Cases reported "Endomyocardial Fibrosis"

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11/26. Progressive dilated cardiomyopathy in a patient with longstanding and complete prednisone-induced hematological remission of idiopathic hypereosinophilic syndrome.

    A female patient is described in whom the diagnosis of idiopathic hypereosinophilic syndrome (HES) with heart disease and peripheral neuropathy was made at the age of 32 years. Although prednisone induced a prompt and longstanding complete hematological remission, progressive and eventually intractable heart failure developed, and the patient died 6 years later. Endomyocardial biopsy at diagnosis showed infiltration with intact and disintegrated eosinophils and Charcot-Leyden crystals. Echocardiographic follow-up (including Doppler-echocardiography) revealed mitral regurgitation with thickening and impaired motility of the posterior mitral leaflet, as well as progressive dilated cardiomyopathy. At autopsy, a diffuse interstitial fibrosis with patchy prominence in an eccentric hypertrophic and highly dilated heart was found. There were no significant endocardial thickening and no mural thrombi. In contrast to the findings of the initial endomyocardial biopsy, autopsy revealed no eosinophilic infiltrate. In this case, eosinophil-induced heart disease manifested as dilated cardiomyopathy, without endocardial fibrosis as originally described by Loffler. We speculate, that eosinophils have been deposited predominantly in the myocard at an early stage of disease, and - activated locally - secreted their granule proteins producing an initial damage to capillary endothelial cells and myocytes. After prednisone-induced clearance of eosinophils from blood and tissues, progressive, self-perpetuating interstitial fibrosis of the myocard and loss of myocytes eventually resulted in end-stage dilated cardiomyopathy.
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ranking = 1
keywords = cardiomyopathy
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12/26. Three autopsy cases of progression to left ventricular dilatation in patients with hypertrophic cardiomyopathy.

    The hearts of three cases of congestive heart failure with dilated left ventricles developing in patients with symptomatic hypertrophic cardiomyopathy (HCM) were morphologically investigated. The results showed that disproportionate hypertrophy and dilatation of the left ventricles, accompanied by massive fibrosis and myocardial disarray, were present in the three patients. The mean percent area of fibrosis of the left ventricle was 34.7% and 47.4% at the upper third and lower third levels, respectively, and was much more frequently associated with disarray (84.4 /- 12.3%). Moreover, the fibrosis was most extensive in the lateral wall of the left ventricle, followed by the posterior, anterior, and interventricular walls. The fibrosis was also diffuse regardless of the subendocardial or subepicardial region of the heart. The findings in the present study suggest that the disarray in this particular series of HCM might be responsible for the mechanism of the fibrosis leading to dilatation of the left ventricle.
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ranking = 0.71428571428571
keywords = cardiomyopathy
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13/26. Aortic and mitral valve replacement in idiopathic hypereosinophilic syndrome.

    Idiopathic hypereosinophilic syndrome is a rare systemic manifestation of eosinophilia that may cause endocardial fibrosis. Endocardial fibrosis and overlying thrombus typically lead to a restrictive cardiomyopathy and heart failure. Occasionally mitral or tricuspid regurgitation (or both) occurs as a result of the fibrosis and thrombus formation. This report describes a patient with hypereosinophilic syndrome who was seen with an unusually localized form of cardiac involvement that resulted in predominantly aortic and mitral stenosis. This was treated with bivalvular replacement.
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ranking = 0.14285714285714
keywords = cardiomyopathy
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14/26. Morphological features of hypertrophic cardiomyopathy with congestive heart failure and a small left ventricular cavity.

    To clarify the morphologic basis of marked congestive heart failure in cases of hypertrophic cardiomyopathy with a small left ventricular cavity, the size of myocytes, the extent of myocardial fiber disarray and fibrosis were quantitatively evaluated in two autopsied cases. Compared with the control hypertrophic cardiomyopathy, both cases had larger myocytes and less fibrosis, and one of them had markedly extensive myocardial fiber disarray. Probably, the hypertrophy of the myocytes or myocardial fiber disarray or both rather than myocardial fibrosis are important in the pathogenesis of severe congestive heart failure in these cases.
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ranking = 0.85714285714286
keywords = cardiomyopathy
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15/26. Evolution of a hereditary cardiac conduction and muscle disorder: a study involving a family with six generations affected.

