that have been used up to now, sepsis and septic shock remain a leading cause of death in critically ill patients. Myocardial dysfunction is a well-described complication of severe sepsis, also referred to as septic cardiomyopathy, which may progress to right and left ventricular pump failure. Many substances and mechanisms seem to be involved in myocardial dysfunction in sepsis, including toxins, cytokines, nitric oxide, complement activation, apoptosis and energy metabolic derangements. Nevertheless, the precise underlying molecular mechanisms as well as their significance in the pathogenesis of septic cardiomyopathy remain incompletely understood. A well-investigated abnormality in septic cardiomyopathy is mitochondrial dysfunction, which likely contributes to cardiac dysfunction by causing myocardial energy depletion. A number of mechanisms have been proposed to cause mitochondrial dysfunction in septic cardiomyopathy, although it remains controversially discussed whether some mechanisms impair mitochondrial function or serve to restore mitochondrial function. The purpose of this review is to discuss mitochondrial mechanisms that may causally contribute to mitochondrial dysfunction and/or may represent adaptive responses to mitochondrial dysfunction in septic cardiomyopathy.

  2. Sepsis-induced Cardiomyopathy

    Science.gov (United States)

    Romero-Bermejo, Francisco J; Ruiz-Bailen, Manuel; Gil-Cebrian, Julián; Huertos-Ranchal, María J

    2011-01-01

    Myocardial dysfunction is one of the main predictors of poor outcome in septic patients, with mortality rates next to 70%. During the sepsis-induced myocardial dysfunction, both ventricles can dilate and diminish its ejection fraction, having less response to fluid resuscitation and catecholamines, but typically is assumed to be reversible within 7-10 days. In the last 30 years, It´s being subject of substantial research; however no explanation of its etiopathogenesis or effective treatment have been proved yet. The aim of this manuscript is to review on the most relevant aspects of the sepsis-induced myocardial dysfunction, discuss its clinical presentation, pathophysiology, etiopathogenesis, diagnostic tools and therapeutic strategies proposed in recent years. PMID:22758615

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1992-07-01

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

  4. TAKOTSUBO CARDIOMYOPATHY: A CASE REPORT

    Directory of Open Access Journals (Sweden)

    Krishna M

    2014-08-01

    Full Text Available Takotsubo cardiomyopathy is a condition caused by intense emotional or physical stress leading to rapid and severe reversible cardiac dysfunction. A 44 year old labourer presented with three days old bilateral traumatic fracture of femur and severe respiratory distress; he was ventilated for one day. Echocardiography ruled out pulmonary embolism. Patient remained stable for the next three days. On the fifth day, he appeared fearful, presented with sudden chest pain, tachycardia and hypotension. Echocardiography revealed ejection fraction of 34%, global hypokinesia of left ventricle with apical ballooning and no regional wall motion abnormalities. Coronary angiography was done which revealed no vascular abnormalities. Diagnosis of Takotsubo cardiomyopathy was made and vasopressors were started. Psychiatric treatment of physical and emotional stress was done. Patient gradually improved with ongoing treatment and on eighth day his cardiac function reverted back to normal. Takotsubo cardiomyopathy can be efficiently managed by early recognition, proper supportive treatment and meticulous management of physical and emotional stress.

  5. Genetic biomarkers in hypertrophic cardiomyopathy.

    Science.gov (United States)

    Coats, Caroline J; Elliott, Perry M

    2013-08-01

    Hypertrophic cardiomyopathy is a common inherited heart muscle disorder associated with sudden cardiac death, arrhythmias and heart failure. Genetic mutations can be identified in approximately 60% of patients; these are commonest in genes that encode proteins of the cardiac sarcomere. Similar to other Mendelian diseases these mutations are characterized by incomplete penetrance and variable clinical expression. Our knowledge of this genetic diversity is rapidly evolving as high-throughput DNA sequencing technology is now used to characterize an individual patient's disease. In addition, the genomic basis of several multisystem diseases associated with a hypertrophic cardiomyopathy phenotype has been elucidated. Genetic biomarkers can be helpful in making an accurate diagnosis and in identifying relatives at risk of developing the condition. In the clinical setting, genetic testing and genetic screening should be used pragmatically with appropriate counseling. Here we review the current role of genetic biomarkers in hypertrophic cardiomyopathy, highlight recent progress in the field and discuss future challenges.

  6. An update on peripartum cardiomyopathy.

    Science.gov (United States)

    Dalzell, Jonathan R; Jackson, Colette E; Gardner, Roy S

    2011-09-01

    Peripartum cardiomyopathy is a rare but potentially devastating complication of pregnancy. Although the definition of this condition has recently been revised by the Heart Failure Association of the European Society of Cardiology, the pathogenesis of peripartum cardiomyopathy is not well understood and relatively little is known about its incidence and prevalence. Hence, peripartum cardiomyopathy is often under-recognized in the clinical setting. A heightened awareness of this condition and its current management options is therefore warranted throughout primary and secondary care. The identification of the putative role of prolactin in the development and progression of this condition has been recently discovered, with preclinical work suggesting beneficial effects of prolactin antagonism. In this article, we review the literature regarding this condition including these recent advances.

  7. 预激性心肌病%Wolff-Parkinson-White syndrome-induced cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    罗亚雄; 龚小鹏; 王福军

    2015-01-01

    Wolff-Parkinson-White (WPW)syndrome can induce dilated cardiomyopathy.Previ-ously,it was frequently reported that WPW syndrome complicating recurrent or persistent tachya-rrhythmias can develop into dilated cardiomyopathy,that is,tachycardia-induced cardiomyopathy. In addition,WPW syndrome may be accompanied with hypertrophic cardiomyopathy.WPW syn-drome can cause another type of dilated cardiomyopathy,WPW syndrome-induced cardiomyopathy, also known as Wolff-Parkinson-mediated cardiomyopathy.It is due to the affected electrical-mechan-ical properties of synchronous ventricular excitation and contraction,leading to influence the me-chanical function of heart and induce ventricular remodeling.Besides the diagnostic criteria for di-lated cardiomyopathy,WPW syndrome-induced cardiomyopathy still has the following two character-istics:(i)Asymptomatic WPW syndrome,that is,no medical history of recurrent or persistent at-tack of tachyarrhythmia clinically;(ii)Twelve lead surface ECG indicates right sided manifest WPW.Radiofrequency ablation is safe and effective in treating the disease.%预激综合征可诱发扩张型心肌病,以往常报道预激综合征伴反复发生或持续性的快速性心律失常可发展为扩张型心肌病,即心动过速性心肌病。另外,预激综合征可合并肥厚型心肌病。而预激综合征还可诱发另一类扩张型心肌病,即预激性心肌病,也称为预激介导性心肌病,是由于心室同步兴奋和收缩的电机械特性受到影响,进而影响心脏的机械功能,造成心室重构而引起的。它除了具备扩张型心肌病的诊断条件外,尚具备下述两个特点:①无症状的预激综合征,即临床上没有反复或持续性快速性心律失常发作史;②体表12导联心电图提示显性预激,并且为右侧旁道。对于预激性心肌病,射频消融术是安全有效的治疗方法。

  8. 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...

