We tend to think of high blood pressure as a problem that affects the heart. But in chronic thromboembolic pulmonary hypertension (CTEPH), the blood pressure in your lungs can rise, often to dangerous levels. While this relatively rare condition can be life threatening, there’s good news: CTEPH is the only type of pulmonary hypertension that can be completely resolved with surgery in some people.\nCTEPH is Rare But Risky\nHigh blood pressure in the blood vessels within your lungs is known as pulmonary hypertension. This problem can force the right side of your heart to work harder, which over time causes your heart to enlarge.\nHigh blood pressure is not just a problem of the heart - it can affect your lungs too. #CTEPH Click To Tweet\nCTEPH is a form of pulmonary hypertension that can occur when a patient develops a chronic pulmonary embolism, where an embolism (clot) gets stuck in one of the lung’s blood vessels and turns into scar-like tissue. This can block blood flow within the lung, leading to symptoms such as chest pain, shortness of breath \u2014 especially during and after physical activity \u2014 and fatigue.\nPulmonary embolisms tend to form in people who have been sedentary for long periods of time due to illness, surgery, or even a long plane flight. Smoking, obesity, use of birth control pills, and conditions like heart disease and cancer can also increase your odds of experiencing a pulmonary embolism.\nCTEPH Causes and Risk Factors\nHaving one or more pulmonary emboli during your lifetime is a major risk factor for developing CTEPH. You’re also more likely to develop CTEPH if you:\n\nHave had your spleen removed\nHave or have had cancer\nHave a chronic inflammatory disease, such as inflammatory bowel disease\nHave a family history of pulmonary hypertension\nAre female\nAre otherwise at high risk for developing blood clots\n\nHowever, some people can develop CTEPH without having these or any other risk factors for the condition.\nCTEPH Tests and Diagnosis\nIf your doctor suspects that you might have CTEPH, he or she may use one or more of the following tests to diagnose it:\n\nVentilation\/Perfusion Scan or “V\/Q Scan” \u2013 This test shows any areas in the lung that are not receiving the appropriate amount of blood flow due to blockage of the arteries by clots.\nEchocardiogram \u2013 Also known as a heart sonogram, this painless procedure is often used to make a preliminary diagnosis by estimating the pressures in the right heart and assessing how well the heart is functioning.\nCT scan or CT angiogram \u2013 This test is to look for blood clots and to evaluate the condition of the rest of the lung besides the arteries.\nPulmonary angiography \u2013 X-rays are used to show blood flow through the lungs.\nHeart catheterization \u2013 This test measures the pressure inside the arteries.\n\nWhen properly treated, people with CTEPH can live as long as those without any history of pulmonary hypertension.\nCTEPH\u00a0Treatment Options\n\nPulmonary thromboendarterectomy (PTE) \u2013 Surgeons open the pulmonary arteries and remove the clot, restoring proper blood flow to the lungs. Depending on how advanced the disease is, this surgery can cure up to 90 percent of all patients.\nMedications \u2013 In the 10 percent of people for whom PTE doesn’t work, medications can provide a secondary treatment option.\nLung transplant \u2013 When surgery or medications fail to cure CTEPH, some people may become candidates for a lung transplant.\n\nYour doctor can tell you more about these options and answer any questions you have about CTEPH.\nTo learn more about CTEPH or to schedule an appointment with a CTEPH specialist, call \u00a0412-648-6161, or toll-free at \u00a01-877-PH4-UPMC, or email PHprogram@upmc.edu.