Sun Yuekun, Lv Lizhi, Lang Xinyue, Zhi Aihua, Zhang Simeng, Wang Cheng, Wang Qiang
Department of Pediatric Cardiac Center, Beijing Anzhen Hospital, Capital Medical University, Beijing, 100029, China.
Department of Cardiac Surgery, Yunnan Fuwai Cardiovascular Hospital, Kunming, 650102, China.
Eur J Med Res. 2025 Jan 13;30(1):26. doi: 10.1186/s40001-024-02257-w.
An effective prognostic nomogram to predict the prognosis for supravalvular aortic stenosis (SVAS) patients is lacking.
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.
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.
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).
目前缺乏一种有效的预测主动脉瓣上狭窄（SVAS）患者预后的列线图。
对2002年至2020年间连续接受手术的SVAS患者进行了一项多中心回顾性研究。患者接受了麦戈恩修复术、多蒂修复术及其他修复术。主要结局是随访时的再次手术或再狭窄。基于Cox回归和Kaplan-Meier方法的列线图用于显示主要结局的危险因素。通过一致性指数（C指数）和校准曲线确定预测准确性。结果采用自抽样重抽样方法进行验证。
在291例SVAS患者中，143例（49.1%）采用麦戈恩修复术，118例（40.5%）采用多蒂修复术，30例（10.3%）采用其他修复术。手术时的中位年龄为4.9岁（四分位间距2.3 - 9.9）。中位随访24个月（四分位间距6.0 - 54.0）后，麦戈恩修复术和多蒂修复术在再次手术或再狭窄方面未发现差异。年龄、性别、SVAS类型、肺动脉狭窄、主动脉瓣狭窄、窦管交界z评分和梯度经Lasso回归分析被认为是独立危险因素，并纳入列线图。列线图的C指数为0.71（95%置信区间0.61至0.81）。再次手术或再狭窄概率的校准曲线显示列线图预测与实际观察结果之间具有良好的一致性。
麦戈恩修复术和多蒂修复术在再次手术和再狭窄风险方面无差异。所提出的列线图对SVAS患者术后再次手术或再狭窄进行了准确预测。试验注册：http://www.chictr.org.cn ，ChiCTR2300067851，2023.01.29（回顾性注册）。
