How is CTEPH treated?

Thankfully, CTEPH is the only type of PH where an operation can provide significant long-term improvements and even potentially cure your disease. This operation is known as pulmonary endarterectomy (PEA) although it is sometimes called pulmonary thrombendarterectomy (PTE).

What happens in a PEA or PTE operation?

The level of activity, quality of life and life expectancy are significantly improved in a large proportion of patients who undergo this operation – and can be thought of as a cure for many of them.1 Highly skilled surgeons perform the operation. It involves opening up the pulmonary arteries and removing the obstructions together with the inner layer of the artery. However, before it can be performed you need to be thoroughly assessed for eligibility for this operation at an expert centre that has proven experience of performing the operation. Indeed, referral to a highly experienced centre has been shown to improve the likelihood of a successful result.

What specialists say about CTEPH and PEA

To hear specialists from Papworth Hospital in the UK talk about CTEPH and PEA, play the video.

What are the potential risks of surgery?

Nowadays, in expert centers the risks of surgery are low (less than 5 % risk of death). Although the operation is performed with phases of deep hypothermic circulatory arrest (without body and brain perfusion) it has been shown that your brain will not be injured by this procedure. There can be other surgical complications like lung infection or postoperative bleeding which are common after other heart and lung operations and can be easily treated by an experienced intensive care team.

Are any medical treatments available?

As with so many diseases, medical science is always looking for new CTEPH treatments, which might be effective if you are considered inoperable by an expert team or if you have some degree of residual PH after surgery. None have been approved for treating CTEPH yet. But speak to your doctor, he or she may be able to keep you up-to-date with any developments.

  1. Papworth Hospital, pulmonary endarterectomy service. http://www.papworthhospital.nhs.uk/content.php?/our_services/thoracic_services_lungs/pulmonary_vascular_disease_unit_pvdu/pulmonary_endarterectomy_service

Further information can be found by watching the above video. You will hear expert speakers Mr John Dunning, Mr David Jenkins and Dr Joanna Pepke-Zaba, all consultants at Papworth Hospital UK, commenting on PEA , patient recovery and aftercare, and giving their thoughts on the current lack of licensed pharmacological treatments.