Long-Term Outcomes of Individualized Repair in Patients with Supravalvular Aortic Stenosis

• Published in: Pediatric cardiology 2024-06
• Main Authors: Wang, Zhangwei, Ma, Kai, Li, Shoujun
• Format: Article
• Language: English
• ISSN: 0172-0643; 1432-1971; 1432-1971
• DOI: 10.1007/s00246-024-03557-y

Various surgical techniques have been introduced to treat supravalvular aortic stenosis (SVAS). However, there is no consensus on the optimal approach. This study aimed to analyze the outcomes of surgical treatment of SVAS and determine the optimal strategy. The Kaplan-Meier curve was used to demonstrate the survival estimates. The Cox proportional hazard model was used to identify risk factors for residual aortic stenosis and residual stenosis-related reintervention. From December 2008 to December 2023, 98 patients with SVAS undergoing surgical repair in our institution were included [McGoon group, n = 62; Doty group, n = 36]. There were 2 in-hospital deaths and 1 late death. The survival rates at 1, 5, and 15 years were 98.0%, 96.7%, and 96.7%, respectively in the whole cohort. Residual aortic stenosis occurred in 18 patients. Multivariable analysis showed that preoperative gradient ≥ 90 mmHg (P = 0.002) and Williams syndrome (P = 0.002) were incremental risk factors for residual aortic stenosis, but surgical technique (P = 0.579) was not a risk factor for residual aortic stenosis. In the McGoon group, patients with diffuse type had worse freedom from residual aortic stenosis than patients with discrete type (P = 0.007). However, in the Doty group, patients with diffuse type had comparable freedom from residual aortic stenosis to patients with discrete type (P = 0.911). Residual stenosis-related reintervention occurred in 15 patients. Fifteen patients all underwent residual aortic stenosis-related reintervention. Of 15 patients, 6 patients also underwent residual pulmonary stenosis-related reintervention. On multivariate analysis, Williams syndrome (P