PROGNOSIS OF SUPRAVALVE AORTIC-STENOSIS IN 81 PATIENTS IN LIVERPOOL (1960-1993)

Objective-To determine the prognosis of supravalve aortic stenosis int o early adult life and the factors affecting this prognosis. Design-81 patients with supravalve aortic stenosis were followed for a median d uration of 8.3 (range 1 to 29) years. Patients-40 patients (49.4%) had Williams' syndrome, 18 (22.2%) familial supravalve aortic stenosis, 1 8 (22.2%) sporadic supravalve aortic stenosis, and five (6.2%) other s yndromes. Nineteen patients had additional levels of left ventricular outflow tract obstruction. Results-47 patients (58%) underwent operati on; 20% within a year of presentation. Multivariable analysis predicte d that 88% of patients would undergo intervention within 30 years of f ollow up. The chance of intervention was increased by more severe aort ic stenosis at presentation and the presence of multilevel obstruction in patients with sporadic supravalve aortic stenosis. Three deaths oc curred before operation and 13 within a month of operation. Ten (62.5% ) of the postoperative deaths were in patients with multilevel obstruc tion. Predicted survival 30 years after presentation was 66%. Risk fac tors for survival were age and severity of aortic stenosis at presenta tion. Multilevel obstruction did not emerge as a significant risk fact or for death because of the high association with the severity of sten osis at presentation. 74% of survivors had mild or insignificant steno sis at follow up. Conclusions-Long-term survival is related to age and the severity of aortic stenosis at presentation. Most patients will r equire intervention, and most survivors will have mild stenosis.