Cases reported "Endomyocardial Fibrosis"

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1/8. Porcelain heart: a case of massive myocardial calcification.

    Reports of massive myocardial calcification are limited mainly to case reports and this type intracardiac calcifications have been usually identified postmortem. We present a very interesting case of massive calcification of the left ventricular myocardium, interventricular and interatrial septae causing restrictive physiology and coronary artery obstruction in a 46-year-old Turkish woman. The diagnosis of myocardial calcification was suspected from chest X-ray and confirmed with computerized tomography and cardiac catheterization. The findings of the chest computed tomography were very similar to those previously reported cases of endomyocardial fibrosis (EMF) with massive calcification of the left ventricle.
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2/8. 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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3/8. 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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4/8. Premature opening of the pulmonary valve in endomyocardial fibrosis.

    Two-dimensional and Doppler echocardiographic findings in a 67-year-old man with endomyocardial fibrosis (EMF) are described. The two-dimensional echocardiogram showed typical features of EMF, right ventricular endomyocardial calcification, a thickened right ventricular wall, obliteration of the apex of the right ventricle and marked dilatation of the right atrium. In addition, premature opening of the pulmonary valve was observed during late diastole. The Doppler echocardiogram revealed forward flow from the right ventricle to the pulmonary artery, indicating the conduit state of the right ventricle. These findings were supported by cardiac catheterization and autopsy. Thus, two-dimensional and Doppler echocardiography are useful not only in making the diagnosis, but also in understanding the hemodynamic condition in EMF.
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5/8. 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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6/8. Right ventricular dysfunction after endocardiectomy for right ventricular endomyocardial fibrosis.

    Early right ventricular failure following a large endocardiectomy in right endomyocardial fibrosis (EMF) is reported. This diagnosis was confirmed upon echocardiography by the presence of a dilated right ventricle, and hemodynamically (upon postoperative control catheterization) by the presence of right ventricular diastolic dysfunction. The role of the endocardiectomy and of the pericardium in the genesis of this ventricular dysfunction are discussed in the light of this case which was documented by a hemodynamic study and surgically confirmed.
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7/8. endomyocardial fibrosis. Preoperative diagnosis and surgical therapy.

    The case of a Nigerian student with biventricular endomyocardial fibrosis is presented. diagnosis was suggested by cardiac catheterization and histologically confirmed by a percutaneous endomyocardial biopsy. Successful surgical repair including mitral valve replacement, tricuspid valve reconstruction, and left ventricular endomyocardial resection was performed through a biatrial approach. The pathologic and surgical considerations are reviewed. This is one of the few cases of endomyocardial fibrosis reported from the united states and the first in which a percutaneous endomyocardial biopsy was used to provide a definitive preoperative histologic diagnosis.
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8/8. methysergide-induced heart disease: a case of multivalvular and myocardial fibrosis.

    methysergide (Sansert) is known to cause mitral and aortic valvular fibrosis and dysfunction, but has generally not been known to damage right heart valves or the myocardium, and cardiac fibrosis has not been considered to be a risk if therapy is intermittently interrupted. The woman who is the subject of this case report developed catheterization-proven severe tricuspid and moderate aortic and mitral regurgitation during noncontinuous therapy with methysergide. In addition, right ventricular endomyocardial biopsy revealed extensive endocardial and intramyocardial fibrosis.
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