Pulmonary hypertension (PH) is divided into varied pathophysiological and etiologic groupings, as classified by the World Health Organization (WHO). Pulmonary arterial hypertension (PAH), which falls under WHO group 1 PH, is a progressive and potentially fatal disease characterized by a vasoconstrictive, proliferative, and thrombotic phenotype, which leads to increased pulmonary artery pressure, right heart failure, and death. Pathologically, in situ thromboses are found in the small distal pulmonary arteries. Dysregulation of coagulation, platelet function, and endothelial cells may contribute to a prothrombotic state. There is mixed evidence for the use of anticoagulation or antiplatelet therapy in PAH patients.