  9. Application of18F-FDG Micro-PET Myocardial Metabolism Imaging for Evaluating Dilated Cardiomyopathy Model in Experimental Rats%18氟-氟脱氧葡萄糖微型正电子发射断层扫描心肌代谢显像技术在大鼠扩张型心肌病模型评价中的应用

    Institute of Scientific and Technical Information of China (English)

    沈丽娟; 陆曙; 周永华; 邢清敏; 李岚; 杨敏; 周春刚

    2016-01-01

    Objective: To explore the application of18F-lfuorodeoxyglucose (FDG) micro- positron emission tomography (PET) myocardial metabolism imaging for evaluating dilated cardiomyopathy model (DCM) in experimental rats. Methods: A total of 12 male SD rats were randomly divided into 2 groups: DCM group, the rats received intraperitoneal injection of adriamycin at 1.0 mg/kg twice per week and Control group, the rats received intraperitoneal injection of normal saline, all animals were treated for 6 weeks followed by 2 weeks observation.n=6 in each group. Echocardiography was performed at pre- and post-modeling,18F-FDG micro-PET myocardial metabolism imaging was conducted after modeling and plasma level of BNP was examined as well. Finally, the rats were scariifed to observe the pathological changes of myocardial tissue. Results: 1 rat died in DCM group and the rest were with successful modeling conifrmed by echocardiography and pathology. Compared with Control group, DCM group showed decreased standard uptake value of18F-FDG (1.23 ± 0.55) vs (6.65 ± 0.41),P<0.01; the standard uptake value of18F-FDG was negatively related to left ventricular end diastolic diameter (LVEDD) (R=-0.709,P=0.015), LVESD (R=-0.924, P=0.000) and plasma level of BNP (R=-0.948,P=0.000), while positively related to LVEF (R=0.968,P=0.000) and fractional shortening (R=0.863,P=0.001). Conclusion:18F-FDG micro-PET myocardial metabolism imaging combining echocardiography, biochemical and pathological examinations may evaluate DCM modeling in rats, which provide a non-invasive and intravital tool for small animal experiment.%目的:探讨18氟-氟脱氧葡萄糖(18F-FDG)微型正电子发射断层扫描(Micro-PET)心肌代谢显像技术在大鼠扩张型心肌病(DCM)模型评价中的应用价值。方法:选用雄性SD大鼠12只,随机分为对照组(n=6)和DCM组(n=6),DCM组腹腔注射阿霉素1.0 mg/kg(生理盐水稀释至1.0 mg/ ml),2次/周,对照组腹腔注

  10. Peripartum cardiomyopathy coexistent with human immunodeficiency virus: A substantial obstetric jeopardy

    Directory of Open Access Journals (Sweden)

    Debasmita Mandal

    2013-01-01

    Full Text Available Peripartum cardiomyopathy (PPCM is a rare cause of pregnancy-related heart failure, which affects a woman during the last months of pregnancy or first months of parturition. Its etiopathogenesis is still unclear. Coexistence of PPCM with human immunodeficiency virus (HIV has been scarcely analyzed. A low CD4 count is proposed to be one of the predictors of dilated cardiomyopathy in HIV. Here, a pregnant woman with HIV presented with signs of congestive heart failure for the first time during her last trimester. Echocardiography revealed a dilated cardiomyopathy with ejection fraction of 34% which proved the diagnosis of PPCM. She underwent cesarean section for impending previous scar rupture. Her status deteriorated subsequently in spite of all efforts and she succumbed due to ventricular tachycardia. This case necessitates an awareness regarding coexistence of HIV with PPCM and dreaded clinical sequences. Patients suffering from HIV should be treated well and their CD4 count should be improved before conception to avoid such complications in pregnancy.

  11. Peripartum cardiomyopathy coexistent with human immunodeficiency virus: a substantial obstetric jeopardy.

    Science.gov (United States)

    Mandal, Debasmita; Dattaray, Chaitalli; Dutta, Mousumi; Sarkar, Gouranga; Sinha, Pooja

    2013-01-01

    Peripartum cardiomyopathy (PPCM) is a rare cause of pregnancy-related heart failure, which affects a woman during the last months of pregnancy or first months of parturition. Its etiopathogenesis is still unclear. Coexistence of PPCM with human immunodeficiency virus (HIV) has been scarcely analyzed. A low CD4 count is proposed to be one of the predictors of dilated cardiomyopathy in HIV. Here, a pregnant woman with HIV presented with signs of congestive heart failure for the first time during her last trimester. Echocardiography revealed a dilated cardiomyopathy with ejection fraction of 34% which proved the diagnosis of PPCM. She underwent cesarean section for impending previous scar rupture. Her status deteriorated subsequently in spite of all efforts and she succumbed due to ventricular tachycardia. This case necessitates an awareness regarding coexistence of HIV with PPCM and dreaded clinical sequences. Patients suffering from HIV should be treated well and their CD4 count should be improved before conception to avoid such complications in pregnancy.

  12. Cocaine cardiomyopathy: A case report

    Directory of Open Access Journals (Sweden)

    Georgiev Antonio

    2014-12-01

    Full Text Available Cocaine is the second most common illicit drug used and the most frequent cause of drug related deaths. The use of cocaine is associated with both, acute and chronic complications, that may involve any system, but the most common system affected is cardiovascular one. Cocaine cardiomyopathy may result from the use of cocaine. This article presents a first case in Republic of Macedonia of 24-year-old male with reversible cocaine-related cardiomyopathy. Clinical presentation, laboratory, X-ray, ultrasound findings and treatment are reviewed.

