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Pulmonary hypertension in infancy and childhood

Published online by Cambridge University Press:  24 May 2005

Matthias Gorenflo ,
Mathias Nelle ,
Ph. A. Schnabel  and
Michael V. Ullmann
Matthias Gorenflo
Affiliation:
Department of Paediatric Cardiology, University Medical Centre, Heidelberg, Germany
Mathias Nelle
Affiliation:
Department of Neonatology, University Medical Centre, Heidelberg, Germany
Ph. A. Schnabel
Affiliation:
Department of Pathology, University Medical Centre, Heidelberg, Germany
Michael V. Ullmann
Affiliation:
Department of Cardiac Surgery, University Medical Centre, Heidelberg, Germany
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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.

Keywords

Aetiologyprimary pulmonary hypertensionbronchopulmonary dysplasiatreatment
Type
Review
Information
Cardiology in the Young , Volume 13 , Issue 3 , June 2003 , pp. 219 - 227
Copyright
© 2003 Cambridge University Press

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