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Drug Treatment of Pulmonary Hypertension in Children

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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.

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Acknowledgments

The Frederick and Margaret L Weyerhaeuser Foundation The Jayden de Luca Foundation NIH/NHLBI R01 HL114753; HL084923 UL1 TR000154 from NCATS/NIH.

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The University of Colorado receives fees for Dr Ivy to be a consultant for Actelion, Bayer, Gilead, Lilly, Pfizer, and United Therapeutics.

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Vorhies, E.E., Ivy, D.D. Drug Treatment of Pulmonary Hypertension in Children. Pediatr Drugs 16, 43–65 (2014). https://doi.org/10.1007/s40272-013-0052-2

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