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

PVRI Member Authors: Maria Jesus del Cerro Marin, Gabriel Diaz, Alexandra Heath, Dr. S. Harikrishnan, Sheila Glennis Haworth, D. Dunbar Ivy, Antonio Augusto Lopes, J. Usha Raj, Dr. Julio Sandoval, Kurt R Stenmark, Ian Adatia

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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Topics

Fetal and Neonatal Lung - Development-Function and Disease/BPD
Pediatric

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

Published in:

Pulmonary Circulation Vol 1: No 2 cover image

June 2011

Pulmonary Circulation Vol 1: No 2

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