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

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.