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Published on: October 19, 2013
Paediatric pulmonary vascular disease
Irene M Lang1, Diana Bonderman, Meinhard Kneussl
1Department of Adult Cardiology, Medical University of Vienna, Vienna, Austria. irene.lang@univie.ac.at
Insights
Pulmonary vascular disease affects intrinsic pulmonary vessels, potentially causing pulmonary hypertension (PH). This review examines pediatric PH based on the 2003 World Health Organization classification.
Area of Science:
- Pediatric Cardiology
- Pulmonology
- Vascular Biology
Background:
- Pulmonary vascular disease involves pathologies of pulmonary arteries and veins.
- Pulmonary hypertension (PH) is defined as mean pulmonary arterial pressure >25 mmHg.
- PH can be pre-capillary or post-capillary, affecting resistance vessels or venous pressure.
Purpose of the Study:
- To review pulmonary vascular disease in children.
- To classify pediatric PH using the 2003 World Health Organization (Venice) classification.
Main Methods:
- Review of existing literature on pediatric pulmonary vascular disease.
- Application of the 2003 WHO classification criteria for PH.
Main Results:
- Pulmonary vascular disease can lead to increased pulmonary vascular resistance.
- Pathological changes include vasoconstriction, remodeling, and thrombosis.
- The primary consequence of increased resistance is right ventricular failure.
Conclusions:
- Pulmonary vascular disease in children requires classification for accurate diagnosis and management.
- The 2003 WHO classification provides a framework for understanding pediatric PH.
- Understanding the mechanisms of PH is crucial for addressing right ventricular dysfunction.
Abstract:
Pulmonary vascular disease comprises any congenital or acquired pathology of the intrinsic pulmonary vessels with the unique feature of pulmonary arteries carrying unsaturated blood and pulmonary veins carrying oxygenated blood. Pulmonary hypertension (PH) ensues when pulmonary vascular disease affects at least 50% of the capillary resistance vessels, i.e. pulmonary pre-acinar and intra-acinar arteries (so-called pre-capillary PH), or when pressure in the pulmonary venous system distal to the capillaries rises above a mean of 15 mmHg (so-called post-capillary PH). PH is defined by a mean pulmonary arterial pressure above 25 mmHg at rest. Vasoconstriction, remodelling and thrombosis of small pulmonary arteries lead to an increase in pulmonary vascular resistance. The consequence is failure of the afterload-intolerant right ventricle. In this review, pulmonary vascular disease in children will be addressed according to the 2003 World Health Organisation (Venice) classification of PH.
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