Late on a Friday afternoon, Robert Palmer sat in the cardiac surgeon’s waiting room, looking at a map on the wall dotted with lights. A race car driver and a senior master technician for Ford, he never expected to find himself here, wondering if he would be able to continue living his life the same way. To him, racing cars is “better than any amusement ride—zipping around the corners so fast that you can’t hold yourself up. The noise, the smell of the tires, the competition.”
Then, the surgeon walked in and explained what he was concerned about. He used an analogy they both understood. “I’m a car guy. You’re a car guy. You have a radiator hose that’s about to blow. We have to get in there and fix it,” the surgeon said.
John Elefteriades, MD, is a Yale Medicine heart surgeon and director of the Yale Center for Advanced Thoracic Aortic Disease at Yale New Haven Hospital’s Aortic Institute. He is a world expert on aortic aneurysm—a thinning of the aorta’s walls that can cause it to bulge—who has spent his career improving care for the body’s main artery. (The map on the wall was one of the world with 32 lights depicting the countries where he has lectured or performed aortic aneurysm surgery.)
But for Palmer (and that helpful radiator hose analogy), Dr. Elefteriades reached back to an earlier chapter in his life. His father had been a mechanic for a Chevrolet dealership in Philadelphia—in fact, the first one owned by Roger Penske, the famous auto racer.
An unsettling discovery
A year earlier, Palmer had visited Michael Fucci, DO, a Yale Medicine cardiologist, to check out episodes of rapid heartbeats. An echocardiogram picked up a surprise: a thoracic aortic aneurysm—a problem Dr. Fucci has seen before when checking patients for another problem. Recently, a second echocardiogram had shown that Palmer’s aneurysm had grown. “I’m going to have to ask you not to race. You’re going to need open heart surgery,” Dr. Fucci told him, and referred him to Dr. Elefteriades.
Palmer was 49 years old and otherwise healthy. He never dreamed he could potentially be one of the 10,000 people who die each year due to aortic aneurysms. Many people never know they have an aneurysm until they experience the sudden, sharp tearing pain that means it is dissecting (splitting into layers) or rupturing—and also that it’s probably too far along to fix. Palmer’s aneurysm was now almost five centimeters—the diameter of a soda can. Palmer was exiting what Dr. Elefteriades called the “green zone of safety” and entering a “yellow danger zone,” similar to the tachometer of a car.
Dr. Elefteriades’ own research has shown that an enlarged aneurysm is at high risk for rupture after physical exertion that is extreme enough to cause a blood pressure spike, such as lifting heavy weights or moving heavy furniture, and as the result of extreme emotional stress. “What could be more anxiety provoking than driving a race car?” Dr. Elefteriades asks. “At any moment you don't know whether that car is going to flip around or catch on fire.”
What, exactly, is an aneurysm?
The aorta has a key function: It carries blood from the heart and distributes it throughout the body. Shaped like a candy cane, it has an ascending portion that rises into an arch at the top of the chest and then descends down to the belly.
Thoracic aneurysms (in the ascending aorta) can grow for years, sometimes becoming large enough to stretch the aortic wall and suddenly dissect or rupture. “Like a heater hose, the aorta is made of layers,” Dr. Elefteriades says. “The layers can come apart. This allows the blood to get between the layers. The thin outer layer is just not strong enough to constrain the blood stream and tolerate the blood pressure. The inner layer tears, and that's when the patient dies.”
Palmer’s aneurysm was primarily in the ascending aorta, stretching down into the root portion, just above the heart muscle. Though Dr. Elefteriades acknowledges that driving race cars is a high-stress pastime, it didn’t cause the problem. Aneurysms in the descending aorta tend to develop from high cholesterol, high blood pressure and smoking. Ascending aneurysms (near the heart) are different. “They are caused by a genetic abnormality that is there from the time the fetus is formed in the womb,” Dr. Elefteriades explains. “That abnormality is usually one irregular letter among the 2.2 billion letters that constitute the genome. It seems like one letter wouldn’t be important, but it is.” So far, medical scientists have found 31 letter changes that can cause a thoracic aneurysm. Dr. Elefteriades believes it’s only a matter of time before all ascending thoracic aneurysms are linked to specific genetic mutations.
A long operation
The day Palmer learned that he needed open heart surgery was an emotional one. His brother Dave, who spends long hours working with Palmer on race cars, told him, “You come first, racing comes second.” So, Palmer set a surgery date for July 19—which happens to be the number on his race cars.
Joelle Buntin, a surgical nurse coordinator, navigated Palmer through the process, providing as much information as possible to support him and alleviate his fears. “I get to know the patients and families personally and try to make them feel more comfortable,” she says. “Even though we operate on patients daily, surgery is usually the biggest experience in their lives and we don’t discount or forget that.”
