How Surgery Can Fix CTEPH, a Rare Type of Pulmonary Hypertension

Early in 2022, Tamara Coleman started to experience episodes of shortness of breath—she’d never had asthma, but now she’d be in the middle of a conversation and find herself struggling to breathe. Gradually, she noticed other confusing symptoms, including weight gain to the point where her clothes no longer fit and pain that limited her ability to clean her house.

She had always been healthy and active. At almost 50 years old, she had owned a home daycare, worked with toddlers in a Head Start program, and taken classes in early childhood education. As her own six children started to grow up and leave home, her plan was to work as long as she could and then spend her retirement enjoying her grandchildren.

Those plans came to a halt in 2018, after surgery for a back injury from a bad fall. That forced her to retire early and confine her work to physically easier temporary positions. But the shortness of breath and other new symptoms were a new problem. In April 2022, with her feet so swollen she could barely put on her shoes, her family took her to the Yale New Haven Hospital emergency department.

Coleman spent 10 days in the hospital, where she received a surprising diagnosis: chronic thromboembolic pulmonary hypertension (CTEPH), a rare, progressive, potentially fatal condition. It develops when blood clots in the lungs (called pulmonary embolisms) block blood vessels in the heart for an extended period of time.

“CTEPH is a condition associated with high mortality,” explains Prashanth Vallabhajosyula, MD, MS, a Yale Medicine cardiac surgeon and surgical director of the Yale Aortic Institute, part of the Yale New Haven Health Heart & Vascular Center. “We can give medications to help clear out the clots, but, at times, there is some remnant clot that stays in the arteries in the lungs.” The gold-standard treatment, he says, is a complex open-heart surgery called a pulmonary thromboendarterectomy that only a few surgeons can perform.

Dr. Vallabhajosyula and his Yale colleagues follow hundreds of patients with the condition, whether they have undergone the surgery or take medication (a comorbidity, or other condition, may make surgery too risky). Some patients have traveled to Yale from other parts of the country to have their CTEPH treated; Coleman lives 12 minutes away.

Below, Tamara Coleman, Dr. Vallabhajosyula, and Yale Medicine pulmonary vascular disease specialist Phillip Joseph, MD, talk to us about CTEPH.

Up to 3,000 cases of CTEPH are diagnosed each year in the United States, and they affect men and women equally, according to the American Lung Association (ALA). People—even children—can develop the condition at any age, although it is most common in adults, especially as they get older.

CTEPH usually starts with a pulmonary embolism caused by a blood clot that forms in the leg or groin and travels up through the body to the lungs—a process that may occur multiple times. Dr. Vallabhajosyula says that in about 3% to 5% of people with a pulmonary embolism, clots get stuck in the lungs and remain there long-term. They clog the arteries in the lungs, and a scar-like tissue forms around them, limiting blood flow.

This leads to a progressive form of pulmonary hypertension. Unlike hypertension (or high blood pressure) in the rest of the body, this affects only the blood vessels in the lungs, and it can cause the blood pressure to rise almost threefold. The right side of the heart is forced to work harder than normal to pump blood into the lungs, which, over time, can cause right-sided heart failure.

It’s difficult to predict who might develop CTEPH before symptoms start, but the ALA notes that people are at higher risk for the condition if they have a history of one or more clots in the lungs, have high blood pressure at the time a blood clot is diagnosed, or have had pulmonary hypertension for at least six months after a clot was diagnosed in the lung.

While estimates vary, Dr. Joseph, who diagnosed Coleman while she was in the hospital, says someone with untreated CTEPH has a life expectancy of three to five years.

But CTEPH often goes undiagnosed and can progress slowly and silently for months or years. “The majority of my patients were never told they had a blood clot,” Dr. Joseph says. But an individual who has one will start to experience shortness of breath with exertion and then other symptoms, such as fatigue, chest pain, dizziness, heart palpitations, tightness in the chest or stomach, and swelling in the legs and abdomen.

If there is any suspicion that a person could have CTEPH—severe shortness of breath being the primary sign—they need to consult a pulmonary hypertension specialist, Dr. Vallabhajosyula says. “Primary care specialists, general cardiologists, and pulmonologists don't deal with the disease on a day-to-day basis,” he says. Patients at Yale have a team of physicians, surgeons, pulmonary hypertension specialists, cardiologists, and radiologists collaborating in a shared decision model to care for them.

These specialists prescribe some combination of tests to diagnose the condition, visualize the clots, and determine the best treatment.

These tests may include (but are not limited to):

• An echocardiogram to assess blood pressure in the lungs
• A V/Q scan, which is actually two tests: a ventilation scan to measure airflow and a perfusion scan to measure blood flow in the lungs
• A pulmonary angiogram, another way to assess blood flow to the lungs; it involves inserting a catheter into the pulmonary artery.
• A right heart catheterization, which measures blood pressure inside the heart and lungs
• A dedicated CT scan, which shows the clots in the lungs and allows the radiologist to see which areas are not getting blood flow

A pulmonary thromboendarterectomy (also known as pulmonary endarterectomy) removes the clots and scar tissue from the arteries.

