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Paediatr Drugs. 2014 Feb;16(1):43-65. doi: 10.1007/s40272-013-0052-2.

Drug treatment of pulmonary hypertension in children.

Author information

1
Division of Pediatric Cardiology, Department of Pediatrics and Communicable Diseases, University of Michigan Medical School, C.S. Mott Children's Hospital, Ann Arbor, MI, USA.

Abstract

Pulmonary arterial hypertension (PAH) is a rare disease in infants and children that is associated with significant morbidity and mortality. The disease is characterized by progressive pulmonary vascular functional and structural changes resulting in increased pulmonary vascular resistance and eventual right heart failure and death. In the majority of pediatric patients, PAH is idiopathic or associated with congenital heart disease and rarely is associated with other conditions such as connective tissue or thromboembolic disease. Although treatment of the underlying disease and reversal of advanced structural changes has not yet been achieved with current therapy, quality of life and survival have been improved significantly. Targeted pulmonary vasodilator therapies, including endothelin receptor antagonists, prostacyclin analogs, and phosphodiesterase type 5 inhibitors, have demonstrated hemodynamic and functional improvement in children. The management of pediatric PAH remains challenging, as treatment decisions continue to depend largely on results from evidence-based adult studies and the clinical experience of pediatric experts. This article reviews the current drug therapies and their use in the management of PAH in children.

PMID:
24114695
PMCID:
PMC3946901
DOI:
10.1007/s40272-013-0052-2
[Indexed for MEDLINE]
Free PMC Article

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