Treatment options for paediatric pulmonary arterial hypertension

R M F Berger*, D Bonnet

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

13 Citations (Scopus)
52 Downloads (Pure)

Abstract

Pulmonary arterial hypertension (PAH) is a serious, progressive condition, which can present idiopathically or secondary to conditions such as systemic sclerosis or congenital heart disease. The condition exists in both adult and paediatric forms, which possess several similar characteristics. Adult and paediatric PAH can, however, be distinguished based on underlying pathology and the presence of age-specific conditions, some of which are related to poor lung development in children. Improved knowledge of vascular biology has led to the development of several PAH-specific therapies, which have demonstrated clinical benefits in adults, including improved exercise capacity and prolonged survival. Treatment data in paediatric PAH are scarce. Although limited, the existing data indicate that current treatments for paediatric PAH are well tolerated and effective, at least in the short- and medium-term. Nevertheless, the current guidelines for clinicians, which recommend use of the adult treatment algorithm in paediatric patients, appear justified when judged according to the available evidence. However, further randomised, controlled trials are necessary to increase the evidence base for treatment of paediatric PAH, especially in relation to age-specific conditions. At present, early initiation of treatment and combination pharmacological therapy may offer the most promising courses of action to improve outcomes in paediatric PAH.

Original languageEnglish
Pages (from-to)321-30
Number of pages10
JournalEuropean Respiratory Review
Volume19
Issue number118
DOIs
Publication statusPublished - Dec-2010

Keywords

  • Adolescent
  • Age Factors
  • Antihypertensive Agents
  • Child
  • Child, Preschool
  • Drug Therapy, Combination
  • Humans
  • Hypertension, Pulmonary
  • Survival Rate
  • Treatment Outcome
  • Vasodilator Agents

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