  13. Peripartum cardiomyopathy: a contemporary review.

    Science.gov (United States)

    Shah, Tina; Ather, Sameer; Bavishi, Chirag; Bambhroliya, Arvind; Ma, Tony; Bozkurt, Biykem

    2013-01-01

    Peripartum cardiomyopathy is a rare and potentially fatal disease. Though approximately half of the patients recover, the clinical course is highly variable and some patients develop refractory heart failure and persistent left ventricular systolic dysfunction. It is diagnosed when women present with heart failure secondary to left ventricular systolic dysfunction towards the end of pregnancy or in the months following delivery, where no other cause of heart failure is found. Etiology remains unclear, and treatment is similar to other cardiomyopathies and includes evidence-based standard heart failure management strategies. Experimental strategies such as intravenous immunoglobulin and bromocriptine await further clinical validation.

  14. A rare case of peripartum cardiomyopathy posted for caesarean section

    Directory of Open Access Journals (Sweden)

    Nalini Kotekar

    2007-01-01

    Full Text Available Post Partum Cardiomyopathy (PPCM is a relatively rare form of heart failure associated with pregnancy. It was recognized first in the 19th century by Ritchie and is defined as the onset of acute heart failure in the last trimester or early post partum period in the absence of infections, metabolic, toxic, ischaemic or valvular causes of myocardial dysfunction. Prognosis depends on the degree of cardiomegaly at presentation and in the following 6 months. Initial high risk period carries a mortality of 25 to 50%. Keeping in mind the reduced contractility and ejection fraction with ventricular dilatation proceeding to cardiac failure, the anesthesiologist managing a case of PPCM faces the challenge of avoiding myocardial depression, hypovolemia and increased SVR, all of which may be hazardous

  15. Physiology and pathophysiology of iron cardiomyopathy in thalassemia.

    Science.gov (United States)

    Wood, John C; Enriquez, Cathleen; Ghugre, Nilesh; Otto-Duessel, Maya; Aguilar, Michelle; Nelson, Marvin D; Moats, Rex; Coates, Thomas D

    2005-01-01

    Iron cardiomyopathy remains the leading cause of death in patients with thalassemia major. Magnetic resonance imaging (MRI) is ideally suited for monitoring thalassemia patients because it can detect cardiac and liver iron burdens as well as accurately measure left ventricular dimensions and function. However, patients with thalassemia have unique physiology that alters their normative data. In this article, we review the physiology and pathophysiology of thalassemic heart disease as well as the use of MRI to monitor it. Despite regular transfusions, thalassemia major patients have larger ventricular volumes, higher cardiac outputs, and lower total vascular resistances than published data for healthy control subjects; these hemodynamic findings are consistent with chronic anemia. Cardiac iron overload increases the relative risk of further dilation, arrhythmias, and decreased systolic function. However, many patients are asymptomatic despite heavy cardiac burdens. We explore possible mechanisms behind cardiac iron-function relationships and relate these mechanisms to clinical observations.

  16. 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

    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 (death from cardiomyopathy or other conditions, and cohort members were followed from 1977 to 2008 for cardiomyopathy diagnosed at ... incidence rate ratios for cardiomyopathy by family history of premature death. Premature cardiomyopathy deaths in first- and second-degree relatives were associated with 29- and 6-fold increases in the rate of cardiomyopathy, respectively. If the first-degree relative died aged

  17. 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.

  18. 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

  19. 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

    2015-01-01

    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. PMID:25993486

  20. 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.

  1. Peripartum Cardiomyopathy: A Case Report

    Directory of Open Access Journals (Sweden)

    Z Basirat

    2006-04-01

    Full Text Available ABSTRACT: Introduction & Objective: Peripartum cardiomyopathy is a rare but sometimes fatal form of heart failure during the period of 1 month antepartum to 5 months postpartum. The aim of this report is to assess the clinical presentation, management and crucial role of echocardiography in women with peripartum cardiomyopathy. Case: A 22 year-old woman, with previously healthy primipara, was admitted to the emergency ward with sever dyspnea, cough, and bloody hemoptesis and a preliminary diagnosis of pulmonary embolism (PE two weeks after cesarean section. Neither perfusion scintigaphy nor Doppler sonography test of lower extremities and pelvis showed any evidence of PE or deep venous thrombosis. Echocardiography revealed features of left ventricular failure. A diagnosis of peripartum cardiomyopathy was made, appropriate treatment was administered and the patient improved. Conclusion: It is possible to misdiagnose peripartum cardiomyopathy with PE. Echocardiography is a valuable tool in the differential diagnosis. As a noninvasive procedure, it should be performed at the bedside as soon as possible to introduce proper treatment and to avoid potentially fatal errors.

  2. Genetic basis of hypertrophic cardiomyopathy

    NARCIS (Netherlands)

    Bos, J.M.

    2010-01-01

    The understanding of hypertrophic cardiomyopathy (HCM) has matured from its cornerstone as a disease of the sarcomere to a compendium of diseases with various clinical, genetic and morphologic substrates. Research has provided us more insights into i) the pathogenetic development of HCM, ii) the pos

  3. 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;

  4. Anabolic steroid-induced cardiomyopathy underlying acute liver failure in a young bodybuilder

    Institute of Scientific and Technical Information of China (English)

    Miguel Bispo; Ana Valente; Rosário Maldonado; Rui Palma; Helena Glória; Jo(a)o Nóbrega; Paula Alexandrino

    2009-01-01

    Heart failure may lead to subclinical circulatory disturbances and remain an unrecognized cause of ischemic liver injury. We present the case of a previously healthy 40-year-old bodybuilder, referred to our Intensive-Care Unit of Hepatology for treatment of severe acute liver failure, with the suspicion of toxic hepatitis associated with anabolic steroid abuse. Despite the absence of symptoms and signs of congestive heart failure at admission, an anabolic steroid-induced dilated cardiomyopathy with a large thrombus in both ventricles was found to be the underlying cause of the liver injury. Treatment for the initially unrecognized heart failure rapidly restored liver function to normal. To our knowledge, this is the first reported case of severe acute liver failure due to an unrecognized anabolic steroid-induced cardiomyopathy. Awareness of this unique presentation will allow for prompt treatment of this potentially fatal cause of liver failure.

  5. Antarctic analog for dilational bands on Europa

    Science.gov (United States)

    Hurford, T. A.; Brunt, K. M.

