Therapeutic effects of treat and repair strategy in pediatric patients with pulmonary arterial hypertension and simple congenital heart defects

Xiaofeng Wang; Shilin Wang; Zhongyuan Lu; Wenlong Wang; Xu Wang

doi:10.1002/pul2.12387

Therapeutic effects of treat and repair strategy in pediatric patients with pulmonary arterial hypertension and simple congenital heart defects

Pulm Circ. 2024 May 15;14(2):e12387. doi: 10.1002/pul2.12387. eCollection 2024 Apr.

Authors

Xiaofeng Wang¹, Shilin Wang¹, Zhongyuan Lu¹, Wenlong Wang¹, Xu Wang¹

Affiliation

¹ 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.

Abstract

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, p = 0.031). There were no differences in postoperative recovery between the two groups (p > 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 (p = 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×m² can predict postoperative persistent low-risk state, PVRi <5.2 WU×m² 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×m², and <5.2 WU×m² is the best choice.

Keywords: congenital heart disease; follow‐up; pediatric patients; pulmonary arterial hypertension; treat and repair strategy.