Aortic Valve Disease in Children: Experience from Raising a Repair-Oriented Program

Objectives: Any aortic valve (AoV) operation in children (repair, Ross or mechanical replacement) is a palliation and reinterventions are frequent. AoV repair is a temporary solution primarily aimed at allowing the patient to grow to an age when more definitive solutions are available. We assessed AoV repair effectiveness across the whole age spectrum of children, excluding AoV disease secondary to congenital heart disease. Established program began in 1989 for neonates and 2003 for older children.

Methods: We retrospectively analyzed survival and freedom from operation/replacement in all neonates (group N) referred for AoV procedure since 1989 and in all children (group C) referred for AoV repair since 2003.

In group N (n = 103), 51 were treated with percutaneous Balloon Valvotomy (BV) and 52 with Surgical Valvoplasty (SV) (commissurotomy shaving).

In group C (n = 182), mean age was 7.4 ± 6.3 years (24% < 1 year); 82 (45%) had a preceding BV. The indications for the procedure were stenotic (n = 119; 65%), regurgitant (n = 56; 31%) or combined (n = 7; 4%) disease. The procedures performed were commissurotomy shaving (n = 71; 39%), leaflet replacement (n = 73; 40%), leaflet extension (n = 20; 11%) and neocommissure creation (n = 18; 11%). Post-repair geometry was tricuspid in 129 (71%) patients.

Results: In group N, 10-year survival was 88% and 94% after BV or SV, respectively. With a median follow-up of 13 years, 10-year freedom from operation/replacement was 36%/60% after BV and 66%/79% after SV. By multivariate analysis, predictors of operation and replacement were: BV, non-tricuspid post-repair geometry, associated left heart malformations, inadequate post-procedural result.

In group C, 10-year survival rate was 97.1%. With a median follow-up of 5 years, 7-year freedom from reoperation/replacement was 57%/68%. By multivariate analysis, predictors of reoperation and replacement were: BV before 6 months, non-tricuspid post-repair geometry, the absence of a developed commissure, cross-clamp duration.

Conclusion: Aortic valve repair in children is safe and effective in delaying the timing for more definitive solution (re-repair, supported Ross or mechanical replacement). In neonates, surgery significantly minimized the need for operation, while balloon dilatation did not postpone age of replacement. Surgical strategy should be individualized to patient age. Avoidance of early balloon dilatation and aiming for a tricuspid post-repair arrangement may improve outcomes.