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Update on pediatric pulmonary arterial hypertension

Dunbar Ivy1, Benjamin S Frank

  • 1Section of Cardiology, Department of Pediatrics, University of Colorado School of Medicine, Aurora, Colorado, USA.

Current Opinion in Cardiology
|November 2, 2020
PubMed

Insights

Pediatric pulmonary arterial hypertension (PAH) diagnosis and treatment have advanced significantly. New therapies, including FDA-approved bosentan, are improving outcomes for children with this serious condition.

Area of Science:

  • Pediatric Cardiology
  • Pulmonary Hypertension Research
  • Medical Therapeutics

Background:

  • Pulmonary arterial hypertension (PAH) presents a significant health challenge in children, leading to severe illness and death.
  • The 2018 World Symposium redefined pulmonary hypertension (PH) criteria, setting mean pulmonary artery pressure above 20 mmHg for adults and children.

Purpose of the Study:

  • To review recent advancements in the diagnosis and treatment of pediatric pulmonary arterial hypertension.
  • To highlight the impact of new therapeutic strategies on outcomes for children with PAH.

Main Methods:

  • Review of recent scientific literature and clinical findings.
  • Analysis of updated diagnostic criteria and therapeutic approvals for PAH.
  • Examination of the use of advanced imaging and clinical monitoring in risk stratification.

Main Results:

  • Improved risk stratification for pulmonary hypertension patients through advanced imaging and clinical monitoring.
  • Increased use of targeted PAH therapies, with bosentan being the first FDA-approved targeted therapy for pediatric use in 2017.
  • Off-label use of adult-approved therapies in pediatric patients has led to better outcomes.

Conclusions:

  • Recent advancements in diagnosis and the introduction of novel therapies have improved the outlook for children with pulmonary arterial hypertension.
  • The updated definition of pulmonary hypertension and targeted therapies mark significant progress in managing pediatric PAH.
Abstract

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