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Pediatr Dev Pathol. 2008 May-Jun;11(3):169-77. doi: 10.2350/07-12-0398.1. Epub 2008 Feb 14.

Pulmonary arterial hypertension from a pediatric perspective.

Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society

Carlyne D Cool, Gail Deutsch


  1. University of Colorado Health Sciences Center, Department of Pathology, Denver, CO, USA. [email protected]

PMID: 18275254 DOI: 10.2350/07-12-0398.1


This review of pediatric pulmonary arterial hypertension provides a framework within which to view pulmonary hypertension in children. Classification schemes, including the latest recommendations from the World Health Organization, are discussed, and the histopathology of severe pulmonary hypertension is reviewed. New information is provided regarding idiopathic and familial forms of the disease. Specific childhood etiologies, including persistent pulmonary hypertension of the newborn and congenital heart disease, are reviewed. Additionally, we examine the role of collagen vascular diseases, portal hypertension, and viruses in the pathogenesis of severe pulmonary arterial hypertension.

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