Objective

To evaluate long-term survival in patients with Turner syndrome after congenital heart surgery with a focus on left heart obstructive lesions (LHOLs).

Study design

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.

Results

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.

Conclusions

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.

中文翻译：

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特纳综合征患者心脏手术后 30 年的生存率

客观的

评估先天性心脏病手术后特纳综合征患者的长期生存率，重点关注左心梗阻性病变 (LHOL)。

学习规划

我们查询了美国先天性心脏病手术登记处小儿心脏护理协会，了解 1982 年至 2011 年间接受先天性心脏手术的特纳综合征患者的年龄 <21 岁。结果来自小儿心脏护理协会和国家死亡和移植登记到 2019 年。通过 Kaplan-Meier 生存曲线和针对年龄、先天性心脏病和时代调整的 Cox 回归比较特纳综合征患者和具有类似 LHOL 的非综合征患者的生存。

结果

我们确定了 179 名接受 LHOL 手术的特纳综合征患者：161 名患有 2 心室病变（缩窄 n = 149，主动脉瓣狭窄 n = 12）和 18 名患有左心发育不全（HLH）变异。157 例 2 心室 LHOL 患者和 6 例 HLH 患者存活至出院。在出院的幸存者中，特纳综合征伴 2 脑室病变的 30 年无移植生存率为 90.4%，非综合征对照者为 90.9%（调整后的风险比 [aHR] 1.15，95% CI 0.64-2.04）。特纳综合征患者的 HLH 出院后生存率为 33%，非综合征患者为 51%，这些数字太小而无法进行有意义的比较。与男性 (aHR 3.72, 95% CI 1.64-8.39) 和女性比较者 (aHR 4.55, 95% CI 1.87-11.

结论

30 年无移植生存期与特纳综合征患者和无先天性心脏病风险过高的 2 心室 LHOL 手术非综合征对照者相似。然而，特纳综合征患者仍面临心血管疾病发病率增加的问题，强调了对这一人群进行终生合并症监测的重要性。