Abstract Background Supravalvular aortic stenosis (SVAS) is a rare condition with limited data on patients with SVAS beyond childhood. Purpose This study aims to investigate the clinical course and outcomes of SVAS in adulthood. Methods All adult (≥18years) patients with SVAS, prospectively registered in the Dutch Congenital Cor Vitia (CONCOR) database between 2001-2019, were included. Survival and event-free survival (composite of mortality, heart failure, arrhythmic events, thrombo-embolic events, SVAS related (re)-intervention, aortic aneurysm surgery, aortic dissection, endocarditis, coronary events and other cardiac surgery) were assessed. The peak velocity was analysed using linear mixed models. Differences in previous operated state, sex, and Williams-Beuren syndrome were explored. Results Sixty-five patients were included (age: 23(IQR:20,31) years, 31% female, 46% previous SVAS correction, 47% Williams-Beuren syndrome). The peak velocity was 2.3±1.0m/s at inclusion. Median follow-up time was 13(IQR:10,17) years. Four patients died (one patient died after cardiac surgery, two patients died of non-cardiac causes and in one patient the cause of death was unknown) translating in a survival of 95%(95%CI:90%-100%) and event-free survival of 83%(95%CI:74%-93%) at 10-years. There were no differences in event-free-survival between previous operated state (p=0.2), sex (p=0.48) and Williams-Beuren syndrome (p=0.85). Thirty-one cardiovascular events occurred in 15 patients (Figure 1), with the majority being arrhythmias. All SVAS related interventions (3 surgeries in 2 patients) occurred in unoperated patients (7 (95%CI:2-21)/1,000patient years). Although no patient showed fast progression (≥0.3m/s/year), the peak velocity evolution over time increased faster in females compared to males (first time spline: 0.8 m/s, p=0.017) (Figure 2). Conclusion In adulthood, patients with SVAS demonstrated a stable clinical course without fast progression and with a limited number of cardiovascular events. However, women exhibited faster disease progression than men. Compared to childhood, a more benign disease course was observed in adults, suggesting that less frequent follow-up may be indicated.Figure 1 Figure 2
Keijzer et al. (Sat,) studied this question.