title: Current management of patients with pulmonary hypertension and right ventricular insufficiency.
abstract: Pulmonary hypertension is a pathologic condition characterized by elevated pulmonary artery pressures and an associated vasculopathy. Primary pulmonary hypertension (PPH) is a rare condition with a sporadic occurrence and a familial form of the disorder. Abnormal vasomotor tone in the pulmonary vasculature results from an imbalance of the action of various vasoconstrictors/ vascular proliferative agents (endothelin and thromboxane) versus vasodilators /anti-proliferative agents (prostacyclin and nitric oxide). The mainstay of outpatient therapy has been the use of digitalis, diuretics, oxygen, and coumadin and the judicious use of vasodilator therapy. Calcium channel blockers in a select group and intravenous prostacyclin have dramatically improved survival for those with primary pulmonary hypertension. Use of prostaglandin I2 (PGI2) in other forms of chronic pulmonary arterial hypertension is not as clear, although evidence of initial beneficial response is promising. Importantly, over the next few years both pharmacologic and nonpharmacologic treatment modalities for pulmonary hypertension may rapidly change as we focus more on the abnormal pulmonary vascular biology and concomitant hemodynamic and neurohormonal milieu.
authors.affiliation: Albert Einstein College of Medicine, 1300 Morris Park Avenue, Bronx, NY 10461, USA.
authors.forename: S R
authors.initials: SR
authors.lastname: Hankins
authors.forename: E M
authors.initials: EM
authors.lastname: Horn
mesh_terms: D000959:Antihypertensive Agents / Q000627:therapeutic use; D002316:Cardiotonic Agents / Q000627:therapeutic use; D004730:Endothelium, Vascular / Q000503:physiopathology; D011464:Epoprostenol / Q000627:therapeutic use; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000503:physiopathology; D014665:Vasodilator Agents / Q000627:therapeutic use; D014666:Vasomotor System / Q000503:physiopathology; D018497:Ventricular Dysfunction, Right / Q000188:drug therapy* / Q000503:physiopathology
chemical_list: D000959:Antihypertensive Agents; D002316:Cardiotonic Agents; D014665:Vasodilator Agents; D011464:Epoprostenol