Advanced Therapies For Pharmacologic Treatment Of Pulmonary Hypertension

Pulmonary hypertension (PH) is a chronic, progressive condition characterized by abnormally high pulmonary vascular pressure. Advanced therapies for PH are specialty medications intended to alter the natural history of the disease. These medications have been approved by the U.S. Food and Drug Administration (FDA) for 2 classes of PH: pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH). Pulmonary arterial hypertension is a rare and debilitating disease associated with abnormal proliferation of smooth muscle cells in the pulmonary arterial system, causing progressive right ventricular dilation and low cardiac output. Advanced therapy medications approved for PAH can be used as single agents or in combination. Chronic thromboembolic pulmonary hypertension is characterized by residual organized thrombi obstructing the pulmonary vasculature following acute or chronic pulmonary embolism. Currently, only 1 medication, the soluble guanylate cyclase stimulator riociguat, has been FDA approved for treatment of CTEPH.