Pulmonary hypertension due to lung disease and or hypoxia

Pulmonary hypertension due to lung disease and or hypoxia

Updated: 10/12/2022

© Jun Wang, MD, PhD

 

General features

  • Group 3 pulmonary hypertension
  • Caused by chronic lung diseases or conditions resulting hypoxemia
  • More common in elder population
  • Usually associated with increased morbidity and mortality
  • Associated disorders include
    • Obstructive lung disease (COPD or bronchiectasis)
    • Restrictive lung disease (eg, interstitial lung disease, kyphoscoliosis)
    • Lung disease with mixed obstruction and restriction (eg, pulmonary fibrosis with emphysema)
    • Hypoxia without lung disease (eg, high altitude, sleep-disordered breathing, obesity hypoventilation)
    • Developmental lung disorders (eg, bronchopulmonary dysplasia, congenital lobar emphysema)

Pathogenesis

  • Pulmonary inflammation/hypoxia result in pulmonary vasoconstriction and remodeling

Clinical presentations

  • Presentations of underlying lung diseases
  • History
    • Exertional hyspnea or hypoxemia unexplainable by current lung disorders
    • Rapid decline of arterial oxygenation
    • Presentations of right heart failure
  • PE
    • Increased P2 heart sound
    • Systolic ejection murmur
    • Signs of right sided heart failure: right ventricular heave, elevated jugular vein, hepatomegaly, etc

Key morphological features

  • Similar to pulmonary arterial hypertension (PAH)
  • Proliferative vasculopathy
  • Involving small muscular arteries
  • Hyperplasia/hypertrophy of all three layers of vascular wall (intima, media, adventitia)
  • Fibrosis
  • Thrombi

Diagnosis

  • Confirmation of pulmonary hypertension
  • Chest X-ray
    • Enlarged central pulmonary arteries and peripheral attenuation
    • Right heart dilation
  • Doppler echocardiography
    • Most reliable non-invasive method
    • Estimate pulmonary artery pressure
    • Tricuspid regurgitation
  • Ventilation-perfusion lung scanning: rule out embolism
  • Right-sided cardiac catheterization
  • Pulmonary function tests, etc

Treatment

  • Management of underlying lung diseases
  • Supportive approaches: Vaccinations, oxygen, diuretics, smoking cessation, etc

 

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