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Rare Aneurysm Condition Cured with Cutting-Edge Surgery

Aug 01, 2017

Marcia Waggoner's primary care doctor had never seen anything like it. The 76-year-old from St. George tried showing the CT scan to her son, a radiology tech, who passed it along to the team of physicians he works with.

One took a look at the scan and said "Dude, is this your mom?"

"Those were his literal words," Waggoner remembered. "It was just too odd. It kept moving up the chain."

Her son began calling around to various experts and health care systems, determined to find someone who could help his mom. She had an abnormality in her aortic arch that had led to the creation of an aneurysm. The technical term is aberrant right subclavian artery (ARSA) with aneurysmal degeneration. It's a congenital problem in which the artery that goes down the arm comes off the wrong place in an area that is weaker. After time, it can cause a dangerous aneurysm near the body's largest artery that, if it ruptures, can be fatal in about three minutes.

Around the same time of the aneurysm discovery, Waggoner suffered a knee injury. Her local doctors declined to surgically repair her knee as long as she had an aneurysm, fearing that if it ruptured during surgery they would not have the resources to save her. Until then, Waggoner had been reluctant to fix the aneurysm, preferring to take her chances.

"My knee [problem] made up my mind for me," Waggoner said. "It was a miracle that occurred in my life. What if my appendix had gone out? I would've died of appendicitis. I had to deal with [the aneurysm]."

She wouldn't have to travel far. The University of Utah Health Aortic Disease Program's vascular surgeons regularly repair and reroute the arteries of patients with ARSA, which itself is not as rare as Waggoner suspected, occurring in about 1 percent of people. What made Waggoner's case unique is that ARSA typically becomes symptomatic in a person's teens, 20s or early 30s. Waggoner had seemingly reached her mid-70s without symptoms.

U of U Health vascular surgeon Claire Griffin, MD, who would become Waggoner's doctor, had never treated a patient as old as Waggoner with this particular disorder. Still, she was matter-of-fact about what it would take to fix it.

"We can take care of things that are a bit less common because they're not scary to us, and they're not rare or strange or intimidating," she said.

Griffin said that patients with aneurysms who go to smaller hospitals often run into the same roadblock that Waggoner encountered: physicians declining to perform less serious surgeries as long as the patient has an aneurysm.

"There isn't actually any sort of known or described increased risk of the aneurysm rupturing at the time of another operation, so it's possible that if she'd come (to have orthopedic surgery at our hospital), they may have done her knee surgery," Griffin said.

Research-based or not, the line of thinking leads to Griffin often caring for patients who don't necessarily care if their aneurysm is fixed.

"They want this other thing fixed that no one will touch because they have the aneurysm," Griffin said.

In Waggoner's case, her aneurysm was far more problematic than the average aneurysm. Not only was it in danger of rupture, it was causing a symptom that Waggoner had never acknowledged or mentioned: a problem with swallowing. Because an aneurysm in this area can compress the esophagus, patients often feel like they can't fully clear their throat. An issue with trying to swallow and feeling like something is stuck, along with hoarseness, is really the only symptom.

"It was sort of only in retrospect that [Waggoner] said 'Oh, I think swallowing has been an issue over time,'" Griffin said. "She's pretty stoic in the sense that I think she's not a complainer, so she may have been having symptoms for a couple of years and just never really thought it merited talking about."

Waggoner, a busy woman who is always on the go, decided to move forward with the operation.

"The idea of sitting home with my cat and the TV and bonbons does not occur to me easily, so all I wanted to do was fight back," she said.

Griffin and a colleague performed the 6-8-hour surgery. Traditionally a large open operation, the surgeons used a far less invasive, cutting-edge method involving an endovascular thoracic endograft, which Griffin described as "a big sleeve-like structure that goes inside the aorta to block the blood from flowing out." The surgeons rerouted her blood flow, which included two bypasses that allowed blood to flow undisturbed from her heart to her arms.

"Dr. Griffin told me 'You can call me your interior designer,'" Waggoner said with a big smile.

Today, Waggoner is doing great, and her prognosis is a good as if she'd never had the problem at all. Following the surgery, she received physical therapy, occupational therapy and nursing care in the comfort of her daughter's home. Within three weeks, she was back in her own home, feeling well enough to work at her daughter's restaurant in St. George.

Don't even try to suggest that the few months of confusion and then surgery slowed her down.

"I could start my car right now and drive to New York," Waggoner said, emphatically. "I have no fear of anything like that."

For Griffin, it's been a satisfying journey.

"When she came to see me, it felt to her like [her ARSA and aneurysm] was this huge deal that no one was going to be able to treat, and it was overwhelming," Griffin said. "It was really nice to be able to talk to her and say, 'Yes, this is something that is rare, but we, as vascular surgeons, handle this kind of thing all the time, so we're going to take good care of you."