Update on pediatric pulmonary arterial hypertension.

Abstract

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