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A 38-year-old man was referred to our hospital because of a heart murmur and mild fever. Physical examination disclosed a systolic murmur of grade 3/6 at the apex and a diastolic murmur of grade 4/6 at the 2nd intercostal space of the left sternal border. The chest radiograph revealed mild cardiomegaly. The ECG showed left ventricular hypertrophy and ST segment depression in leads I, aVL and V4 to V6. Aortogram demonstrated supravalvular aortic stenosis (SAS) and aortic regurgitation. At the operation, we found the right coronary ostium was severely narrowed by the thickened right coronary cusp and the fibrous ring causing SAS. Aortic valve replacement and extended aortoplasty were performed. He had been doing well but suddenly died 11 months after the operation.
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