Online Health Chat with Gustavo Heresi, MD, MPH
November 24, 2014
Pulmonary hypertension is a rare and serious lung disorder. It occurs in people of all ages, races and ethnic backgrounds. One of the more severe causes of pulmonary hypertension is due to unresolved blood clots in the lungs, which is called chronic thromboembolic pulmonary hypertension (CTEPH). Although CTEPH can cause serious illness and can even be fatal in certain cases, it can be cured in some patients through a surgical procedure that removes the obstructions in the lung. Other causes of pulmonary hypertension may require a lung transplant to treat the patient.
The treatment of choice for CTEPH is a complex surgery called pulmonary thromboendarterectomy, which can potentially cure this disease. Cleveland Clinic is one of the few centers in the country with expertise in this procedure.
About the Speakers
Gustavo Heresi, MD, is a staff physician in the Department of Pulmonary, Allergy and Critical Care Medicine at Cleveland Clinic. His specialties include pulmonary hypertension, CTEPH, acute respiratory distress syndrome (ARDS), pneumonia, pulmonary fibrosis, scleroderma and sepsis.
Dr. Heresi-Davila completed his fellowship in pulmonary and critical care medicine at the Cleveland Clinic following his residency in internal medicine at Jackson Memorial Hospital in Miami. Dr. Heresi-Davila graduated from medical school at Universidad Nacional de San Agustin Facultad de Medicina in Arequipa, Peru. Dr. Heresi-Davila is board-certified in the fields of internal medicine, critical care medicine and pulmonary disease.
Let’s Chat About CTEPH
Moderator: Let's get started with our questions...
ABCs of CTEPH
MegN: What is CTEPH?
Gustavo_Heresi,_MD: CTEPH means chronic thromboembolic pulmonary hypertension. This means high blood pressure in the lungs due to blood clots that, for some reason, never went away after an episode or more of pulmonary embolism and got stuck inside the pulmonary arteries. This is a type of pulmonary hypertension that is very important to diagnosis because it is the only type that can be cured with an operation.
MegN: What causes CTEPH?
Gustavo_Heresi,_MD: The risk factors for CTEPH are the same as the risk factors for blood clots in the legs or the lungs. These are a variety of medical conditions and situations, such as being sick in the hospital, fractures, taking birth control pills, smoking, obesity and cancer, among others. People with these conditions tend to get blot clots, yet most of them, with the help of blood thinners, are able to clear them. A minority of people go on and develop CTEPH after having blood clots for reasons that remain unclear.
MegN: Are there pre-existing conditions that may trigger CTEPH?
Gustavo_Heresi,_MD: Yes. They include having one or more episodes of blood clots in the legs or lungs, cancer, permanent intravenous catheters, spleen removed, infected pacemakers, blood group other than Type O, and certain antibodies such as lupus anticoagulant and antiphospholipid antibodies. However, it is important to note that even without these pre-existing conditions, CTEPH can develop. As a matter of fact, up to a quarter of the patients that we see with CTEPH do not tell us about a prior history of blood clots.
LucyJ: If someone had blood clots in their legs, are they more apt to get CTEPH?Gustavo_Heresi,_MD: Yes. Blood clots in the legs are the most common source of blood clots in the lungs, and CTEPH starts off with blood clots in the lungs that do not go away even when taking blood thinners. However, most people with blood clots do not go on and develop CTEPH. Yet, because blood clots are so common, CTEPH is probably more common than we think, and it is certainly underappreciated as a cause of shortness of breath and elevated blood pressure in the lungs
MegN: How is CTEPH diagnosed?
Gustavo_Heresi,_MD: The best screening test for CTEPH is something called the V/Q scan. This is a nuclear medicine test that looks for areas of the lungs that are not getting enough blood flow. This test is absolutely necessary in everybody with pulmonary hypertension of unclear reasons and in those with persistent shortness of breath or fatigue after having blood clots. If the V/Q scan is abnormal, we further study this with a CT scan of the chest and detailed pictures of the pulmonary arteries with a pulmonary angiogram. Finally, to precisely measure the pressures in the lungs, we always need a right heart catheterization.
