Long-term outcomes after supravalvar aortic stenosis repair: Impact of residual haemodynamics on reintervention

OBJECTIVE: Surgical repair of supravalvar aortic stenosis (SVAS) provides excellent early outcomes; however, late reintervention remains common. The determinants of left ventricular outflow tract (LVOT) failure, particularly the role of residual haemodynamics, remain incompletely defined. We sought to identify predictors of LVOT reintervention in a contemporary cohort. METHODS: A retrospective study of consecutive patients aged <18 years undergoing SVAS repair between 2005 and 2024 was performed. Primary endpoints were overall survival and freedom from LVOT reintervention. Survival and freedom from reintervention were assessed using Kaplan-Meier analysis. Multivariable Cox regression was used to identify independent predictors of LVOT reintervention. RESULTS: Seventy-seven patients underwent repair at a median age of 3 (1-8) years, with median follow-up of 9.3 (6-14) years. There was no early or late mortality, nineteen patients (25%) required reintervention, including 16 (21%) LVOT-related procedures. Freedom from LVOT reintervention was 74% at 10 years and 71% at 15-20 years. Discharge LVOT velocity >3 m/s was independently associated with LVOT reintervention (HR 11.66, 95% CI 3.56-38.18; p < 0.001). Younger age was also independently associated with increased risk (HR per year 0.74, 95% CI 0.59-0.94; p = 0.013) for LVOT re-intervention. Coronary abnormalities were documented in 17% of patients, with one early coronary reintervention. CONCLUSION: SVAS repair is associated with excellent long-term survival. Residual discharge velocity emerged as an independent predictor of late LVOT reintervention supporting the importance of achieving optimal haemodynamic relief at the index operation and structured lifelong surveillance.