Long-term follow-up of extended aortoplasty for supravalvular aortic stenosis

Extended aortoplasty is an operation that was designed to provide a symmetric reconstruction of the aortic root in patients with supravalvular aortic stenosis. The aim of this report is to provide long-term follow-up of the original cohort of 15 patients who underwent extended aortoplasty between 1975 and 1983. Follow-up was obtained in 14 patients. One patient was lost to follow-up 3 years after operation; he was included in this report. An echocardiogram, chest radiograph, and electrocardiogram were obtained for each surviving patient. The median length of follow-up was 141 months (range 36 to 238). The median preoperative gradient was 90 mm Hg (range 55 to 150). The median immediate postoperative gradient was 20 mm Hg (range 0 to 50, p < 0.05 compared with preoperative gradient) and the median long-term gradient was 32 mm Hg (range 6 to 96, p < 0.05 compared with preoperative gradient; p = not significant compared with immediate postoperative gradient). Two patients died: one of left ventricular failure after a subsequent aortic valve replacement and one of chronic left ventricular failure. The Kaplan-Meier estimate of survival at 218 months for all patients was 77.4% (70% confidence limits 62% to 93%). The estimated freedom from reoperation for all patients was 69% at 218 months (70% confidence limits 56% to 82%). Univariate analysis revealed that the presence of a bicuspid valve is a significant risk factor for reoperation (p = 0.038), but not for death (p = 0.51). The Kaplan-Meier estimate of freedom from reoperation for patients with a bicuspid aortic valve was 42.9% at 141 months (70% confidence limits 21% to 65%). Extended aortoplasty provides effective long-term relief of the pressure gradient across the supravalvular ridge. However, a significant number of patients require subsequent operations, particularly those with a bicuspid aortic valve.