title: Understanding primary pulmonary hypertension.
abstract: Primary pulmonary hypertension (PPH) is a severe and progressive disease. It is characterized by persistent elevation of pulmonary artery pressure without any known cause. The hemodynamics demonstrates high pulmonary artery pressure and resistance to blood flow through the lung circulation. PPH is a diagnosis of exclusion. The diagnosis is often delayed because the initial symptoms may be mild and the exam findings subtle. The mean age of survival is 2.8 years without treatment. With treatment patients can survive for more than 10 years. Current lifetime treatment includes anticoagulation, oxygen, digoxin, calcium channel blockers, vasodilators, and diuretics. Promising results have been obtained with prostacyclin and endothelin receptor antagonists. Lung transplantation can be considered for patients with advances PPH and those not responding to medical therapy.
authors.affiliation: The University of Pittsburgh Medical Center, Cardiovascular Institute, Pittsburgh, Pa, USA.
authors.forename: Diane Schleyer
authors.initials: DS
authors.lastname: Berkowitz
authors.forename: Nancy Grimes
authors.initials: NG
authors.lastname: Coyne
mesh_terms: D000328:Adult; D000959:Antihypertensive Agents / Q000494:pharmacology / Q000627:therapeutic use; D000077300:Bosentan; D011464:Epoprostenol / Q000031:analogs & derivatives* / Q000494:pharmacology / Q000627:therapeutic use; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000451:nursing / Q000503:physiopathology / Q000628:therapy; D013449:Sulfonamides / Q000494:pharmacology / Q000627:therapeutic use
chemical_list: D000959:Antihypertensive Agents; D013449:Sulfonamides; D011464:Epoprostenol; D000077300:Bosentan; C427248:treprostinil