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Background <p>Perioperative management of pediatric patients with congenital heart disease (CHD) and severe pulmonary arterial hypertension (PAH) remains challenging because of limited evidence-based guidelines.</p> Case presentation <p>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.</p> Conclusions <p>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.</p>

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Early postoperative treprostinil in for severe pulmonary arterial hypertension in a high-risk pediatric patient with complete atrioventricular septal defect: a case report

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  • 发表日期：

    2026年5月16日

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  • Sha Zhao,
  • Zhong-yuan Lu,
  • Pantao Zhou,
  • Li Xiong,
  • Xiao-Bin Li

摘要

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.