Modern medicine sometimes can provide the comfort of clear-cut reasons for our illnesses and disease. Your genetics might explain your cancer. Your insistence on eating fatty, calorie-ridden foods and leading a sedentary lifestyle could tell the tale of clogged arteries that caused your heart attack. A stray particle from a blood clot might be the culprit that plugged a blood vessel, leading to your stroke.
But the clues to puzzling medical problems can be far more elusive, even for skilled and experienced providers. Plain luck, both good and bad, that unexpectedly changes lives is hard to account for.
Dr. Christopher Lieu can speak to that. Lieu, an associate professor of Medicine-Medical Oncology with the University of Colorado School of Medicine, was hardly a candidate for a serious heart problem back in 2014. He was just 36, in good health, and had a busy practice seeing patients at the UCHealth University of Colorado Cancer Center on the Anschutz Medical Campus. He stayed active on the basketball court and running trails and at home, where he and his wife cared for two kids, then ages 1 and 3.
“I’d gone through life pushing myself in every way possible,” Lieu said. Then some family news put a damper on that.
Family heart history, medical mystery
An uncle of Lieu’s had died in Korea, seemingly of heart problems. At that time, the precise cause wasn’t known, but Lieu’s mother had survived an aortic dissection: a tear in the inner wall of the aorta, the artery responsible for transporting blood pumped from the heart to the rest of the body. His mother survived, but the death of her brother prodded Lieu to question whether family history made him vulnerable to aortic problems.
Lieu posed the question to his primary care physician. The potential genetic link led to approval for an echocardiogram to obtain pictures of Lieu’s heart. The resulting images revealed a bulge, or aneurysm, in the aortic root, the top portion of the artery, which is attached to the heart. The root includes the aortic valve and openings to the coronary arteries, which feed the heart blood. If an aneurysm ruptures in that area, the internal bleeding that results can be fatal.
The news was an unpleasant surprise, as Lieu felt no symptoms. That’s not unusual, said his cardiologist, Dr. Amber Khanna, an associate professor of Medicine-Cardiology with the CU School of Medicine and a specialist in adult congenital heart disease at UCHealth University of Colorado Hospital and the UCHealth Heart and Vascular Center.
“Some people may have chest pain, but an aortic aneurysm is usually a silent killer,” Khanna said. Lieu’s aneurysm also remains a bit of a medical mystery and a testament to the role that chance can play in the course of a person’s life.
Khanna said that an aortic aneurysm at Lieu’s age “is almost always genetic, especially with his family history.” Tests showed he had a pair of gene mutations not normally seen in otherwise healthy people, she said, but she and her colleagues aren’t convinced the changes caused Lieu’s aortic problem.
“His genetic testing is indeterminate,” Khanna said.
There was a further twist. The cause of death for Lieu’s uncle – a heavy smoker with emphysema – was ultimately diagnosed as pneumonia. Lieu said that had he known there was no aortic connection to his uncle’s death, he would never have mentioned it to his PCP and probably would never have gotten the echocardiogram that revealed the aneurysm.
Caught in the middle
The echocardiogram painted a clear picture of the problem in Lieu’s aorta, but the questions it raised were murky. For one thing, the aneurysm at that point, 4.6 centimeters, wasn’t big enough to demand surgery to repair it, Khanna said. It was on the borderline for surgery for someone with a confirmed genetic risk, which was uncertain in Lieu’s case.
In addition, unlike a person suffering typical signs of heart problems, like shortness of breath, chest tightness or jaw pain, Lieu wouldn’t feel tangible benefits from a surgical repair, Khanna said.
“It’s fixing one of the pipes, but the pipe isn’t causing the person any problems,” she explained. In removing the aneurysm, “we don’t make the heart stronger, we don’t make the valves work better, we don’t fix the electrical system. It’s just preventing future problems.”
Khanna said, however, that Lieu’s relative youth and otherwise good health pointed in favor of surgery.
“Younger patients have their whole life in front of them, and they are more likely to do well with surgery,” she said. “I was confident that Chris would do well with it.”
That didn’t make the decision a slam-dunk for Lieu. He was in the frustrating position of not having a definitive diagnosis – the aneurysm wasn’t small enough to be confident that he could safely watch and wait or big enough to go to surgery immediately.
