单心室姑息治疗非均衡性房室间隔缺损的长期结果：Fontan 生存者比之前认为的要好。
Long-term outcomes of single-ventricle palliation for unbalanced atrioventricular septal defects: Fontan survivors do better than previously thought.
机构信息
Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Australia; Department of Paediatrics, University of Melbourne, Melbourne, Australia; Murdoch Children's Research Institute, Melbourne, Australia.
Department of Paediatrics, University of Melbourne, Melbourne, Australia; Murdoch Children's Research Institute, Melbourne, Australia; Department of Cardiology, Royal Children's Hospital, Melbourne, Australia.
出版信息
J Thorac Cardiovasc Surg. 2017 Feb;153(2):430-438. doi: 10.1016/j.jtcvs.2016.09.051. Epub 2016 Sep 24.
BACKGROUND
Single-ventricle palliation (SVP) for children with unbalanced atrioventricular septal defect (uAVSD) is thought to carry a poor prognosis, but limited data have been reported.
METHODS
We performed a retrospective review of children with uAVSD who underwent SVP at a single institution. Data were obtained from medical records and correspondence with general practitioners and cardiologists.
RESULTS
Between 1976 and 2016, a total of 139 patients underwent SVP for uAVSD. A neonatal palliative procedure was performed in 83.5% of these patients (116 of 139), and early mortality occurred in 11.2% (13 of 116). Ninety-four patients underwent stage II palliation, with an early mortality of 6.4% (6 of 94). Eighty patients (57.6%) underwent Fontan completion, with an early mortality of 3.8% (3 of 80). Interstage mortality was 11.7% (12 of 103) between stages I and II and 17.0% (15 of 88) between stage II and Fontan. Long-term survival was 66.5% (95% confidence interval [CI], 57.9%-73.9%) at 5 years, 64.4% (95% CI, 55.5%-72.0%) at 15 years, and 57.8% (95% CI, 47.5%-66.8%) at 25 years. Survival post-Fontan was 94.9% (95% CI, 86.9%-98.0%) at 5 years, 92.0% (95% CI, 80.6%-96.8%) at 15 years, and 82.4% (95% CI, 61.5%-92.6%) at 25 years. Risk factors associated with death or transplantation were aortic atresia (hazard ratio [HR], 5.3; P = .03) and hypoplastic aortic arch (HR, 2.5; P = .02). Atrioventricular valve operations were required in 31.7% of the patients (44 of 139), with 31.8% of them (14 of 44) requiring a further operation.
CONCLUSIONS
Children undergoing SVP for uAVSD have substantial mortality, with <60% survival at 25 years. However, survival of children who achieve Fontan completion is better than has been reported previously.
背景
对于患有房室间隔缺损伴非均衡型（uAVSD）的儿童，单心室姑息治疗（SVP）被认为预后较差，但目前仅有有限的数据报道。
方法
我们对在一家医院接受 uAVSD 单心室姑息治疗的患儿进行了回顾性分析。数据来自病历以及与全科医生和心脏病专家的通信。
结果
1976 年至 2016 年期间，共有 139 例 uAVSD 患儿接受了 SVP 治疗。其中 83.5%（116/139）的患儿接受了新生儿姑息性手术，早期死亡率为 11.2%（13/116）。94 例患儿接受了二期姑息治疗，早期死亡率为 6.4%（6/94）。80 例（57.6%）患儿接受了 Fontan 完成手术，早期死亡率为 3.8%（3/80）。I 期至 II 期的中期死亡率为 11.7%（12/103），II 期至 Fontan 的中期死亡率为 17.0%（15/88）。I 期至 II 期的长期死亡率为 11.7%（12/103），II 期至 Fontan 的长期死亡率为 17.0%（15/88）。5 年时的长期生存率为 66.5%（95%可信区间[CI]，57.9%-73.9%），15 年时为 64.4%（95% CI，55.5%-72.0%），25 年时为 57.8%（95% CI，47.5%-66.8%）。Fontan 完成术后的 5 年生存率为 94.9%（95% CI，86.9%-98.0%），15 年生存率为 92.0%（95% CI，80.6%-96.8%），25 年生存率为 82.4%（95% CI，61.5%-92.6%）。死亡或移植的相关危险因素为主动脉瓣闭锁（危险比[HR]，5.3；P=0.03）和主动脉弓发育不良（HR，2.5；P=0.02）。需要进行三尖瓣手术的患儿占 31.7%（139 例中的 44 例），其中 31.8%（44 例中的 14 例）需要进一步手术。
结论
对于患有房室间隔缺损伴非均衡型的儿童，接受单心室姑息治疗的死亡率很高，25 年生存率不到 60%。然而，Fontan 完成手术患儿的生存率优于之前的报道。
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