Pulmonary arterial hypertension (PAH) includes a heterogeneous group of diseases characterized by pulmonary vasoconstriction and remodeling of the lung circulation. Although PAH is a disease of the lungs, patients with PAH frequently die of right heart failure. Indeed, it has been increasingly recognized that patient survival depends on the adaptive response of the right ventricle to the changes in the lung circulation. Many PAH specific drugs can affect the function of right ventricle, however whether these drug effects are direct or indirect remains incompletely understood.

In this review, we sought to recapitulate some of the known effects of standard PAH treatments on the heart, as well as to explore potentially new additional therapies to directly treat right ventricular failure.