Surgical techniques and prognostic nomogram for patients with supravalvular aortic stenosis

Abstract

Abstract Background An effective prognostic nomogram to predict the prognosis for supravalvular aortic stenosis (SVAS) patients is lacking. Methods A multi-center retrospective study of consecutive SVAS patients with surgery between 2002 and 2020 was conducted. Patients underwent McGoon repairs, Doty repairs, and other repairs. The primary outcome was the re-operation or restenosis at follow-up. The nomogram based on Cox regression and Kaplan–Meier method was used to show the risk factors of the primary outcome. The predictive accuracy was determined by the concordance index (C-index) and calibration curve. The results were validated using the bootstrap resampling method. Results Of the 291 SVAS patients, 143 (49.1%) used McGoon repairs, 118 (40.5%) used Doty repairs and 30 (10.3%) used other repairs. The median age at operation was 4.9 years (IQR 2.3–9.9). After a median follow-up of 24 months (IQR 6.0–54.0), no difference in re-operation or restenosis was found between McGoon repairs and Doty repairs. Age, gender, SVAS type, pulmonary artery stenosis, aortic valve stenosis, sinotubular junction z-score and gradient were considered independent risk factors by Lasso regression and were included in the nomogram. The C-index of the nomogram was 0.71 (95% CI 0.61 to 0.81). The calibration curve for the probability of re-operation or restenosis showed good agreement between prediction by nomogram and actual observation. Conclusions McGoon repairs and Doty repairs had no difference in re-operation and restenosis risk. The proposed nomogram gave an accurate prediction of re-operation or restenosis for patients with SVAS after surgery. Trial registration http://www.chictr.org.cn , ChiCTR2300067851, 2023.01.29 (retrospectively registered).

Keywords

• Supravalvular aortic stenosis
• McGoon repairs
• Doty repairs
• Nomogram
• Risk factors