Thirty-Year Survival after Cardiac Surgery for Patients with Turner Syndrome.
Thirty-Year Survival after Cardiac Surgery for Patients with Turner Syndrome.
复制标题
DOI：
10.1016/j.jpeds.2021.08.034
复制
发表时间：
2021-12
期刊：
影响因子：
--
通讯作者：
Wechsler SB
中科院分区：
文献类型：
--
作者：
Alam S;Claxton JS;Mortillo M;Sassis L;Kefala-Karli P;Silberbach M;Kochilas L;Wechsler SB
评估特纳综合征患者在先天性心脏手术后的长期生存，重点是左心阻塞性病变（LHOLs）。我们查询了儿科心脏护理协会（一家美国先天性心脏手术登记机构）1982年至2011年间年龄<21岁的特纳综合征患者接受先天性心脏手术的情况。结果来自儿科心脏护理联盟和截至2019年的国家死亡和移植登记处。采用Kaplan-Meier生存曲线和Cox回归校正年龄、先天性心脏病和年龄，比较Turner综合征患者和无综合征相似LHOL患者的生存。我们确定了179例因LHOL手术的特纳综合征患者：161例为双心室病变（缩窄n = 149，主动脉狭窄n = 12）， 18例为左心发育不良（HLH）变异。2室LHOL 157例，HLH 6例存活出院。在出院的幸存者中，伴有2脑室病变的特纳综合征患者30年无移植生存率为90.4%，无综合征比较者为90.9%（校正风险比[aHR] 1.15, 95% CI 0.64-2.04）。Turner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，无法进行有意义的比较。除心力衰竭死亡外，特纳综合征患者发生心血管疾病事件的风险高于男性（aHR 3.72, 95% CI 1.64-8.39）和女性（aHR 4.55, 95% CI 1.87-11.06）。Turner综合征患者的30年无移植生存期与2室LHOL手术的非综合征比较者相似，没有额外的先天性心脏病风险。然而，特纳综合征患者仍然面临心血管疾病发病率增加的问题，这就强调了在这一人群中终生共病监测的重要性。
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.
登录
查看更多内容
影响因子：
2.1
作者：
Chou, Yu-Yu;Wang, Chao-Jan;Lo, Fu-Sung
通讯作者：
Lo, Fu-Sung
影响因子：
8
作者：
Sybert, VP
通讯作者：
Sybert, VP
影响因子：
1.9
作者：
Swerdlow, AJ;Hermon, C;Youings, S
通讯作者：
Youings, S
影响因子：
5.4
作者：
Spector, Logan G.;Menk, Jeremiah S.;Kochilas, Lazaros K.
通讯作者：
Kochilas, Lazaros K.
影响因子：
5.8
作者：
Schoemaker, Minouk J.;Swerdlow, Anthony J.;Jacobs, Patricia A.
通讯作者：
Jacobs, Patricia A.