    2014-09-01

    Europa's surface shows signs of extension, which is revealed as lithospheric dilation expressed along ridges, dilational bands and ridged bands. Ridges, the most common tectonic feature on Europa, comprise a central crack flanked by two raised banks a few hundred meters high on each side. Together these three classes may represent a continuum of formation. In Tufts' Dilational Model ridge formation is dominated by daily tidal cycling of a crack, which can be superimposed with regional secular dilation. The two sources of dilation can combine to form the various band morphologies observed. New GPS data along a rift on the Ross Ice Shelf, Antarctica is a suitable Earth analog to test the framework of Tufts' Dilational Model. As predicted by Tufts' Dilational Model, tensile failures in the Ross Ice Shelf exhibit secular dilation, upon which a tidal signal can be seen. From this analog we conclude that Tufts' Dilational Model for Europan ridges and bands may be credible and that the secular dilation is most likely from a regional source and not tidally driven.

  6. Antarctic Analog for Dilational Bands on Europa

    Science.gov (United States)

    Hurford, T. A.; Brunt, K. M.

    2014-01-01

    Europa's surface shows signs of extension, which is revealed as lithospheric dilation expressed along ridges, dilational bands and ridged bands. Ridges, the most common tectonic feature on Europa, comprise a central crack flanked by two raised banks a few hundred meters high on each side. Together these three classes may represent a continuum of formation. In Tufts' Dilational Model ridge formation is dominated by daily tidal cycling of a crack, which can be superimposed with regional secular dilation. The two sources of dilation can combine to form the various band morphologies observed. New GPS data along a rift on the Ross Ice Shelf, Antarctica is a suitable Earth analog to test the framework of Tufts' Dilational Model. As predicted by Tufts' Dilational Model, tensile failures in the Ross Ice Shelf exhibit secular dilation, upon which a tidal signal can be seen. From this analog we conclude that Tufts' Dilational Model for Europan ridges and bands may be credible and that the secular dilation is most likely from a regional source and not tidally driven.

  7. Distribution of late gadolinium enhancement in various types of cardiomyopathies:Significance in differential diagnosis, clinical features and prognosis

    Institute of Scientific and Technical Information of China (English)

    Hiroshi; Satoh; Makoto; Sano; Kenichiro; Suwa; Takeji; Saitoh; Mamoru; Nobuhara; Masao; Saotome; Tsuyoshi; Urushida; Hideki; Katoh; Hideharu; Hayashi

    2014-01-01

    The recent development of cardiac magnetic resonance(CMR)techniques has allowed detailed analyses of cardiac function and tissue characterization with high spatial resolution.We review characteristic CMR features in ischemic and non-ischemic cardiomyopathies(ICM and NICM),especially in terms of the location and distribution of late gadolinium enhancement(LGE).CMR in ICM shows segmental wall motion abnormalities or wall thinning in a particular coronary arterial territory,and the subendocardial or transmural LGE.LGE in NICM generally does not correspond to any particular coronary artery distribution and is located mostly in the mid-wall to subepicardial layer.The analysis of LGE distribution is valuable to differentiate NICM with diffusely impaired systolic function,including dilated cardiomyopathy,end-stage hypertrophic cardiomyopathy(HCM),cardiac sarcoidosis,and myocarditis,and those with diffuse left ventricular(LV)hypertrophy including HCM,cardiac amyloidosis and Anderson-Fabry disease.A transient low signal intensity LGE in regions of severe LV dysfunction is a particular feature of stress cardiomyopathy.In arrhythmogenic right ventricular cardiomyopathy/dysplasia,an enhancement of right ventricular(RV)wall with functional and morphological changes of RV becomes apparent.Finally,the analyses of LGE distribution have potentials to predict cardiac outcomes and response to treatments.

  8. Cardiomyopathy as presenting sign of glycogenin-1 deficiency-report of three cases and review of the literature.

    Science.gov (United States)

    Hedberg-Oldfors, Carola; Glamuzina, Emma; Ruygrok, Peter; Anderson, Lisa J; Elliott, Perry; Watkinson, Oliver; Occleshaw, Chris; Abernathy, Malcolm; Turner, Clinton; Kingston, Nicola; Murphy, Elaine; Oldfors, Anders

    2017-01-01

    We describe a new type of cardiomyopathy caused by a mutation in the glycogenin-1 gene (GYG1). Three unrelated male patients aged 34 to 52 years with cardiomyopathy and abnormal glycogen storage on endomyocardial biopsy were homozygous for the missense mutation p.Asp102His in GYG1. The mutated glycogenin-1 protein was expressed in cardiac tissue but had lost its ability to autoglucosylate as demonstrated by an in vitro assay and western blot analysis. It was therefore unable to form the primer for normal glycogen synthesis. Two of the patients showed similar patterns of heart dilatation, reduced ejection fraction and extensive late gadolinium enhancement on cardiac magnetic resonance imaging. These two patients were severely affected, necessitating cardiac transplantation. The cardiomyocyte storage material was characterized by large inclusions of periodic acid and Schiff positive material that was partly resistant to alpha-amylase treatment consistent with polyglucosan. The storage material had, unlike normal glycogen, a partly fibrillar structure by electron microscopy. None of the patients showed signs or symptoms of muscle weakness but a skeletal muscle biopsy in one case revealed muscle fibres with abnormal glycogen storage. Glycogenin-1 deficiency is known as a rare cause of skeletal muscle glycogen storage disease, usually without cardiomyopathy. We demonstrate that it may also be the cause of severe cardiomyopathy and cardiac failure without skeletal muscle weakness. GYG1 should be included in cardiomyopathy gene panels.

  9. Patterns of delayed-enhancement in MRI of ischemic and non-ischemic cardiomyopathies; Muster der spaeten Kontrastmittelanreicherung in der MRT bei ischaemischen und nicht-ischaemischen Kardiomyopathien

    Energy Technology Data Exchange (ETDEWEB)

    Stork, A.; Bansmann, P.M.; Koops, A.; Adam, G. [Klinik und Poliklinik fuer Diagnostische und Interventionelle Radiologie, Universitaetsklinikum Hamburg-Eppendorf (Germany); Muellerleile, K.; Meinertz, T. [Universitaeres Herzzentrum, Universitaetsklinikum Hamburg-Eppendorf (Germany); Lund, G.K. [Kardiovaskulaere Bildgebung, Roentgeninstitut Duesseldorf (Germany)