“The operation is technically difficult because it’s not always easy to find the casts [scar tissue]—they get stuck to the inner wall of the arteries, and we have to peel them off carefully without damaging the vessels,” Dr. Vallabhajosyula says.

Like other open-heart surgeries, a pulmonary thromboendarterectomy involves making an incision in the chest and dividing the breastbone to get to the heart—and, in this case, opening the arteries to the lungs. The patient is hooked up to a heart-lung bypass machine, which takes over the work of the heart, allowing the surgeon to stop the heart and work on the arteries. The blood is cooled as it passes through the heart-lung machine, then spreads through the body, cooling it down, which protects the brain and organs during the operation.

“The only way to safely remove the chronic casts inside the pulmonary arteries is to do it during a brief period of circulatory arrest, when all blood flow in the body is completely halted,” Dr. Vallabhajosyula says. The surgery takes four to six hours, but a surgeon’s time to remove the casts is typically limited to 20 minutes for each lung.

Coleman was extremely nervous about proceeding with surgery. Dr. Joseph says some patients with CTEPH want the surgery as soon as possible, but others are not quick to agree. He gave her medication to treat the condition temporarily and continued to monitor her. “I kept telling her to come back and then we'll talk about it again,” he says. “For some people, it takes time to feel comfortable with it.”

She finally agreed to have the operation, which was scheduled for February 2023. In the days leading up to it, Coleman took medication for anxiety, which helped. “I went into the hospital knowing I would have to go through this battle,” she says.

When the day arrived, the surgery started in the morning and finished in the evening, and she was surprised by how she felt as she woke up. “When I came out of it, I was not in pain. I realized everything was intact, and my heart was beating. My first thought was, ‘Thank you, God, I’m alive,’” she says.

“People notice improvements in their symptoms right away,” says Dr. Vallabhajosyula. There is still the recovery process from open-heart surgery, which requires a hospital stay of a week to 10 days. Then, most patients go home, where they are visited by physical therapists, visiting nurses, and other support services. While they can’t lift anything heavy for the first two months, many are back to driving locally a couple of weeks later, and most report feeling much better in six to eight weeks, Dr. Vallabhajosyula says.

People who have had a pulmonary thromboendarterectomy are more prone than other people to developing more clots, so they must take a blood thinning medication for the rest of their lives.

A pulmonary thromboendarterectomy has a 96% success rate, meaning it significantly improves symptoms. Many patients are cured of the disease, meaning their pulmonary hypertension goes away and their symptoms disappear. “I'd say there’s a 75% cure rate,” Dr. Joseph says. In some patients, pulmonary hypertension goes down dramatically in the operating room. It’s assessed six months later with a repeat right heart catheterization.

“Our goal is to get them to a normal life,” Dr. Joseph says.

However, success can depend on such factors as the patient’s age and general health, whether they have other medical conditions, and how advanced the disease is. “Many people who come to our clinic have advanced disease and surgery helps a lot, but it doesn't cure it,” Dr. Joseph says. Those patients may need additional therapy after their surgery, such as medication, along with a balloon pulmonary angioplasty (more on that below).

About 30% of people with the condition aren’t candidates for a pulmonary thromboendarterectomy—they may not be healthy enough, or they don’t want to proceed with the surgery. Some medications help clear out the lungs, but they may not work well enough, Dr. Vallabhajosyula says.

Some of these patients may be able to have a much less invasive balloon pulmonary angioplasty, in which a catheter with a small balloon at its tip is inserted into the distal pulmonary arteries, then inflated to open the artery and let more blood flow through. The balloon is then inflated to widen the narrowed or blocked blood vessels, improving blood flow and reducing shortness of breath.

While it’s not “the gold standard treatment, “this was recently shown to provide better outcomes than medical management,” Dr. Vallabhajosyula says.

Other patients are treated with medications that help clear out the lungs, “but some remnant of the clot can remain in the lungs and in the arteries in the lungs, and eventually it will form the casts that close off the blood vessels,” Dr. Vallabhajosyula says.

Based on her experience, Coleman now tells friends and loved ones to pay attention to their bodies. “If something is changing in your body, keep a running record of it,” she says. “If you have shortness of breath or anything that could be a sign that your body is imbalanced, check it out.”

A year after her operation, her shortness of breath is long gone—Dr. Joseph says her symptoms improved significantly within six months after surgery. She is no longer working and walks with a cane, partly because of her previous back injury. “But I’m able to sit upright with my feet on solid ground without my feet swelling,” she says. “I feel like I never had that surgery.”

Most of Coleman’s family lives nearby, and she spends as much time as she can with her grandchildren. There are eight of them now, ages 5 months to 11 years. “Being around children again brings me so much joy,” she says, adding that she’s happy to be alive. “It’s a beautiful thing,” she says.