Chronic thromboembolic pulmonary hypertension

Chronic thromboembolic pulmonary hypertension (CTEPH) is a distinct subgroup of pulmonary hypertension that most frequently develops following massive or repeated pulmonary embolism.

The term CTEPH should be used for patients with chronic thromboembolic disease and pulmonary hypertension as defined by a mean pulmonary arterial pressure (mPAP) ≥25 mmHg at rest determined by right heart catheterisation. By contradistinction, chronic pulmonary emboli are mainly a consequence of incomplete resolution of pulmonary thromboembolism without pulmonary hypertension.

Chronic thromboembolic pulmonary hypertension is a rare disease. The exact prevalence and annual incidence of CTEPH is unknown. Data from the United Kingdom suggest that CTEPH may occur in approximately five individuals per million population per year 2. A cumulative incidence of 0.1–9.1% within the first two years after a symptomatic pulmonary embolus has been reported 3. However, some patients develop CTPEH in the absence of previous acute pulmonary embolus 4.

Clinical symptoms and signs are non-specific or absent in early stage of the disease, the impact of right heart failure only becomes evident in advanced disease. When present, the symptoms of CTEPH may resemble those of acute pulmonary embolism, idiopathic pulmonary arterial hypertension, asthma or chronic obstructive pulmonary disease 1,5. The non-specificity of symptoms and signs may contribute to a delay in diagnosis, often up to three years.

Chronic thromboembolic pulmonary hypertension is characterised by organised tissue and the presence of a variable small vessel arteriopathy leading to an increased pulmonary vascular resistance and finally resulting in progressive right heart failure.

Signs of chronic thromboembolic pulmonary hypertension can be identified on unenhanced CT, contrast-enhanced CT (CE-CT) and CT pulmonary angiography (CTPA).

  • dilatation of the central/proximal pulmonary arteries (pulmonary artery diameter: ≥29 mm in men and ≥27 mm in women)
    • visible on unenhanced CT, CE-CT and CTPA
  • eccentric organised thrombi: eccentric intraluminal filling defects adherent to the pulmonary arterial wall
    • visible in CE-CT and CTPA
    • compare with the central intraluminal filling defects in acute pulmonary embolism, the so-called polo mint sign (dark thrombus in the centre of a pulmonary artery, surrounded by the contrast medium in white)
  • total occlusions (pouch defects), or partial occlusions
    • visible in CE-CT and CTPA
  • stenoses, indicated by variability in the size of lobar and segmental level vessels, and/or irregular vessel calibre
    • Visible in CTPA
  • intraluminal fibrous webs or bands, these appear as linear intraluminal filling defects; intraluminal fibrous bands are ribbon-like structures anchored to the vessel wall at two points with a free mid-portion (often oriented along long axis of vessel) while intraluminal fibrous webs are branching networks of bands
    • visible in CTPA

  • enlargement of the right ventricle
    • visible on unenhanced CT, CE-CT and CTPA
  • thickening of the right ventricular wall
    • visible on unenhanced CT, CE-CT and CTPA
  • mosaic perfusion pattern of the lung parenchyma
    • visible in unenhanced CT and CE-CT
  • residual scars from infarcts (peripheral irregular, linear, or wedge-shaped densities)
    • visible in unenhanced CT, CE-CT and CTPA
  • large bronchial artery collaterals

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Article information

rID: 56593
System: Chest, Cardiac
Synonyms or Alternate Spellings:
  • Chronic embolic pulmonary hypertension (CTEPH)
  • CTEPH

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