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Updated: Jul 14, 2026

Increasing Pulmonary Artery Pulsatile Flow Improves Hypoxic Pulmonary Hypertension in Piglets
Published on: May 11, 2015
[Pulmonary arterial hypertension in children: new therapeutic approaches]
1Unité de cardiologie pédiatrique, département de pédiatrie, hôpital des enfants, hôpitaux universitaires de Genève, Genève, Switzerland. maurice.beghetti@hcuge.ch <maurice.beghetti@hcuge.ch>
Insights
Pediatric pulmonary arterial hypertension (PAH) treatments have advanced significantly due to better understanding of its mechanisms. New therapies target endothelial dysfunction by balancing vasodilatory and vasoconstrictive substances.
Area of Science:
- Cardiology
- Pediatric Pulmonology
- Pharmacology
Context:
- Pulmonary arterial hypertension (PAH) is a major cause of illness and death in pediatric cardiopulmonary diseases.
- Recent advancements in understanding PAH pathophysiology have revolutionized treatment strategies.
Purpose:
- To review novel therapeutic approaches for pediatric pulmonary arterial hypertension (PAH).
- To discuss the application of new PAH therapies in children, building on adult treatment successes.
Summary:
- Endothelial dysfunction in PAH involves reduced vasodilators (prostacyclins, nitric oxide) and increased vasoconstrictors (endothelin, thromboxanes).
- Current treatments aim to restore balance by administering prostacyclins, nitric oxide, and endothelin receptor antagonists.
Impact:
- These new therapies offer improved management for pediatric PAH, including idiopathic, familial, and associated forms.
- The review highlights the rapid translation of adult PAH treatment advancements to the pediatric population.
Abstract:
Pulmonary arterial hypertension (PAH) contributes significantly to the morbidity and mortality of diverse cardiopulmonary disorders in paediatrics. The treatment of PAH has dramatically changed over the last decade. A better understanding of the pathophysiological mechanisms has lead to the introduction of several new therapies both in the field of idiopathic and familial PAH or PAH associated with underlying conditions (i.e. congenital heart disease, connective tissue disease, HIV) as well as in the field of acute PAH. The endothelial dysfunction, considered a one of the striking problems of this disease, is characterized by a decreased production of vasodilatory-antiproliferative substances (prostacyclins, nitric oxide) and increased production of vasoconstrictive-proliferative substances (endothelin, thromboxanes). It is currently possible to try to palliate to these deficits by administering prostacyclins or nitric oxide, as well as antagonizing the effect of endothelin through endothelin receptor antagonists. These progresses have first appeared in the adult population but have been rapidly applied to the paediatric population. This short review will discuss these new therapeutic approaches and refers to several large reviews recently published for a more comprehensive description.
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