Journal article
Treating pulmonary hypertension in pediatrics.
- Lador F Hôpitaux Universitaires de Genève, Département des Spécialités de Médecine, Service de Pneumologie, Programme Hypertension Pulmonaire , Geneva , Switzerland.
- Sekarski N
- Beghetti M
- 2015-02-11
Published in:
- Expert opinion on pharmacotherapy. - 2015
pediatrics
pulmonary hypertension
registries
strategy
treatment goals
treatments
Child
Drug Therapy, Combination
Endothelin Receptor Antagonists
Epoprostenol
HIV Infections
Humans
Hypertension, Pulmonary
Phosphodiesterase 5 Inhibitors
Quality of Life
English
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.
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.
- Language
-
- English
- Open access status
- closed
- Identifiers
-
- DOI 10.1517/14656566.2015.1013937
- PMID 25665593
- Persistent URL
- https://sonar.ch/global/documents/205979
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