Department of Pediatrics, Emory University School of Medicine, Atlanta, GA; Children's Healthcare of Atlanta and Sibley Heart Center Cardiology, Atlanta, GA.
Department of Pediatrics, Emory University School of Medicine, Atlanta, GA.
J Pediatr. 2021 Dec;239:187-192.e1. doi: 10.1016/j.jpeds.2021.08.034. Epub 2021 Aug 24.
To evaluate long-term survival in patients with Turner syndrome after congenital heart surgery with a focus on left heart obstructive lesions (LHOLs).
We queried the Pediatric Cardiac Care Consortium, a US-based registry of congenital heart surgery, for patients with Turner syndrome undergoing congenital heart surgery at <21 years of age between 1982 and 2011. Outcomes were obtained from the Pediatric Cardiac Care Consortium and from national death and transplant registries through 2019. Survival of patients with Turner syndrome and nonsyndromic patients with similar LHOL was compared by Kaplan-Meier survival curves and Cox regression adjusted for age, congenital heart disease, and era.
We identified 179 patients with Turner syndrome operated for LHOL: 161 with 2-ventricle lesions (coarctation n = 149, aortic stenosis n = 12) and 18 with hypoplastic left heart (HLH) variants. There were 157 with 2-ventricle LHOL and 6 with HLH survived to discharge. Among survivors to hospital discharge, the 30-year transplant-free survival was 90.4% for Turner syndrome with 2-ventricle lesions and 90.9% for nonsyndromic comparators (adjusted hazard ratio [aHR] 1.15, 95% CI 0.64-2.04). The postdischarge survival for HLH was 33% for Turner syndrome and 51% for nonsyndromic patients, with these numbers being too small for meaningful comparisons. There was a higher risk for cardiovascular disease events in patients with Turner syndrome vs male (aHR 3.72, 95% CI 1.64-8.39) and female comparators (aHR 4.55, 95% CI 1.87-11.06) excluding heart failure deaths.
The 30-year transplant-free survival is similar for patients with Turner syndrome and nonsyndromic comparators with operated 2-ventricle LHOL without excess congenital heart disease risk. However, patients with Turner Syndrome still face increased cardiovascular disease morbidity, stressing the importance of lifelong comorbidity surveillance in this population.
评估特纳综合征患者先天性心脏病手术后的长期生存情况，重点关注左心梗阻性病变（LHOL）。
我们在美国小儿心脏护理协会注册中心查询了特纳综合征患者的资料，该注册中心收集了 1982 年至 2011 年期间接受先天性心脏病手术且年龄<21 岁的患者信息。研究结果从小儿心脏护理协会注册中心和全国死亡与移植登记处获取，随访至 2019 年。采用 Kaplan-Meier 生存曲线和 Cox 回归比较特纳综合征患者和具有相似 LHOL 的非综合征患者的生存情况，校正年龄、先天性心脏病和时代因素。
我们确定了 179 例接受 LHOL 手术的特纳综合征患者：161 例为 2 心室病变（缩窄 149 例，主动脉瓣狭窄 12 例），18 例为左心发育不良（HLH）变异型。2 心室 LHOL 患者中有 157 例存活至出院，6 例 HLH 患者存活至出院。在出院存活的患者中，特纳综合征 2 心室病变患者 30 年无移植生存率为 90.4%，非综合征对照患者为 90.9%（校正后的危险比 [aHR] 1.15，95%CI 0.64-2.04）。特纳综合征 HLH 患者的出院后生存率为 33%，非综合征患者为 51%，但这些数字太小，无法进行有意义的比较。与男性（aHR 3.72，95%CI 1.64-8.39）和女性对照者（aHR 4.55，95%CI 1.87-11.06）相比，特纳综合征患者心血管疾病事件风险更高，但不包括心力衰竭死亡。
对于接受 2 心室 LHOL 手术且无先天性心脏病风险增加的特纳综合征患者和非综合征对照患者，30 年无移植生存率相似。然而，特纳综合征患者仍然面临更高的心血管疾病发病率，这强调了在该人群中进行终身合并症监测的重要性。
