Chronic Thromboembolic Pulmonary Hypertension (CTEPH) Chronic Thromboembolic Pulmonary Hypertension (CTEPH) Share on email Share on print To understand

this condition, it is important to understand the role of the pulm onary circulation. It is also helpful to understand the key words associated wit h the condition. The Pulmonary Circulation The pulmonary circulation is made up of the blood vessels that carry oxygen-rich blood through the lungs (the pulmonary system). As the blood flows though the l ungs, it loads up with oxygen and loses the carbon dioxide it is carrying. Then, the oxygen-rich blood returns to the heart through the left upper chamber (atri um), flows into the left lower chamber (ventricle) and out through the aorta to all cells in the body. Once the oxygen is lost from the blood, the veins carry it back to the heart fro m all over the body. It flows into the heart s right atrium. After the atrium is f illed with blood, it squeezes (contracts) and sends the blood into the right ven tricle. When the right ventricle fills and contracts, it pushes blood into the p ulmonary artery. The pulmonary artery goes into the lungs through the pulmonary circulation. Key Words Chronic is a medical term that describes a disease or condition that continues f or a period of months or years. A Pulmonary Thromboembolism refers to the clots that travel from veins in the bo dy (emboli) and clog the arteries in the lungs. Once lodged in the lung arteries , they can cause more clots to form (thrombosis) and add more resistance to the blood flow through the lung. This increased resistance from the clots increases the pressure inside the lungs . The medical term for increased pressure is Hypertension. Combining these key w ords gives us the meaning of chronic thromboembolic pulmonary hypertension: High blood pressure in the pulmonary arteries that lasts six months or longer. The c ondition often happens after there is a pulmonary embolism. What causes CTEPH? When the body cannot resorb a pulmonary embolism (PE), it can trigger CTEPH. How ever, up to half of all patients with CTPEH do not have a history of PE. In 2008 , the World Council on Pulmonary Embolism reported that approximately 2 to 4 per cent of people with PE develop CTEPH. It is a relatively rare disease. Only abou t 5,000 people in the United States are diagnosed with CTEPH each year. Because many patients with CTEPH have no history of PE, or don t know they have PE, it can be overlooked or not suspected. Risk factors for PE and CTEPH Risk factors for pulmonary embolism include: Long periods of inactivity. This can include bed rest due to surgery or illn ess, or even a long trip by car or plane. Certain types of surgery, such as joint replacement of the hip or knee Older age Certain diseases, including peripheral artery disease, heart disease and can cer Family or personal history of blood clots Smoking

Obesity A history of taking synthetic estrogen in the form of birth control pills or hormone replacement therapy The risk factors for developing CTEPH include: Large PE or more than one episode of PE Previous surgical removal of the spleen Chronic inflammatory diseases, such as inflammatory bowel disease and osteom yelitis History of cancer Female sex Hypercoagulability disorders What are the symptoms of CTEPH? Many people who develop pulmonary hypertension after PE go through a so-called ho neymoon period when they do not have any symptoms. When symptoms develop, they ar e vague and non-specific in the early stages of the disease. These include: Shortness of breath with exercise Chest discomfort Fatigue Symptoms of later-stage disease include fainting and signs of right heart failur e, such as fluid retention (edema) and blue-tinged fingers and toes (cyanosis). Some patients with CTEPH never have any early symptoms. Most times, the disease is discovered in these patients when they develop late-stage symptoms or during an autopsy. How is CTEPH diagnosed? If a physician suspects a patient has CTEPH, the next step is to perform imaging studies and specialized tests. These may include: Transthoracic echocardiogram (TTE) an ultrasound of the heart. TTE is used t o screen for pulmonary hypertension. Ventilation-perfusion (V/Q) scan - radioactive material (radioisotopes) is i njected to how well air moves through the lungs and how well the blood circulate s through the lungs. In most cases, a normal result from a V/Q scan means the pa tient does not have CTEPH. Pulmonary angiography X-rays that show blood flow through the lungs. This te st is used to confirm a diagnosis of CTEPH after the patient has an abnormal V/Q scan. The combination of a V/Q scan and pulmonary angiography is considered the gold standard for diagnosing CTEPH. Heart catheterization - checks blood pressure and flow in the heart s arteries and chambers. At Cleveland Clinic, a heart catheterization is a routine part of the diagnostic work-up for patients with any type of pulmonary hypertension. Computed tomography (CT) pulmonary angiography a specialized type of CT scan that shows visual slices of the pulmonary arteries and gives more details about t heir physical condition. This test also shows visual slices of the lung tissue to help the physician determine the health of the tissue. Pulmonary function tests used to make sure the lung tissue is not significan tly diseased. How is CTEPH treated? A surgical procedure called a pulmonary thromboendarterectomy is currently the r ecommended and only effective treatment for patients with CTEPH. During this com plex procedure, the surgeon cleans out the blockages in the pulmonary arteries i

