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Peer-reviewedCC BY-NCPulmonary circulation

Salvage Use of Sotatercept for Potts Shunt in Refractory Pulmonary Hypertension.

  • Zhuoyuan Xu1
  • Mingjie Zhang2
  • Yiwei Chen1
  • Xiafeng Yu2
  • Yanjun Sun2
  • Jie Bai3
  • Zhuoming Xu2
  • Lijun Fu1
  • Hao Zhang2,4
4 affiliations
  1. 1Department of Cardiology, National Children's Medical Center, Shanghai Children's Medical Center Shanghai Jiao Tong University School of Medicine Shanghai China.
  2. 2Department of Cardiothoracic Surgery, National Children's Medical Center, Shanghai Children's Medical Center Shanghai Jiao Tong University School of Medicine Shanghai China.
  3. 3Department of Anesthesiology, National Children's Medical Center, Shanghai Children's Medical Center Shanghai Jiao Tong University School of Medicine Shanghai China.
  4. 4Shanghai Clinical Research Center for Rare Pediatric Diseases, Shanghai Institute of Pediatric Congenital Heart Disease Shanghai China.
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Abstract

The reverse Potts shunt (RPS) is a promising palliative intervention for pediatric patients with drug-refractory severe pulmonary arterial hypertension (PAH), yet it carries substantial risks of perioperative mortality and hemodynamic instability. We report two high-risk pediatric female patients who developed acute hemodynamic instability and recurrent pulmonary hypertensive crises following successful percutaneous RPS creation. Despite maximal targeted triple therapy and vasoactive support, no sustained improvement was observed. Following multidisciplinary consensus, a single salvage dose of sotatercept (0.3 mg/kg) was administered. Both patients demonstrated rapid, hemodynamic stabilization within 48 h, enabling the swift withdrawal of vasoactive support and inhaled nitric oxide within 1 week. Follow-up echocardiography revealed significant recovery of right ventricular function, and both patients were successfully weaned off treprostinil. These findings suggest that short-term perioperative sotatercept can serve as an effective acute rescue therapy to safeguard high-risk surgical interventions in critical pediatric PAH.

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Cite

BibTeXRIS
BibTeX
@article{xu2026salvage,
  title = {{Salvage Use of Sotatercept for Potts Shunt in Refractory Pulmonary Hypertension.}},
  author = {Zhuoyuan Xu and Mingjie Zhang and Yiwei Chen and Xiafeng Yu and Yanjun Sun and Jie Bai and Zhuoming Xu and Lijun Fu and Hao Zhang},
  journal = {Pulmonary circulation},
  year = {2026},
  month = oct,
  doi = {10.1002/pul2.70436},
  url = {https://europepmc.org/article/MED/42924047},
  note = {CC BY-NC},
}
RIS
TY  - JOUR
TI  - Salvage Use of Sotatercept for Potts Shunt in Refractory Pulmonary Hypertension.
AU  - Zhuoyuan Xu
AU  - Mingjie Zhang
AU  - Yiwei Chen
AU  - Xiafeng Yu
AU  - Yanjun Sun
AU  - Jie Bai
AU  - Zhuoming Xu
AU  - Lijun Fu
AU  - Hao Zhang
PY  - 2026
DA  - 2026/10/22
JO  - Pulmonary circulation
DO  - 10.1002/pul2.70436
UR  - https://europepmc.org/article/MED/42924047
AB  - The reverse Potts shunt (RPS) is a promising palliative intervention for pediatric patients with drug-refractory severe pulmonary arterial hypertension (PAH), yet it carries substantial risks of perioperative mortality and hemodynamic instability. We report two high-risk pediatric female patients who developed acute hemodynamic instability and recurrent pulmonary hypertensive crises following successful percutaneous RPS creation. Despite maximal targeted triple therapy and vasoactive support, no sustained improvement was observed. Following multidisciplinary consensus, a single salvage dose of sotatercept (0.3 mg/kg) was administered. Both patients demonstrated rapid, hemodynamic stabilization within 48 h, enabling the swift withdrawal of vasoactive support and inhaled nitric oxide within 1 week. Follow-up echocardiography revealed significant recovery of right ventricular function, and both patients were successfully weaned off treprostinil. These findings suggest that short-term perioperative sotatercept can serve as an effective acute rescue therapy to safeguard high-risk surgical interventions in critical pediatric PAH.
SN  - 2045-8932
SN  - 2045-8940
ER  -