Abstract

Pulmonary hypertension (PH) is a deadly cardiovascular disease that follows a progressive and symptomatic course. This disease presents in approximately 15 cases per million each year and is categorized into five different groups based on established or presumed etiology. The poorly understood role of BMPR II mutation, early onset and severity of symptoms and increased fatality rate associated with IPAH make treatment a challenge for physicians. Current treatment options focus on the three main mechanisms of endothelial dysfunction which is the hallmark of PAH, namely Prostacyclin, Endothelin-1, and Nitric oxide pathways. This review summarizes different mechanisms of action for drugs that are currently available, recently approved or still under investigation. Despite all the progress made in the past decade, a number of obstacles still exist in the way of optimal treatments and outcomes. Currently available drugs (Amlodipine, Ambrisentan, and Sildenafil) may no longer be as effective as the newer generation of drugs, due to better outcomes and favorable modification of disease profile. Some clinical trial drugs have shown significant health improvements in IPAH patients by prolonging vasodilation and inhibiting vasoconstriction. Understanding the mechanism of action for IPAH still remains the key for researchers and physicians to develop novel drugs to treat PAH patients thereby increasing survival rates.

Authors and Affiliations