Innovative procedure breathes new life into woman with rare pulmonary disorder

February 29 is Rare Disease Day. Meet a Florida woman, with a rare pulmonary disorder, who is breathing easier after undergoing an innovative procedure.

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CLEVELAND – Pulmonary hypertension was robbing Peggy Notman of her life, one breath at a time.

In her early sixties, Notman experienced a blood clot following her surgery for a double hip replacement. A pulmonologist near her Florida home prescribed several months of blood thinners and oxygen, as needed, but her condition did not improve; it slowly worsened.

“I would take a short walk, with my son and his dog, and be totally out of breath,” said Notman. “They changed my medication and sent me to respiration rehabilitation, but I wasn’t making any headway.”

Notman, a paralegal, and paralegal studies instructor, was forced to retire from her jobs early when she wasn’t able to maintain enough oxygen to complete her two-hour lectures, two times a week. She would quickly get lightheaded and fatigued, even while on oxygen, and soon, she could barely function.

Notman was eventually referred to Jinesh Mehta, M.D., a pulmonologist at Cleveland Clinic Florida.

When testing revealed a number of blood clots throughout her lungs, Notman learned she actually suffered from chronic thromboembolic pulmonary hypertension (CTEPH) – a rare and progressive form of pulmonary hypertension, caused by blood clots that don’t dissolve in the lungs.

Left untreated, CTEPH can lead to heart failure, and in some cases, even death.

“I’d heard of blood clots and embolisms, but I had never heard of CTEPH,” she said. “And I had no real (treatment) options other than to try a bunch of medications, stay on oxygen 24/7 and do the best that I could.”

While a complex surgical procedure — pulmonary thromboendarterectomy — is the gold-standard curative treatment for CTEPH, Notman was among the 30-40 percent of patients who aren’t candidates for the procedure, due to a variety of reasons.

However, in late 2017, Notman learned she was a likely candidate for a new form of CTEPH treatment: balloon pulmonary angioplasty (BPA). The catheter-based procedure is a well-established treatment for blocked vessels in the heart and brain, but has only recently been revived for patients with clots in the lungs.

BPA requires tiny punctures in the neck or groin, through which a balloon catheter is fed into the lungs. The catheter dilates the clot-filled vessels to let blood flow more smoothly. Because pulmonary circulation can be more complex than cardiac circulation, the procedure must be conducted incrementally to avoid hemorrhaging or other injuries.

“After undergoing several of these procedures, Peggy feels much better,” said Gustavo Heresi, M.D., director of the CTEPH and pulmonary vascular program at Cleveland Clinic. “Her shortness of breath is almost gone, her oxygen levels have improved dramatically, and she’s almost essentially back to normal. That’s very satisfying to see.”

“After the second one, I could walk past three houses and back in the neighborhood where we were temporarily living in Cleveland,” said Notman. “After the third procedure, I was able to walk past seven houses. I realized this was really working.”

Today, Notman is able to live without regular use of her oxygen tank, and does not feel lightheaded or weak, as she did before.

“Now I can do the things I need and I want to do,” she said. “I’m even considering going back to teaching after the procedures are complete. I have a new lease on life.”

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