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Published on: February 14, 2019

Childhood idiopathic pulmonary arterial hypertension: a national cohort study

S Moledina1, A A Hislop, H Foster

  • 1Great Ormond Street Hospital for Children, London, UK.

Heart (British Cardiac Society)
|April 22, 2010
PubMed

Insights

Childhood idiopathic pulmonary arterial hypertension (IPAH) is rare, with lower incidence than in adults. Early disease severity predicts poor outcomes, but survival has improved significantly over time.

Area of Science:

  • Pediatric Cardiology
  • Pulmonology
  • Rare Diseases

Background:

  • Idiopathic pulmonary arterial hypertension (IPAH) in children is a rare condition with a poor prognosis.
  • Limited data exists on its clinical characteristics and epidemiology in pediatric populations.
  • Understanding long-term outcomes is crucial for improving patient management.

Purpose of the Study:

  • To determine the incidence and prevalence of childhood IPAH.
  • To describe the clinical features and long-term outcomes of pediatric IPAH.
  • To identify factors associated with survival in children with IPAH.

Main Methods:

  • A retrospective analysis of 7 years of data from the UK Service for Pulmonary Hypertension in Children.
  • Inclusion of 64 children diagnosed with IPAH.
  • Assessment of treatment strategies including prostanoids, bosentan, sildenafil, combination therapy, and calcium channel antagonists.

Main Results:

  • The incidence of IPAH was 0.48 cases per million children per year, with a prevalence of 2.1 cases per million.
  • Syncope was present in 31% of cases; edema was rare.
  • Survival rates at 1, 3, and 5 years were 89%, 84%, and 75%, respectively. Transplant-free survival was 89%, 76%, and 57%.

Conclusions:

  • Childhood IPAH has a lower incidence than adult IPAH and distinct clinical features.
  • Clinical status at presentation, including WHO functional class and nutritional status (height/weight z-scores), significantly predicts outcomes.
  • This 7-year study demonstrates a marked improvement in survival compared to historical data.
Abstract

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