This article provides an overview of pulmonary arterial hypertension (PAH), beginning with the initial pathologic recognition of pulmonary hypertension more than 100 years ago and progressing to the current diagnostic categorization of PAH. It reviews the epidemiology, pathophysiology, genetics, and modern treatment of PAH. The article discusses several important recent studies that have highlighted the importance of new management strategies, including serial risk assessment and combination pharmacotherapy.
Keywords: BMPR2; Endothelin receptor antagonists; Phosphodiesterase-5 inhibitors; Prostacyclin; Pulmonary arterial hypertension; Pulmonary hypertension; REVEAL Registry.
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