Reoperation after supravalvular aortic stenosis repair

Background Supravalvular aortic stenosis (SVAS) is the rarest type of left ventricular outflow tract obstruction. We reviewed our experience with this anomaly and analyzed risk factors for death or reoperation. Methods Between 1984 and 2009, 49 patients had surgery for SVAS. A single-patch technique was used in 3, two-sinus enlargement in 39, and three-sinus enlargement in 7. Variables evaluated included age at surgery (<2 versus >2 years old), presence of pulmonary artery stenosis, type of SVAS (focal versus diffuse), presence of valvular aortic stenosis, and era of surgery. Results The only early death occurred in a patient who experienced cardiac arrest during anesthesia induction and could not be separated from bypass after surgery. There were 2 late deaths at 3 and 11 years after SVAS repair, both related to treatment for pulmonary artery stenosis. Actuarial survival at 5, 10, and 20 years was 95%, 95%, and 90%, respectively. Sixteen patients required 23 reoperations: for pulmonary artery stenosis (n = 10), distal aortic stenosis (n = 9), aortic valve stenosis (n = 4), and coronary artery stenosis (n = 1). Actuarial reoperation-free survivals at 5, 10, and 20 years were 73%, 58%, and 52%, respectively. Coexistent pulmonary artery stenosis, young age at surgery, and diffuse type SVAS were predictors of lower freedom from death or reoperation by both univariate and multivariate analyses. Conclusions Survival after surgical repair of SVAS is excellent. However, reoperation is frequent, especially when the patients also have pulmonary artery stenosis, diffuse type SVAS, and initial surgery at a young age.