A Consensus Approach to the Classification of Pediatric Pulmonary Hypertensive Vascular Disease: Report from the PVRI Pediatric Taskforce, Panama 2011

Overview

Journal Pulm Circ

Publisher Wiley

Specialty Pulmonary Medicine

Date 2011 Aug 30

PMID 21874158

Citations 91

Authors

Maria Jesus Del Cerro

Steven Abman

Gabriel Diaz

Alexandra Heath Freudenthal

Franz Freudenthal

S Harikrishnan

Sheila G Haworth

Dunbar Ivy

Antonio A Lopes

J Usha Raj

Julio Sandoval

Kurt Stenmark

Ian Adatia

Affiliations

Soon will be listed here.

Abstract

Current classifications of pulmonary hypertension have contributed a great deal to our understanding of pulmonary vascular disease, facilitated drug trials, and improved our understanding of congenital heart disease in adult survivors. However, these classifications are not applicable readily to pediatric disease. The classification system that we propose is based firmly in clinical practice. The specific aims of this new system are to improve diagnostic strategies, to promote appropriate clinical investigation, to improve our understanding of disease pathogenesis, physiology and epidemiology, and to guide the development of human disease models in laboratory and animal studies. It should be also an educational resource. We emphasize the concepts of perinatal maladaptation, maldevelopment and pulmonary hypoplasia as causative factors in pediatric pulmonary hypertension. We highlight the importance of genetic, chromosomal and multiple congenital malformation syndromes in the presentation of pediatric pulmonary hypertension. We divide pediatric pulmonary hypertensive vascular disease into 10 broad categories.

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