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.
How many people have CTEPH? How successful is the surgery—and are there other alternatives? Tamara Coleman, Dr. Vallabhajosyula, and Yale Medicine pulmonary vascular disease specialist Phillip Joseph, MD, talked to us about CTEPH.
