Xiafeng Yu, Yanjun Sun, Lijun Fu, Zhuoming Xu, Yumin Zhong, Wenjing Hong, Yi Yan, Yinyu Yang, Hao Zhang
Shanghai Children’s Medical Center and Shanghai Jiao Tong University School of Medicine.
China
Journal of the American College of Cardiology Case Reports
JACC Case Rep 2025; 30:
DOI: 10.1016/j.jaccas.2025.105153
Abstract
Background: Infantile idiopathic pulmonary arterial hypertension complicated by recurrent episodes of pulmonary hypertensive crisis is an extremely rare and critical condition with high mortality.
Case summary: We present the case of a 5-month-old infant with a pulmonary hypertension crisis refractory to medical therapy, who underwent successful treatment with an emergency reversed Potts shunt (RPS), along with postoperative venopulmonary extracorporeal membrane oxygenation (V-P ECMO) support.
Discussion: From an imaging perspective, the mechanism by which RPS improves symptoms in patient with sustained pulmonary hypertension crises is explained. Additionally, this is the first reported case of successful postoperative use of V-P ECMO support after RPS.
Take-home messages: It is important to recognize the importance of RPS and V-P ECMO in the treatment of pulmonary hypertension crises. By comparing pre- and postoperative imaging data, the mechanism by which RPS improves cardiac function is better understood.
Category
Class I. Heritable Pulmonary Hypertension
Surgical and Catheter-mediated Interventions for Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes