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Pathogenesis of pulmonary hypertension

Lewis J Rubin, MD
William Hopkins, MD
Section Editors
Jess Mandel, MD
Andrew Nicholson, MD
Deputy Editor
Geraldine Finlay, MD


Pulmonary hypertension (PH) is a disease characterized by elevated pulmonary artery pressure, which often results in right ventricular failure. It may be idiopathic, familial, or associated with multiple other diseases. PH occurs in men and women of any race or age.

The pathogenesis of PH is described in this review. The definition, classification, epidemiology, etiology, clinical manifestations, diagnosis, treatment, and prognosis of PH are discussed separately. (See "Overview of pulmonary hypertension in adults" and "Clinical features and diagnosis of pulmonary hypertension in adults" and "Treatment of pulmonary hypertension in adults".)


The classification of primary or secondary pulmonary hypertension no longer exists. The revised World Health Organization (WHO) classification system includes five groups [1]. Patients in the first group are considered to have pulmonary arterial hypertension (PAH), whereas patients in the remaining four groups are considered to have pulmonary hypertension (PH) (table 1). When all five groups are discussed collectively, PH is generally used. We adhere to this nomenclature in the discussion that follows. The WHO classification system is listed in brief below and described in detail separately. (See "Overview of pulmonary hypertension in adults", section on 'Classification'.)

Group 1 – PAH

Group 2 – PH due to left heart disease


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