WHAT IS. Helping your patients understand their type of PH. Educational program by. Material endorsed by

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1 Educational program by Material endorsed by WHAT IS CTEPH Helping your patients understand their type of PH Provided by Bayer to help education of the PH community

2 What is ph? B lood travels from your heart to the lungs, where it picks up oxygen, by way of the pulmonary artery As a result, the right heart has to work harder than normal to pump blood to the lungs to be oxygenated I n pulmonary hypertension (PH for short), narrowing of the blood vessels or obstruction within the pulmonary artery restrict this blood flow, causing blood pressure to rise The medical definition of PH is blood pressure in the pulmonary arteries which is greater than 25 mmhg (the normal value is between 8 and 20 mmhg) 4 Oxygen-rich blood is pumped back out to the body 1 3 Blood returns to the heart after delivering oxygen to the body If your lungs don t receive as much blood as they should with each pump, less oxygen gets picked up If your body doesn t get the amount of oxygen it needs to function normally, it can leave you feeling breathless Blood returns to the heart from the lungs 2 Blood gets pumped to the lungs via the pulmonary artery to pick up a fresh supply of oxygen 01

3 What is CTEPH? CTEPH stands for: chronic refers to an illness that lasts a long time thromboembolic the blocking of a blood vessel which can initially arise from a blood clot pulmonary means relating to the lungs hypertension is the medical term for high blood pressure 02

4 What is CTEPH? CTEPH is 1 of 5 different types of PH: 1. Pulmonary arterial hypertension (PAH) 2. PH due to left heart disease 3. PH due to lung diseases and/or hypoxia 4. Chronic thromboembolic pulmonary hypertension (CTEPH) 5. PH with unclear and/or multifactorial mechanisms While all types of PH share some common characteristics, there are certain differences that occur with CTEPH it is not the same as other forms, such as the more commonly known PAH 03

5 What CAUSES CTEPH? It is believed that, in some cases, people who develop CTEPH have a history of pulmonary embolism that is, a blood clot in the lungs It is thought that these blood clots are not completely resolved and remain stuck to the wall of the pulmonary artery Because the blockage is fibrous in CTEPH and becomes part of the vessel wall, it is not possible to simply dissolve it with blood thinners Difference between pulmonary embolism and CTEPH Coagulated blood clogs the artery in a pulmonary embolism A more fibrous blockage is responsible for CTEPH Blockage restricts blood flow increasing blood pressure in the artery Over time, extra tissue forms within the clot so that it becomes more fibrous, creating a blockage that restricts the blood flow Images courtesy of Prof B Yildizeli, Marmara University Hospital, Turkey 04

6 What are the symptoms of CTEPH? One of the problems with CTEPH is that its symptoms are quite general and so may initially be attributed to more common heart or lung conditions Most frequent symptoms of CTEPH: 99.1% 40.5% 31.5% 15.3% 13.7% dyspnea (breathlessness) edema (swelling of the tissues) tiredness chest pain syncope (fainting) Pepke-Zaba J et al. Circulation 2011; 124:

7 How is PH diagnosed? Echocardiography An echocardiogram is a painless procedure that uses ultrasound to create moving pictures of your heart it s a good way to see how your heart is beating and pumping blood, and to check for signs of disease it can help identify a problem with the valves and measure the size of the chambers of your heart it can also provide a good estimate of your pulmonary arterial pressure (PAP for short) Gel will be spread on your chest and then a probe, which looks a bit like a microphone, will be pressed firmly against your skin This sends the sound waves into the chest and picks up the echoes that bounce back which are then displayed as a picture on a screen 06

8 How is PH diagnosed? Right heart catheterization In order to measure your PAP exactly, a catheter with pressure sensor is placed directly into your pulmonary artery After injection of a local anesthetic, a special, small, hollow plastic tube (the catheter) is inserted through your arm or leg and guided into a lung artery Once in the artery, blood pressure at the tip of the catheter will then be measured Depending on the results of these and other tests, you may need a type of scan called a ventilation/ perfusion (or V/Q) scan 07

9 How is CTEPH confirmed? A V/Q scan is used to help tell the difference between CTEPH and other types of PH This scan is in two parts: 1 The first measures your breathing (ventilation) 2 The second measures blood flow in the lung (perfusion) A V/Q scan uses a very small amount of radioactive material and a special camera to detect the radiation After an injection of a mildly radioactive solution, you will also be asked to breathe a tasteless, odorless gas which also contains a very small amount of radiation Multiple pictures of your chest will then be taken from different angles 08

10 How is CTEPH confirmed? A normal V/Q scan rules out CTEPH A normal scan Ventilation A person with CTEPH Ventilation Perfusion Perfusion 09

11 How is CTEPH treated? Pulmonary endarterectomy (PEA), is a surgical operation in which the blood vessels of the lungs are cleared of the fibrous blockage PEA is a major operation and can only be performed by a small number of highly skilled surgeons Before deciding you are suitable to have this surgery, you need to be thoroughly assessed at an expert center to make sure that it is appropriate for you: if the blockage is too deep within the lungs, surgery may not be able to reach it your suitability to undergo major surgery will also be assessed Many people assessed for PEA by an expert team do have CTEPH that can be operated on Quality of life and life expectancy are improved in a significant proportion of people who undergo this operation, and can be thought of as a cure for many of them PEA is suitable for about 2 out of 3 people with CTEPH 10

12 What if PEA is not appropriate, or CTEPH recurs? Sometimes, CTEPH may not be appropriate for surgery because of where the blockage is, or a person may be too unwell to undergo the operation, or the potential risks might outweigh the benefits Even after surgery, CTEPH may persist or recur at a later date and so may need further management by your specialist While no medical treatments have been approved for treating CTEPH, the science is always evolving and scientists are always researching new and alternative treatments Your doctor will track your condition carefully and he or she will also be able to keep you up-to-date with any developments 11

13 References: Corsico AG et al. Long-term outcome after pulmonary endarterectomy. Am J Respir Crit Care Med 2008; 178: Galiè N et al. Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J 2009; 30: Jenkins D et al. State-of-the-art chronic thromboembolic pulmonary hypertension diagnosis and management. Eur Respir Rev 2012; 21: Pepke-Zaba J et al. Chronic thromboembolic pulmonary hypertension (CTEPH). Results from an international prospective registry. Circulation 2011; 124: The International CTEPH Association Bayer CTEPH information CTEPH *For non-us and non-uk visitors G.GM.RIO Scan for it, for the chance to surgically cure it