Burke A P, Virmani R
Department of Cardiovascular Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA.
Hum Pathol. 1998 Oct;29(10):1124-7. doi: 10.1016/s0046-8177(98)90424-5.
We report four cases of sudden unexpected death in three males and one female aged 12 to 31 years. Death occurred during exercise in three of four cases, and there was no history of sudden death or previous cardiac history in any patient. At autopsy, there was marked intramural coronary artery dysplasia of the ventricular septum, accompanied in three of the four cases by myocardial fibrosis. The arterial dysplasia was characterized by severe medial thickening with smooth muscle cell disorganization and marked luminal narrowing. There was no evidence of myofiber disarray or asymmetric septal hypertrophy to suggest hypertrophic cardiomyopathy. Other than an ostium secundum type atrial septal defect in one case, there were no associated cardiac or extracardiac lesions found at complete autopsy of these individuals. We conclude that small vessel disease of intramural coronary arteries of the ventricular septum may be an isolated finding leading to sudden cardiac death in young adults.
我们报告了4例突然意外死亡病例，患者为3名男性和1名女性，年龄在12至31岁之间。4例中有3例死亡发生在运动期间，所有患者均无猝死史或既往心脏病史。尸检时，室间隔壁内冠状动脉存在明显发育异常，4例中有3例伴有心肌纤维化。动脉发育异常的特征为中膜严重增厚，平滑肌细胞排列紊乱，管腔明显狭窄。没有证据表明存在肌纤维紊乱或不对称性室间隔肥厚以提示肥厚型心肌病。除1例继发孔型房间隔缺损外，这些个体的完整尸检未发现相关的心脏或心脏外病变。我们得出结论，室间隔壁内冠状动脉的小血管疾病可能是导致年轻人心脏性猝死的一个独立发现。
