[A case of congenital absence of the right coronary aortic cusp]

A 12-year-old boy with absence of the right coronary cusp underwent aortic valve replacement with a 21 mm St. Jude Medical prosthetic valve. He was known to have noncyanotic congenital heart disease at the age of 2 months. The diagnosis of aortic insufficiency was first made when he was 1 1/4 years old. Intensive medical treatment was continued while he was growing up. Cardiac catheterization was performed repeatedly at ages 3 1/2 and 7. The findings on the fourth cardiac catheterization at 12 years of age were pulmonary arterial pressure of 90/60 mmHg, left ventricular end-diastolic pressure of 25 mmHg and poor contraction of the left ventricle. Although IABP support was necessary for treatment of severe low cardiac output state in the early postoperative period, he was discharged at 1 month after operation, Now, he goes to school under anticoagulant therapy. Intensive medical care followed by elective aortic valve replacement may be a useful treatment for isolated congenital absence of the aortic valve cusp.