Ivan M. Robbins, MD

abstract: Pulmonary arterial hypertension (PAH) is 1 of 5 types of pulmonary hypertension (PH). Symptoms may include dyspnea on exertion, fatigue, near-syncope, and palpitations. Physical findings include lower extremity edema, jugular venous distention, and a loud P2. Findings on chest radiography, transthoracic echocardiography, and electrocardiography can suggest the presence of PAH; however, right heart catheterization is the gold standard for confirming the diagnosis and for differentiating PAH from other forms of PH. It is essential to exclude chronic thromboembolic PH, since this can be surgically corrected. The treatment of PAH depends on the severity. In addition to the standard treatments, such as diuretics and anticoagulation, more advanced treatment options include prostaglandin therapy (epoprostenol, treprostinil, and iloprost), endothelin receptor antagonists (bosentan), and phosphodiesterase inhibitors (sildenafil).