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Pulmonary hypertension due to lung disease and/or hypoxemia (group 3 pulmonary hypertension): Epidemiology, pathogenesis, and diagnostic evaluation in adults

Elizabeth S Klings, MD
Section Editors
Jess Mandel, MD
Talmadge E King, Jr, MD
Deputy Editor
Geraldine Finlay, MD


Patients with pulmonary hypertension (PH) due to diffuse lung disease (eg, chronic obstructive pulmonary disease, interstitial lung disease, or overlap syndromes) or conditions that cause hypoxemia (eg, obstructive sleep apnea, alveolar hypoventilation disorders) are classified as having group 3 PH (table 1).

The prevalence, pathogenesis, and diagnosis of PH due to lung disease and/or hypoxemia are presented here. The treatment and prognosis of patients with group 3 PH are reviewed separately. (See "Pulmonary hypertension due to lung disease and/or hypoxemia (group 3 pulmonary hypertension): Treatment and prognosis".)


The classification and definitions of PH and cor pulmonale are discussed below:

Classification – The World Health Organization (WHO) classifies patients with PH into five groups based upon etiology [1]. Patients in group 1 are considered to have pulmonary arterial hypertension (PAH; also sometimes referred to as pre-capillary pulmonary hypertension), whereas patients in group 2 (due to left-sided heart disease), group 3 (due to chronic lung disorders and hypoxemia), group 4 (due to chronic thromboembolic disease), and group 5 (due to unidentified mechanisms) are considered to have PH (table 1). When all five groups are discussed collectively, the term PH is used. (See "Classification and prognosis of pulmonary hypertension in adults", section on 'Classification'.)

Pulmonary hypertension – PH is defined as a mean pulmonary artery pressure (mPAP) ≥25 mmHg at rest, measured by right heart catheterization [2]. PH is considered severe if mPAP is ≥35 mmHg or the mPAP is ≥25 mmHg with an elevated right atrial pressure and/or the cardiac index is <2 L/min/m2 [3]. (See "Clinical features and diagnosis of pulmonary hypertension in adults", section on 'Diagnostic criteria'.)

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Literature review current through: Dec 2017. | This topic last updated: Mar 07, 2017.
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