Left sided arrhythmogenic ventricular dysplasia in siblings.
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.
De Pasquale
C G
CG
Cardiac Services, Flinders Medical Centre, Flinders Drive, Bedford Park, 5042, South Australia, Australia. carmine.depasquale@fmc.sa.gov.au
Heddle
W F
WF
D000328:Adult; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000235:genetics* / Q000473:pathology; D016757:Death, Sudden, Cardiac / Q000473:pathology; D006801:Humans; D008297:Male; D009679:Nuclear Family; D010375:Pedigree; D018487:Ventricular Dysfunction, Left / Q000235:genetics* / Q000473:pathology