Postoperative root size predicts regurgitation in supravalvular aortic stenosis repair

Supravalvular aortic stenosis (SVAS) repair carries a moderate risk for postoperative aortic regurgitation (AR). This may be related to the targeted sinotubular junction diameter z score (STJDZ) at reconstruction, traditionally sized to zero. We investigated relationships between achieved STJDZ and aortic valve function after repair.

Sixty-six children underwent patch repair from 2010 to 2024 at a median age of 1.2 years (interquartile range [IQR], 0.4, 3.5 years) via Brom (n = 53), Doty (n = 9), and McGoon (n = 4) techniques. Relationships between STJDZ and aortic valve function were assessed via Jonckheere-Terpstra nonparametric test for trend over median follow-up of 5.6 years (IQR, 2.4, 8.4 years).

Preoperatively, AR was none/trivial in 52 (79%), left ventricular outflow tract obstruction (LVOTO) moderate or greater in 54 (82%), and median STJDZ -3.8 (IQR, -4.4, -2.9). At discharge, AR and LVOTO were mild or greater in 45 (68%) and 18 (27%), and median STJDZ was -0.3 (IQR, -1.3, -1.1). STJDZ increased by a median of 3.0 (IQR, 1.6, 4.4) preoperatively to postoperatively. Discharge AR was none/trivial at median STJDZ -1.3 (IQR, -1.8, -0.1) and worsened with increasing diameters (mild: 0.4 [IQR, -1.0, 1.8], mild+: 0.0 [IQR, -1.2, 1.5]; P = .038), with similar results at follow-up (P = .048). LVOTO worsened with lower median STJDZ (none/trivial: 0.2 [IQR, -1.2, 1.5], mild: -1.3 [IQR, -3.2, -0.1]; P = .015). Subanalysis of those with none/trivial preoperative AR (n = 52) showed that increase in STJDZ by ≥4 demonstrated worse follow-up AR (P = .016).

Achieving STJDZ zero using patch repair techniques to relieve SVAS obstruction demonstrated mild or greater postoperative AR compared with none/trivial regurgitation at median z score -1.3. Mild LVOTO was noted with median z score -1.3. Targeting STJDZ of -1 in pediatric SVAS repair appears appropriate to balance residual regurgitation and LVOTO. Further follow-up is required to understand the long-term impact of this target on aortic growth.