WHAT IS CTEPH. Helping you understand your type of PH. Provided by Bayer to help education of the PH community
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1 Educational program by Material endorsed by WHAT IS CTEPH Helping you understand your type of PH Provided by Bayer to help education of the PH community
2 WHAT IS PH? lood travels from your heart to the B lungs, where it picks up oxygen, by way of the pulmonary arteries I n pulmonary hypertension (PH for short), narrowing of the blood vessels or obstruction within the pulmonary arteries restrict this blood flow, causing blood pressure to rise 4 Oxygen-rich blood is pumped back out to the body 1 3 Blood returns to the heart after delivering oxygen to the body Blood returns to the heart from the lungs 2 Blood gets pumped to the lungs via the pulmonary arteries to pick up a fresh supply of oxygen A s a result, the right heart has to work harder than normal to pump blood to the lungs to be oxygenated I f your lungs don t receive as much blood as they should with each pump, less oxygen gets picked up he medical definition of PH is blood T pressure in the pulmonary arteries which is greater than 25 mmhg at rest (the normal value is between 8 and 20 mmhg)1 If your body doesn t get the amount of oxygen it needs to function normally, it can leave you feeling breathless 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 initially arises from a blood clot pulmonary means relating to the lungs hypertension is the medical term for elevated blood pressure 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 02
4 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 2 It is thought that these blood clots are not completely resolved and remain in the pulmonary arteries 1,3 Over time, extra tissue forms within the clot so that it becomes more fibrous, creating a blockage that restricts the blood flow 3 Blockage restricts blood flow increasing blood pressure in the 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 Images courtesy of Prof B Yildizeli, Marmara University Hospital, Turkey 03
5 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 3 Most frequent symptoms of CTEPH: % 40.5% 31.5% dyspnea (breathlessness) edema (swelling of the tissues) tiredness 15.3% 13.7% chest pain syncope (fainting) 04
6 HOW IS CTEPH 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 05
7 HOW IS CTEPH DIAGNOSED? A V/Q scan is used to help tell the difference between CTEPH and other types of PH 3 This scan is in two parts: 1 2 The first measures The second measures your breathing blood flow in the lung (ventilation) (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 06
8 HOW IS CTEPH DIAGNOSED? A normal V/Q scan rules out CTEPH 3 A normal scan Ventilation Perfusion A person with CTEPH Ventilation Perfusion 07
9 HOW IS CTEPH CONFIRMED? Diagnosis is confirmed in 2 ways: 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 Pulmonary angiogram* To find the blockages in your lungs, a dye is injected into your pulmonary arteries A catheter is inserted into a lung artery (as in right heart catheterization) and an X-ray dye is injected through it The size and shape of the blood vessels can then be imaged * or computed tomography pulmonary angiogram/magnetic resonance angiogram 08
10 HOW IS CTEPH TREATED? CTEPH is the only type of PH that can potentially be cured with a special surgery, pulmonary endarterectomy (PEA), and without the need for a lung transplant. PEA is a surgical operation in which the blood vessels of the lungs are cleared of the fibrous blockage 3 PEA is a major operation and can only be performed by very skilled surgeons Before deciding if you are suitable for having this surgery, you need to be thoroughly assessed at an expert center to make sure that it is appropriate for you: 1 - 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 due to other conditions you may suffer from Many people assessed for PEA by an expert team do have CTEPH that can be operated on 2 Quality of life and life expectancy are improved in a significant proportion of people who undergo this operation, and it can be thought of as a cure for many of them 4 PEA is suitable for about 2 out of 3 people with CTEPH 2 09
11 WHAT IF PEA IS NOT APPROPRIATE, OR CTEPH RECURS? Sometimes, CTEPH may not be appropriate for surgery. This could be down to where the blockage is, what other conditions a person has or whether the person is too unwell to undergo the operation. In these cases 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 Until recently there was no targeted medical therapy available for CTEPH. Now, however, there is an approved pharmacological treatment 5,6 Your doctor will track your condition carefully and he or she will also be able to keep you up-to-date with any developments The best resource is always your PH specialist team. However, here are some useful and informative websites you might want to take a look at: PHA Europe CTEPH Association website Bayer CTEPH information *for non-us and non-uk visitors 10
12 References: 1. Galiè N et al. Eur Heart J 2009; 30: Pepke-Zaba J et al. Circulation 2011; 124: Jenkins D et al. Eur Respir Rev 2012; 21: Corsico AG et al. Am J Respir Crit Care Med 2008; 178: Simmoneau G et al. Eur Respir J 2014:erj Ghofrani HA et al. N Eng J Med 2013; 369: CTEPH Association website Bayer CTEPH information *For non-us and non-uk visitors CTEPH G.MKT.GM.RIO
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