Pulmonary hypertension due to lung disease and/or hypoxemia (group 3 pulmonary hypertension): Treatment and prognosis
- Jay H Ryu, MD
Jay H Ryu, MD
- Professor of Medicine
- Mayo Clinic College of Medicine
- Robert P Frantz, MD
Robert P Frantz, MD
- Professor of Medicine
- Mayo Clinic College of Medicine
- Section Editors
- Talmadge E King, Jr, MD
Talmadge E King, Jr, MD
- Editor-in-Chief — Pulmonary and Critical Care Medicine
- Section Editor — Interstitial Lung Disease
- Dean, School of Medicine
- Vice Chancellor, Medical Affairs
- University of California San Francisco
- Jess Mandel, MD
Jess Mandel, MD
- Section Editor — Pulmonary Vascular Disease
- Professor of Medicine
- University of California, San Diego
Patients with pulmonary hypertension (PH) due to chronic 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 treatment and prognosis of patients with group 3 PH are reviewed here. The prevalence, pathogenesis, and diagnostic evaluation of patients with suspected group 3 PH are presented separately. (See "Pulmonary hypertension due to lung disease and/or hypoxemia (group 3 pulmonary hypertension): Epidemiology, pathogenesis, and diagnostic evaluation in adults".)
The World Health Organization (WHO) classifies patients with PH into five groups based upon etiology . Patients in group 1 are considered to have pulmonary arterial hypertension (PAH; also sometimes referred to as precapillary pulmonary hypertension), whereas patients in group 2 (due to left-sided heart disease), group 3 (due to lung disorders and hypoxemia), group 4 (due to chronic thromboembolic disease), and group 5 (associated with a variety of conditions) are considered to have PH (table 1). When all five groups are discussed collectively, the term PH is generally used. Classification of PH is discussed in detail separately. (See "Classification and prognosis of pulmonary hypertension in adults", section on 'Classification'.)
Treatment of associated condition — Treatment of the associated condition (eg, chronic obstructive pulmonary disease [COPD], interstitial lung disease [ILD], sleep disordered breathing [SDB]) is indicated in all patients with group 3 PH. However, while some strategies (eg, continuous positive airway pressure) modestly reduce pulmonary artery pressures (PAP), evidence to suggest significant reductions in PH or improved clinically impactful outcomes (eg, reduced mortality, improved exercise capacity, delayed progression) is lacking [2,3]. Nonetheless, disease-specific therapies are assumed to result in improved alveolar hypoxia, which is thought to contribute to the pathogenesis of PH and to progression of pulmonary hypertension. (See "Pulmonary hypertension due to lung disease and/or hypoxemia (group 3 pulmonary hypertension): Epidemiology, pathogenesis, and diagnostic evaluation in adults", section on 'Pathogenesis'.)
Treatment of the associated conditions are discussed separately. (See "Treatment of idiopathic pulmonary fibrosis" and "Management of stable chronic obstructive pulmonary disease" and "Management of obstructive sleep apnea in adults" and "Obstructive sleep apnea and cardiovascular disease in adults", section on 'Impact of treatment'.).
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- GENERAL MEASURES
- Treatment of associated condition
- Conventional and supportive therapies
- OUR APPROACH
- Mild to moderate pulmonary hypertension
- - Observation
- Severe pulmonary hypertension
- - Pulmonary arterial hypertension-directed therapy
- Chronic obstructive disease
- Interstitial lung disease
- Sleep disordered breathing and hypoxia
- Overlap syndromes
- Patients who fail therapy
- - Lung transplantation
- SUMMARY AND RECOMMENDATIONS