Long-Term Outcomes after Surgical Repair of Supravalvular Aortic Stenosis in Pediatric Patients: 30 Years’ Single-Center Outcome

 

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Background: Supravalvular aortic stenosis (SVAS) can be focal with a distinct stenosis localization or with a diffuse narrowing of the ascending aorta. Surgical techniques consist of the single patch repair technique (McGoon's repair), the pantaloon- or Y-shaped patch which extends into two aortic valve sinuses (Doty's technique and the three-patch technique where three separate triangular patches are inserted in the three sinuses (Brom's technique).

Method: A retrospective chart review of all patients aged below 18 years at the time of surgery, who had undergone surgery for SVAS from May 1985 until April 2020, was conducted. Mortality was cross-checked with the national health insurance database and could be provided for all, but one patient, who was transferred only for surgery from a foreign country.

Results: From May 1985 until April 2020, 20 repairs of SVAS were performed in 19 patients (63.2% males; 52.6% Williams–Beuren syndrome; familial SVAS (Eisenberg's syndrome in one patient). The corrective surgeries consisted of a single-patch repair in four cases, a pantaloon-shaped patch in eight cases and a three-patch technique in eight cases. Median age at time of surgery was 1.3 years (IQR: 0.8–2.8 years). In 10 cases, concomitant patch plasty of the pulmonary arteries was performed and in two cases concomitant aortic valve repair was performed. There were no early deaths. Two patients required postoperative extracorporeal membrane oxygenation (ECMO) support and in five cases, delayed sternal closure (including the two ECMO patients) was necessary. One late death occurred in a 6-month-old Williams–Beuren patient after reoperation for coarctation of the aorta 15 months after correction of the SVAS with a pantaloon-shaped patch. Kaplan–Meier estimated survival was 94.7 ± 5.1% at 30 years. Reoperation (one pantaloon-patch repair, two Ross's procedures) for re-SVAS occurred in three cases and freedom from reoperation for re-SVAS was 93.3 ± 6.4% at 5 years, 86.2 ± 9.1% at 10 years and 75.4 ± 12.8% at 30 years.

Conclusion: Early outcomes are excellent as no early deaths occurred but perioperative management regarding anesthesia and cardiopulmonary bypass weaning is complicated due to higher than usual pressures are often required to ensure myocardial perfusion via the thick-walled coronary arteries and ostial stenoses. In this complex patient cohort, close follow-up is warranted, though long-term survival and reoperation rates are good.

Publication History

Article published online:
12 February 2022

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