Pulmonary Hypertension

Pulmonary Hypertension Vallerie V. McLaughlin, MD,* and Stuart Rich, MD Abstract: Pulmonary hypertension, in its simplest sense, is elevation of the pulmonary artery pressure above normal. A multitude of diseases may increase the pulmonary artery pressure and result in right ventricular dysfunction. The treatments of pulmonary hypertension are as varied as its causes. The past decade has realized remarkable growth in knowledge of the mechanisms of pulmonary arterial hypertension and, concurrently, therapies for this once uniformly fatal disease. In addition to continuous intravenous epoprostenol, subcutaneous treprostinil and oral bosentan are now FDA approved for the treatment of pulmonary arterial hypertension. Other forms of pulmonary hypertension, such as pulmonary venous hypertension, pulmonary hypertension related to diseases of the respiratory system, and thromboembolic pulmonary hypertension will be discussed. (Curr Probl Cardiol 2004;29:575-634. ) Introduction PP ulmonary hypertension, in its simplest sense, refers to any elevation in the pulmonary artery pressure above normal. The presence of pulmonary hypertension may reflect serious underlying pulmonary vascular disease which can be progressive and fatal, or simply an obligatory passive elevation in the pulmonary artery pressure in response to elevated pressures in the left heart. The treatment of pulmonary hypertension is as varied as the etiologies. Therefore, it is important to critically evaluate the nature of the pulmonary hypertension Dr. McLaughlin is a consultant for United Therapeutics, Actelion, and Encysive, and CoTherix; receives research support from Pfizer, Myogen, and Encysive; and received an honorarium from Actelion and United Therapeutics. Dr. Rich is Chief Medical Officer of United Therapeutics Corporation. *Address correspondence to: Vallerie V. McLaughlin, MD, University of Michigan, 1500 E. Medical Center Drive, L3119 Women’s Hospital, Ann Arbor, MI 48109-0273. E-mail: vmclaugh@umich. edu. Curr Probl Cardiol 2004;29:575-634. 0146-2806/$ – see front matter doi:10. 1016/j. cpcardiol. 2004. 04. 001 Curr Probl Cardiol, October 2004 575