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Paediatric pulmonary hypertension and sildenafil: current practice and controversies

A J Wardle1, R M R Tulloh

  • 1University of Bristol, Bristol, UK.

Archives of Disease in Childhood. Education and Practice Edition
|June 18, 2013
PubMed

Insights

Sildenafil is a key treatment for pediatric pulmonary hypertension, improving quality of life and survival. Despite some controversies and recent setbacks, its use in managing pediatric pulmonary hypertension remains significant.

Area of Science:

  • Pediatric Cardiology
  • Pharmacology
  • Neonatology

Background:

  • Pediatric pulmonary arterial hypertension (PAH) management has shifted towards disease-modifying strategies.
  • Sildenafil, a phosphodiesterase-V inhibitor, is central to this paradigm shift.
  • Its application extends to related conditions like persistent pulmonary hypertension of the newborn and bronchopulmonary dysplasia.

Purpose of the Study:

  • To review the evolving role of sildenafil in pediatric pulmonary hypertension.
  • To address controversies and challenges associated with its use in younger patients.
  • To examine the impact of recent data on long-term outcomes and regulatory approvals.

Main Methods:

  • Literature review of studies on sildenafil in pediatric pulmonary hypertension.
  • Analysis of clinical trial data and post-marketing surveillance.
  • Discussion of regulatory changes and their implications.

Main Results:

  • Sildenafil has demonstrated efficacy in improving quality of life and survival in pediatric PAH.
  • Alternative formulations and expanded indications have emerged.
  • Concerns regarding long-term mortality and regulatory actions have introduced complexities.

Conclusions:

  • Sildenafil remains a cornerstone in the contemporary management of pediatric pulmonary hypertension.
  • Its role is expected to persist across various clinical contexts.
  • Ongoing research and careful consideration of risks are essential for its continued use.

Keywords:
1. Pulmonary arterial hypertension2. Paediatric3. Sildenafil4. Review

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