Pulmonary hypertensive disorders may occur as result of various cardiac, pulmonary parenchymal, and vascular diseases. The genetic basis for the development of inherited primary pulmonary hypertension has recently been reported. Improved recognition and better understanding of the pathophysiology of these disorders has led to new treatment options. A new classification system was introduced in 1998. Once patients are accurately diagnosed according to the correct classification, specific therapy can be directed. Pulmonary artery vasodilators are the mainstay of therapy for patients with pulmonary arterial hypertension. Others require specific medical therapy. Select patients may be candidates for surgical therapies, and it is imperative to identify patients who may benefit from pulmonary endarterectomy. This article reviews the current treatment options and potential future therapies for patients with pulmonary hypertension.