Pulmonary hypertension is an uncommon disease that carries a significant morbidity and mortality. Pulmonary arterial hypertension is a subtype of pulmonary hypertension that describes a group of disease entities that lead to an elevation in precapillary pulmonary artery pressure. Despite advances in the diagnosis and treatment of pulmonary arterial hypertension, it remains a difficult disease to recognize and manage. In this review article, we will discuss the definition and diagnosis of pulmonary arterial hypertension. Additionally, we will discuss the ever-expanding management options, their mechanisms and strategies, including combination therapy and the most recent advances and future directions.
Keywords: calcium channel blockers; combination therapy; endothelin receptor antagonists; phosphodiesterase-5 inhibitor; prostacyclin analog; pulmonary arterial hypertension; pulmonary hypertension.