    2007-01-15

    Contrast-enhanced MRI using the delayed-enhancement technique (DE-MRI) is widely applied in the clinical work-up of myocardial diseases. Myocardial diseases of varying etiology result in myocardial changes, such as necrosis, fibrosis, edema and metabolite deposition, which can be visualized by DE-MRI. Acute and chronic ischemic diseases based on a coronary artery disease as well as non-ischemic cardiomyopathies display DE. Cardiomyopathies often show a characteristic enhancement pattern. While ischemic lesions are localized in the subendocardium, non-ischemic cardiomyopathies often display an intramyocardial or subepicardial pattern. The typical pattern for dilated cardiomyopathies is band-like and intramyocardial with septal involvement. Arrhythmogenic right-ventricular dysplasias/cardiomyopathies are frequently associated with right-ventricular DE. In the case of amyloid cardiomyopathies which are often restrictive cardiomyopathies, subendocardial and circular DE is typically observed. Hypertrophic cardiomyopathies display patchy intramyocardial DE usually in the anteroseptal region. Acute myocarditis is typically accompanied by intramyocardial or subepicardial DE affecting the lateral wall. In the case of chronic myocarditis, intramyocardial or subepicardial DE is observed most frequently. Cardiac sarcoidosis typically entails patchy subepicardial DE with right- and left-ventricular involvement. Since there is an overlap between the enhancement patterns of cardiomyopathies, the diagnostic accuracy of DE-MRI is limited and the diagnosis must be based on additional clinical and MRI findings. The amount of DE often corresponds with cardiac functional parameters as well as with the frequency of cardiac events so that DE-MRI may be useful for risk stratification. Furthermore, DE-MRI can be helpful in the planning and evaluation of myocardial biopsies and electrophysiological examinations. (orig.)

  10. Keshan disease and mitochondrial cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    YANG Fuyu

    2006-01-01

    Keshan disease (KD) is a potentially fatal form of cardiomyopathy (disease of the heart muscle) endemic in certain areas of China. From 1984 to 1986, a national comprehensive scientific investigation on KD in Chuxiong region of Yunnan Province in the southwest China was conducted. The investigation team was composed of epidemiologists, clinic doctors, pathologists, biochemists, biophysicists and specialists in ecological environment. Results of pathological, biochemical and biophysical as well as clinical studies showed: an obvious increase of enlarged and swollen mitochondria with distended crista membranes in myocardium from patients with KD; significant reductions in the activity of oxidative phosphorylation (succinate dehydrogenase, cytochrome oxidase, succinate oxidase, H+-ATPase) of affected mitochondria; decrease in CoQ, cardiolipin, Se and GSHPx activity, while obvious increase in the Ca2+ content. So, it was suggested that mitochondria are the predominant target of the pathogenic factors of KD. Before Chuxiong KD survey only a few cases of mitochondrial cardiomyopathy were studied. During the multidisciplinary scientific investigation on KD in Chuxiong a large amount of samples from KD cases and the positive controls were examined. On the basis of the results obtained it was suggested that KD might be classified as a "Mitochondrial Cardiomyopathy" endemic in China. This is one of the achievements in the three years' survey in Chuxiong and is valuable not only to the deeper understanding of pathogenic mechanism of KD but also to the study of mitochondrial cardiomyopathy in general.Keshan disease is not a genetic disease, but is closely related to the malnutrition (especially microelement Se deficiency). KD occurs along a low Se belt, and Se supplementation has been effective in prevention of such disease. The incidence of KD has sharply decreased along with the steady raise of living standard and realization of preventive measures. At present, patients of

  11. Abnormal atrial activation is common in patients with arrhythmogenic right ventricular cardiomyopathy

    DEFF Research Database (Denmark)

    Platonov, Pyotr G; Christensen, Alex H; Holmqvist, Fredrik;

    2011-01-01

    INTRODUCTION: Structural right atrial abnormalities have been described in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). However, little is known about electrocardiographic signs of atrial involvement in ARVC because no systematic studies have been conducted. METHODS: P......%, whereas 15 patients (37%) had atypical P-wave positive in all 3 leads (P right ventricular abnormality. CONCLUSIONS: Patients with ARVC commonly demonstrate deteriorated...... atrial activation expressed either as prolonged P-wave duration or abnormal P-wave morphology. The P-wave abnormalities were not secondary to right ventricular dilatation. These findings show that atrial involvement is common in ARVC and may represent yet another manifestation of the disease...

  12. Peripartum cardiomyopathy (A literature review

    Directory of Open Access Journals (Sweden)

    Farveh Vakilian

    2014-07-01

    Full Text Available Heart failure (HF is a serious and growing public health concern, which has many causes. Pregnancy is a critical condition with significant hemodynamic and immunologic changes. Peripartum cardiomyopathy (PPCM is a disease of unknown cause in which left ventricular (LV dysfunction occurs during the last trimester of pregnancy or the early puerperium. PPCM is known to be the most common cardiovascular cause of severe complications in pregnancy.  Risk factors for peripartum cardiomyopathy include advanced maternal age, twin pregnancy, smoking, pregnancy-related hypertension and preeclampsia, multiparity, African descent, and long-term tocolysis. Oxidative stress and some inflammatory markers have been diagnosed in PPCM pathophysiology. Recent observations have suggested that bromocriptine might favor recovery of LV systolic function in patients with PPCM. Patients developed peripartum cardiomiopathy treated with bromocriptine showed significantly improved LV ejection fraction and heart failure symptoms. This article tries to have a short review on this clinical scenario.

  13. Takotsubo cardiomyopathy: a historical note

    Institute of Scientific and Technical Information of China (English)

    2009-01-01

    @@ To the Editor: I read with interest the case report of Takotsubo cardiomyopathy in a Chinese woman.1 Unfortunately, the authors mistakenly attributed the original description of this syndrome to Tsuchihashi et al in 2001.2Actually Dote et al3 first described this syndrome in 1991, ten years before Tsuchihashi et al did.2 Incidentally, the authors did make a reference in their case report to the article by Dote et al3 which was cited as reference 1.

  14. Peripartum Cardiomyopathy Presenting as Bradycardia

    Science.gov (United States)

    Rose, Carl H.; Tweet, Marysia S.; Hayes, Sharonne N.; Best, Patricia J. M.; Blauwet, Lori A.

    2017-01-01

    Peripartum cardiomyopathy (PPCM) is a disease that typically affects young otherwise healthy women. As PPCM is associated with significant mortality, timely diagnosis is necessary to ensure appropriate care. To our knowledge, this represents the first reported case of PPCM presenting as symptomatic bradycardia. We describe the patient's clinical presentation and relevant findings and review the potential etiology and ramifications of bradycardia in patients with PPCM.

