Recent Advances in the Diagnosis and Management of Pulmonary Arterial Hypertension.

Abstract

Pulmonary arterial hypertension (PAH) is a rare, progressive, debilitating and life shortening condition characterized by raised pulmonary arterial pressures. PAH includes a group of conditions sharing similar pathophysiology, clinical features and response to therapy. The commonest sub-groups of PAH include idiopathic pulmonary arterial hypertension (IPAH), and PAH associated with connective tissue disease. Recently published international guidelines emphasize the need for disease awareness and early referral to expert centres in suspected cases. Following diagnosis and careful risk stratification, combination therapy is recommended using drugs targeting the nitric oxide, endothelin and prostacyclin signaling pathways. Promising new therapies are on the horizon, however, the survival remains disappointing with a median survival of 7 years. In this review, we focus on the diagnostic evaluation, risk stratification, available treatment options and future directions in PAH.