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Original Articles: Pediatric Cardiac

Reoperation After Supravalvular Aortic Stenosis Repair

^a Division of Pediatric Cardiothoracic Surgery, Arkansas Children's Hospital, Little Rock, Arkansas
^b Department of Pediatrics, Arkansas Children's Hospital, Little Rock, Arkansas

Accepted for publication July 30, 2010.

^_* Address correspondence to Dr Imamura, Pediatric Cardiothoracic Surgery, Arkansas Children's Hospital, 1 Children's Way, Slot 677, Little Rock, AR 72202 (Email: imamuramichiaki{at}uams.edu).

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.

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