Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2021 Jan;36(1):67-79.
doi: 10.1097/HCO.0000000000000822.

Update on pediatric pulmonary arterial hypertension

Dunbar Ivy  1 , Benjamin S Frank
Affiliations
Review

Update on pediatric pulmonary arterial hypertension

Dunbar Ivy et al. Curr Opin Cardiol. 2021 Jan.

Abstract

Purpose of review: Pulmonary arterial hypertension (PAH) causes high morbidity and mortality in children. In this review, we discuss advances in diagnosis and treatment of this disorder.

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 more than 20 mmHg. Targeted PAH therapy is increasingly used off-label, but in 2017, bosentan became the first Food and Drug Administration-targeted PAH therapy approved for use in children.

Summary: In recent years, advanced imaging and clinical monitoring have allowed improved risk stratification of pulmonary hypertension patients. New therapies, approved in adults and used off-label in pediatric patients, have led to improved outcomes for affected children.

PubMed Disclaimer

Similar articles

See all similar articles

Cited by

References

    1. Frank BS, Ivy DD. Diagnosis, evaluation and treatment of pulmonary arterial hypertension in children. Children 2018; 5:44.
    1. Frank BS, Ivy DD. Pediatric pulmonary arterial hypertension: evaluation and treatment. Curr Treat Opt Pediatr 2020; 6:12–28.
    1. Frank BS, Ivy DD. Pediatric pulmonary arterial hypertension. Pediatr Clin North Am 2020; 67:903–921.
    1. Ivy DD, Abman SH, Barst RJ, et al. Pediatric pulmonary hypertension. J Am Coll Cardiol 2013; 62: (25 Suppl): D117–D126.
    1. Galie N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Respir J 2015; 37:67–119.

Publication types

MeSH terms

Substances