Surgical techniques and prognostic nomogram for patients with supravalvular aortic stenosis

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, Dot...

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Main Authors: Yuekun Sun, Lizhi Lv, Xinyue Lang, Aihua Zhi, Simeng Zhang, Cheng Wang, Qiang Wang
Format: Article
Language:English
Published: BMC 2025-01-01
Series:European Journal of Medical Research
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Online Access:https://doi.org/10.1186/s40001-024-02257-w
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author Yuekun Sun
Lizhi Lv
Xinyue Lang
Aihua Zhi
Simeng Zhang
Cheng Wang
Qiang Wang
author_facet Yuekun Sun
Lizhi Lv
Xinyue Lang
Aihua Zhi
Simeng Zhang
Cheng Wang
Qiang Wang
author_sort Yuekun Sun
collection DOAJ
description 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).
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spelling doaj-art-c2c02152de88402492547d126f58b7ea2025-01-19T12:14:49ZengBMCEuropean Journal of Medical Research2047-783X2025-01-0130111010.1186/s40001-024-02257-wSurgical techniques and prognostic nomogram for patients with supravalvular aortic stenosisYuekun Sun0Lizhi Lv1Xinyue Lang2Aihua Zhi3Simeng Zhang4Cheng Wang5Qiang Wang6Department of Pediatric Cardiac Center, Beijing Anzhen Hospital, Capital Medical UniversityDepartment of Pediatric Cardiac Center, Beijing Anzhen Hospital, Capital Medical UniversityDepartment of Pharmacy and Clinical Trial Unit, Beijing Anzhen Hospital, Capital Medical UniversityDepartment of Radiology, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical CollegeDepartment of Cardiac Surgery, Peking University People’s HospitalCenter for Pediatric Cardiac Surgery, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical CollegeDepartment of Pediatric Cardiac Center, Beijing Anzhen Hospital, Capital Medical UniversityAbstract 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).https://doi.org/10.1186/s40001-024-02257-wSupravalvular aortic stenosisMcGoon repairsDoty repairsNomogramRisk factors
spellingShingle Yuekun Sun
Lizhi Lv
Xinyue Lang
Aihua Zhi
Simeng Zhang
Cheng Wang
Qiang Wang
Surgical techniques and prognostic nomogram for patients with supravalvular aortic stenosis
European Journal of Medical Research
Supravalvular aortic stenosis
McGoon repairs
Doty repairs
Nomogram
Risk factors
title Surgical techniques and prognostic nomogram for patients with supravalvular aortic stenosis
title_full Surgical techniques and prognostic nomogram for patients with supravalvular aortic stenosis
title_fullStr Surgical techniques and prognostic nomogram for patients with supravalvular aortic stenosis
title_full_unstemmed Surgical techniques and prognostic nomogram for patients with supravalvular aortic stenosis
title_short Surgical techniques and prognostic nomogram for patients with supravalvular aortic stenosis
title_sort surgical techniques and prognostic nomogram for patients with supravalvular aortic stenosis
topic Supravalvular aortic stenosis
McGoon repairs
Doty repairs
Nomogram
Risk factors
url https://doi.org/10.1186/s40001-024-02257-w
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