“It’s one thing if you are one edge or the other, where you have to get it done because it’s life-threatening or you have some time to wait. I was right in the middle,” Lieu said.
And he felt fine, which complicated the prospect of an open-heart procedure and putting his practice on hold for at least six weeks of recovery.
After consulting with Khanna, Lieu decided to wait while he had regular echocardiograms to see if the aneurysm grew. Late in 2015, the bulge had extended to 4.9 centimeters, a worrisome sign that increased the pressure for surgery.
He and his wife, Caitlyn, spent October and November discussing the situation between themselves and with Khanna. They also met with Dr. Brett Reece, professor of Cardiac Surgery and director of the Thoracic Aorta Program at CU, who would perform the aortic repair if Lieu approved it.
The David Procedure
“We’re like primary care physicians for aortas that are larger than normal,” Reece said. He added that the Thoracic Aorta Program sees patients of all ages, with the number of those in the 20- to 50-year range at least equaling the number of older patients.
Both Khanna and Reece explained the benefits and risks of the procedure and answering as many questions as possible.
Lieu finally decided that the risk of a rupture at his age and the resulting anxiety it would wreak on Caitlyn and their kids, were too great to bear. He chose surgery.
In February 2016, Reece repaired Lieu’s aorta with a technique called the David Procedure. In simplified terms, the open-heart surgery involves cutting the upper portion of the aorta, removing the section of tissue with the aneurysm, replacing the tissue with a graft, re-implanting the patient’s own aortic valve in the graft (rather than putting in an artificial valve) and attaching the graft to the remaining aorta.
Reece said the David Procedure is an option for patients like Lieu whose aortic valves are “relatively normal” or even those that don’t close completely. It’s not used in patients whose valves have become stenotic, or stiffened. He said the Thoracic Aorta Program is now doing nearly 50 such procedures a year for patients of Lieu’s age or younger.
With his own aortic valve still in place, Lieu does not have to take blood thinners for the rest of his life, as do those who receive artificial valves.
“His valve is now perfect and I hope it serves him for the rest of his life,” Reece said.
The operation was successful. After a roughly week-long stay at UCH, Lieu went home for six weeks of recovery, during which he didn’t work or drive. He couldn’t lift anything for four weeks. He returned to work at the six-week mark and two weeks later flew to New York City to give a presentation. That went well, he said, but the physical challenges were severe enough that he labeled it “not the brightest decision I have ever made.”
Khanna was aware of the trip, stressing that she treats adults. They make their own choices about their recovery. In general, she added, she talks to all her patients about the risks of activity after surgery and ways to minimize it.
“I try to put it into a context of the degree of risk but that’s very difficult,” she said. Lieu was no exception.
“He knew he couldn’t put his own suitcase in the overhead bin,” she said of Lieu’s decision, adding she was not surprised by his struggles with the trip.
“It takes so much energy to recover, even if you are young and healthy,” she said. “I tell patients, ‘Give yourself time.’ They learn that if they push themselves too hard one day, they are going to pay for it the next couple of days.” In fact, she added, full recovery generally takes at least six months and often a year.
The experience in New York was one of several lessons Lieu said he learned from switching roles, from provider to patient. For example, many of his patients go through aggressive surgeries to treat their cancer. Many lose their desire to eat and face debilitating fatigue. He found himself encountering the same challenges after his heart surgery and gaining a new appreciation for his patients’ battles.
“Any time you put yourself in your patients’ shoes, it has a benefit because you have a better sense of what a good day and a bad day really feels like,” Lieu said.
As he approaches the three-year surgical mark, Lieu said he feels no ill effects. He gets periodic imaging tests and takes a blood pressure medication to protect the aorta. He’s returned to life as it was – with the addition of a third child – but remnants of the unexpected ordeal stay with him.
“You have the scar to help you remember,” he said. He still hits the basketball court but sometimes a nagging thought accompanies him and slows the fast break a step or two.
“There is always something in the back of my mind that something could happen in the future,” Lieu said.
Chris Lieu serves as a different kind of reminder for Brett Reece.
“I love seeing him around campus now because I know we gave him the best care possible,” Reece said.