2021 Volume 37 Issue 3 Pages 173-183
Despite deeper understanding of the pathophysiology of pulmonary artery hypertension (PAH), idiopathic and heritable PAH remains fatal. Specific therapies have advanced the clinical management of this challenging disease. Clinical trials have provided evidence of the effectiveness of targeted therapies, but data are limited for pediatric patients with PAH. Potential therapeutic approaches have been assessed, such as new pulmonary vasodilators, cell-based therapy and devices. The present investigation describes the novel and potential treatments that are available or under development. This paper provides an overview of the novel treatment options for PAH.