Key result
Family screening of a sudden cardiac death victim identified a left-sided form of arrhythmogenic ventricular dysplasia in his asymptomatic 36-year-old brother.
Case Report (n=2)
This case report highlights a rare left-sided variant of arrhythmogenic ventricular dysplasia in siblings and underscores the importance of screening family members of young sudden cardiac death victims.
Supports family screening after young SCD; hypothesis-generating for left-dominant arrhythmogenic phenotypes.
A 32 year old man with no previous medical history suffered a sudden cardiac death. Post mortem examination revealed circumferential fibro-fatty infiltration of the left ventricular myocardium. Histological appearance was characteristic of arrhythmogenic right ventricular dysplasia but unusual for its localisation only to the left ventricle. As a result of this sudden cardiac death the family of the deceased was screened for cardiac disease. A brother of the index case was 36 years old and free of cardiac history and symptoms. Cardiac investigations revealed a functionally and electrically abnormal left ventricle with apparent sparing of the right ventricle. The brothers 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.
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Pasquale et al. (2001) conducted a case report in Left sided arrhythmogenic ventricular dysplasia (n=2). Family screening was evaluated on Detection of cardiac disease. Family screening of a sudden cardiac death victim identified a left-sided form of arrhythmogenic ventricular dysplasia in his asymptomatic 36-year-old brother.
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