Supravalvular aortic stenosis in adults

Supravalvular aortic stenosis in adults, in contrast to the form seen in infants and children, is usually not associated with mental retardation, peculiar facies or severe peripheral pulmonic stenosis. Subtle clinical findings serve to distinguish it from valvular aortic stenosis, a differentiation of great importance if surgery is a consideration. Diagnosis is made by aortic and left ventricular angiograms. The severity of the clinical disease correlates better with abnormalities of the coronary arteries than with the severity of the obstruction, although both are frequently significant. Once symptoms occur, the prognosis is poor without surgical correction. Surgical mortality rates are high, primarily as a result of incomplete preoperative diagnosis.