Wang Xiaofeng, Wang Shilin, Lu Zhongyuan, Wang Wenlong, Wang Xu
Department of Pediatric Intensive Care Unit, National Center for Cardiovascular Disease, FuWai Hospital Chinese Academy of Medical Sciences and Peking Union Medical College Beijing China.
Pulm Circ. 2024 May 15;14(2):e12387. doi: 10.1002/pul2.12387. eCollection 2024 Apr.
Surgical indications for patients with pulmonary arterial hypertension (PAH) and congenital heart defects are controversial. The treat and repair strategy has demonstrated efficacy in adult populations, but there have been no studies on pediatric patients. This study included pediatric patients with PAH and simple congenital heart defects who underwent corrective repair between 2012 and 2021. According to the preoperative treatment strategies, the patients were divided into a regular strategy group (Group 1) and a treat-and-repair strategy group (Group 2). Postoperative recovery and follow-up results were compared between the two groups. A total of 33 patients were included in this study. Group 1 consisted of 19 patients, whereas Group 2 consisted of 14 patients. The pulmonary vascular resistance index in Group 2 was higher than that in Group 1 (10.9 ± 4.1 vs. 8.2 ± 1.6 WU, = 0.031). There were no differences in postoperative recovery between the two groups ( > 0.05). During follow-up, five patients were lost (three in Group 1 and two in Group 2). The median follow-up period was 59 months. One patient died in Group 1, and two patients died in Group 2. There was no significant difference in the survival curve ( = 0.39). At the last follow-up, another seven patients had experienced a non-low-risk condition, with a total of three non-low-risk patients in Group 1 and seven in Group 2, including one patient in each group who had a history of ICU admission. According to the ROC curve, a preoperative PVRi <8.2 WU×m can predict postoperative persistent low-risk state, PVRi <5.2 WU×m can avoid postoperative death and/or ICU administration. In pediatric patients with PAH and simple congenital heart defects, the treat and repair strategies may provide surgery opportunities, PVRi should be <8 WU×m, and <5.2 WU×m is the best choice.
肺动脉高压（PAH）合并先天性心脏缺陷患者的手术指征存在争议。治疗与修复策略已在成人患者中显示出疗效，但尚无关于儿科患者的研究。本研究纳入了2012年至2021年间接受矫正修复的PAH合并简单先天性心脏缺陷的儿科患者。根据术前治疗策略，将患者分为常规策略组（第1组）和治疗与修复策略组（第2组）。比较两组患者术后恢复情况及随访结果。本研究共纳入33例患者。第1组有19例患者，第2组有14例患者。第2组的肺血管阻力指数高于第1组（10.9±4.1 vs. 8.2±1.6 WU，P = 0.031）。两组术后恢复情况无差异（P>0.05）。随访期间，5例患者失访（第1组3例，第2组2例）。中位随访期为59个月。第1组1例患者死亡，第2组2例患者死亡。生存曲线无显著差异（P = 0.39）。在最后一次随访时，另有7例患者出现非低风险情况，第1组共有3例非低风险患者，第2组有7例，每组各有1例患者有入住重症监护病房的病史。根据ROC曲线，术前肺血管阻力指数（PVRi）<8.2 WU×m可预测术后持续低风险状态，PVRi<5.2 WU×m可避免术后死亡和/或入住重症监护病房。在PAH合并简单先天性心脏缺陷的儿科患者中，治疗与修复策略可能提供手术机会，PVRi应<8 WU×m，<5.2 WU×m是最佳选择。
