Isolated fibromuscular dysplasia of the coronary ostium: a rare cause of sudden death. Case report and review of the literature

We present a unique case of sudden death in a 21-year-old man with history of cocaine use and a solitary fibromuscular dysplastic lesion completely occluding the left coronary artery ostium. We document intimal proliferation of myofibroblasts at the opening of the left coronary ostium without other concomitant lesions. This report discusses the gross and histologic features of the lesion, explores in careful detail the possible etiologies, and gives a comprehensive literature review of isolated coronary ostial fibromuscular dysplasia presenting with sudden death.