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Diagnosis and treatment of pediatric pulmonary arterial hypertension

Nesrine Farhat1, Frederic Lador2,3, Maurice Beghetti1,3,4

  • 1a Pediatric Cardiology Unit , University Children's Hospital of Geneva , Geneva , Switzerland.

Expert Review of Cardiovascular Therapy
|January 31, 2019
PubMed

Insights

Pediatric pulmonary arterial hypertension (PAH) is a severe condition requiring thorough diagnosis and targeted therapies. While outcomes have improved, research continues to seek a cure for this rare disease.

Area of Science:

  • Pediatric cardiology
  • Pulmonology
  • Rare diseases

Background:

  • Pediatric pulmonary arterial hypertension (PAH) is a rare, severe condition with a poor prognosis.
  • PAH can be idiopathic, heritable, or associated with conditions like congenital heart disease.
  • Despite improved outcomes due to better diagnostics and targeted therapies, a cure remains elusive.

Purpose of the Study:

  • To provide an overview of current diagnostic and treatment strategies for pediatric PAH.
  • To highlight the need for adapted clinical studies to define optimal therapeutic approaches.
  • To emphasize ongoing research and registries that will inform future management.

Main Methods:

  • Review of current diagnostic approaches for pediatric PAH.
  • Analysis of existing and emerging targeted therapies.
  • Discussion of expert consensus and data from ongoing studies and registries.

Main Results:

  • Improved outcomes in pediatric PAH over the last decade due to enhanced diagnostics and therapies.
  • Current treatment is largely based on adult data and expert consensus.
  • Significant progress is still needed to find a cure for pediatric PAH.

Conclusions:

  • A comprehensive diagnostic approach is crucial for managing pediatric PAH.
  • Targeted therapies have improved prognosis, but a cure is not yet available.
  • Further research and adapted clinical studies are essential for advancing pediatric PAH treatment.
Abstract

Keywords:
Paediatricdiagnosispulmonary arterial hypertensiontreatment

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