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Current and advancing treatments for pulmonary arterial hypertension in childhood

Willemijn M H Zijlstra1, Mark-Jan Ploegstra, Rolf M F Berger

  • 1Center for Congenital Heart Diseases, Department of Pediatric Cardiology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, P.O. Box 30.001, 9700 RB Groningen, The Netherlands.

Expert Review of Respiratory Medicine
|July 23, 2014
PubMed

Insights

Pediatric pulmonary arterial hypertension (PAH) treatment lacks specific guidelines. This review examines current PAH therapies and their limited pediatric data, aiming to guide effective treatment strategies for children.

Area of Science:

  • Cardiology
  • Pediatric Pulmonology
  • Pharmacology

Background:

  • Pulmonary arterial hypertension (PAH) is a severe lung vasculature disease.
  • Current PAH treatments and guidelines are primarily based on adult data.
  • Pediatric PAH presents unique characteristics, making adult guideline extrapolation hazardous.

Purpose of the Study:

  • To review current PAH treatments and their efficacy/safety in children.
  • To address the lack of pediatric-specific treatment strategies and response assessment.
  • To discuss potential treatment goals for pediatric PAH.

Main Methods:

  • Literature review of existing PAH treatments.
  • Analysis of available pediatric data on efficacy and safety.
  • Discussion of treatment goals and strategy translation for pediatric PAH.

Main Results:

  • Limited efficacy and safety data exist for current PAH treatments in children.
  • Pediatric data on treatment response assessment is virtually absent.
  • Significant differences exist between adult and pediatric PAH disease characteristics.

Conclusions:

  • There is a critical need for evidence-based treatment strategies in pediatric PAH.
  • Current adult guidelines cannot be directly extrapolated to children.
  • Further research is required to establish optimal pediatric PAH management.

Keywords:
PAH-targeted drugsgoal-oriented treatmentpediatricspulmonary arterial hypertensionsurvivaltreatment goal

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