Minimally invasive approaches are changing surgery almost across the board, but an ascending thoracic aneurysm is a traditional (and difficult) open surgery that can take several hours. It starts with cutting a footlong incision through the breastbone and opening the ribcage to reach the heart. The heart is then temporarily stopped so that the surgeon can delicately mobilize the arteries, go in and cut out the damaged section of aorta and sew a new artificial tube (called a graft) into place.
Palmer’s surgery also involved replacing his aortic valve, which allows blood to flow from the heart to the aorta. Here Palmer had a choice—he could use the “gold standard” Dacron valve, which is used for most patients, and would last a lifetime but require him to take a mild blood thinner every day; or, he could use an animal valve, which wouldn’t require the blood thinners. He opted for the animal valve. “I didn’t want to be on any blood thinners, especially driving a race car,” he says.
As a man accustomed to being in the driver’s seat, Palmer felt uneasy about having to undergo major surgery. Racing is an experience you control by honing driving skills and fine-tuning the vehicles. “For the operation, I literally had no control,” Palmer says. But Dr. Elefteriades told Palmer he has done thousands of these operations and had a 98 percent success rate for elective (planned, non-emergency) surgeries. “You’re going to be fine,” he told his patient.
When he went under anesthesia around 7 a.m., Palmer says, “I was dead set that I was going to wake up in a couple of hours. But when I woke up, my last memory of the day was the loudspeaker in intensive care saying visiting hours were over. So, I knew the entire day had gone by.” The surgery was a success.
Later, Dr. Elefteriades told Palmer, “We’ve been looking at your aorta under the microscope. It was extremely inflamed. And the layers were starting to come apart. I think we got this just in time.”
Searching for answers
As a Yale Medicine aneurysm patient, Palmer’s name is now included in a database of more than 5,000 patients and 15,000 imaging studies. The best-known database of its kind, it has become a rich source of information about such issues as how fast the aorta grows, at what size it ruptures and the risk factors for rupture. For example, in addition to physical exertion, doctors now know that dramatic life events, like the death of a loved one, can a precede a rupture. (The accompanying tissue bank of excised aneurysms supplies materials for research to laboratories around the world.)
This information will be critical as doctors consider the possibility that aortic aneurysms may be on the rise—some studies have shown them doubling in a 10-year period. In Japan, hospitals started tracking autopsies with total body CT scans to determine the precise cause of death. “I would have thought the number of patients dying from aneurysms would be maybe 1/100th of 1 percent,” Dr. Elefteriades says. “But the Japanese studies came out with a staggering finding: The deaths of 8.5 percent of all people coming into an emergency department in cardiac arrest were caused by a splitting apart of the ascending aorta. So, this is much more common than we ever thought. When it's in one family member, it will be in many other family members.”
What you should know about aneurysms
Routine screening can help save lives. Dr. Elefteriades urges everyone with a family history of aneurysm disease anywhere in the body—or a family member who died suddenly at a young age, without prior history of heart disease—to consider being checked with an echocardiogram. People with inherited connective tissue disorders, such as Marfan and Ehlers-Danlos syndromes, are also at higher risk for thoracic aneurysms.
For people with a family history of aneurysm, the Yale Center for Advanced Thoracic Aortic Disease at Yale New Haven Hospital’s Aortic Institute has one of the most sophisticated genetic testing programs for thoracic aortic aneurysm in the world, helping hundreds of parents, siblings and children of people who have had aneurysms understand their genetic risk. “We look for that abnormal letter. And we find it in about one out of three patients. If family members have the same abnormal letter as their loved one, then we know they have to be watched very closely,” Dr. Elefteriades says.
When Palmer was a teenager, his 30-year-old brother died suddenly, supposedly of a heart attack. Now Dr. Elefteriades says there is a good chance that his brother died from a ruptured aneurysm. So, Palmer has now convinced his two adult sons, Adam and Ryan, to undergo genetic testing at Yale. “Both are physical fitness trainers, so they are lifting weights and exerting themselves. If they have an aneurysm they don’t know about, that might put them at high risk,” he says.
Promising new developments are taking shape. Dr. Elefteriades is working with a gene company on what he is calling an RNA Signature Test for thoracic aortic aneurysm—a possible step toward developing a general screening technique that is affordable and accurate enough for widespread use. (Final validation studies are in progress.)
In the meantime, as in Palmer’s case, most aortic aneurysms are picked up on a chest X-ray or ultrasound ordered for a different reason. For him that worked out. One year after his operation, Palmer says he is 100 percent recovered. He is grateful for the support of his wife, Kate, and his sons Adam and Ryan, and from family and friends.
This spring he raced at several tracks in Connecticut, including Thompson Speedway and Stafford Motor Speedway. He expects to live a long life—after all, his father lived to 98. “This is not the end of the road,” Palmer says. “But it makes you realize how little time you have here. So, I do what I love and keep racing cars. If I’m not racing or working on a car, I’ll watch it on TV, or go see races in New Jersey and Daytona. You can’t replace the thrill. It’s what I do.”