title: Medical management of primary pulmonary hypertension.
abstract: Primary pulmonary hypertension (PPH) is a rare disease effecting the pulmonary vasculature. Patients present with dyspnoea on exertion, chest pain, near syncope and evidence of right heart failure. The natural history of PPH was described in the 1980s and the survival rate was quite poor. The introduction of iv. epoprostenol in the early 1990s has significantly altered the natural history of this disease. Due to the complicated nature of this therapy, inhaled, sc. and oral prostacyclins have also been evaluated for treatment of PPH. The most recent class of medications added to the armamentarium for the treatment of PPH are the endothelin receptor antagonists. This paper reviews the available therapies for the treatment of PPH.
authors.affiliation: Rush-Presbyterian-St. Luke's Medical Centre, 1725 W Harrison Street, Suite 020, Chicago, IL 60612, USA. vmclaugh@rush.edui
authors.forename: Vallerie V
authors.initials: VV
authors.lastname: McLaughlin
mesh_terms: D000077300:Bosentan; D002121:Calcium Channel Blockers / Q000627:therapeutic use*; D065128:Endothelin Receptor Antagonists; D011464:Epoprostenol / Q000031:analogs & derivatives / Q000627:therapeutic use*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy*; D016032:Randomized Controlled Trials as Topic; D013449:Sulfonamides / Q000627:therapeutic use; D016896:Treatment Outcome
chemical_list: D002121:Calcium Channel Blockers; D065128:Endothelin Receptor Antagonists; D013449:Sulfonamides; D011464:Epoprostenol; D000077300:Bosentan