Learn About Chronic Thromboembolic Pulmonary Hypertension (CTEPH)
There are many causes of pulmonary hypertension, or high blood pressure affecting the arteries of the lungs. In some patients, pulmonary embolism or blood clots in these arteries can become organized and form scar-like tissue which block or narrow these vessels, leading to a form of pulmonary hypertension called chronic thromboembolic pulmonary hypertension (CTEPH). This condition is difficult to diagnose leading to a delay in diagnosis and even in some cases, it may be missed. However, once diagnosed, there are a number of treatment options available to patients at medical centers that specialize in this disease.
- After a blood clot in the lungs, CTEPH develops in about 1 in 25 patients despite adequate anticoagulation (treatment with oral or injectable blood thinner medications over time). About one-quarter of patients with CTEPH do not have a known history of prior blood clots. CTEPH can be a cause of persistent shortness of breath that is otherwise unexplained.
- The main symptoms of CTEPH are non-specific and include fatigue, shortness of breath, especially with exercise such as climbing stairs or walking up inclines, swelling of the legs, and dizziness or passing out with exertion.
- CTEPH is screened for by a lung ventilation-perfusion scan (V/Q scan) and the diagnosis is confirmed by other procedures including a right heart catheterization, pulmonary angiography, CT scan or magnetic resonance imaging of the pulmonary vessels and lungs.
- CTEPH is potentially a curable disease if patients are found to be candidates for a surgical procedure called a pulmonary thromboendarterectomy (PTE). During this procedure, the chronic clot is removed. In those patients who are not candidates for surgery, medications and other procedures (such as balloon pulmonary angioplasty) can improve symptoms.
What is CTEPH?
After a blood clot in the lungs (called a pulmonary embolism), the majority of patients will regain normal blood flow in the affected arteries after a few months of anticoagulation with medications such as warfarin, enoxaparin, rivaroxaban or apixaban. In a minority of patients (about 1 in 25) the clot is organizes into scar-like tissue in the vessel despite proper anticoagulation. As these “scars” block blood flow to the pulmonary arteries, the blood pressure increases in these vessels. The normal pressure in the pulmonary arteries is very low (about 25/10 mmHg) and the right side of the heart that pumps the blood to the lungs to get oxygen is normally thin-walled. But in CTEPH, as the pressure in the pulmonary arteries increases, the right side of the heart enlarges and eventually begins to fail as the heart becomes over-stretched. This strain on the right side of the heart leads to the symptoms of CTEPH.
How CTEPH Affects Your Body
The main complaints of patients with CTEPH are primarily due to the right heart straining to pump blood against the high pressures in the pulmonary arteries. The symptoms begin with shortness of breath, especially with exercise like climbing stairs or walking up inclines. As the disease progresses, fatigue (or feeling excessively tired) and possibly light-headedness or passing out with exercise may develop as the heart fails. Given the difficulty pumping blood forward to the pulmonary arteries due to the high pressure, fluid may back up into the abdomen or cause the legs to swell.
How Serious is CTEPH?
If untreated, CTEPH is a chronic and progressive disease that leads to heart failure and can result in death. The good news is that many patients can be cured with pulmonary thromboendarterectomy (PTE) surgery. In those that are not surgical candidates, symptoms can be managed with a medication to treat pulmonary hypertension.
CTEPH Symptoms, Causes and Risk Factors
What are the Symptoms of CTEPH?
The first symptom of CTEPH is typically shortness of breath with exertion. Fatigue, a decline in exercise capabilities, palpitations, and chest heaviness with exertion can also occur. This can progress to light-headedness and even passing out with exercise. Some patients have swelling of their legs. Rarely, coughing up blood can be a problem.
Exercise intolerance, fatigue and dyspnea are the most common symptoms. As the disease progresses, patients may report chest pain, dizziness, leg swelling and lightheadedness.
When the disease is severe, it can lead to syncope. Many of these symptoms can start very mild and progress with time. Many of the symptoms are similar to other diseases. CTEPH is often underdiagnosed and/or misdiagnosed as one of these other disease processes.
