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Recent advances in the clinical management of end-stage pediatric pulmonary hypertension

Oscar van der Have1,2, Rachel K Hopper3, Rebecca J Kameny3

  • 1Department of Experimental Medical Science and The Wallenberg Center for Molecular Medicine, Lund University.

Current Opinion in Pulmonary Medicine
|May 20, 2026
PubMed

Insights

This review covers advanced treatments for children with end-stage pulmonary hypertension. Early assessment and intervention, including new therapies and transplantation, are crucial for improving outcomes in pediatric pulmonary vascular disease.

Area of Science:

  • Pediatric Cardiology
  • Pulmonary Vascular Disease
  • Critical Care Medicine

Background:

  • Pediatric pulmonary hypertension management is evolving with increased understanding of pulmonary vascular disease.
  • Risk prediction tools are improving, highlighting the need for serial re-assessment in children.
  • Pediatric-specific complications of pulmonary hypertension require further investigation despite adult therapy advances.

Purpose of the Study:

  • To provide an overview of recent clinical management advances for pediatric end-stage pulmonary hypertension.
  • To discuss strategies for children refractory to conventional medical therapy.
  • To highlight the importance of early consideration of advanced therapies.

Main Methods:

  • Review of current literature on pediatric pulmonary hypertension management.
  • Analysis of emerging pharmaceutical and interventional therapies.
  • Discussion of lung transplantation and mechanical circulatory support.

Main Results:

  • Advanced pharmaceutical and interventional therapies, alongside lung transplantation, improve outcomes.
  • Ongoing trials are exploring pediatric-specific pulmonary hypertension complications.
  • Interventional strategies to support the right ventricle are increasingly used, though evidence is limited.

Conclusions:

  • Management of pediatric right ventricular failure is complex, integrating hemodynamic assessment, vasodilator therapy, and ventilatory strategies.
  • Extra-corporeal membrane oxygenation can serve as a bridge to transplantation or other interventions.
  • Timely consideration of advanced therapies and mechanical support is vital for improving outcomes in pediatric pulmonary hypertension.
Abstract

Keywords:
pediatric intensive carepediatric lung transplantationpulmonary arterial hypertension

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