Surgical Techniques and Prognostic Nomogram for Patients with Supravalvular Aortic Stenosis

Background An effective prognostic nomogram to predict the prognosis of patients with supravalvular aortic stenosis (SVAS) is lacking. This study sought to report the outcomes of different surgical techniques, identify the risk factors for postoperative re-operation or restenosis, and establish a prognostic nomogram. Methods This multi-centred retrospective cohort study included consecutive patients with SVAS who underwent surgery between 2002 and 2020. Patients underwent McGoon, Doty, and other repairs. The primary outcome was re-operation or restenosis at follow-up. The nomogram based on Cox regression and the Kaplan–Meier method was used to identify the risk factors for the primary outcome. The predictive accuracy was determined using the concordance index (C-index) and calibration curve. The results were validated using the bootstrap resampling method. Results Of the 291 patients with SVAS, 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 (inter-quartile range (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 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 least absolute shrinkage and selection operator 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 and Doty repairs showed no difference in re-operation and restenosis risk. The nomogram accurately predicted re-operation or restenosis in patients with SVAS after surgery. Trial Registration :