  15. Peripartum Cardiomyopathy: A Case Report

    Directory of Open Access Journals (Sweden)

    Afzal Azim

    2009-04-01

    Full Text Available Peripartum cardiomyopathy (PPCM is an uncommon but life threatening disease that affects women in the last month of pregnancy or within the first five months after delivery. Very few Indian case reports are available. However, it is essential for the practitioner dealing with such population to have a high degree of clinical suspicion for early diagnosis and management. Echocardiography is used to diagnose this entity and monitor the therapy.

  16. Peripartum Cardiomyopathy Presenting as Bradycardia

    Directory of Open Access Journals (Sweden)

    Elisabeth Codsi

    2017-01-01

    Full Text Available Peripartum cardiomyopathy (PPCM is a disease that typically affects young otherwise healthy women. As PPCM is associated with significant mortality, timely diagnosis is necessary to ensure appropriate care. To our knowledge, this represents the first reported case of PPCM presenting as symptomatic bradycardia. We describe the patient’s clinical presentation and relevant findings and review the potential etiology and ramifications of bradycardia in patients with PPCM.

  17. Recurrent takotsubo cardiomyopathy in a child.

    Science.gov (United States)

    Srivastava, Nayan T; Parent, John J; Hurwitz, Roger A

    2016-02-01

    Takotsubo cardiomyopathy or transient apical ballooning syndrome very rarely presents in children. In all patients with takotsubo, it is estimated that only 3.5% will have recurrence. In this study, we describe a case of recurrent takotsubo cardiomyopathy in a child, likely triggered by status epilepticus.

  18. Cardiomyopathy in becker muscular dystrophy:Overview

    Institute of Scientific and Technical Information of China (English)

    Rady Ho; My-Le Nguyen; Paul Mather

    2016-01-01

    Becker muscular dystrophy(BMD) is an X-linked recessive disorder involving mutations of the dystrophin gene. Cardiac involvement in BMD has been described and cardiomyopathy represents the number one cause of death in these patients. In this paper, the pathophysiology, clinical evaluations and management of cardiomyopathy in patients with BMD will be discussed.

  19. Peripartum cardiomyopathy : Euro Observational Research Program

    NARCIS (Netherlands)

    Hoes, M. F.; van Hagen, I.; Russo, F.; Van Veldhuisen, D. J.; Van den Berg, M. P.; Roos-Hesselink, J.; van Spaendonck-Zwarts, K. Y.; van der Meer, P.

    2014-01-01

    Peripartum cardiomyopathy is a rare but potentially life-threatening form of heart failure affecting women late in pregnancy or in the first months after delivery. Peripartum cardiomyopathy is difficult to diagnose and its onset and progression are variable between individuals. The pathophysiology r

  20. Outpatient experience with oesophageal endoscopic dilation.

    Science.gov (United States)

    Jani, P G; Mburugu, P G

    1998-07-01

    Between March 1990 and August 1997, outpatient endoscopic balloon dilation was performed for oesophageal strictures which developed secondary to malignancies, peptic strictures, post surgical narrowing, achalasia cardia, corrosive ingestion and other causes. A total of 169 dilations were performed in the 92 cases with an average of 1.8 dilation/case (Range 1 to 8). Dilation was possible in all 92 cases without the need for fluoroscopic monitoring. Twenty three (13.6%) of the dilations were performed using pneumatic balloon while in 146(86.4%) cases wire guided metal olives were used. There were nine minor complications which were treated with medication on an outpatient basis and four major complications which required inpatient care. Three of these had perforation of the oesophagus and one died. One other patient developed aspiration pneumonia and subsequently died.

  1. Radionuclide evaluation of renal artery dilatation

    Energy Technology Data Exchange (ETDEWEB)

    Born, M.L.; Gerlock, A.J. Jr.; Goncharenko, V.; Hollifield, J.W.; MacDonell, R.C. Jr.

    1981-01-01

    Radionuclide studies were used in three patients to evaluate renal perfusion and function within 24 hours following transluminal dilatation. In one patient, technetium-99 m pertechnetate showed good renal perfusion one and 12 hours after a post-dilatation arteriogram had shown a renal artery intimal defect. Improved clearance of iodine-131 ortho-iodohippurate from the blood demonstrated an increase in renal function 18 hours following dilatation of a stenosis at a renal allograft anastomosis in the second patient, while technetium-99 m-labeled DTPA showed an improved total glomerular filtration rate 24 hours after dilatation of a saphenous vein bypass graft in the third patient. It was concluded that renal radionuclide studies are of benefit in evaluating patients in the immediate post-dilatation period.

  2. Takotsubo cardiomyopathy associated with severe hypothyroidism in an elderly female

    Directory of Open Access Journals (Sweden)

    Jorge A Brenes-Salazar

    2016-01-01

    Full Text Available Takotsubo cardiomyopathy, also known as stress cardiomyopathy, is a syndrome that affects predominantly postmenopausal women. Despite multiple described mechanisms, intense, neuroadrenergic myocardial stimulation appears to be the main trigger. Hyperthyroidism, but rarely hypothyroidism, has been described in association with Takotsubo cardiomyopathy. Herein, we present a case of stress cardiomyopathy in the setting of symptomatic hypothyroidism.

  3. Hypertrophic obstructive cardiomyopathy in an infant with an adrenocortical tumor.

    Science.gov (United States)

    Hauser, Jakob; Riedl, Stefan; Michel-Behnke, Ina; Minkov, Milen; Perneczky, Eva; Horcher, Ernst

    2013-08-01

    Nonfamilial cardiomyopathies in childhood have been only sporadically ascribed to endocrine disorders. We report on a 4-month-old male infant presenting with Cushing's syndrome associated with excessive body weight (8.9 kg; >97th percentile) and features of virilization (Tanner stage 2 for pubic hair development). Abdominal sonography showed a large adrenal tumor. Echocardiography revealed myocardial hypertrophy with severe subaortic obstruction. Blood tests showed excessive androgen and cortisol serum levels with absent circadian rhythm as well as suppressed corticotropin. Urine catecholamine levels were within the normal range. Tumor resection with general anesthesia was performed after preparation with antihypertensive and anticongestive drug therapy. Continuous intravenous hydrocortisone substitution was started intraoperatively and subsequently tapered and switched to oral administration after 12 days. A gradual reduction in glucocorticoid substitution and its discontinuation after a total duration of 9 months were well tolerated. Histopathologic workup revealed an adrenocortical tumor of intermediate dignity. Postoperative tumor staging excluded both residual primary tumor and metastases. Both a normalization of body weight and myocardial mass were observed. The present article is, to our knowledge, the first to describe severe hypertrophic obstructive cardiomyopathy caused by an adrenocortical tumor and provides novel detailed data on postoperative glucocorticoid management.

