Recent advances in the understanding of pulmonary arterial hypertension have led to new therapeutic options, although the disease remains incurable and continues to cause substantial morbidity and mortality. Disease-specific therapies have been approved for use in the US, including epoprostenol and its various analogs, endothelin receptor antagonists, and phosphodiesterase 5 inhibitors. The use of combination therapy with agents from more than one of these drug classes is becoming increasingly common, although guidelines establishing optimal combinations are lacking. Meanwhile, potential future therapeutic options are actively being pursued.