Long-term outcomes after primary intervention for congenital aortic valve stenosis: a 30-year single-centre experience
TL;DR
Long-term survival following intervention for severe congenital AVS is excellent and comparable between BAV and SAV, and reintervention is common, particularly in neonates, but progression to AVR less so.
Abstract
Abstract Objective To compare long-term outcomes following balloon aortic valvuloplasty (BAV) and surgical aortic valvotomy (SAV), including surgical valvuloplasty for isolated severe congenital aortic valve stenosis (AVS). Data for mechanical aortic valve replacement and pulmonary autograft replacement are also presented to provide a complete institutional cohort overview. Methods Retrospective single-centre cohort study including patients aged <18 years undergoing primary intervention for isolated severe AVS between 1995 and 2024. Severe AVS was defined as peak Doppler gradient >65 mm Hg and/or mean gradient >40 mm Hg. Primary outcome was all-cause mortality. Secondary outcomes were time to reintervention and time to aortic valve replacement (AVR)—mechanical or autograft. Kaplan-Meier and Cox regression analyses were performed. Results A total of 231 patients met inclusion criteria (96 BAV, 112 SAV, 23 other). Median age at intervention was lower in the SAV group (0.2 years vs 3.74 years, p<0.001). Overall survival was 99.4% at 10 years and 92.3% at 25 years, with no statistically significant difference between BAV and SAV (p=0.083), though a trend may exist. Reintervention occurred in 49% of patients overall, with similar rates between BAV and SAV (p=0.950). Freedom from mechanical or autograft AVR remained high (75% at 10 years; 67% at 25 years), with no significant difference between strategies (p=0.190). Neonatal intervention was associated with significantly lower freedom from reintervention (p<0.0001). Conclusions Long-term survival following intervention for severe congenital AVS is excellent and comparable between BAV and SAV. Reintervention is common, particularly in neonates, but progression to AVR less so. These findings support continued multidisciplinary, anatomy-guided decision-making and highlight the importance of life-course management strategies. Development of a national AVS registry is indicated.