How is CTEPH Treated?

There is a potential cure for CTEPH trough pulmonary endarterectomy (PEA) surgery. Approved medical treatment may also slow disease progression and alleviate symptoms.

Medical Therapy

  • Riociguat (Adempas®) – approved in Canada in 2013 for treatment of non-operative or residual post-operative CTEPH (WHO class IV)

Surgical Treatment

The standard and potentially curative treatment for patients who have developed CTEPH and are suitable for surgery is a procedure called pulmonary endarterectomy (PEA). PEA is major surgery that clears the blood vessels of the lungs of clots and scar material presented in the disease.[i] To determine whether you are a suitable candidate for surgery, you need to be thoroughly assessed at a centre specialized in the treatment of CTEPH. CTEPH specialists will assess:

  • If the blockage is reachable through surgery - if the blockage is too deep within the lungs, it may not be reachable;
  • Your overall suitability to undergo surgery. 

Some people assessed for PEA by an expert team do have operable CTEPH. Successful surgical outcomes can include an improved life expectancy.[ii]

Pulmonary Endarterectomy Surgery

Adapted from a patient education brochure produced by Patient and Family Education Program.

© University Health Network, Patient and Family Education

Information for Patients with Chronic Thromboembolic Pulmonary Hypertension (CTEPH) and Their Families

Click on the headings below to learn:

  • how to prepare for your surgery;
  • what to expect while in hospital;
  • what to expect after you return home;
  • and who to call if you have any questions.
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Additional Resources

Patient Education

Pulmonary Thromboendarterectomy: A Guide for Patients and Families
Published by the University of Ottawa Heart Institute 


 

[i] Ali, JM et al Chronic thromboembolic pulmonary hypertension: An underdiagnosed entity? Hosp Pract 2012;40:71–9.

[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.