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

Clinical Presentation

Patients with IPAH often have nonspecific symptoms, which confound its recognition and result in a mean length of time from onset of the initial symptoms to diagnosis of approximately 2 years.[4] Initial symptoms include fatigue and shortness of breath. As the disease progresses, additional clinical features may include dizziness or lightheadedness on exertion, peripheral edema, exertional chest pain, and syncope. Clinical signs often include an increase in the pulmonic component of the second heart sound, indicative of elevated pulmonary artery pressure.[4] Radiographic changes such as a prominent central pulmonary artery, and electro-cardiographic abnormalities including large P waves, right ventricular hypertrophy, or right axis deviation, are commonly seen.[2,4] Echocardiography frequently shows enlargement of the right side of the heart, with a reversal of the normal septal curvature. Pressures in the left side of the heart are generally normal, although compression of the left chambers by extreme dilatation of the right chambers can result in decreased left ventricular filling and, consequently, small increases in diastolic pressures.[11]