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Pulmonary arterial hypertension: a comparison between children and adults

R J Barst1, S I Ertel, M Beghetti

  • 1Division of Paediatric Cardiology, Columbia University College of Physicians and Surgeons, 31 Murray Hill Road, Scarsdale, New York, NY 10583, USA. robyn.barst@gmail.com

The European Respiratory Journal
|March 2, 2011
PubMed

Insights

Pulmonary arterial hypertension (PAH) shares many similarities in children and adults, including pathology and symptoms. Current guidelines recommend similar diagnostic and treatment approaches for both age groups, improving survival rates.

Area of Science:

  • Pediatric Cardiology
  • Pulmonary Medicine
  • Vascular Biology

Background:

  • Pulmonary arterial hypertension (PAH) affects both children and adults, presenting with shared and unique characteristics.
  • Histopathology in pediatric PAH mirrors adult forms, with children exhibiting more medial hypertrophy and unique lung growth abnormalities contributing to the condition.

Purpose of the Study:

  • To review and compare the pathology, symptoms, diagnosis, and treatment of pulmonary arterial hypertension (PAH) in pediatric and adult populations.
  • To highlight similarities and differences in PAH presentation and management between children and adults.

Main Methods:

  • Review of existing literature on pediatric and adult pulmonary arterial hypertension (PAH).
  • Comparison of histopathology, clinical presentation, diagnostic criteria, and treatment outcomes.
  • Analysis of hemodynamic assessment and vasoreactivity definitions in different age groups.

Main Results:

  • Shared features include vascular and endothelial dysfunction, elevated pulmonary vascular resistance, and pulmonary artery pressure at diagnosis.
  • Children present with more medial hypertrophy and unique lung growth abnormalities; adults experience more heart failure with edema, while syncope is more common in children with idiopathic PAH.
  • Adult PAH therapies show efficacy in children, leading to comparable survival rates between pediatric and adult patients.

Conclusions:

  • Pulmonary arterial hypertension (PAH) in children and adults exhibits more similarities than differences.
  • Current diagnostic and therapeutic guidelines for PAH are largely applicable to both pediatric and adult populations, based on expert opinion for children and evidence for adults.

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