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The Korean Journal of Pathology 1997;31(11): 1233-1236.
Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy: Report of an autopsy case.
Tae Yub Kim, Young Min Kim, Jae Gul Chung, Gyung Yub Gong, Su Kil Park, In Chul Lee, Joo Ryung Huh
1Department of Pathology, Asan Medical Center, College of Medicine, University of Ulsan, Seoul 038-040, Korea.
2Department of Nephrology, Asan Medical Center, College of Medicine, University of Ulsan, Seoul 038-040, Korea.
ABSTRACT
A 35-year-old man was admitted with a 20 day history of generalized edema and muscular weakness of the lower extremities. He was alert with a pale puffy face and an ejection murmur was heard at the cardiac apex. The electrocardiogram disclosed low voltage, first degree atrioventricular block, and a right bundle branch block. During the hospitalization an intractable diastolic hypotension developed, which measured 0 mmHg at the lowest point. At that time the echocardiogram revealed a dilated, akinetic right ventricle. Eventually a multiorgan failure developed and an autopsy following his death presented a fibrofatty replacement of the right ventricular myocardium. This might be a case of an arrhythmogenic right ventricular dysplasia/cardiomyopathy, which is usually characterized clinically by a ventricular tachycardia and may cause a sudden death in young adults.
Key Words: Right ventricular dysplasia/cardiomyopathy; Arrhythmogenic dysplasia; Sudden death
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