What Causes CTEPH? What are the Risk Factors?
CTEPH is caused by chronic blood clots in the lungs that become scar-like tissue blocking or narrowing the pulmonary vessels. About 75% of patients with CTEPH have had a history of having a prior blood clot in their lungs called a pulmonary embolism. Having recurrent blood clots or experiencing a large initial blood clot may predispose to the development of CTEPH. Certain medical conditions such having a known clotting tendency, having your spleen removed, chronic inflammatory conditions like ulcerative colitis, having an infected pacemaker wire, or having a history of cancer can also increase the risk of developing CTEPH.
When to See Your Doctor
If you have had a blood clot or pulmonary embolism and do not return to your expected level of function after three months of anticoagulation, you should discuss the possibility that chronic blood clots are causing your shortness of breath. CTEPH develops in about 1 in 25 patients after a blood clot in the lungs. Furthermore, if you are experiencing shortness of breath that is otherwise not well explained, then the possibility that this symptom might be due to chronic blood clots should explored.
Diagnosing and Treating CTEPH
What to Expect
The diagnosis of CTEPH involves screening for the disease with a lung ventilation-perfusion scan and an echocardiogram (an ultrasound of the heart). If these tests are normal, then CTEPH is ruled out. However, if abnormal, these studies should be followed by confirmatory diagnostic tests. A right heart catheterization will better define the degree of pulmonary hypertension and right heart strain that is present. To confirm the diagnosis of CTEPH and to locate the chronic clots, pulmonary angiography, CT angiography scan or magnetic resonance imaging of the pulmonary vessels and lungs will be necessary.
How is CTEPH Diagnosed?
A ventilation-perfusion scan (performed in the nuclear medicine section of the hospital) is the recommended screening test for CTEPH. In this test, you will inhale a gas to see where air is distributed in the lungs. Then, a radioactive protein will be injected into your vein to see where blood is flowing. If there are blockages to blood flow in the lungs, this is concerning for the presence of blood clots in the lungs.
The heart is evaluated with an ultrasound called an echocardiogram. Signs of pulmonary hypertension seen on the echocardiogram include right heart enlargement or a reduction in right heart function. However, definitive diagnosis for pulmonary hypertension is achieved with a right heart catheterization to measure pulmonary artery pressures and to determine how much blood the heart is pumping over time. Do they want to discuss the RHC procedure?
To establish the diagnosis of chronic blood clots, and to accurately locate the clots, a pulmonary angiogram, CT angiography or magnetic resonance imaging of the pulmonary arteries is necessary. For each study, contrast is injected into the pulmonary arteries so that blockages in these vessels can be identified, and an expert interpreter will determine if these clots are in locations that they can be surgically removed. Ideally, patients should be evaluated by medical centers that specialize in the care and management of patients with pulmonary hypertension, and in particular, CTEPH.
How is CTEPH Treated?
The definitive treatment for CTEPH is a surgery called a pulmonary thromboendarterectomy (PTE), also referred to as a pulmonary endarterectomy. This is an open heart surgery that involves cooling the patient’s body while on heart-lung bypass. Once cool, the heart is then stopped and the chronic blood clots are carefully removed from the pulmonary arteries. PTE surgery is curative in over 90% of patients. And if there’s some residual pulmonary hypertension after surgery, medications to lower the pressures can be prescribed. Not all hospitals that perform open heart surgery perform PTE, thus referral to a specialized center is often necessary.
For those patients who might not be good candidates for PTE, either due to chronic clots that are not easily accessible to the surgeon or who have other medical conditions which make PTE too risky, there are other options. A newer procedure called balloon pulmonary angioplasty uses a tiny balloon which is inflated inside the pulmonary artery to reduce the narrowing, and sometimes open the vessel. This procedure can reduce pulmonary artery pressure and help relieve right heart strain. There is now also a medication called riociguat which has been approved for inoperable CTEPH or residual pulmonary hypertension after PTE surgery. In addition, depending on the best care plan, other pulmonary hypertension medications may be needed to improve symptoms and decrease pulmonary artery pressures for CTEPH patients who might not be candidates for surgery.
