Successful atrioventricular valve repair improves long-term outcomes in children with unbalanced atrioventricular septal defect
Successful atrioventricular valve repair improves long-term outcomes in children with unbalanced atrioventricular septal defect
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DOI：
10.1016/j.jtcvs.2017.06.042
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发表时间：
2017-12-01
影响因子：
6
通讯作者：
Konstantinov, Igor E.
中科院分区：
文献类型：
--
作者：
Buratto, Edward;Ye, Xin Tao;Konstantinov, Igor E.
背景：房室瓣关闭不全是房室间隔缺损不平衡患者发病和死亡的重要原因。然而，对不平衡房室间隔缺损和单心室生理学儿童房室瓣修复的结果了解有限。方法：我们对在皇家儿童医院接受单心室姑息治疗并接受房室瓣手术的所有不平衡房室间隔缺损儿童进行回顾性分析。结果：1976年至2016年间，139名不平衡房室间隔缺损儿童接受了房室瓣修复术。单心室姑息治疗，其中 31.7%（44/139）需要房室瓣手术。 97.7% (43/44) 的患者尝试修复房室瓣，其中 4.7% (2/43) 在初次手术期间转为置换。 2.3% (1/44) 的患者在未尝试修复的情况下更换了房室瓣。早期死亡率为 18.2% (8/44)。 10 年免于死亡或移植的率为 66.0%（95% 置信区间，49.1-78.5），20 年免于死亡或移植的率为 53.3%（95% 置信区间，32.1-70.6）。在多变量分析中，显着的出院前房室瓣关闭不全（风险比，6.4；P=.002）、年龄小于 1 岁（风险比，8.3；P=.01）和 II 期姑息治疗前的修复（风险比，3.4；P=.04）与死亡相关。 10 年免再手术率为 61.9%（95% 置信区间，41.9-76.8），20 年免再手术率为 56.3%（95% 置信区间，35.3-72.8）。出院时中度或较大的房室瓣关闭不全与再次手术风险增加相关（风险比，1.8；P=.03）。在最近一次随访中，无移植幸存者中，60.0% (15/25) 的房室瓣关闭不全程度低于中度。结论：房室间隔缺损不平衡患者的房室瓣手术与较高的死亡率和较高的再手术率相关。成功的房室瓣修复与更好的生存和免于再次手术有关。
Background: Atrioventricular valve regurgitation is a significant cause of morbidity and mortality in patients with unbalanced atrioventricular septal defect. However, knowledge of the outcomes of atrioventricular valve repair in children with unbalanced atrioventricular septal defect and univentricular physiology is limited.Methods: We conducted a retrospective review of all patients with unbalanced atrioventricular septal defect treated with single-ventricle palliation who underwent atrioventricular valve surgery at The Royal Children's Hospital.Results: Between 1976 and 2016, 139 children with unbalanced atrioventricular septal defect underwent single-ventricle palliation, of whom 31.7% (44/139) required atrioventricular valve surgery. Repair of the atrioventricular valve was attempted in 97.7% (43/44) of patients, of whom 4.7% (2/43) were converted to replacement during the initial operation. Replacement of the atrioventricular valve without attempted repair was performed in 2.3% (1/44) of patients. Early mortality was 18.2% (8/44). Freedom from death or transplantation at 10 years was 66.0% (95% confidence interval, 49.1-78.5) and at 20 years was 53.3% (95% confidence interval, 32.1-70.6). In multivariable analysis, significant predischarge atrioventricular valve regurgitation (hazard ratio, 6.4; P=.002), age less than 1 year (hazard ratio, 8.3; P=.01), and repair before stage II palliation (hazard ratio, 3.4; P=.04) were associated with death. Freedom from reoperation at 10 years was 61.9% (95% confidence interval, 41.9-76.8) and at 20 years was 56.3% (95% confidence interval, 35.3-72.8). Moderate or greater atrioventricular valve regurgitation at discharge was associated with an increased risk of reoperation (hazard ratio, 1.8; P=.03). Of transplant-free survivors, atrioventricular valve regurgitation was less than moderate in 60.0% (15/25) at the most recent follow-up.Conclusions: Atrioventricular valve surgery in patients with unbalanced atrioventricular septal defect is associated with substantial mortality and a high rate of reoperation. Successful atrioventricular valve repair is associated with better survival and freedom from reoperation.