Pharmacotherapy for Idiopathic Pulmonary Arterial Hypertension During the Past 25 Years

Anna M. Hackman, Pharm.D.; Thomas E. Lackner, Pharm.D., FASCP


Pharmacotherapy. 2006;26(1):68-94. 

In This Article


In a healthy individual, the right ventricle is a thin-walled muscular pump, and normal pulmonary circulation is a high-flow, low-resistance system. In pulmonary hypertension, however, pulmonary artery pressure and pulmonary vascular resistance are elevated. A consequence of the increased pulmonary artery pressure is right ventricular hypertrophy, progressing eventually to right-sided heart failure. Initially, cardiac output remains normal at rest but is limited during exercise. Right myocardial perfusion may be restricted by increases in right ventricular pressures, resulting in right ventricular ischemia. As the disease progresses, cardiac output becomes compromised even at rest. In addition, tricuspid regurgitation may develop, contributing to heart failure.[11] The three most common causes of death in IPAH are right-sided heart failure, pneumonia, and sudden death caused, individually or in combination, by acute pulmonary embolism, right ventricular ischemia, pulmonary hemorrhage, or arrhythmias due to arterial hypoxia and acidosis.[2,11]