Chronic Severe Aortic Regurgitation in a Child Due to Congenital Hypoplastic Right Coronary Cusp: A Report of a Rare Case

Congenital hypoplasia of the aortic valve cusp is extremely rare. Such anomalies may predispose to valvular dysfunction, endocarditis, and, in some cases, sudden cardiac events. We report a very rare case of a 13-year-old girl presenting with failure to thrive and exertional dyspnea. Clinical examination revealed a grade 3/6 to-and-fro ejection systolic and early diastolic murmur at the right upper sternal border. Transthoracic echocardiography revealed a tricuspid aortic valve with a markedly hypoplastic right coronary cusp, leading to severe eccentric aortic regurgitation. CT angiography confirmed asymmetric cusp morphology. The patient underwent surgical aortic valve replacement with a mechanical prosthesis. Postoperative recovery was uneventful. Hypoplastic right coronary cusp is a rare anomaly of the aortic valve. Early recognition using multimodality imaging and timely surgical intervention are essential for preventing adverse outcomes.