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  • Review Article
  • Published:

Treatment of pulmonary arterial hypertension in children

Nature Reviews Cardiology volume 12, pages 244–254 (2015)Cite this article

Subjects

Key Points

  • Pulmonary arterial hypertension (PAH) is a symptom of various pathologies and is frequently associated with progressive deterioration; PAH is an important cause of morbidity and mortality in children

  • Current therapies for PAH target the vascular calcium channel as well as abnormalities in the endothelin, nitric oxide, and prostacyclin signalling pathways

  • Advances in basic and clinical research into PAH have led to improved understanding of disease pathogenesis and identification of novel therapeutic targets

  • Only a minority of drugs for PAH are officially approved in children, because randomized controlled studies are limited by the small number of paediatric patients with PAH

  • Interventional and surgical strategies are increasingly performed in children with severe PAH refractory to medical therapy, to relieve right ventricular pressure overload, improve left ventricular performance, and support systemic circulation

  • Developments in PAH-specific therapy as well as surgical and interventional procedures for severe PAH might delay or even avoid the need for lung transplantation

Abstract

Pulmonary arterial hypertension (PAH) is an important cause of morbidity and mortality in children. Improved understanding of the pathophysiological mechanisms of the underlying diseases has resulted in the development of effective, but not yet curative, therapies. Currently, drugs from three main pharmacological groups targeting specific aberrant pathways (endothelin, nitric oxide, and prostacyclin) and four routes of administration (inhaled, intravenous, oral, and subcutaneous) have been approved for adult patients with PAH. However, only a minority of these drugs has been officially approved for children, mainly because randomized controlled studies are limited by the small number of paediatric patients with PAH worldwide. In children with progressive, severe PAH and an inadequate response to drug therapy, advances in interventional and surgical approaches have provided promising new strategies to avoid right ventricular deterioration. These techniques can delay or even avoid the need for lung transplantation. In this Review, we present an update on developments in drug therapy for patients with PAH and highlight the current status of these treatments in children. We also describe the new surgical, interventional, and hybrid procedures, as well as their practical application in children with severe PAH.

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Figure 1: Treatment algorithm for children with idiopathic or familial pulmonary arterial hypertension.
Figure 2: Treatment strategy in patients with suprasystemic hypertension owing to borderline left ventricular morphology.
Figure 3: Contrast-enhanced angiographic images of a stented persistent ductus arteriosus (arrows) in two patients with suprasystemic pulmonary hypertension allowing decompression of the right ventricle by a right-to-left shunt through the persistent ductus arteriosus into the descending aorta (asterisks), thereby causing a decrease in pulmonary arterial pressures to systemic level.

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Authors and Affiliations

  1. Pediatric Heart Center, University Children's Hospital, Feulgenstrasse 12, Giessen, D-35392, Germany

    Heiner Latus, Dietmar Schranz & Christian Apitz

  2. Department of Biomedical Engineering, University of Maastricht, Universiteitssingel 50, Maastricht, 6229 ER, Netherlands

    Tammo Delhaas

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H.L. and C.A. researched data for the article, and all the authors discussed its content. H.L. and C.A. wrote the manuscript. T.D. and D.S. reviewed and edited the article before submission.

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Latus, H., Delhaas, T., Schranz, D. et al. Treatment of pulmonary arterial hypertension in children. Nat Rev Cardiol 12, 244–254 (2015). https://doi.org/10.1038/nrcardio.2015.6

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