Open AccessA Consensus Approach to the Classification of Pediatric Pulmonary Hypertensive Vascular Disease: Report from the PVRI Pediatric Taskforce, Panama 2011
Author(s) -
Cerro Maria Jesus,
Abman Steven,
Diaz Gabriel,
Freudenthal Alexandra Heath,
Freudenthal Franz,
Harikrishnan S.,
Haworth Sheila G.,
Ivy Dunbar,
Lopes Antonio A.,
Raj J. Usha,
Sandoval Julio,
Stenmark Kurt,
Adatia Ian
Publication year - 2011
Publication title -
pulmonary circulation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.791
H-Index - 40
ISSN - 2045-8940
DOI - 10.4103/2045-8932.83456
Subject(s) - medicine , pulmonary hypertension , intensive care medicine , disease , maldevelopment , pulmonary hypoplasia , heart disease , maladaptation , presentation (obstetrics) , pediatrics , pathology , pregnancy , surgery , fetus , psychiatry , genetics , biology , anatomy
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.