Sha Zhao, Zhong-yuan Lu, Pantao Zhou, Li Xiong, Xiao-Bin Li
Affiliated Traditional Chinese Medicine Hospital and Southwest Medical University. Beijing No.6 Hospital. Fuwai Hospital. Gulin Municipal Hospital of Traditional Chinese Medicine.
China
BioMedical Central Cardiovascular Disorders
BMC Cardiovasc Disord 2026;
DOI: 10.1186/s12872-026-05954-y
Abstract
Background: Perioperative management of pediatric patients with congenital heart disease (CHD) and severe pulmonary arterial hypertension (PAH) remains challenging because of limited evidence-based guidelines.
Case presentation: We present the case of a 14-year-old female with a complete atrioventricular septal defect (Rastelli type A) and a single atrium with a biventricular connection. The preoperative pulmonary vascular resistance (PVR) was 6.3 Wood units after oxygen inhalation. Following a multidisciplinary evaluation, fenestrated patch closure was performed to reduce the right ventricular afterload. With standard perioperative management in place, early intravenous Treprostinil was initiated and carefully titrated. Thereafter, the patient maintained hemodynamic stability, was successfully extubated, and was discharged in stable condition.
Conclusions: Early intravenous treprostinil, combined with individualized surgical protection, may help maintain hemodynamic stability and support recovery in high-risk pediatric patients with CHD-associated PAH.
Category
Class I. Pulmonary Hypertension Associated with Congenital Cardiovascular Disease
Medical Therapy. Efficacy or Lack of Efficacy
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