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Royal Papworth Hospital is the only centre in the UK offering pulmonary endarterectomy (PEA, historically known as PTE) surgery and has performed more than 2,000 operations since 1996. We are one of the most active centres in the world, carrying out 170-190 PEA operations per year, with some of the best long-term outcomes internationally.
Pulmonary endarterectomy is a surgical operation in which the blood vessels of the lungs are cleared of clot and scar material. In a large proportion of patients who undergo this operation, their level of activity, quality of life and life expectancy are significantly improved and pulmonary endarterectomy surgery can be thought of as a cure for most patients.
CTEPH is a relatively rare disease, but it can occur in 2-4% of people following a pulmonary embolism.
It is a form of pulmonary hypertension that occurs as the result of blood clots blocking off the arteries of the lungs. While most of these blood clots originate in the veins, many people with this condition may be unaware that these clots are forming.
Over time, the blood clots build up in the blood vessels of the lungs which become permanently scarred with narrowing and decreased blood flow. This results over time in increased pressure and resistance to blood flow in the lungs leading to pulmonary hypertension and right heart failure. When severe, this situation leads to increasing breathlessness, exercise limitation and reduced life expectancy.
PEA is a complex and highly-skilled surgical operation in which the blood vessels of the lungs are cleared of the clot and scar material and often takes up to 10 hours.
The remarkable part of the operation is the need to drain all the blood out of a patient's body to allow the surgical team to see enough to clear the clots. This can only be achieved by cooling the patient's body to 20°C - almost half the normal body temperature - to prevent damage to vital organs, including the brain.
This is achieved by attaching the patient to a heart-lung bypass machine, which is operated by perfusionists. The blood exits the patient's body, enters the bypass machine where it is cooled, and then returns to the patient in a circuit; it's this cold blood that cools them down.
Once the body reaches a suitable temperature, all the blood is removed from the patient through bypass (technical cardiac arrest or suspended animation) to create a bloodless field, and the heart-lung machine turned off for up to 20 minutes. This allows the surgeon to begin clearing the clots and CTEPH disease from the lining of the pulmonary artery, but once the 20-minute limit has been reached the bypass machine is then turned back on to re-perfused the body's organs with blood.
This process is then repeated to remove the clots and scarring from the other lung. Research led by the Royal Papworth PEA team has shown that this is safest way to perform this operation, but the heart-lung machine can only be switched off - and the patient in a state of suspended animation - for a maximum of 60 minutes.
Meanwhile, the anaesthetic team monitor oxygen levels in the brain, which is cooled using a device that wraps around the patient's head.
The surgery is demanding because it is technically very difficult to get access to the arteries of the lungs and the procedure itself is a major undertaking. For this reason it is necessary to ensure that patients who might potentially benefit from this operation are thoroughly evaluated.
Not everyone with CTEPH will be suitable for this operation, and a number of additional investigations must be performed in order to assess the appropriateness of surgery and the level of risk involved. However, in a large proportion of patients who undergo this operation, their level of activity, quality of life and life expectancy are significantly improved and pulmonary endarterectomy surgery can be thought of as a cure for most patients.
Prior to being accepted for surgery, you will have the opportunity to discuss the operation in detail with the surgeon and other members of the team at Royal Papworth Hospital. Should you or your family have any questions or concerns they will be happy to address them prior to any decisions being made. You can download a copy of the patient’s guide and agreement to consent information booklet.
The majority of patients (70-80%) referred to our service will benefit from surgery. Occasionally the type of disease may not be appropriate for surgery, or the patient may be too well or unwell to undergo the operation. In this circumstance you will be followed carefully and there may be some medical treatments available that can help improve the severity of pulmonary hypertension.
At present, Royal Papworth Hospital is working closely with other centres around the world to develop more effective treatments for this condition.
Clinical Director of Surgery and Transplantation
Mr David Jenkins
Mr Steven Tsui
Mr Choo Yen Ng
Mr John Taghavi
If you would like further information regarding CTEPH or pulmonary endarterectomy (PEA) please email our PEA Specialist Nurses at phn-tr.PTENurses@nhs.net.
This film has been produced to help patients and their families prepare for undergoing pulmonary endarterectomy surgery at Royal Papworth; it outlines practical ways of helping to prepare for going home after surgery.