Pulmonary hypertension due to pulmonary artery obstruction

Pulmonary hypertension due to pulmonary artery obstruction

Updated: 08/25/2022

© Jun Wang, MD, PhD

 

General features

  • Group 4 pulmonary hypertension
  • Two subgroups
    • Chronic thromboembolic pulmonary hypertension (CTEPH)
    • Other pulmonary artery obstructions
      • Tumors of pulmonary artery
      • Pulmonary artery stenosis
      • Arteritis
      • Mediastinal fibrosis, etc

Chronic thromboembolic pulmonary hypertension

  • Definition
    • Mean pulmonary arterial pressure > 20 mm Hg
    • Presence of organized, nonacute, thromboembolic material and altered vascular remodeling in the pulmonary vasculature
  • Rare but might be life threatening
  • Likely due to underlying hypercoagulable state
  • Commonly with history of pulmonary embolism
  • Worse prognosis if > 70 years, residual PH, comorbidities, etc

Pathogenesis

  • Incomplete thrombus resolution resulting organization and remodeling of pulmonary vessels
  • Other causes resulting narrowing pulmonary vessels 



Clinical presentations

  • Features of pulmonary hypertension: progressive dyspnea, etc
  • Features of thromboembolism
    • History of acute pulmonary thromboembolic events
    • History of acute deep veinous thromboembolic events
    • Flow murmurs over the lung fields: due to partially obstructed vessels

Diagnosis

  • Confirmation of pulmonary hypertension
  • Chest radiography: Enlarged right ventricle
  • Ventilation perfusion lung scan: Recommended initial study
  • Pulmonary angiography: complete/partial vascular obstruction
  • Laboratory tests: CBC, coagulation panels

Treatment

  • Surgery: Pulmonary thromboendarterectomy
  • Medical care
    • If inoperable
    • o   Targeted therapy: Riociguat, macitentan, phosphodiesterase type 5 inhibitors, etc
    • Improve right heart function: Diuretics
    • Anticoagulation: Warfarin
  • Long term monitoring: 6-minute walk distance testing, transthoracic echocardiography, right heart catheterization

 

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