Diagnosing CTEPH

Symptoms and Risk Factors

Symptoms of CTEPH

The most frequent presenting symptoms of CTEPH include:[i]

  • Shortness of breath (dyspnea)
  • Swelling of the feet or ankles (edema)
  • Fatigue
  • Chest pain
  • Fainting (syncope)

After pulmonary embolism, up to 4% of patients may develop CTEPH within 2 years.

Risk Factors

Those at greater risk of developing CTEPH include:[ii]

  • People with a history of cancer, which increases the tendency to form blood clots
  • People with any chronic inflammatory diseases of the bowels or bones
  • People who have had their spleen removed
  • People with surgically implanted shunts in the brain, which drain spinal fluid from around the brain
  • People who have very large pulmonary emboli
  • People who already manifest some pulmonary hypertension at the time of diagnosis of a pulmonary embolism
  • People with underlying blood clotting disorders, though not all clotting disorders increase risk

Diagnosis and Referral

Investigations

  • Echocardiogram
  • Blood tests
  • Chest X-rays
  • ECG
  • Pulmonary function tests

Referral to Specialized Centres for Confirmation Tests

  • Exercise tolerance test
  • CT scanning/imaging
  • Ventilation/perfusion lung scan
  • Right heart catheterization

How is CTEPH Confirmed? 

In patients with suspected CTEPH, it is strongly recommended that a nuclear ventilation-perfusion (VQ) scan be performed.[iii] The VQ scan is a critical step to early, accurate diagnosis of CTEPH and to pursuing optimal disease management and survival. The VQ scan assesses air and blood flow in the lungs and helps to identify blood clots or pulmonary embolism (PE). A normal VQ scan rules out CTEPH.

Normal-CTEPH_VQ_scans_-pg_8.jpeg

 


[i] Pepke-Zaba K et al. Circulation 2011; 124:1973-81.

[ii] Mehta, S et al Diagnostic evaluation and management of chronic thromboembolic pulmonary hypertension: A clinical practice guideline. Can Respir J 2010; 17(6):301-334.

[iii] Mehta, S et al Diagnostic evaluation and management of chronic thromboembolic pulmonary hypertension: A clinical practice guideline. Can Respir J 2010; 17(6):301-334.