Arrhythmogenic right ventricular dysplasia as a cause of sudden death

The American Journal of Forensic Medicine and Pathology
J D SchiønningI B Kristensen

Abstract

Arrhythmogenic right ventricular dysplasia (ARVD) is a poorly understood and often underdiagnosed disorder of the right ventricle, characterized by replacement of myocardium by fibroadipose tissue, arrhythmic manifestations, and sudden death. The disease occurs in families and is inherited as an autosomal dominant trait. This report describes five cases of ARVD identified by autopsy. In three of the cases, sudden death occurred in the young (16-28 years old) during or shortly after exercise. In another case, a 46-year-old man with no relevant medical history was found dead in his bathroom. In the last case, a 57-year-old woman died from pulmonary thromboembolism. In none of the subjects had the disease been diagnosed or suspected before death. Only one (a 21-year-old man) had previous typical symptoms of the disease. Autopsy examination showed right ventricle dilation and, in four cases, cardiomegaly. The right ventricular myocardium of all hearts was almost replaced by adipose tissue and to a variable degree by fibrous tissue, while the left ventricle myocardium demonstrated no, or only scattered, fibro-fatty infiltration. Postmortem diagnosis of ARVD can be important in identifying possible affected family members in order to...Continue Reading

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Citations

Jul 11, 2001·The American Journal of Forensic Medicine and Pathology·E N MichalodimitrakisI Stiakakis
Aug 28, 2007·The American Journal of Forensic Medicine and Pathology·Osman CelbisBora Ozdemir
Jun 5, 2007·Forensic Science International : Synergy·Lisa WingenfeldThomas Bajanowski
Mar 20, 2004·Journal of Cardiovascular Electrophysiology·Chandra BommaHugh Calkins
Aug 2, 2014·Journal of Cardiovascular Electrophysiology·Lingmin WuShu Zhang
Dec 5, 2006·European Journal of Cardiovascular Prevention and Rehabilitation : Official Journal of the European Society of Cardiology, Working Groups on Epidemiology & Prevention and Cardiac Rehabilitation and Exercise Physiology·Karin BilleErik J Meijboom
Jun 14, 2013·Clinical Medicine Insights. Cardiology·Jorge RomeroRichard Lucariello
Aug 26, 1998·Medicine, Science, and the Law·P Lunetta, A Penttilä
Jun 9, 2006·Europace : European Pacing, Arrhythmias, and Cardiac Electrophysiology : Journal of the Working Groups on Cardiac Pacing, Arrhythmias, and Cardiac Cellular Electrophysiology of the European Society of Cardiology·Elzbieta Katarzyna WlodarskaPiotr Hoffman

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Arrhythmogenic Right Ventricular Dysplasia

Arrhythmogenic right ventricular dysplasia is a congenital cardiomyopathy that is characterized by infiltration of adipose and fibrous tissue into the right ventricle wall and loss of myocardial cells. Primary injuries usually are at the free wall of the right ventricular and right atria resulting in ventricular and supraventricular arrhythmias. Discover the latest research on arrhythmogenic right ventricular dysplasia here.