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Published on: January 16, 2013
Pulmonary hypertension in infancy and childhood
Matthias Gorenflo1, Mathias Nelle, Ph A Schnabe
1Department of Paediatric Cardiology, University Medical Centre, Heidelberg, Germany. Matthias_Gorenflo@med.uni-heidelberg.de
Insights
This review covers pediatric pulmonary hypertension, focusing on left-to-right shunting, bronchopulmonary dysplasia, and primary pulmonary hypertension. Effective treatments like nitric oxide and prostanoids are discussed for improved outcomes in children.
Area of Science:
- Pediatric Cardiology
- Pulmonology
- Vascular Biology
Background:
- Pediatric pulmonary hypertension (PPH) involves functional and morphological changes in pulmonary vessels.
- Key conditions include left-to-right shunting, bronchopulmonary dysplasia, and primary pulmonary hypertension.
- Pathogenesis involves an imbalance of vasoconstricting and vasodilating mechanisms.
Purpose of the Study:
- To review current concepts in the pathogenesis of PPH in infants and children.
- To discuss contemporary management strategies for PPH.
- To highlight special considerations for left-to-right shunting, bronchopulmonary dysplasia, and primary pulmonary hypertension.
Main Methods:
- Literature review of pathogenesis and management strategies for pediatric pulmonary hypertension.
- Analysis of functional and morphological contributions to PPH.
- Evaluation of current and emerging therapeutic interventions.
Main Results:
- Nitric oxide is effective for PPH post-intra-cardiac repair with left-to-right shunting.
- Prostanoids are under investigation for left-to-right shunting and effective for primary pulmonary hypertension.
- Therapeutic strategies for PPH with bronchopulmonary dysplasia target chronic lung disease and utilize vasodilators.
Conclusions:
- Targeted therapies based on pathophysiological understanding improve PPH management.
- Further research is needed for primary pulmonary hypertension pathogenesis.
- Prostanoids show promise for improving long-term prognosis in pediatric pulmonary hypertension.
Abstract:
In this review, we discuss current concepts in the pathogenesis and management of pulmonary hypertension affecting infants and children, with special focus on left-to-right shunting, bronchopulmonary dysplasia, and primary pulmonary hypertension. In patients of these ages, functional aspects, such as an imbalance between vasoconstricting and vasodilating mechanisms, and morphological alterations of the vessel wall, contribute to the pulmonary hypertension. In the past decades, strategies have emerged for treatment that are targeted at the pathophysiological basis. Thus, in patients with left-to-right shunting and pulmonary hypertension after intra-cardiac repair, treatment with nitric oxide has been introduced effectively, while treatment with prostanoids, such as iloprost, is under investigation. In patients with pulmonary hypertension and bronchopulmonary dysplasia, therapeutic strategies focus on the underlying chronic lung disease and use of vasodilators. The pathogenesis of primary pulmonary hypertension in children remains as yet unclear, although treatment with prostanoids has proven effectively to improve the long-term prognosis.
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