Department of Pediatrics, Washington University, St. Louis, Missouri, USA.
Department of Surgery, Washington University, St. Louis, Missouri, USA.
J Am Coll Cardiol. 2021 Aug 3;78(5):468-477. doi: 10.1016/j.jacc.2021.05.039.
The placement of a pulmonary-to-systemic arterial shunt in children with severe pulmonary hypertension (PH) has been demonstrated, in relatively small studies, to be an effective palliation for their disease.
The aim of this study was to expand upon these earlier findings using an international registry for children with PH who have undergone a shunt procedure.
Retrospective data were obtained from 110 children with PH who underwent a shunt procedure collected from 13 institutions in Europe and the United States.
Seventeen children died in-hospital postprocedure (15%). Of the 93 children successfully discharged home, 18 subsequently died or underwent lung transplantation (20%); the mean follow-up was 3.1 years (range: 25 days to 17 years). The overall 1- and 5-year freedom from death or transplant rates were 77% and 58%, respectively, and 92% and 68% for those discharged home, respectively. Children discharged home had significantly improved World Health Organization functional class (P < 0.001), 6-minute walk distances (P = 0.047) and lower brain natriuretic peptide levels (P < 0.001). Postprocedure, 59% of children were weaned completely from their prostacyclin infusion (P < 0.001). Preprocedural risk factors for dying in-hospital postprocedure included intensive care unit admission (hazard ratio [HR]: 3.2; P = 0.02), mechanical ventilation (HR: 8.3; P < 0.001) and extracorporeal membrane oxygenation (HR: 10.7; P < 0.001).
A pulmonary-to-systemic arterial shunt can provide a child with severe PH significant clinical improvement that is both durable and potentially free from continuous prostacyclin infusion. Five-year survival is comparable to children undergoing lung transplantation for PH. Children with severely decompensated disease requiring aggressive intensive care are not good candidates for the shunt procedure.
在患有严重肺动脉高压（PH）的儿童中，放置肺动脉至体动脉分流术已在相对较小的研究中证明，对其疾病是一种有效的姑息治疗。
本研究旨在通过对接受分流术的 PH 儿童国际登记处的数据进行扩展，来进一步证实这一发现。
从欧洲和美国的 13 个机构收集了 110 名接受分流术的 PH 儿童的回顾性数据。
17 名患儿术后院内死亡（15%）。93 名成功出院回家的患儿中，18 名随后死亡或接受肺移植（20%）；平均随访时间为 3.1 年（范围：25 天至 17 年）。总体 1 年和 5 年免于死亡或移植的生存率分别为 77%和 58%，出院回家的患儿分别为 92%和 68%。出院回家的患儿的世界卫生组织功能分级（P < 0.001）、6 分钟步行距离（P = 0.047）和脑利钠肽水平（P < 0.001）均显著改善。术后，59%的患儿完全停止了前列环素输注（P < 0.001）。术后院内死亡的术前危险因素包括重症监护病房收治（危险比[HR]：3.2；P = 0.02）、机械通气（HR：8.3；P < 0.001）和体外膜肺氧合（HR：10.7；P < 0.001）。
肺动脉至体动脉分流术可为患有严重 PH 的儿童提供显著的临床改善，且这种改善持久且可能无需持续使用前列环素输注。5 年生存率与接受肺移植治疗 PH 的儿童相当。需要积极重症监护治疗的严重失代偿疾病患儿不适合进行分流术。
