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


A diagnosis of IPAH is essentially a diagnosis of exclusion. If symptoms of pulmonary arterial hypertension (PAH) are present, chest radiography and pulmonary function tests can exclude emphysema, pulmonary or cystic fibrosis, and thoracic cage abnormalities. Echocardiography will rule out left-sided heart disease, valvular anomalies, and congenital heart disease, whereas ventilation-perfusion scanning and angiography can eliminate the diagnosis of thromboembolic disease. Finally, blood tests such as for antinuclear antibody, rheumatoid factor, human immunodeficiency virus (HIV), hepatitis, and aspartate aminotransferase and alanine aminotransferase levels can rule out causes of pulmonary hypertension such as lupus, scleroderma, rheumatoid arthritis, acquired immunodeficiency syndrome, and liver disease.

The lack of an identifiable cause confers the term “idiopathic” on this condition. Ultimately, although invasive, catheterization of the right side of the heart revealing elevated pulmonary artery pressure, increased right atrial pressure, and a decreased cardiac index provides the final diagnosis. Mortality in IPAH has been shown to correlate directly with the degree of derangement in these three hemodynamic variables.[1] Diagnosis of IPAH follows the American College of Chest Physicians' guideline parameters of mean pulmonary artery pressure greater than 25 mm Hg with a left atrial pressure less than 15 mm Hg. In comparison, the mean pulmonary artery pressure in a healthy adult is approximately 14 mm Hg at rest.