Surgical Outcome of Supra-Valvular Aortic Stenosis: A 50-Year Single-Centre Experience

This study aimed to evaluate and compare the long-term outcomes of 3 different surgical techniques (McGoon, Doty, and Brom) for supra-valvular aortic stenosis (SVAS) and to identify the risk factors affecting mortality and morbidity.

We performed a single-centre retrospective analysis of all patients who underwent surgery for SVAS between August 1974 and January 2025. The outcomes were analysed using survival and competing risk analysis, and risk factors were identified using Cox regression models.

A total of 75 patients were identified, 26 (35%) profited from the McGoon technique, 33 (44%) patients received a Doty repair, and 16 (21%) underwent Brom repair. Williams-Beuren syndrome was associated with 45 (60%) patients. Associated anomalies included pulmonary stenosis in 40 (53%) patients, aortic coarctation in 24 (32%), and abnormal coronary arteries in 14 (19%). Transplant-free survival at 15 years was 92%, and no difference was found between surgical techniques (P = .339). Abnormal coronary arteries were a risk factor for major adverse cardiac events (odds ratio 8.666, P = .011) and mortality (hazard ratio [HR] 4.285, P = .030). Cumulative incidence of reoperation at 15 years was 18%, with no difference between surgical techniques (P = .299). However, patients with pulmonary stenosis (HR 3.450, P = .020) had a higher risk of reoperation.

Survival after surgical repair of SVAS is over 90% at 15 years regardless of the surgical technique. Abnormal coronary arteries are responsible for higher operative mortality. Concomitant pulmonary artery stenosis is at a higher risk of reoperation, suggesting more severe arterial disease.