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Pediatric Drugs
Erschienen in: Pediatric Drugs 2/2020

01.04.2020 | Review Article

Drug Treatment of Pulmonary Hypertension in Children

verfasst von: Catherine M. Avitabile, Erika E. Vorhies, David Dunbar Ivy

Erschienen in: Pediatric Drugs | Ausgabe 2/2020

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Abstract

Pulmonary arterial hypertension (PAH) is a rare disease in infants and children that is associated with significant morbidity and mortality. The disease is characterized by progressive pulmonary vascular functional and structural changes resulting in increased pulmonary vascular resistance and eventual right heart failure and death. In many pediatric patients, PAH is idiopathic or associated with congenital heart disease and rarely is associated with other conditions such as connective tissue or thromboembolic disease. PAH associated with developmental lung diseases such as bronchopulmonary dysplasia or congenital diaphragmatic hernia is increasingly more recognized in infants and children. Although treatment of the underlying disease and reversal of advanced structural changes have not yet been achieved with current therapy, quality of life and survival have improved significantly. Targeted pulmonary vasodilator therapies, including endothelin receptor antagonists, prostacyclin analogs, and phosphodiesterase type 5 inhibitors have resulted in hemodynamic and functional improvement in children. The management of pediatric PAH remains challenging as treatment decisions depend largely on results from evidence-based adult studies and the clinical experience of pediatric experts. This article reviews the current drug therapies and their use in the management of PAH in children.
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Metadaten
Titel
Drug Treatment of Pulmonary Hypertension in Children
verfasst von
Catherine M. Avitabile
Erika E. Vorhies
David Dunbar Ivy
Publikationsdatum
01.04.2020
Verlag
Springer International Publishing
Erschienen in
Pediatric Drugs / Ausgabe 2/2020
Print ISSN: 1174-5878
Elektronische ISSN: 1179-2019
DOI
https://doi.org/10.1007/s40272-019-00374-2

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