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1/5. Pacing in right ventricular dysplasia after disconnection surgery.

    This report describes a 33-year-old patient with arrhythmogenic right ventricular (RV) dysplasia who had a dual chamber pacemaker implanted at age 23 years for drug-induced bradycardia. Pacing was continued after right ventricular free-wall disconnection (RVFWD) at age 24 years. Her pacemaker was not replaced after battery depletion 7 years later. She presented the following year in severe right-sided heart failure. Her old pacemaker generator was replaced. This was followed by rapid resolution of her clinical failure and return to a full, active, physical lifestyle. This observation suggests the potential benefit of dual chamber pacing in patients with RV dysplasia after RVFWD.
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2/5. Arrhythmogenic right ventricular cardiomyopathy and sudden cardiac death in young Koreans.

    The aim of this study was to assess the frequency and clinical characteristics of arrhythmogenic right ventricular cardiomyopathy (ARVC) in young victims of sudden cardiac death (SCD). From January 1999 to December 2000, postmortem studies were conducted in 38 cases of SCD (age < or =35 (27 /-7) years old, 26 male) from the Taegu-Kyungpook region of southeastern korea. Cases of sudden infant death syndrome were excluded. The causes of SCD were ARVC in 42%, acute myocardial infarction in 11%, myocarditis in 11%, pulmonary embolism in 8%, hypertrophic cardiomyopathy in 5%, aortic rupture in 3%, aortic stenosis in 3%, and unknown in 18%. The mean age of the 16 ARVC victims was 27 /-5 years and 10 were male. None were competitive athletes, or had been suspected of having cardiovascular disease before death. SCD was not related to vigorous physical or competitive activity and occurred during sleep in 7 cases, during work in 4, during bathing in 2, while driving, praying and eating in 1 case each. ARVC is an important cause of SCD in young people in this area of korea.
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3/5. Misdiagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy.

    INTRODUCTION: diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) has major implications for the management of patients and their first-degree relatives. diagnosis is based on a set of criteria proposed by the International Task Force for cardiomyopathies. We report our experience in providing a re-evaluation for patients who previously have been diagnosed with ARVD/C. methods AND RESULTS: We studied 89 patients who requested a re-evaluation for diagnosis of ARVD/C at our center. Each of these patients had been diagnosed with ARVD/C at their initial evaluation. Each patient was re-evaluated with clinical history, physical examination, and noninvasive testing at our center. Invasive testing, which included electrophysiologic testing, right ventricular angiography, and endomyocardial biopsy, was performed when clinically indicated. Sixty (92%) of the 65 patients who had undergone magnetic resonance imaging (MRI) at an outside institution were reported to have an abnormal MRI consistent with ARVD/C. Among these patients, the only abnormality identified was the qualitative finding of intramyocardial fat/wall thinning in 46 patients. On re-evaluation, these qualitative findings were not confirmed. None of these 46 patients ultimately were diagnosed with ARVD/C. Among the entire patient group, only 24 (27%) of the 89 patients met the Task Force criteria for ARVD/C. CONCLUSION: This study demonstrates that the high frequency of "misdiagnosis" of ARVD/C is due to over-reliance on the presence of intramyocardial fat/wall thinning on MRI, incomplete diagnostic testing, and lack of awareness of the Task Force criteria. diagnosis of ARVD/C cannot rely solely upon qualitative features on MRI.
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4/5. Arrhythmogenic right ventricular cardiomyopathy/dysplasia mimicking rape homicide: a case report.

    Arrhythmogenic right ventricular cardiomyopathy (ARVC), also known as arrhythmogenic right ventricular dysplasia, is a known cause of sudden unexplained death in young, otherwise healthy adults. In the forensic setting, several cases of fatal ARVC have been reported, mostly occurring during physical exercise. However, a very few cases present where the death scene is mistaken for a homicide. We report here the case of a 23-year-old woman, found dead, lying in the snow on an isolated property. The woman's genitals were exposed, with her trousers down over her thighs. rape homicide was strongly suspected, but autopsy findings proved otherwise.
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5/5. pathology of arrhythmogenic right ventricular cardiomyopathy/dysplasia--an autopsy study of 20 forensic cases.

    arrhythmogenic right ventricular dysplasia/ cardiomyopathy (ARVC) is characterized histologically by massive infiltration of the right ventricular wall by fat tissue, with surviving strands of cardiomyocytes bordered by or embedded in fibrosis. ARVC has been recognized as a cause of sudden death, especially in the young. The purpose of our autopsy study was to examine the clinical characteristics and the pathological patterns in the hearts of 20 people who died suddenly of ARVC. In view of our findings and the literature, we discussed the possible causes and pathogenesis of ARVC, as well as the mechanisms by which sudden death occurs in this disease. During the 7-year study period, 20 hearts from 9 men and 11 women fulfilled the criteria for ARVC. The mean age was 41 years (range, 17 to 80). The disease was unknown prior to death in all cases. Fourteen persons died at rest, and six on effort. In 9 of the 20 cases, the trigger of sudden death was an acute emotional stress, sometimes associated with a moderate physical activity. The mean heart weight was 380 g (range, 280 to 520). Both ventricles were involved in 40% of the cases. Inflammatory infiltrates consisting of lymphocytes were present in 60% of the cases, but myocyte necrosis was found in only one case. ARVC is more likely to result from a degenerative process than a congenital disorder. Genetic factors, viral or autoimmune inflammation or both, and apoptosis are also involved in the degenerative disorder.
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