Sudden Death Due to Coronary Tree Hypoplasia

A 20-year-old woman complained for several weeks before her death of unspecific epigastric pains. Such episodes were labeled as "dyspeptic episode.'' A month later, once again she started to complain about stomach pains, then she collapsed unconscious. She was rushed to a hospital, where she was admitted in cardiorespiratory arrest. An electrocardiogram revealed a ventricular fibrillation. All resuscitation efforts were unsuccessful. The autopsy revealed nonspecific general changes (subpleural petechiae and blood pooling). Dissection of the coronary arteries revealed a normal topography of coronary arteries but a marked hypoplasia of all the subepicardial coronary arteries (maximum diameter of left anterior descending coronary artery = 1.9 mm, maximum diameter of right coronary artery = 1.43 mm). Furthermore, the left anterior descending coronary artery was slightly compressed by a fibrous bridge at about 1.5 cm of its origin; a fibrinoleukocytic thrombus has formed immediately above this bottleneck, obstructing the entire lumen of the artery. Microscopic examination revealed an area of recent myocardial necrosis; diffuse subendocardial fibrosis, with extension into the subjacent myocardium, disorganizing the myocardial fibers; and variable thickening of the vascular walls with periarteriolar fibrosis. Weigert stain has shown marked intimal hyperplasia, either focal or diffuse, in different segments of vascular wall, accompanied by disruption of the internal elastic lamina due to subintimal cellular proliferation, in the medium branches of coronary arteries. The micrometric measurements showed that intimal thickening was at least the size of the media (similar to 358 vs 356.8 mu m) and sometimes double.