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Pediatric Pulmonary Arterial Hypertension: Evaluation and Treatment

  • Cardiology/CT Surgery (K Gist, Section Editor)
  • Published:
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Abstract

Purpose of Review

Pediatric pulmonary arterial hypertension (PAH) is a rare but severe disease with no cure. In this review, we discuss an approach to the evaluation and management of a pediatric patient with suspected or confirmed PAH.

Recent Findings

Proceedings published from the 2018 World Symposium updated the definition of pulmonary hypertension to include all adults and children with mean pulmonary artery pressure > 20 mmHg. A classification scheme for patients with PAH associated with congenital heart disease is now also well recognized. Recent research and guidelines have described a series of risk stratification criteria as well as specific criteria for operability in children with unrepaired systemic-to-pulmonary shunt lesions. Although multiple medications have been used off-label, in 2017, bosentan became the first targeted PAH therapy approved for use in children.

Summary

In recent years, advanced imaging and clinical monitoring have allowed improved risk stratification of PH patients. New therapies, approved in adults and used off-label in pediatric patients, have led to improved outcomes for affected children. However, no targeted pulmonary hypertension medications are approved for children < 3 years of age at this time. Future studies focused on safe, effective, pediatric-specific strategies to improve outcomes are needed.

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Funding

This review was supported by the Frederick and Margaret L. Weyerhaeuser Foundation (NIH Grant No. R01HL114753), the Jayden de Luca Foundation (NIH Grant No. U01HL121518), and the NIH/NCATS Colorado CTSA (Grant No. UL1 TR001082).

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  1. Department of Pediatrics, Section of Cardiology, University of Colorado School of Medicine, 13123 E. 16th Ave Box B100, Aurora, CO, 80045, USA

    Benjamin S. Frank MD & D. Dunbar Ivy MD

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  1. Benjamin S. Frank MD
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  2. D. Dunbar Ivy MD
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Correspondence to Benjamin S. Frank MD.

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Conflict of Interest

The University of Colorado receives fees for Dr. Ivy to be a consultant and perform clinical trials for Actelion, Bayer, Lilly and United Therapeutics. Dr. Ivy also receives research funding from the FDA and NIH. Dr. Frank declares no conflict of interest.

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All reported studies/experiments with human or animal subjects performed by the authors have been previously published and complied with all applicable ethical standards (including the Helsinki declaration and its amendments, institutional/national research committee standards, and international/national/institutional guidelines).

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Frank, B.S., Ivy, D.D. Pediatric Pulmonary Arterial Hypertension: Evaluation and Treatment. Curr Treat Options Peds 6, 12–28 (2020). https://doi.org/10.1007/s40746-020-00186-4

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