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Occlusion of the right coronary artery (RCA) ostium by an aortic cusp is a rare anomaly and can be a cause of sudden death. We report the case of a child with progressive stenosis of the right coronary ostium caused by a hypoplastic right coronary cusp that adhered to the aortic wall resulting in severe myocardial ischemia. The patient underwent a Ross-Konno operation with mitral valvuloplasty for congenital aortic valvular stenosis and mitral regurgitation caused by ischemia-induced posteromedial papillary muscle dysfunction. The myocardial ischemia was relieved by resection of the deformed pouch-like cusp to disclose the RCA ostium. Postoperative myocardial scintigraphy demonstrated no myocardial ischemia and multidetector computed tomography showed no coronary ostial stenosis.

Occlusion of the right coronary artery ostium by an aortic cusp attachment