Meccanici F, Notenboom ML, Meijssen J, Smit V, van de Woestijne PC, van den Bosch AE, Helbing WA, Bogers AJJC, Takkenberg JJM, Roos-Hesselink JW. Eur J Cardiothorac Surg. 2024 Jan 2;65(1):ezad360. doi: 10.1093/ejcts/ezad360.PMID: 37889257
Take home points:
- The 30-year estimated survival rate after congenital SVAS repair was 91% compared to that of the matched general population.
- The estimated 30-year reintervention rate (31%) and the risk of myocardial infarction (8%) were notably high.
Summary:
This systematic review and meta-analysis included 23 publications reporting long-term outcomes following surgical repair for congenital supravalvular aortic stenosis (SVAS), encompassing a total of 1472 patients (median age at surgical repair: 5.8 years). The majority of the petients had the discrete type of SVAS (71.7%), and nearly half (49.6%) were diagnosed with Williams-Beuren syndrome. The repair techniques reported were single-patch repair (43.6%), pantaloons-shaped patch repair (33.2%), and three-patch repair (14.2%). The pooled early mortality rate following SVAS repair was 4.2% (95% CI: 3.2-5.5) and the late mortality was 0.61% (95%CI: 0.45 0.83) per patient-year. Additionally, the risk of sudden unexpected death was 0.36% (95% CI: 0.24 0.55) per patient-year. A microsimulation model over a 30-year time horizon estimated that life expectancy after SVAS repair was 90.7% (95%CI: 90.0-91.6) of the expected life expectancy of the matched general population.
Over the 30-year period, the estimated risk of myocardial infarction was 8.1% (95%CI: 7.3-9.9), and the reintervention rate was 31.3% (95%CI: 29.6–33.4), with 27.2% (95%CI: 25.8-29.1) of these reinterventions attributed to repair dysfunction (Figure).
Comment:
Most studies on surgical outcomes for SVAS are based on small patient cohorts from single instisutions, with significant variations in patient characteristics and disease severity. This heterogeneity makes it challengint to develop a comprehensive understanding of long-term outcomes. The authors addressed this limitation by conducting a systematic review and meta-analysis and incorporated microsimulation models to predict 30-year survival and reoperation rates.
- The relative life expectancy after SVAS repair was considerable. However, the risk of sudden unexpected death (0.36% per patient-year) and myocardial infarction (8% over 30 years) remain concerning. In general, patients requiring surgery before age of 1 year present with more severe disease and poorer prognoses. However, the findings emphasize the need for close, long-term follow-up for all patients to monitor the progression of coronary artery disease and biventricular outflow tract obstruction, both of which can lead to sudden death.
- The projected 30-year reintervention rate of 31% was notably high, with most reinterventions resulting from repair dysfunction. Although the three-sinus techniques theoretically restore the symmetrical aortic root and may prevent post-repair aortic insufficency, no consensus exists regarding the optimal repair approach. Nearly half of the patients in the study underwent single-patch repair, while only 14 received three-patch repair. The small sample size and limited follow-up for the three-patch technique hinder direct comparisons of outcomes across repair strategies. Ultimately, an individualized approach that ensures complete relief of the obstruction and restores root anatomy is essential to minimize the need for late reinterventions after SVAS repair.
