Current Cardiology Reports

, 21:162 | Cite as

Management of Pulmonary Arterial Hypertension in the Pediatric Patient

  • Jordan E. Ezekian
  • Kevin D. HillEmail author
Congenital Heart Disease (RA Krasuski, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Congenital Heart Disease


Purpose of Review

Pediatric pulmonary arterial hypertension (PAH) is associated with significant morbidity and mortality. Herein we review the diagnosis and classification for pediatric PAH and detail the current therapeutic options available for use in the pediatric PAH population.

Recent Findings

Classification and treatment of pediatric PAH is guided by adult criteria and treatment algorithms, yet the distribution of factors contributing to PAH in children differs significantly from that seen in adults. It is necessary to understand these differences in order to appropriately tailor therapy to the needs of the child or adolescent. An expanding array of targeted PAH drugs are now approved for use in adults, and many of these drugs are used “off-label” to treat children and adolescents with PAH. Use of these novel therapies has coincided with marked improvement in outcomes, suggesting significant benefit. However, because most of these drugs have not been studied in rigorous randomized, controlled trials in children, it is critical that physicians understand their mechanisms of action, potential benefits, and safety profiles.


Pediatric PAH outcomes have improved substantially in the modern era, coinciding with the “off-label” use of targeted PAH drugs in children and adolescents. Ideally, care should be provided at centers with specialized expertise in the diagnosis and treatment of pediatric PAH by providers who understand the appropriate diagnostic algorithms, classification schemes, and treatment approaches.


Pediatric pulmonary arterial hypertension Pulmonary hypertension Phosphodiesterdase-5 inhibitors Endothelin receptor antagonists Nitric oxide Prostacyclins 


Compliance with Ethical Standards

Conflict of Interest

Jordan E. Ezekian declares no conflict of interest.

Kevin D. Hill reports another from Actelion (he is a site PI for a multicenter trial of macitentan for treatment of PAH in children).

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.


Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

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Authors and Affiliations

  1. 1.Department of Pediatrics, Division of Pediatric CardiologyDuke University Medical CenterDurhamUSA

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