n the right and left lungs using specialized tools. The surgery is performed thr ough a chest incision while the patient s heart is stopped. A heart-lung machine t akes over the function of the heart and lungs during the procedure. This surgery is considered a highly specialized procedure that should be perform ed only by an experienced team that includes cardiothoracic surgeons, anesthesio logists, perfusionists and intensivists at a medical center that has a successfu l track record with the procedure. How well do patients do after the surgery? When performed by an experienced team at a medical center with successful outcom es for this procedure, pulmonary thromboendarterectomy results in a complete cur e in about 90 percent or more of patients. Success is measured by heart size and function and return of pulmonary blood pressure to normal limits. As surgical t echniques and technology advance, outcomes continue to improve, and surgical mor tality is now less than 3 percent. Who can be considered for surgical treatment? At Cleveland Clinic, all patients with CTEPH are screened and evaluated for poss ible pulmonary thromboendarterectomy. The decision to perform surgery is based o n how severe the patients symptoms are, how high the pulmonary blood pressure is and abnormalities of the pulmonary circulation, whether the diseased areas in t he pulmonary arteries can be reached during surgery, and the patient s other medic al conditions. Patients with severe lung disease or disease of the heart s left ventricle are not eligible for surgery. For patients who have coronary artery disease, coronary a rtery bypass grafting (CABG) surgery can be performed at the same time as the pu lmonary thromboendarterectomy. Lung transplantation may be an option for some pa tients. What are the potential risks or complications of pulmonary thromboendarterectomy ? About 10 percent of patients who have a pulmonary thromboendarterectomy continue to have pulmonary hypertension after the surgery. This condition causes about 7 5 percent of the deaths that occur within 30 days after surgery and 50 percent o f deaths in the long-term. Studies show that patients who have pulmonary hyperte nsion after the procedure were usually not good candidates for the surgery. This is because most of these patients have disease in small arteries that cannot be reached by the surgeon. About 60 percent of patients develop fluid in the lungs after surgery. This is u sually caused by slight injury to the lungs during surgery. The fluid goes away with time. For a small percentage of patients, the condition is severe enough to be life-threatening, and they may need ventilator support until the fluid is go ne. Is a medical treatment available for CTEPH? Several different classes of drugs (prostanoids, endothelin receptor antagonists , phosphodiesterase-5 inhibitors) that are used for other types of pulmonary hyp ertension have been suggested as treatment of patients with CTEPH. To date, ther e has been only one randomized, controlled trial (the type of clinical study the U.S. Food and Drug Administration [FDA] requires for approval of a new treatmen t) to see how well these drugs work against CTEPH. The results of the trial were negative. Because there is no evidence of any benefit to patients with CTEPH, a ll these drugs are considered experimental and are not a suggested treatment. At Cleveland Clinic, patients who are not candidates for surgery and those who d evelop pulmonary hypertension after surgery are treated with specialized medicin es known to be effective against pulmonary hypertension .

How can I learn more about CTEPH or find a physician who treats patients with th is disease? To learn more CTEPH and treatment or for a referral to a physician, contact us o r call: Dr. Gustavo Heresi Davila 216-636-5327 Dr. Nicholas Smedira 216-445-7052 References/Suggested Reading Kim N. Chronic Thromboembolic pulmonary hypertension. Medscape. www.medscape.org/viewarticle/556058_6.2007. Accessed June 2, 2012.