Left sided arrhythmogenic ventricular dysplasia in siblings

A 32 year old man with no previous medical history suffered a sudden cardia c death. Post mortem examination revealed circumferential fibro-fatty infil tration of the left ventricular myocardium. Histological appearance was cha racteristic of arrhythmogenic right ventricular dysplasia but unusual for i ts localisation only to the left ventricle. As a result of this sudden card iac death the family of the deceased was screened for cardiac disease. A br other of the index case was 36 years old and free of cardiac history and sy mptoms. Cardiac investigations revealed a functionally and electrically abn ormal left ventricle with apparent sparing of the right ventricle. The brot hers may have a left sided form of arrhythmogenic ventricular dysplasia and illustrate the importance of screening family members of young victims of sudden cardiac death.