Congenital aortic regurgitation caused by absence of the right coronary cusp.

We describe a patient with congenital aortic regurgitation, well documented from the age of 1 month. Surgery was performed at the age of 7 years, when echocardiography showed progressive dilatation of the left ventricle with decreasing shortening fraction. Inspection of the valve during the operation showed a basically tricuspid valve, but with a rudimentary right coronary cusp. At histological examination, myxoid dysplasia was found in all the cusps. An aortic homograft was inserted and the left ventricular internal dimensions returned to normal within 3 months after the operation.