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1/33. A patient with hypertrophic cardiomyopathy accompanied by right ventricular dilation of unknown cause.

    Hypertrophic cardiomyopathy (HCM) is a disease characterized by an unknown cause of hypertrophy in the left or right ventricle. The dilated phase of HCM shows disease conditions resembling dilated cardiomyopathy, such as ventricular dilation, thin ventricular wall, and reduction of the ejection fraction. A patient presented with left ventricular concentric hypertrophy accompanied by right ventricular dilatation of unknown cause. Right ventricular endomyocardial biopsy specimens showed characteristic myocardial disarray. Therefore, there is the possibility that the patient had right and left ventricular HCM in the process toward the dilated phase, in which dilatation first occurred in the right ventricle.
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ranking = 1
keywords = cardiomyopathy
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2/33. heart transplantation after successful donor postpartum pulmonary embolectomy.

    A fulminant pulmonary embolism can be treated surgically if thrombolytic therapy is contraindicated. A 31-year-old woman developed a fulminant pulmonary embolism after right-sided deep venous thrombosis 1 day after undergoing a cesarean section. A pulmonary embolectomy with cardiopulmonary bypass was performed, but the patient was brain-dead. After 2 days of echocardiographic observation, her heart was explanted for a 61-year-old man with ischemic cardiomyopathy. His right heart data were unremarkable, and he remains well 16 months after transplantation. Despite the sudden strain on the right ventricle that occurs with a pulmonary embolism, such a heart may be transplanted successfully after a pulmonary embolectomy.
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ranking = 0.16666666666667
keywords = cardiomyopathy
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3/33. Ventricular tachycardia in an adolescent with arrhythmogenic right ventricular dysplasia.

    We report the case of an adolescent boy with exertional syncope and ventricular tachycardia caused by arrhythmogenic right ventricular dysplasia. diagnosis was determined by transthoracic echocardiography and definitive management with an automatic internal cardiac defibrillator. Emergency physicians must be aware of this serious but treatable cause of adolescent exertional syncope.
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ranking = 4374.0228809784
keywords = ventricular dysplasia, right ventricular dysplasia, arrhythmogenic, arrhythmogenic right, arrhythmogenic right ventricular dysplasia, dysplasia
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4/33. A case of arrhythmogenic right ventricular cardiomyopathy in sinus rhythm associated with thrombus in the right atrium.

    We describe a patient with arrhythmogenic right ventricular cardiomyopathy (ARCV) in sinus rhythm associated with thrombus in the right atrium. The occurrence of a right heart thrombus in ARCV is extremely rare and, to our knowledge, has been previously reported only in the right ventricle. In our case, ARCV most probably led to right atrial spontaneous echo contrast, and later, right atrial thrombus formation by blood stasis caused by right ventricular systolic dysfunction. In conclusion, our case suggests that right atrial thrombus may occur in ARCV, even in sinus rhythm.
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ranking = 2442.3779255109
keywords = arrhythmogenic right ventricular cardiomyopathy, right ventricular cardiomyopathy, arrhythmogenic, arrhythmogenic right, ventricular cardiomyopathy, cardiomyopathy
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5/33. The impact of cocaine on the donor heart: a case report.

    With a limited supply of donor hearts in the united states and a prevalent history of cocaine abuse among potential heart donors, the question of transplanting the hearts of cocaine users presents a dilemma to the surgeon. We report a patient who died of the acute right ventricular failure of a heart from a donor with a history of binge drinking and cocaine abuse and who had sustained traumatic brain death. The donor's serum was positive for cocaine prior to transplantation, and autopsy findings were consistent with cocaine cardiomyopathy. This case illustrates the importance of accurate donor history and toxicologic screen prior to heart transplantation and suggests that hearts of cocaine users should not be transplanted, especially in a setting of traumatic brain death.
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ranking = 0.16666666666667
keywords = cardiomyopathy
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6/33. A case with Uhl's anomaly presenting with severe right heart failure.

    Uhl's anomaly was first reported by Uhl in 1952 and is characterized by congenital partial or complete absence of right ventricular myocardium. It is a very rare anomaly with unknown aetiology. Associations with other congenital heart diseases, familial occurrency, sudden death and arrhythmia with Uhl's anomaly have been reported. Pathologic findings vary with the patient's age and severity of the right ventricular disorder. In infancy, it may occur with severe right-sided heart failure as well as asymptomatic cardiomegaly. Despite its rarity, Uhl's anomaly may be considered in patients with right ventricular failure due to dilated cardiomyopathy of the right ventricle. We report the case of six-year-old boy presenting with striking ascites due to severe right heart failure of Uhl's anomaly.
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ranking = 0.16666666666667
keywords = cardiomyopathy
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7/33. Right ventricular dysplasia in an asymptomatic young man: an uncommon case with biventricular involvement and no known family history.

