Surgical Treatments

• Atrial Sepostomy
• Transplant Surgery
• Pulmonary Endarterectomy

Atrial Septostomy

Some people with pulmonary hypertension faint occasionally when there is a sudden increase in pressure in the lungs. Fainting, also called syncope, can be helped by making a small hole in the septum, (wall) between the right and left atria. This little hole is made during the course of a cardiac catheterisation. The hole acts as a safety valve and allows the right heart to ‘decompress’ when the pressure increases suddenly.

Transplant Surgery

There has been a considerable change in the approach to both the assessment and possible listing of patients with severe PH in the past decade. The dramatic development in new therapies for PH along with the setting up and strengthening of centres of excellence which manage patients with PH has clearly impacted on the timing for transplant and even the need for it in many cases. Studies have now shown 60 - 70% of PH patients previously listed for transplantation (based on the criteria used before the development of prostacyclin therapies) can now be de-listed because their condition has improved so much on the new treatments. PH patients do not generally get on transplant lists unless they fail medical therapy.

For a small number of PH patients however, quality of life and life expectancy can be moderately to substantially improved by lung transplantation. It is impossible to predict how long they may survive after transplantation but the most critical period for survival is the first year after transplantation. This is the period when surgical complications, rejection and infection are the greatest threat to survival. Patients who survive the first year are more likely to survive 3 years or longer after transplantation. There are patients alive today who had lung transplantation 5 or even more years ago.

Life expectancy after lung transplantation is shorter than for heart, liver or kidney transplantation, particularly for PH patients. Rejection and infection are the two major complications of lung transplantation. Immunosuppressive (anti rejection) medications help keep the rejection process "turned off." Other medications may be necessary to control and treat rejection.

The timing of the operation depends on the quality of life of the individual with PH. It also depends on how fit the person is for surgery and the availability of compatible donor organs. The major limiting factor is lack of donor organs. The donated heart and lungs come from a healthy donor who has just died. The heart and lungs must be transplanted as soon as possible after death.

What are the different types of lung transplant?
There are several types of lung transplantation. Patients may undergo either heart and lung, bilateral lung or in rare cases single lung transplantation for PH.

All these types of surgery seem alike, but in fact they are quite different. Mainly because of the way the surgeon gains access into the chest cavity and where and how she or he joins up the blood vessels and airways.

Transplant assessment
Before a patient is put on the transplant waiting list they are carefully assessed in order to define their suitability to undergo the operation. This process is carried out because it is known that patients with certain physical, mental or social characteristics do not tolerate this sort of major surgery very well and would therefore not represent a justified use of rare organs, compared with other more suitable patients. This is the harsh reality of organ transplantation. Guidelines are set by certain official bodies that define a suitable patient. Factors such as the following play a major role:

• Age
• Life expectancy (difficult to accurately calculate)
• Other concurrent diseases that the individual may have

In addition patients with psychological problems or a tendency to abuse alcohol, drugs or cigarettes would not be seen as acceptable behaviour and would prevent them being accepted onto a transplant list. Ideally patients would demonstrate a willingness to undergo transplantation and would have a supportive family or a close circle of friends.

Generally the age limit for transplantation is around 60 years of age. Patients are expected to have good kidney and liver function before a transplant is carried out and their blood should have no clotting abnormalities.

Patients should not have any form of infection immediately prior to surgery due to the need for immunosuppression afterwards. Immunosuppression would mean that the patient would be unable to recover from even a relatively minor infection.

If a patient has suffered a previous heart attack, this would be considered a contraindication (a reason that makes it inadvisable to employ a particular procedure or treatment). Previous chest surgery is also considered to be a complicating factor. The reason being that if the chest wall has been opened previously, disturbing the scars during another open chest operation can lead to excessive blood loss. However, this is where the different types of transplantation offer differing degrees of success. The sequential single lung transplant allows the operation to be carried out without cutting the chest open down the sternum (breast bone). Access is gained down either side of the chest.

How does transplantation affect daily life?
Advice on coping with the pressures that transplantation imposes on the lives of patients and families is a subject best covered by those who have lived through it. Jo Hatton was one of the first patients to receive a heart-lung transplant in the UK, in 1985. She set up The Transplant Support Network; an organisation that as its name suggests provides support for those people who need or have undergone a transplant. She wrote a book detailing her life before and after her operation.

Transplant Centres in the UK

Pulmonary Endarterectomy

Not only is this a big word, it is a big operation! Pulmonary Endarterectomy (thankfully often shortened to PEA) is an operation to remove old blood clots from the pulmonary arteries in the lungs. In some individuals where blood clots are the cause of the PH, this operation may be a cure as it can restore almost normal blood flow to the lungs. In the UK, this operation is only performed at Papworth Hospital (near Cambridge).

This type of PH is due to thrombo-embolic disease. In chronic thrombo-embolic pulmonary hypertension (CTEPH) the disease begins with blood clots, usually from the deep veins of the legs or pelvis moving in the circulation and lodging in the pulmonary arteries (this is known as a pulmonary embolism). In most people these blood clots dissolve and cause no further problems. In a small proportion of people the blood clots partially dissolve or don’t dissolve at all and leave a permanent blockage in the pulmonary artery – these people develop CTEPH.

It is important to understand that not all people with PH due to these blood clots (CTEPH) are suitable for this operation. The location of such clots is very important in the decision about the feasibility of this surgery. The surgeon can remove large clots in the big pulmonary arteries but not small clots which may be scattered in the small pulmonary arteries throughout the lung.

Over time the cells lining the blood vessel wall grow over and through the clot, walling it off. The clot itself changes into fibrous, creamy tissue, which is attached to the lining of the blood vessel wall and cannot then be dissolved or sucked out. Pulmonary artery branches are narrowed or completely blocked off.

The surgeon opens the pulmonary arteries and removes the inner lining to clear the obstructions. It is a major operation involving the “heart-lung bypass” machine. The operation involves some risk, but if successful leads to a significant improvement in symptoms and life expectancy.

If your doctors tests show that you have CTEPH you will need some further investigations to establish whether you might be suitable to be treated by the PTE operation. Some of these investigations may be carried out at your local hospital or at your specialist centre. For some you may need to go to Papworth Hospital.

This is a high risk operation – about 10% of people don’t survive. This does mean though that 9 out of 10 people do and they are often returned back to normal quality and length of life. Some people (about 10% again) however still have residual PH that needs to be treated with standard PH treatments, such as Tracleer or Iloprost.

It is also common for individuals awaiting surgery to be treated with standard PH treatments prior to operation, this can improve their quality of life in this waiting period and, perhaps more importantly, can improve overall outcomes following surgery.