Year: 2026
Type: article
Study design: Observational Study
Abstract: Objective Supravalvular aortic stenosis (SVAS) repair carries 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 (AoV) function following repair. Methods Sixty-six children underwent patch repair from 2010-2024 at median age 1.2[IQR:0.4,3.5]yrs via Brom (n=53), Doty (n=9), and McGoon (n=4) techniques. Relationships between STJDZ and AoV function were assessed via Jonckheere-Terpstra nonparametric test for trend over median follow-up of 5.6[IQR:2.4,8.4]yrs. Results Preoperatively, AR was none/trivial in 52 (79%), 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] pre- to post-operative. 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=0.038), with similar results at follow-up (p=0.048). LVOTO worsened with lower median STJDZ (none/trivial: 0.2[IQR:-1.2,1.5], mild: -1.3[IQR:-3.2,-0.1]; p=0.015). Sub-analysis of those with none/trivial preoperative AR (n=52) showed that increase in STJDZ by ≥4 demonstrated worse follow-up AR (p=0.016). Conclusions Achieving STJDZ zero using patch repair techniques to relieve SVAS obstruction demonstrated mild or greater postoperative AR, compared to 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 long-term impacts of this target on aortic growth.
Source: JTCVS Open
Institutions: Boston Children's Hospital, Harvard University
Language: English
Subfield: Cardiology and Cardiovascular Medicine
Field: Medicine
Domain: Health Sciences
Open Access status: gold
Estimated APC paid: $2,431
OpenAlex ID: W7165764623