    A 33-year-old man had cardiomegaly on a routine x-ray examination. He was asymptomatic with no history of infarction, syncope, or palpitations. There was no family history of congenital heart disease or sudden death. Two-dimensional transthoracic echocardiography demonstrated marked enlargement of the right atrium and ventricle with severely depressed right and left ventricular function that was consistent with right ventricular dysplasia. The patient was treated with an angiotensin-converting enzyme inhibitor and did well for 6 months, but then developed symptomatic left-sided congestive heart failure. Short-term improvement was obtained with intravenous inotropic therapy, but he continued to have progressive symptoms of heart failure. Approximately 7 months after his initial presentation, the patient underwent orthotopic heart transplantation for intractable congestive heart failure. Pathologic examination of the explanted heart established the diagnosis of right ventricular dysplasia with left ventricular involvement. This is an uncommon presentation of right ventricular dysplasia with biventricular involvement and no known family history.
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ranking = 3501.370549365
keywords = ventricular dysplasia, right ventricular dysplasia, dysplasia
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8/33. Long-term follow-up of right ventricular monomorphic extrasystoles.

    OBJECTIVES: The purpose of this study was to verify in a long-term follow-up whether frequent monomorphic right ventricle extrasystoles may progress to arrhythmogenic right ventricular dysplasia (ARVD). BACKGROUND: Frequent monomorphic right ventricle extrasystoles are generally considered benign. However, in patients with this pattern, cardiac magnetic resonance (MR) has recently shown anatomical and functional abnormalities of the right ventricle. methods: Sixty-one patients who had been classified by noninvasive examinations as having frequent idiopathic right ventricle ectopy were contacted after 15 /- 2 years (12 to 20) and submitted to clinical examination, electrocardiogram (ECG), Holter monitoring, stress test, signal averaged ECG, echocardiography and, in 11 patients, cardiac MR. The primary end point was to ascertain the presence of cases of sudden death or progression to ARVD. RESULTS: At the end of the follow-up, 55 patients were alive; six died, none of sudden death; eight stated to be well but refused further examinations. The 47 patients examined had normal ECG; in 24 patients (51%), extrasystoles were no longer present at Holter monitoring; late potentials were present in up to 15% of the patients; the right ventricle was normal at echocardiography. In 8 of 11 patients (73%), cardiac MR showed focal fatty replacement and other abnormalities of the right ventricle. CONCLUSIONS: In this long-term follow-up study, no patient died of sudden death nor developed ARVD; two-thirds of the patients were asymptomatic, and, in half of the patients, ectopy had disappeared. Focal fatty replacement in the right ventricle was present in most.
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ranking = 874.80457619568
keywords = ventricular dysplasia, right ventricular dysplasia, arrhythmogenic, arrhythmogenic right, arrhythmogenic right ventricular dysplasia, dysplasia
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9/33. Noncompaction of the right ventricle following Senning repair.

    A three-year-old boy presented with generalized edema, respiratory distress, prominent right ventricular impulse and hepatosplenomegaly. He had undergone Senning repair at one year of age. On his echocardiography, there were numerous prominent trabeculations and deep intertrabecular recesses measuring approximately 5 mm in depth along the free wall and right ventricular apex. Echogenicity of the endocardial surface was increased suggesting a fibrotic process in progress. Intertrabecular recesses were observed to be filling from the ventricle by color Doppler which is consistent with noncompaction. Various semilunar valve obstructions were shown to be responsible for the persistence of deep endomyocardial spaces surrounded by exeggerated hypertrophy of the trabeculae. This report presents the echocardiographic findings of right ventricular cardiomyopathy associated with dextroposition of the great arteries following Senning operation resembling noncompaction. Thus, this rare entity needs to be clarified regarding morphological criteria in distinction from other cardiomyopathies.
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ranking = 156.27867943688
keywords = right ventricular cardiomyopathy, ventricular cardiomyopathy, cardiomyopathy
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10/33. Right ventricular cardiomyopathy accompanied by protein-losing enteropathy and chylous effusion.

    Severe right-side heart failure developed in a 47-year-old Japanese woman who suffered from hypoalbuminemia and a massive right side chylous pleural effusion. She had been diagnosed as having protein-losing enteropathy with right ventricular cardiomyopathy. autopsy showed congenital anomalies of the lymph ducts and abnormal deposition of fibrous and fatty tissue in the right ventricular myocardium. The clinical and pathological findings are consistent with the nonarrythmogenic form of the arrythmogenic right ventricular dysplasia.
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ranking = 1063.9671923192
keywords = ventricular dysplasia, right ventricular dysplasia, right ventricular cardiomyopathy, ventricular cardiomyopathy, cardiomyopathy, dysplasia
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