  4. PERIPARTUM CARDIOMYOPATHY--REPORT OF 16 CASES

    Institute of Scientific and Technical Information of China (English)

    杨佳欣; 刘俊涛; 边旭明

    2002-01-01

    Objective.To analyze the clinical characteristics of peripartum cardiomyopathy and to evaluate the different factors that influence the prognosis of the peripartum cardiomyopathy.Method.A retrospective review was undertaken on records of women who were diagnosed with peripartum cardiomyopathy at Peking Union Medical College Hospital between Jan.1983 and May 1999.Results.During the research period,only 16 pregnant women were documented as peripartum cardiomyopathy.Some of the women undertook ultrasonic cardiographic (UCG) examination that showed decreased systolic function.Seven women were complicated with pregnancy induced hypertension.Three died of disseminated intravascular coagulation,embolism and cardiogenic shock respectively.Conclusion.Early diagnosis of the peripartum cardiomyopathy is extremely important.The UCG can provide helpful information on disease progression or regression.

  5. Small interfering RNA therapy against carbohydrate sulfotransferase 15 inhibits cardiac remodeling in rats with dilated cardiomyopathy.

    Science.gov (United States)

    Watanabe, Kenichi; Arumugam, Somasundaram; Sreedhar, Remya; Thandavarayan, Rajarajan A; Nakamura, Takashi; Nakamura, Masahiko; Harima, Meilei; Yoneyama, Hiroyuki; Suzuki, Kenji

    2015-07-01

    Carbohydrate sulfotransferase 15 (CHST15) is a sulfotransferase responsible for biosynthesis of chondroitin sulfate E (CS-E), which plays important roles in numerous biological events such as biosynthesis of proinflammatory cytokines. However, the effects of CHST15 siRNA in rats with chronic heart failure (CHF) after experimental autoimmune myocarditis (EAM) have not yet been investigated. CHF was elicited in Lewis rats by immunization with cardiac myosin, and after immunization, the rats were divided into two groups and treated with either CHST15 siRNA (2μg/week) or vehicle. Age matched normal rats without immunizations were also included in this study. After 7weeks of treatment, we investigated the effects of CHST15 siRNA on cardiac function, proinflammatory cytokines, and cardiac remodeling in EAM rats. Myocardial functional parameters measured by hemodynamic and echocardiographic studies were significantly improved by CHST15 siRNA treatment in rats with CHF compared with that of vehicle-treated CHF rats. CHST15 siRNA significantly reduced cardiac fibrosis, and hypertrophy and its marker molecules (left ventricular (LV) mRNA expressions of transforming growth factor beta1, collagens I and III, and atrial natriuretic peptide) compared with vehicle-treated CHF rats. CHF-induced increased myocardial mRNA expressions of proinflammatory cytokines [interleukin (IL)-6, IL-1β], monocyte chemoattractant protein-1, and matrix metalloproteinases (MMP-2 and -9), and CHST15 were also suppressed by the treatment with CHST15 siRNA. Western blotting study has confirmed the results obtained from mRNA analysis as CHST15 siRNA treated rats expressed reduced levels of inflammatory and cardiac remodeling marker proteins. Our results demonstrate for the first time, that CHST15 siRNA treatment significantly improved LV function and ameliorated the progression of cardiac remodeling in rats with CHF after EAM.

  6. Prognostic Significance of Frontal QRS-T Angle in Patients with Idiopathic Dilated Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Sheng-Na Li

    2016-01-01

    Conclusions: The frontal QRS-T angle is a powerful predictor of all-cause mortality, cardiac mortality, and worsening heart failure in IDC patients, independent of well-established prognostic factors. Optimized therapy significantly narrows the QRS-T angle, which might be an indicator of medication compliance, but this requires further investigation.

  7. From the risk-stratification of patients with dilated cardiomyopathy to the optimal treatment strategy

    Directory of Open Access Journals (Sweden)

    Frolov A.V.

    2016-03-01

    Conclusions. Application of the original model of risk stratification will allow to optimize the general management in DCM and the strategy of timely selection of potential candidates for implantation of cardioverter- defibrillator for the primary prevention of SCD.

  8. Charcot-Marie-Tooth disease and dilated cardiomyopathy. A rare combination.

    Directory of Open Access Journals (Sweden)

    Rafael Pila Pérez

    2011-07-01

    Full Text Available Se presenta el caso de un paciente de 50 años de edad, con 14 años de evolución de manifestaciones clínicas, destacándose las alteraciones musculoesqueléticas de los cuatro miembros con atrofia de las prominencias tenar e hipotenar y de la musculatura de ambas piernas. Se destacó la presencia de alteraciones sensitivas en miembros inferiores con distribución en calcetín, atrofia, atonía, arreflexia y marcha equina. Desde el punto de vista cardiaco, el paciente presentaba un fibriloaleteo. La radiografía de tórax mostró un aumento marcado del área cardiaca y la ecocardiografía puso de manifiesto una miocardiopatía dilatada. El estudio histopatológico confirmó la presencia de la enfermedad de Charcot-Marie-Tooth asociada a miocardiopatía dilatada. El diagnóstico se basó en las características clínicas, la velocidad de conducción motora, y el estudio histopatológico, que demostró desmielinización con lesiones en “cebolla”, si bien faltaron los estudios genéticos. La enfermedad de Charcot-Marie-Tooth es una enfermedad rara; aproximadamente un 60 % de los pacientes que la padecen, son portadores de una duplicación del cromosoma 17. Por ello, se consideró oportuno transmitir la experiencia de este caso.

  9. Bridge to recovery in two cases of dilated cardiomyopathy after long-term mechanical circulatory support

    OpenAIRE

    Pacholewicz, Jerzy; Zakliczyński, Michał; Kowalik, Violetta; Nadziakiewicz, Paweł; Kowalski, Oskar; Kalarus, Zbigniew; Zembala, Marian

    2014-01-01

    Ventricular assist devices (VADs) have become an established therapeutic option for patients with end-stage heart failure. Achieving the potential for recovery of native heart function using VADs is an established form of treatment in a selected group of patients with HF. We report two cases of VAD patients with different types of pump used for mechanical circulatory support, a continuous flow pump (Heart-Ware®) and a pulsatile pump (POLVAD MEV®), which allow regeneration of the native heart....

