Treating pulmonary hypertension in pediatrics

Abstract

Introduction: Pulmonary hypertension is a hemodynamic condition occurring rarely in pediatrics. Nevertheless, it is associated with significant morbidity and mortality. When characterized by progressive pulmonary vascular structural changes, the disease is called pulmonary arterial hypertension (PAH). It results in increased pulmonary vascular resistance and eventual right ventricular failure. In the vast majority of cases, pediatric PAH is idiopathic or associated with congenital heart disease, and, contrary to adult PAH, is rarely associated with connective tissue, portal hypertension, HIV infection or thromboembolic disease.

Areas covered: This article reviews the current drug therapies available for the management of pediatric PAH. These treatments target the recognized pathophysiological pathways of PAH with endothelin-1 receptor antagonists, prostacyclin analogs and PDE type 5 inhibitors. New treatments and explored pathways are briefly discussed.

Expert opinion: Although there is still no cure for PAH, quality of life and survival have been improved significantly with specific drug therapies. Nevertheless, management of pediatric PAH remains challenging, and depends mainly on results from adult clinical trials and pediatric experts. Further research on PAH-specific treatments in the pediatric population and data from international registries are needed to identify optimal therapeutic strategies and treatment goals in the pediatric population.

Keywords:

• pediatrics
• pulmonary hypertension
• registries
• strategy
• treatment goals
• treatments

Acknowledgments

The authors would like to thank Catherine Torriani Hammon and Chloé Mégevand Lador for technical support and help in revising the article.

Declaration of interest

F Lador has served as consultant and/or advisory board member for Novartis and Beyer Schering, has received conference fees from Actelion, Bayer-Schering, Lilly, GlaxoSmithKline, Novartis and Pfizer, Inc., and has received an educational grant from GlaxoSmithKline. M Beghetti has served as a consultant and/or advisory board member for Actelion, Bayer-Schering, Lilly, GlaxoSmithKline, Novartis and Pfizer, Inc. and has received investigator-initiated research funding from Actelion and Beyer-Schering. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

Notes

This box summarizes key points contained in the article.