Although a small number of atrial myxomas are found in association with congenital cardiac defects, ventricular myxomas coexisting with congenital cardiac anomalies are extremely rare. We report a case of right ventricular (RV) myxoma coexistent with atrioventricular septal defect in an adolescent. Echocardiography showed an RV mass, a small ostium primum atrial septal defect, and a cleft of the left atrioventricular valve. Magnetic resonance imaging revealed a mass, suggesting a myxoma. The mass was excised simultaneously with repair of the anomalies and was histologically confirmed as a myxoma.
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