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Peer-reviewedCC BY-NC-NDJTCVS open

Postoperative root size predicts regurgitation in supravalvular aortic stenosis repair.

  • Dominic P Recco1,2
  • Lauren Marshall1
  • Matthew Schildmeier3
  • Kimberlee Gauvreau3,4
  • Joseph Confessore1
  • Luis G Quinonez1,2
  • Pedro J Del Nido1,2
  • Christopher W Baird1,2
  • Peter E Hammer1,2
  • David M Hoganson1,2
4 affiliations
  1. 1Department of Cardiac Surgery, Boston Children's Hospital, Boston, Mass.
  2. 2Department of Surgery, Harvard Medical School, Boston, Mass.
  3. 3Department of Cardiology, Boston Children's Hospital, Boston, Mass.
  4. 4Department of Pediatrics, Harvard Medical School, Boston, Mass.
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Abstract

Objective

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.Methods

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).Results

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).Conclusions

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.

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Cite

BibTeXRIS
BibTeX
@article{recco2026postoperative,
  title = {{Postoperative root size predicts regurgitation in supravalvular aortic stenosis repair.}},
  author = {Dominic P Recco and Lauren Marshall and Matthew Schildmeier and Kimberlee Gauvreau and Joseph Confessore and Luis G Quinonez and Pedro J Del Nido and Christopher W Baird and Peter E Hammer and David M Hoganson},
  journal = {JTCVS open},
  year = {2026},
  month = jun,
  doi = {10.1016/j.xjon.2026.101941},
  url = {https://europepmc.org/article/MED/42948104},
  note = {CC BY-NC-ND},
}
RIS
TY  - JOUR
TI  - Postoperative root size predicts regurgitation in supravalvular aortic stenosis repair.
AU  - Dominic P Recco
AU  - Lauren Marshall
AU  - Matthew Schildmeier
AU  - Kimberlee Gauvreau
AU  - Joseph Confessore
AU  - Luis G Quinonez
AU  - Pedro J Del Nido
AU  - Christopher W Baird
AU  - Peter E Hammer
AU  - David M Hoganson
PY  - 2026
DA  - 2026/06/24
JO  - JTCVS open
DO  - 10.1016/j.xjon.2026.101941
UR  - https://europepmc.org/article/MED/42948104
AB  - Objective  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.Methods  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).Results  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).Conclusions  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.
SN  - 2666-2736
ER  -