Cases reported "Endomyocardial Fibrosis"

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1/26. An unusual case of recurrent Loffler endomyocarditis of the aortic valve.

    Idiopathic hypereosinophilic syndrome is a rare systemic disease with an unexplained elevated eosinophil count. Loffler endomyocarditis is hypereosinophilic syndrome with endocardial fibrosis and restrictive cardiomyopathy. The atrioventricular valves are frequently involved, causing valvular regurgitation. Previously, there has been one case report of combined aortic and mitral valve involvement with Loffler endomyocarditis that was treated with bivalvular replacement. We describe a previously healthy 50-year-old man diagnosed with Loffler endomyocarditis complicated by peripheral thromboembolism and severe aortic regurgitation due to valve fibrosis and fibrotic vegetation on the aortic valve. He underwent embolectomy and aortic valve replacement in addition to treatment for his hypereosinophilia. He later presented with cardiomyopathy with severe aortic insufficiency due to the destruction of the aortic valve prosthesis by sterile fibrinous vegetation. To our knowledge, this is the second case in the literature in which Loffler endomyocarditis involves the aortic valve and the first patient in whom only the aortic valve is involved.
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ranking = 1
keywords = cardiomyopathy
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2/26. Functionally atrialized parchment-like right ventricle with extensive myocardial fibrosis of left ventricle.

    A 18-year-old female with unusual type of parchment-like right ventricle died of intractable congestive heart failure was reported. A catheter study revealed the absence of systolic contraction of the right ventricle. The pressure tracing curve in the right ventricle was virtually the same as in the right atrium. At autopsy, there was an extensive myocardial fibrosis of the left ventricle in addition to the almost total absence of the myocardium of the right ventricle. The case was considered to be a unique type of idiopathic cardiomyopathy.
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ranking = 0.5
keywords = cardiomyopathy
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3/26. Tropical endomyocardial fibrosis (Davies' disease): case report demonstrating the role of magnetic resonance imaging.

    Tropical endomyocardial fibrosis (TEMF), a restrictive cardiomyopathy of unclear etiology, is an endemic disease in equatorial africa, south america and india. The patients are usually young, the onset of the disease and its clinical manifestations insidious, and the prognosis poor. We currently present a 50-year-old Congolese female who was referred with symptoms of progressive right-sided heart failure due to isolated TEMF of the right ventricle. Surgical resection of regional endomyocardial fibrosis was not possible and our patient was referred for cardiac transplantation. Cardiac magnetic resonance imaging (CMR) demonstrated the primary and secondary structural and functional abnormalities. CMR seems ideally suited to diagnose this condition and monitor response to medical and/or surgical therapy.
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ranking = 0.5
keywords = cardiomyopathy
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4/26. Typical clinical aspect of endomyocardial fibrosis.

    endomyocardial fibrosis is a restrictive cardiomyopathy, of unknown etiology, which occurs most commonly in tropical and subtropical areas. Typical clinical aspect of the disease shows striking ascites, regardless of which ventricle is involved, contrasting with little or no peripheral edema.
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ranking = 0.5
keywords = cardiomyopathy
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5/26. Cardiomyopathy in Becker muscular dystrophy--does regional fibrosis mimic infarction?

    We present a case of a 39-year-old man with Becker muscular dystrophy and severe congestive cardiac failure. Cardiac magnetic resonance imaging revealed subendocardial late gadolinium enhancement, similar to that seen in myocardial infarction. He had no risk factors for atherosclerotic coronary artery disease and coronary angiography was normal. We propose that regional subendocardial myocardial fibrosis which has been described histologically in the cardiomyopathy associated with Becker muscular dystrophy may resemble previous infarction at contrast enhanced cardiac magnetic resonance imaging.
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ranking = 0.5
keywords = cardiomyopathy
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6/26. Dilated cardiomyopathy masquerading as long qt syndrome.

    atrioventricular block has been described in association with cases of long qt syndrome and mortality is increased in this subgroup. We describe an infant with congenital QT prolongation and atrioventricular block with normal cardiac function, leading to the initial diagnosis of long qt syndrome. She subsequently developed dilated cardiomyopathy requiring cardiac transplantation. We postulate that the presenting electrocardiograph abnormalities were early manifestations of the myocardial disease, preceding the development of myocardial dysfunction by several months. The need for heightened surveillance in cases of QT prolongation with atrioventricular block is amplified by the possibility of an evolving cardiomyopathy.
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ranking = 3
keywords = cardiomyopathy
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7/26. An autopsy case of cardiomyopathy with restrictive physiology in a child.

    An autopsy case of cardiomyopathy with restrictive physiology associated with subendocardial myocardial necrosis and fibrosis in a seven-year-old child is described. cardiac catheterization showed high left ventricular end-diastolic pressure with a dip-and-plateau pattern. Macroscopically, marked dilatation with fibroelastosis in both atria and mild dilatation in both ventricles were observed. Histologic examination revealed extensive subendocardial necrosis with marked disorganization of myocardial cells and moderate arteriolosclerosis. We speculate that these extensive myocardial lesions, which contributed to the restrictive hemodynamic changes, could have been induced by anoxia or a Ca2 transport abnormality of unknown cause.
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ranking = 2.5
keywords = cardiomyopathy
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8/26. Long-term follow-up of a child with idiopathic restrictive cardiomyopathy.

    A ten-year-old male with palpebral edema was proved to have a restrictive hemodynamic pattern by cardiac catheterization. Sequential echocardiographic examination revealed uniformly depressed left ventricular diastolic properties, well-maintained left ventricular systolic function, and a progression of obliterative change of the left ventricular cavity. Myocardial biopsy at 17 years of age disclosed a normal endocardium and marked interstitial fibrosis of the myocardium. Consequently, a decrease of cardiac output and a marked biatrial enlargement have developed in association with persistent atrial fibrillation.
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ranking = 2
keywords = cardiomyopathy
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9/26. Fatal hypertrophic cardiomyopathy in an infant of a diabetic mother.

    The case is reported of a male infant with hypertrophic cardiomyopathy (HCM), attributed to maternal diabetes, who, contrary to the usually good prognosis of this condition, died at 11 weeks of age. The clinical, echocardiographic, and pathological findings were indistinguishable from familial HCM, except for the lesser degree of myocardial fiber disarray on microscopic examination.
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ranking = 2.5
keywords = cardiomyopathy
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10/26. Histological evidence of left ventricular involvement in arrhythmogenic right ventricular dysplasia.

    A 40-year-old female with arrhythmogenic right ventricular dysplasia (ARVD) demonstrated a reduced motion of the left ventricular (LV) apex. Specimens of LV free wall, obtained by endomyocardial biopsy, histologically revealed prominent interstitial fibrosis with sparse distribution of myocytes. The myocytes were hypertrophic and disrupted with loss of myofibrils. This is a case of ARVD, where LV involvement was histologically verified.
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ranking = 1128.7996780332
keywords = ventricular dysplasia, right ventricular dysplasia, arrhythmogenic right ventricular dysplasia, arrhythmogenic, arrhythmogenic right, dysplasia
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