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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.

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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.

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