Primary Pulmonary Hypertension

doi:10.1056/NEJM199701093360207

Volume 336:111-117		January 9, 1997		Number 2

Primary Pulmonary Hypertension

Lewis J. Rubin, M.D.

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Primary pulmonary hypertension is a condition characterizedby sustained elevations of pulmonary-artery pressure withouta demonstrable cause. The diagnostic criteria used in the NationalInstitutes of Health (NIH) registry¹ include a mean pulmonary-arterypressure of more than 25 mm Hg at rest, or more than 30 mm Hgwith exercise, and the exclusion of left-sided cardiac valvulardisease, myocardial disease, congenital heart disease, and anyclinically important respiratory, connective-tissue, or chronicthromboembolic diseases. Pulmonary vascular disease with clinicaland pathological features similar to those of primary pulmonaryhypertension can occur in patients with portal hypertension,²infection with the human immunodeficiency . . . [Full Text of this Article]

Incidence

Pathogenesis and Pathophysiology

Diagnosis

Therapy

Vasodilators

Transplantation

Anticoagulation

Other Supportive Measures

Survival and Natural History

Future Directions

Source Information

From the Division of Pulmonary and Critical Care Medicine, Departments of Medicine and Physiology, University of Maryland School of Medicine, Baltimore.

Address reprint requests to Dr. Rubin at 10 South Pine St., Rm. 800, Baltimore, MD 21201.

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