Living with CTEPH
Living with CTEPH can be difficult and will affect your life due to shortness of breath. It is important to learn about CTEPH, the medications used to prevent blood clots, and the treatment options including surgery and medications to improve pulmonary hypertension. Some patients with CTEPH may require supplemental oxygen.
What to Expect
All patients with CTEPH need to be involved with their care. First, patients need to ensure that that they are adherent to their medications, especially their anticoagulation. All patients with CTEPH should remain on lifelong anticoagulation to prevent new blood clots from forming. Even patients who undergo the PTE surgery will still need to remain on anticoagulation after the surgery for life. There are many options for anticoagulation available and you and your doctor can discuss which option is best for you. No matter which anticoagulation medication a patient is prescribed, there are risks of bleeding.. The prescribing physician will go through these risks prior to starting these medications.
Additionally, patients should seek referrals to physicians who specialize in CTEPH so that the best treatment option for them can be determined. If you are deemed to be a candidate for PTE surgery, then you should be sent to center with experience doing this operation. Though the needs of each individual may be different, most patients who are candidates for PTE surgery should expect to be in the hospital for about two weeks after surgery. The majority of patients feel their shortness of breath is improved immediately after surgery, but many have pain from the large incision over the breastbone. It takes about 2 months for the bone to heal after it is wired back together. During this time, patients should not drive, lift anything heavier than 10 pounds or submerge the wound in water (i.e. no swimming or baths).
As mentioned, lifelong anticoagulation is recommended to prevent worsening CTEPH from recurrent blood clots. Additionally, a high salt diet and excessive fluid intake can worsen the strain on the right heart. You should limit yourself to about 1 teaspoon (2400 mg) of salt intake per day. If you are on warfarin for anticoagulation, you should strive to eat the same amounts of food containing vitamin K every day. Green, leafy vegetables are sources of vitamin K. Variations in vitamin K intake can lead to fluctuations in the INR, the measure of how thin your blood is on warfarin. Finally, exercise is important. While most patients with CTEPH are limited by shortness of breath, you should be able to exercise at a low intensity like walking. Your body will tell you if you are over exerting yourself. If you develop severe shortness of breath or light-headedness, you should stop to rest.
There are many resources for patients with CTEPH. The Pulmonary Hypertension Association assists patients in finding CTEPH specialists, and provides access to support groups for others with CTEPH, including patients who have undergone PTE surgery. Another excellent patient and physician resource is the website, www.CTEPH.com where patients can view videos of experts discussing the diagnosis and treatment of CTEPH.
Questions to Ask Your Doctor about CTEPH
Making notes before your visit, as well as taking along a trusted family member or friend, can help you through the first appointment with your doctor.
- Do I need a ventilation/perfusion scan (VQ scan) to screen for chronic blood clots and an echocardiogram to screen for pulmonary hypertension?
- Do I need a right heart catheterization to measure the pressures in the pulmonary arteries and or other diagnostic tests to confirm the presence of chronic blood clots?
- Am I a candidate for pulmonary thromboendarterectomy (PTE)?
- What are my options for anticoagulation? What are the risks and benefits of each of these medications. How are these medications monitored.
Questions for Surgical Candidates
- What are the risks of PTE surgery?
- How many PTE surgeries has this center done?
- What are the possible complications from PTE surgery and am I at increased risk for any of these complications?
- How long is expected hospital stay and what will the expected recovery be like?
- Are there any alternatives to PTE surgery?
- Will I need medications for pulmonary hypertension after surgery?
Questions for Patients Deemed Not to be Surgical Candidates
- Can I get a second opinion at another center to determine if I am a candidate for PTE surgery there?
- Am I a candidate for balloon pulmonary angioplasty?
- What medications are available to treat my pulmonary hypertension, if I am not a surgical candidate?
Sandeep Sahay, MD, FCCP
Deborah Jo Levine, MD, MS, FCCP
Jay Peters, MD, FCCP
Date Last Reviewed