title: A consensus approach to the classification of pediatric pulmonary hypertensive vascular disease: Report from the PVRI Pediatric Taskforce, Panama 2011.
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.
authors.affiliation: La Paz Children's Hospital, Madrid, Spain.
authors.forename: Maria Jesus Del
authors.initials: MJ
authors.lastname: Cerro
authors.forename: Steven
authors.initials: S
authors.lastname: Abman
authors.forename: Gabriel
authors.initials: G
authors.lastname: Diaz
authors.forename: Alexandra Heath
authors.initials: AH
authors.lastname: Freudenthal
authors.forename: Franz
authors.initials: F
authors.lastname: Freudenthal
authors.forename: S
authors.initials: S
authors.lastname: Harikrishnan
authors.forename: Sheila G
authors.initials: SG
authors.lastname: Haworth
authors.forename: Dunbar
authors.initials: D
authors.lastname: Ivy
authors.forename: Antonio A
authors.initials: AA
authors.lastname: Lopes
authors.forename: J Usha
authors.initials: JU
authors.lastname: Raj
authors.forename: Julio
authors.initials: J
authors.lastname: Sandoval
authors.forename: Kurt
authors.initials: K
authors.lastname: Stenmark
authors.forename: Ian
authors.initials: I
authors.lastname: Adatia