Key result
Early postoperative IV treprostinil with fenestrated patch closure maintains hemodynamic stability in severe CHD-associated PAH.
Why the study?
Perioperative management of pediatric patients with congenital heart disease and severe pulmonary arterial hypertension remains challenging due to limited evidence-based guidelines.
Case Report (n=1)
No
Early intravenous treprostinil may help maintain hemodynamic stability and support recovery in high-risk pediatric patients with CHD-associated PAH undergoing surgery.
May inform individualized perioperative strategies in complex pediatric CHD-PAH; hypothesis-generating and requires prospective validation.
Perioperative management of pediatric patients with congenital heart disease (CHD) and severe pulmonary arterial hypertension (PAH) remains challenging because of limited evidence-based guidelines. 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. 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.
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Zhao et al. (2026) conducted a case report in Complete atrioventricular septal defect with severe pulmonary arterial hypertension (n=1). Intravenous Treprostinil was evaluated on Hemodynamic stability and pulmonary artery pressure. Early postoperative intravenous treprostinil, combined with fenestrated patch closure, maintained hemodynamic stability and facilitated successful extubation in a 14-year-old with severe CHD-associated PAH.
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