BMC Cardiovasc Disord. 2026 May 16. doi: 10.1186/s12872-026-05954-y. Online ahead of print.
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.
PMID:42143244 | DOI:10.1186/s12872-026-05954-y