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J Am Coll Cardiol. 2013 Dec 24;62(25 Suppl):D117-26. doi: 10.1016/j.jacc.2013.10.028.

Pediatric pulmonary hypertension.

Author information

1
Pediatric Cardiology, Children's Hospital Colorado, University of Colorado School of Medicine, Aurora, Colorado. Electronic address: dunbar.ivy@childrenscolorado.org.
2
Pediatric Pulmonary Medicine, Children's Hospital Colorado, University of Colorado School of Medicine, Aurora, Colorado.
3
Columbia University, College of Physicians and Surgeons, New York, New York.
4
Centre for Congenital Heart Diseases, Pediatric Cardiology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.
5
Centre de Référence Malformations Cardiaques Congénitales Complexes, Necker Hospital for Sick Children, Assistance Publique des Hôpitaux de Paris, Pediatric Cardiology, University Paris Descartes, Paris, France.
6
Department of Biostatistics, University of Washington, Seattle, Washington.
7
Great Ormond Street Hospital, London, United Kingdom.
8
Department of Pediatrics, University of Illinois at Chicago, Chicago, Illinois.
9
Department of Pediatrics, University of California Davis Children's Hospital, Davis, California.
10
Pediatric Cardiology Unit, University Hospital, Geneva, Switzerland.

Abstract

Pulmonary hypertension (PH) is a rare disease in newborns, infants, and children that is associated with significant morbidity and mortality. In the majority of pediatric patients, PH is idiopathic or associated with congenital heart disease and rarely is associated with other conditions such as connective tissue or thromboembolic disease. Incidence data from the Netherlands has revealed an annual incidence and point prevalence of 0.7 and 4.4 for idiopathic pulmonary arterial hypertension and 2.2 and 15.6 for pulmonary arterial hypertension, respectively, associated with congenital heart disease (CHD) cases per million children. The updated Nice classification for PH has been enhanced to include a greater depth of CHD and emphasizes persistent PH of the newborn and developmental lung diseases, such as bronchopulmonary dysplasia and congenital diaphragmatic hernia. The management of pediatric PH remains challenging because treatment decisions continue to depend largely on results from evidence-based adult studies and the clinical experience of pediatric experts.

KEYWORDS:

APAH-CHD; AVT; CHD; HPAH; IPAH; PAPm; PH; PHVD; PPHN; PVR; SVR; acute vasodilator testing; congenital heart disease; hereditary pulmonary arterial hypertension; idiopathic pulmonary arterial hypertension; mean pulmonary artery pressure; pediatrics; persistent pulmonary hypertension of the newborn; pulmonary arterial hypertension associated with congenital heart disease; pulmonary hypertension; pulmonary hypertensive vascular disease; pulmonary vascular resistance; systemic vascular resistance

Republished in

PMID:
24355636
DOI:
10.1016/j.jacc.2013.10.028
[Indexed for MEDLINE]
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