Intermountain Medical Center Team Performs Pioneering Operations to Save the Life of Utah Woman With Rare Genetic Disorder

Jess Gomez

 Jess.Gomez@imail.org

 5/19/2011

MURRAY, UT – A 28-year-old Kanab, Utah, woman is alive today after a team of doctors from Intermountain Medical Center performed a series of complex surgeries they believe have never been done before in Utah. In fact, similar operations are incredibly rare worldwide.

The surgeries were done three weeks ago to treat an aortic aneurysm — a balloon-like bulge in the aorta that can burst, causing serious bleeding. Aortic aneurysms are not uncommon, but this case was unlike any the doctors had seen before.

Their patient, Vera Davis, was born with Turner syndrome, a chromosomal disorder that affects only about 1 in 2,000 newborn girls. The condition can cause cardiovascular malformations, including aortic aneurysms and valve problems. In Vera’s case, she also had unusual branching of the great vessels of the aorta. The aneurysm had developed a tear and was in danger of rupturing.

“Vera had a very serious aneurysm that was located right next to the vessels supplying blood flow to her brain,” said John Doty, MD, a cardiovascular thoracic surgeon at Intermountain Medical Center. “There was no question we had to fix it or eventually it would have ruptured and she would have died.”

But because of her unusual anatomy and the location of the aneurysm, a traditional approach would have cut off blood flow to her brain or arms.

So three physicians at Intermountain Medical Center — each an expert in a different area of medicine — worked together to develop a plan to treat Vera: Duane Blatter, MD, an interventional radiologist; the cardiac surgeon Dr. Doty; and Douglas Wirthlin, MD, a vascular surgeon.

The doctors consulted with colleagues around the country, who offered differing opinions on the best solutions. In the end, they settled on a plan that included three complicated — and risky — operations.

“Vera had five major interventions and every single one carried a risk,” said Dr. Wirthlin. “But doing nothing was an even bigger risk.”

The treatment plan went like this:

Surgery one, April 25: Two teams of surgeons led by Dr. Doty and Dr. Wirthlin worked simultaneously on each side of Vera’s body to perform two bypasses from the arteries in her neck to the arteries in her arms. The bypasses created a new path for the blood to flow after closing the aneurysm.

Surgery two, April 27: Dr. Blatter, Dr. Doty, and Dr. Wirthlin worked together to place an endovascular stent-graft inside the aneurysm, which sealed it off. The stent-graft had to be placed absolutely perfectly, or it would have cut off blood flow to Vera's brain. Dr. Blatter then used a catheter to deploy specialized devices, including more than 50 tiny coils, into the abnormal artery to her right arm. These devices would cause Vera’s blood to clot and prevent blood from flowing back into the aneurysm.

Surgery three, May 2: Dr. Blatter placed more coils to prevent blood backflow into the artery to her left arm and to further seal the aneurysm. "It looked really good after the last surgery, and we knew we had completely sealed the aneurysm. We knew it was going to work," said Dr. Blatter.

It was a tremendous relief to the whole team, who said the outcome was never certain. “We were working under the possibility that Vera could have had a catastrophic problem with each step,” said Dr. Wirthlin. “Fortunately, every step went well and her body responded perfectly.”

Vera says she expects to have a long, healthy life now, and she gives the credit to three physicians at Intermountain Medical Center, who all donated their time for this rare case.

“I was scared I might not come out of the surgery,” she said. “But I thought, these doctors know what they’re doing, and they’re going to do best they can. It’s hard to grasp how lucky I am to be alive today.”

Dr. Blatter said that the surgeries would not have been possible without the expertise of the entire group of physicians.

“This was a challenging case — a very, very rare case. We could not have done this except as a team and no where else other than Intermountain Medical Center, which has the unique depth and breath of resources that we needed to successfully perform this procedure,” he said.

Added Dr. Doty: “This is not about two or three doctors coming together, but entire teams from different special specialties coming together to do what was best for the patient. This includes the team from Dixie Regional Medical Center who diagnosed Vera and sent her to us. This is a great success for her and her family.”

Intermountain Medical Center is one of the premier hospitals in the country, and serves as the flagship medical facility for the Intermountain Healthcare system. 

Intermountain Medical Center Team Performs Pioneering Operations to Save the Life of Utah Woman With Rare Genetic Disorder

 

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