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Review Article
Mechanisms of Disease
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Volume 351:1655-1665 October 14, 2004 Number 16
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Pulmonary Arterial Hypertension
Harrison W. Farber, M.D., and Joseph Loscalzo, M.D., Ph.D.

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Pulmonary hypertension is usually classified as primary (idiopathic) or secondary.1 It is now clear, however, that there are conditions within the category of secondary pulmonary hypertension that resemble primary pulmonary hypertension in their histopathological features and their response to treatment. For this reason, the World Health Organization (WHO) classified pulmonary hypertension into five groups on the basis of mechanisms, rather than associated conditions (Table 1). The most recent revision of the WHO classification uses consistent terminology and defines pulmonary hypertension more precisely than previous versions.2 Group I of the WHO classification, designated pulmonary arterial hypertension, is the principal . . . [Full Text of this Article]

Imbalance of Vascular Effectors

Prostacyclin and Thromboxane A2

Endothelin-1

Nitric Oxide

Serotonin

Adrenomedullin

Vasoactive Intestinal Peptide

Vascular Endothelial Growth Factor

Associated Environmental Factors

Hypoxia

Anorexigens

Central Nervous System Stimulants

Other Associated Conditions

Scleroderma

Infection with HIV

Human Herpesvirus

Portal Hypertension

Thrombocytosis

Hemoglobinopathies

Hereditary Hemorrhagic Telangiectasia

Associated Genetic Abnormalities

TGF-{beta} Receptor Pathway

Serotoninergic Pathway

Molecular Basis of Treatment Strategies

Conclusions


Source Information

From the Evans Department of Medicine (H.W.F., J.L.), the Pulmonary Center (H.W.F.), and the Whitaker Cardiovascular Institute (J.L.), Boston University School of Medicine, Boston.

Address reprint requests to Dr. Loscalzo at the Department of Medicine, Boston University School of Medicine, 88 E. Newton St., Boston, MA 02118, or at jloscalz@bu.edu.


Related Letters:

Pulmonary Hypertension
McLoughlin P., Hyvelin J.-M., Howell K., Tektonidou M., Farber H. W., Loscalzo J.
Extract | Full Text | PDF  
N Engl J Med 2005; 352:418-419, Jan 27, 2005. Correspondence

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