Center for Congenital Heart Diseases, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
Great Ormond Street Hospital for Children, London, UK.
Heart. 2017 Aug;103(16):1244-1249. doi: 10.1136/heartjnl-2016-310624. Epub 2017 Jan 23.
Paediatric pulmonary arterial hypertension (PAH) after neonatal arterial switch operation (ASO) for transposition of the great arteries (TGA) is a clinically recognised entity with an estimated incidence of 0.6%-1.0%. Nevertheless, a clinical characterisation is lacking. We present an international cohort of children with PAH after neonatal ASO for TGA and describe epidemiology and clinical course.
Data were collected of children with PAH after neonatal ASO (≤6 weeks after birth) for simple TGA without residual shunt defects, identified in four national paediatric PAH networks in Europe and one US referral centre.
Twenty-five children were identified between 1989 and 2014. In 17 children (68%), PAH was detected <1 year after ASO. In the remaining children, PAH was detected after median 64 months (IQR 19.5, 94.5). Nineteen children (96%) received PAH-targeted therapies. During follow-up after ASO (median 5.2 years), eight children died, four underwent lung transplantation and two received a Potts shunt. 1-year and 5-year Potts shunt- and transplantation-free survival after ASO was 100% and 73%. From first PAH detection, this was 100% and 58%, respectively, which did not differ between children with early (<1 year after ASO) or late PAH detection.
The occurrence of PAH after ASO for TGA represents a specific association. PAH onset may be early or late after ASO, with similar fatal course from first PAH detection. Mechanisms leading to PAH in this association are unknown, but may include abnormal prenatal pulmonary haemodynamics and/or genetic susceptibility. Routine, lifelong follow-up for children who undergo ASO for TGA should include screening for PAH.
新生儿动脉调转术（ASO）治疗大动脉转位（TGA）后发生的儿科肺动脉高压（PAH）是一种已被临床认可的病症，其发病率估计为 0.6%-1.0%。然而，目前对其临床特征还缺乏了解。我们报告了一组国际儿童病例，他们在新生儿期接受 ASO 治疗 TGA 后发生了 PAH，并描述了其流行病学和临床病程。
我们在欧洲的四个儿科肺动脉高压网络和一个美国转诊中心收集了在新生儿期（出生后≤6 周）接受 ASO（无残余分流缺陷的单纯 TGA）后发生 PAH 的儿童的数据。
在 1989 年至 2014 年期间，共确定了 25 名儿童。在 17 名儿童（68%）中，ASO 后<1 年发现了 PAH。在其余儿童中，PAH 是在中位时间 64 个月（IQR 19.5，94.5）后发现的。19 名儿童（96%）接受了针对 PAH 的治疗。在 ASO 后（中位 5.2 年）的随访期间，有 8 名儿童死亡，4 名接受了肺移植，2 名接受了 Potts 分流术。ASO 后 1 年和 5 年的 Potts 分流术和移植无失败生存率分别为 100%和 73%。从首次 PAH 检测开始，这分别为 100%和 58%，这在 ASO 后早期（<1 年）和晚期发现 PAH 的儿童之间没有差异。
TGA 患者接受 ASO 后发生 PAH 代表一种特定的关联。PAH 发病可以在 ASO 后早期或晚期发生，从首次 PAH 检测开始，其致命病程相似。导致这种关联中发生 PAH 的机制尚不清楚，但可能包括异常的产前肺血流动力学和/或遗传易感性。所有接受 TGA ASO 的儿童都应进行常规的、终生的随访，包括 PAH 的筛查。
