Primary Pulmonary Hypertension.

Primary pulmonary hypertension has become a very treatable disease given the recent advances in medical therapy. Any patient with unexplained right ventricular enlargement or pulmonary hypertension needs a thorough workup to determine the cause. Patients with primary pulmonary hypertension should be referred to a specialized "Center of Excellence" to evaluate potential therapies on a case-by-case basis. Warfarin anticoagulation is generally recommended in all patients. Some patients (approximately 20%) will have a dramatic response to high doses of calcium channel blockers; the remainder are generally helped by the use of continuous intravenous prostacyclin therapy. Lung transplantation remains the final treatment option for patients in whom medical therapy fails.