Tommy: What is a V/Q scan that you mentioned for diagnosing CTEPH?
Gustavo_Heresi,_MD: The V/Q scan stands for ventilation/perfusion scan. You go to nuclear medicine facility; they will place an IV in your arm. Then they have you inhale a gas that is labeled with radioactive marker. Then they will inject a similar radioactive marker substance into your IV. Finally, they will scan your chest and will put the pictures together to find areas of your lungs that are not getting enough blood flow, as they are occluded by chronic blood clots. This test is very safe, has few to no side effects and is extremely effective in detecting blood clots as the reason for pulmonary hypertension or shortness of breath.
Living with CTEPH
Buriser: Does having CTEPH affect my ability to work?
Gustavo_Heresi,_MD: Unfortunately yes. Most people with CTEPH are limited by shortness of breath and fatigue with activities. So, depending on the kind of job that you have, it can certainly be an issue. The good news, however, is that CTEPH can be effectively treated and cured in the vast majority of patients with an operation called pulmonary thromboendarterectomy (PTE). The key is to get an evaluation at a medical center with experience in CTEPH and PTE. After PTE, most people are able to go back and resume normal levels of activity. Here at Cleveland Clinic, we have an experienced CTEPH and PTE center with a dedicated team of specialists and would be more than happy to evaluate your case.
Elizabeth: Can you travel if you have CTEPH and, if so, what distances? What precautions do I need to plan for?
Gustavo_Heresi,_MD: You need to be cautious about travel, but you can travel if you take certain precautions. First of all, if you use oxygen, you need to make arrangements with the airline and your doctor for a portable oxygen concentrator to be used during the flight. Second, you need to be sure you take blood thinners recommended by your doctor, stretch your legs and walk around every hour or so. Finally, make sure you take your CTEPH medication consistently.
Elizabeth: Are there any diet restrictions or a special diet that should be followed?
Gustavo_Heresi,_MD: The diet restrictions are the same as those that people on warfarin (Coumadin) need to follow, such as being consistent with the amount of green, leafy vegetables that you eat every week. There are no special diets or restrictions regarding CTEPH specifically.
ChloeMc: With CTEPH, I have a lot of fatigue, but I still want to exercise as much as possible. Are there any exercise restrictions?
Gustavo_Heresi,_MD: Yes. You should avoid weight lifting, particularly objects heavier than 20 pounds. If you are more stable on therapy, aerobic exercise – such as walking or light jogging – is actually recommended and is likely to help your breathing and fatigue. If you are a candidate for PTE surgery, you should pursue that because you would recover normal stamina if the operation is successful.
The Topic is Treatment
MegN: Would you explain pulmonary thromboendarterectomy (PTE) surgery?
Gustavo_Heresi,_MD: PTE is a complex operation. It requires a median sternotomy, which is the same incision performed during coronary bypass surgery. You are connected to the heart-lung machine, meaning that your breathing and circulation are done by a machine. The surgeon makes an incision in the pulmonary arteries and then removes all of the blood clots from your lungs, which at this point are hard like a keloid scar. During certain periods of time, the heart-lung machine needs to be stopped so that there is no blood flow through the body. This is called circulatory arrest. The reason for circulatory arrest is to provide the surgeon with a completely bloodless field so the chronic blood clots can be seen and removed. To protect the body during these periods of circulatory arrest, the body is cooled down to 68 degrees Fahrenheit.
OnWaNo: Is there any type of medical therapy or treatment for CTEPH, or is surgery the only option?
Gustavo_Heresi,_MD: Yes there is, however, it is important to remember that PTE surgery is the treatment of choice for most patients with CTEPH. Only in patients who have been evaluated at an expert PTE center and deemed not to be candidates for the operation is medical therapy indicated. Medical therapy can also be used in patients who underwent PTE surgery but are left with persistently elevated pulmonary pressures. There is one FDA-approved medicine for these situations called riociguat.
Elizabeth: Are there any clinical trials for CTEPH?
