Abstract

Hypoplastic coronary artery disease (HCAD) is a rare abnormality with a high rate of sudden death and poor outcome. HCAD was revealed by myocardial infarction in a teenager with objective evidence of silent ischaemia on myocardial scintigraphy. After four years of follow up, he suddenly collapsed during exercise and subsequently died. Although HCAD is very uncommon, it should be actively excluded in children and young adults who experience sudden cardiac death. Aggressive treatment such as implantable cardioverter-defibrillator or heart transplantation may be indicated for this rare coronary abnormality.