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.
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.
Authors: Jelena Kasnar-Samprec; Jürgen Hörer; Hanna Bierwirth; Zsolt Prodan; Julie Cleuziou; Andreas Eicken; Rüdiger Lange; Christian Schreiber Journal: Pediatr Cardiol Date: 2012-03-22 Impact factor: 1.655
Authors: Rosa Roemers; Jolanda Kluin; Frederiek de Heer; Sara Arrigoni; Regina Bökenkamp; Joost van Melle; Tjark Ebels; Mark Hazekamp Journal: World J Pediatr Congenit Heart Surg Date: 2018-03