  10. T1 mapping in differentiation of diffuse myocardial disease in hypertrophic and dilative cardiomyopathy

    NARCIS (Netherlands)

    Puntmann, V.O.; Pastor, A.; Chen, Z.; Voigt, T.; Karim, R.; Rhode, K.; Razavi, R. S.; Schaeffter, T.; Nagel, E.

    2013-01-01

    T1 mapping was proposed as potentially valuable in quantitative assessment of diffuse myocardial fibrosis. We aimed to determine its role in differentiation of healthy myocardium from diffuse fibrosis in clinical setting. Conclusions: We demonstrate that nativeand post-contrast T1 values and their r

  11. Bridge to recovery in two cases of dilated cardiomyopathy after long-term mechanical circulatory support.

    Science.gov (United States)

    Pacholewicz, Jerzy; Zakliczyński, Michał; Kowalik, Violetta; Nadziakiewicz, Paweł; Kowalski, Oskar; Kalarus, Zbigniew; Zembala, Marian

    2014-06-01

    Ventricular assist devices (VADs) have become an established therapeutic option for patients with end-stage heart failure. Achieving the potential for recovery of native heart function using VADs is an established form of treatment in a selected group of patients with HF. We report two cases of VAD patients with different types of pump used for mechanical circulatory support, a continuous flow pump (Heart-Ware(®)) and a pulsatile pump (POLVAD MEV(®)), which allow regeneration of the native heart. Patients were qualified as INTERMACS level 3-4 for elective implantation of an LVAD. Implantations were performed without complications. The postoperative course was uncomplicated. In the HeartWare patient the follow-up was complicated by episodes of epistaxis and recurrent GIB as well as driveline infection. The follow-up of the POLVAD MEV patient was uneventful. Recurrent GIB forced us to withdraw aspirin and warfarin therapy and maintain only clopidogrel in the HeartWare patient.. In mid-February 2013 the patient was admitted due to dysfunction of the centrifugal pump with a continuous low-flow alarm and increase power consumption. Under close monitoring of the patient a decision was made to stop the pump immediately and evaluate cardiac function. The serial echocardiography studies showed significant improvement in LVEF up to 45% and no significant valvular pathology. In February 2013 LVAD explant was performed by left thoracotomy without complications. At six-month follow-up the patient was in a good clinical condition, in NYHA class I/II, and on pharmacological treatment.

  12. Efficacy of ivabradine in idiopathic dilated cardiomyopathy patients with chronic heart failure

    Directory of Open Access Journals (Sweden)

    Sherief Mansour

    2011-06-01

    Conclusion: Adding ivabradine to optimal medical treatment in HF patients improved symptoms, quality of life, effort tolerance, and echocardiographic parameters, and reduced hospitalization. This beneficial ivabradine effect is probably due to its heart rate–reducing properties.

  13. Ivabradine Improves Heart Rate Variability in Patients with Nonischemic Dilated Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Ertugrul Kurtoglu

    2014-10-01

    Full Text Available Background: Ivabradine is a novel specific heart rate (HR-lowering agent that improves event-free survival in patients with heart failure (HF. Objectives: We aimed to evaluate the effect of ivabradine on time domain indices of heart rate variability (HRV in patients with HF. Methods: Forty-eight patients with compensated HF of nonischemic origin were included. Ivabradine treatment was initiated according to the latest HF guidelines. For HRV analysis, 24-h Holter recording was obtained from each patient before and after 8 weeks of treatment with ivabradine. Results: The mean RR interval, standard deviation of all normal to normal RR intervals (SDNN, the standard deviation of 5-min mean RR intervals (SDANN, the mean of the standard deviation of all normal-to-normal RR intervals for all 5-min segments (SDNN index, the percentage of successive normal RR intervals exceeding 50 ms (pNN50, and the square root of the mean of the squares of the differences between successive normal to normal RR intervals (RMSSD were low at baseline before treatment with ivabradine. After 8 weeks of treatment with ivabradine, the mean HR (83.6 ± 8.0 and 64.6 ± 5.8, p < 0.0001, mean RR interval (713 ± 74 and 943 ± 101 ms, p < 0.0001, SDNN (56.2 ± 15.7 and 87.9 ± 19.4 ms, p < 0.0001, SDANN (49.5 ± 14.7 and 76.4 ± 19.5 ms, p < 0.0001, SDNN index (24.7 ± 8.8 and 38.3 ± 13.1 ms, p < 0.0001, pNN50 (2.4 ± 1.6 and 3.2 ± 2.2 %, p < 0.0001, and RMSSD (13.5 ± 4.6 and 17.8 ± 5.4 ms, p < 0.0001 substantially improved, which sustained during both when awake and while asleep. Conclusion: Our findings suggest that treatment with ivabradine improves HRV in nonischemic patients with HF.

  14. Successful reversal of propionic acidaemia associated cardiomyopathy: evidence for low myocardial coenzyme Q10 status and secondary mitochondrial dysfunction as an underlying pathophysiological mechanism.

    Science.gov (United States)

    Baruteau, J; Hargreaves, I; Krywawych, S; Chalasani, A; Land, J M; Davison, J E; Kwok, M K; Christov, G; Karimova, A; Ashworth, M; Anderson, G; Prunty, H; Rahman, S; Grünewald, S

    2014-07-01

    Dilated cardiomyopathy is a rare complication in propionic acidaemia (PA). Underlying pathophysiological mechanisms are poorly understood. We present a child of Pakistani consanguineous parents, diagnosed with late-onset PA at 18months of age. He presented a mild phenotype, showed no severe further decompensations, normal growth and psychomotor development on a low protein diet and carnitine supplementation. At 15years, a mildly dilated left ventricle was noticed. At 17years he presented after a 2-3month history of lethargy and weight loss with severe decompensated dilated cardiomyopathy. He was stabilised on inotropic support and continuous haemofiltration; a Berlin Heart biventricular assist device was implanted. He received d,l-hydroxybutyrate 200mg/kg/day, riboflavin and thiamine 200mg/day each and coenzyme Q10 (CoQ10). Myocardial biopsy showed endocardial fibrosis, e