Chronic thromboembolic pulmonary hypertension

Lancet Respir Med. 2014 Jul;2(7):573-82. doi: 10.1016/S2213-2600(14)70089-X. Epub 2014 Jun 2.


Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare but debilitating and life-threatening complication of acute pulmonary embolism. CTEPH results from persistent obstruction of pulmonary arteries and progressive vascular remodelling. Not all patients presenting with CTEPH have a history of clinically overt pulmonary embolism. The diagnostic work-up to detect or rule out CTEPH should include ventilation-perfusion scintigraphy, which has high sensitivity and a negative predictive value of nearly 100%. CT angiography usually reveals typical features of CTEPH, including mosaic perfusion, part or complete occlusion of pulmonary arteries, and intraluminal bands and webs. Patients with suspected CTEPH should be referred to a specialist centre for right-heart catheterisation and pulmonary angiography. Surgical pulmonary endarterectomy remains the treatment of choice for CTEPH and is associated with excellent long-term results and a high probability of cure. For patients with inoperable CTEPH, various medical and interventional therapies are being developed.

Publication types

  • Review

MeSH terms

  • Angioplasty, Balloon*
  • Bosentan
  • Chronic Disease
  • Endarterectomy
  • Endothelin Receptor Antagonists / therapeutic use
  • Enzyme Activators / therapeutic use
  • Humans
  • Hypertension, Pulmonary / diagnosis*
  • Hypertension, Pulmonary / etiology
  • Hypertension, Pulmonary / therapy*
  • Phosphodiesterase 5 Inhibitors / therapeutic use
  • Piperazines / therapeutic use
  • Pulmonary Embolism / complications*
  • Purines / therapeutic use
  • Pyrazoles / therapeutic use
  • Pyrimidines / therapeutic use
  • Sildenafil Citrate
  • Sulfonamides / therapeutic use
  • Thromboembolism / diagnosis*
  • Thromboembolism / etiology
  • Thromboembolism / therapy*


  • Endothelin Receptor Antagonists
  • Enzyme Activators
  • Phosphodiesterase 5 Inhibitors
  • Piperazines
  • Purines
  • Pyrazoles
  • Pyrimidines
  • Sulfonamides
  • Sildenafil Citrate
  • Bosentan
  • riociguat