Gustavo_Heresi,_MD: Yes. A very important national registry of CTEPH is about to be launched in the US. In this registry, several expert CTEPH and PTE centers will collect a lot of information from patients with CTEPH that will allow us to understand and answer a lot of open questions that we still have regarding CTEPH. Cleveland Clinic CTEPH and PTE center will participate in this registry. I would like to encourage every CTEPH patient to participate in this registry, as there are no risks involved. The registry will collect information that is routinely obtained for clinical reasons.
LucyJ: Are most patients with CTEPH on oxygen therapy as well as other medications?
Gustavo_Heresi,_MD: Many CTEPH patients are on oxygen but not all. We routinely test for the need of oxygen in our patients. All CTEPH patients should be on blood thinners for the rest of their lives. All CTEPH patients should be evaluated for PTE surgery. Some patients can be treated will a medication called riociguat to lower their pulmonary pressures, but only if they cannot get PTE surgery as determined by an expert center.
What About Recovery?
MegN: What restrictions are there post pulmonary thromboendarterectomy (PTE) surgery?
Gustavo_Heresi,_MD: The hospital stay for PTE surgery is usually one to two weeks. After discharge, it takes about three to six weeks to recover from the effects of the operation. During this time, physical activity should be limited, and driving is usually not possible as patients cannot turn their torso from side-to-side. After six weeks, most patients are able to resume physical activities, and we actually recommend pulmonary rehabilitation around this time. By three to six months, most patients feel much better and are able to return to an essentially normal life.
Sby475: Can you get CTEPH again and why?
Gustavo_Heresi,_MD: Yes you can. The main reason this could happen is not being diligent with anticoagulation therapy after the PTE surgery. Aside from anticoagulants, a way to prevent recurrence is to have an inferior vena cava (IVC) filter inserted prior to the operation. This is a metallic, umbrella-like device that prevents blood clots from the legs from getting to the lungs. Thus, with appropriate anticoagulation (blood thinners) and the IVC filter, CTEPH will most likely not come back.
Moderator: That is all the time we have today for questions. Thank you everyone for participating today; and thank you, Gustavo Heresi MD, for your insightful answers to our questions about the management and treatment of CTEPH.
Gustavo_Heresi,_MD: I would like to thank everyone who joined today. CTEPH, when diagnosed correctly, can be cured with surgery. Unfortunately, CTEPH is frequently not recognized. This is why we encourage everybody to screen for this disease with a V/Q scan and to refer patients to an expert CTEPH center. Here at Cleveland Clinic’s CTEPH program, we are always available to answer any questions that you may have.
To make an appointment with Gustavo Heresi-Davila, MD, or any of the other specialists in Pulmonary and Critical Care Medicine at Cleveland Clinic, please call 216.444.6697 or toll-free 800.223.2273, ext. 55763. You can also visit us online at http://www.clevelandclinic.org/respiratory.
For More Information
On Cleveland Clinic
The pulmonary program at Cleveland Clinic Respiratory Institute is ranked third in the United States by U.S .News & World Report. For nearly 20 years, we have been caring for patients with all forms of pulmonary hypertension, including idiopathic pulmonary arterial hypertension (PAH), chronic thromboembolic pulmonary hypertension, portopulmonary hypertension and pulmonary hypertension associated with connective tissue diseases.
Our physicians and nurses have special expertise and interest in pulmonary hypertension, and are dedicated to the evaluation and care of patients with pulmonary hypertension. Teams are comprised of pulmonary and critical care physicians, advanced practice nurses, research nurse coordinators and research fellows. We also find it important to collaborate closely with the departments of cardiovascular medicine, cardiovascular imaging, cardiothoracic surgery and lung transplantation, as well as specialists in hepatology, liver transplantation, sleep medicine and rheumatology. This enables us to provide the most comprehensive care for our patients and the best treatment options available for pulmonary hypertension.
On CTEPH: Health Information
Learn more about symptoms, causes, diagnostic tests and treatments for COPD
For additional health information visit clevelandclinic.org/health.
For additional information about clinical trials: http://my.clevelandclinic.org/lungs-breathing-allergy/departments-centers/pulmonary-hypertension.aspx.
For additional information about clinical trials: ClinicalTrials.gov.
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