    This study describes six generations of a family with autosomal dominant cardiac conduction system and myocardial disease with recognizable clinical stages. A 20 year follow-up of nine family members, a medical questionnaire of 196, electrocardiographic screening of 91, noninvasive testing of 20, and catheterization with endomyocardial biopsy of six are the basis of this report. The clinical stages are as follows: Stage I occurs in the second and third decades of life and is characterized by an absence of symptoms, normal heart size, sinus bradycardia, and premature atrial contractions. Stage II is marked by first-degree atrioventricular block in the third and fourth decades. Stage III occurs in the fourth and fifth decades and is accompanied by chest pain, fatigue, lightheadedness, and advanced atrioventricular block followed by the development of atrial fibrillation or flutter. Stage IV, in the fifth and sixth decades of life, is characterized by congestive heart failure and recurrent ventricular arrhythmias. light microscopy of right ventricular endomyocardial biopsy specimens from patients in stage II revealed very mild fibrosis; electron microscopy of the specimens demonstrated mild dilatation of tubules, mitochondrial swelling, and minimal myofibrillar loss. biopsy specimens from patients with stage III disease were similar to those from patients with stage II disease except for an increase of myofibrillar loss. The stage IV specimens had diffuse fibrosis and more severe tubular dilatation, mitochondrial cristolysis, and myofibrillar loss. At autopsy in the proband, the atrial changes were more severe than the ventricular and were especially marked in the sinoatrial and atrial myocardium. Early recognition of the disease and use of pacemakers and antiarrhythmic agents have proved beneficial for affected family members. Thorough family studies of patients with conduction system disease and/or dilated cardiomyopathy are necessary to better understand the hereditary basis and natural course of this category of disease.
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ranking = 0.14285714285714
keywords = cardiomyopathy
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16/26. Eosinophilic endomyocardiopathy.

    A young patient with a severely elevated eosinophil count died from manifestations of severe eosinophilic endomyocardiopathy. Echocardiographic and hemodynamic evaluation were diagnostic of a rapidly progressive restrictive cardiomyopathy. Progression of cardiac disease in the face of medical treatment is an ominous sign and may argue for earlier surgical intervention in this syndrome.
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ranking = 0.14285714285714
keywords = cardiomyopathy
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17/26. Histologic abnormalities of the left ventricle in a patient with arrhythmogenic right ventricular dysplasia.

    Right ventricular histologic abnormalities have been described in association with arrhythmogenic right ventricular dysplasia, but the cause of the disease is unknown. Identical abnormalities were noted in endomyocardial biopsy specimens from the right and left ventricles of a 20-year-old male patient with the clinical syndrome of arrhythmogenic right ventricular dysplasia. Left ventricular function was normal at rest and during bicycle exercise. Eighteen months previously, he had shown clinical and serologic evidence of mycoplasmal myocarditis. arrhythmogenic right ventricular dysplasia may represent predominant involvement of the right ventricle in a generalized cardiomyopathic process, possibly as a consequence of healed myocarditis.
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ranking = 427.80096159204
keywords = ventricular dysplasia, right ventricular dysplasia, arrhythmogenic right ventricular dysplasia, arrhythmogenic, arrhythmogenic right, dysplasia
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18/26. Constrictive endocarditis. Report of a case with successful surgery.

    An 18-year old white youth presented with severe right heart failure and was found to have an obliterated and funnel-shaped right ventricle, massive tricuspid regurgitation, and mitral regurgitation. The haemodynamic findings were those of cardiomyopathy with obliteration on the right side. He underwent surgery consisting of decortication and peeling off of a thickened right ventricular endocardium, tricuspid valve replacement, and mitral valve repair. After surgery there was pronounced haemodynamic as well as clinical improvement. The pathological picture was that of constrictive endocarditis. We recommend this method of treatment for patients with obliterate cardiomyopathy on either side of the heart.
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ranking = 0.28571428571429
keywords = cardiomyopathy
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19/26. Congestive cardiomyopathy with segmental wall motion abnormalities and a non-uniform pattern of fibrosis.

    A case of congestive cardiomyopathy in a 46-year-old smoker with single-vessel right coronary artery disease is presented. Segmental contraction abnormalities were present in the anterior wall. Pathologic analysis showed diffuse fibrosis consistent with a cardiomyopathy in areas of abnormal contraction and normal muscle in areas of normal contractility. Segmental wall motion abnormalities have been reported but none with microscopic studies of areas of both abnormal and normal contractility.
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ranking = 0.85714285714286
keywords = cardiomyopathy
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20/26. endomyocardial fibrosis with calcification of the myocardium.

    A case of endomyocardial fibrosis with intramyocardial calcification was characterized by symptoms of restrictive cardiomyopathy resembling constrictive pericarditis. Pathological findings were typical of those found in classical Davies' disease, originally encountered and described in uganda.
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ranking = 0.14285714285714
keywords = cardiomyopathy
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