Primary pulmonary hypertension. Practical therapeutic recommendations.
Primary pulmonary hypertension (PPH) is a rare disease of unknown aetiology which typically results in right heart failure and death within several years of the onset of symptoms. While there is no cure for PPH, several pharmacological and surgical approaches to treatment have been developed over the past decade which proved useful in a significant proportion of patients. In particular, vasodilator therapy may produce sustained haemodynamic and symptomatic improvement in up to approximately two-thirds of patients; in the remaining patients, vasodilators may either produce no benefit or result in deterioration. The calcium channel blocking agents are the most widely used oral vasodilators; continuous intravenous infusions of epoprostenol (prostacyclin; prostaglandin I2) have been used in some patients who are refractory to oral therapy, particularly as a bridge to transplantation. While combined heart-lung transplantation has been considered the surgical procedure of choice for severe pulmonary hypertension, single lung transplantation has been performed successfully in a small number of patients, and may be the preferred approach in patients with reasonably preserved right heart function.
Division of Pulmonary and Critical Care Medicine, University of Maryland School of Medicine, Baltimore 21201.
L J
LJ
Rubin
D000925:Anticoagulants / Q000627:therapeutic use; D002121:Calcium Channel Blockers / Q000627:therapeutic use; D002301:Cardiac Glycosides / Q000627:therapeutic use; D003131:Combined Modality Therapy; D004232:Diuretics / Q000627:therapeutic use; D011464:Epoprostenol / Q000627:therapeutic use; D016041:Heart-Lung Transplantation; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000601:surgery; D010102:Oxygen Inhalation Therapy; D011379:Prognosis; D014665:Vasodilator Agents / Q000627:therapeutic use
D000925:Anticoagulants; D002121:Calcium Channel Blockers; D002301:Cardiac Glycosides; D004232:Diuretics; D014665:Vasodilator Agents; D011464:Epoprostenol