Pulmonary arterial hypertension (PAH) continues to be a condition associated with a high morbidity and mortality despite the advances in treatment strategies over the past 25 years. Although defined by strict hemodynamic criteria, PAH is a syndrome based on diverse etiologies and pathogenesis. PAH is characterized by excessive pulmonary vascular remodeling, pulmonary arterial obstruction and elevated pulmonary vascular resistance (PVR), which result in a marked increase in right ventricle (RV) afterload. Eventually, the RV is not able to cope with the increased load and HF develops.

Currently, available treatments for PAH were developed to restore the imbalance of vasoactive factors, arising as a result of endothelial and smooth muscle cell (SMC) dysfunction, and primarily working by inducing vasodilation. These traditional medications include the prostacyclin analogues and receptor agonists, phosphodiesterase 5 inhibitors, endothelin-receptor antagonists, and cGMP activators. The progress observed in medical therapy of PAH patients over the past 15 years is not related to the discovery of new pathways, but to the evolution and testing of new drugs and strategies for combination therapy, and on escalation of treatments based on systematic assessment of clinical response. These medications prolong life, but mortality remains unacceptably high. Although PAH is a disorder of the pulmonary vasculature, RV function is the main prognostic marker, and should therefore also be the focus of more detailed analyses for novel drug developments in PAH.

Given the success of the first volume, this second volume will continue to focus on the recent advances in PAH research. We encourage investigators to submit original (basic and translational) research articles and reviews to this Research Topic with the purpose to address mechanistic insights, and to decipher novel diagnostic tools and treatments aimed at PAH.

Topics include, but are not limited to:

1) Cellular, molecular, and/or hemodynamic mechanisms as therapeutic targets in PAH.

2) Targeting Epigenetics for PAH therapy.

3) Potential new treatments for PAH.

4) Repurposing established drugs for the resolution of PAH.

5) Metabolic drugs in PAH.

6) Restoring microbiota in PAH.

7) New perspectives on direct therapies for right heart failure in PAH.

Are you interested in contributing a manuscript to this Research Topic?

Therapeutics in Pulmonary Arterial Hypertension: Volume II