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Bicuspid Aortic Valve

patient story jerry

Jerry Tarrant has a hard time sitting still.

For the better part of 40 years, he’s worked in the information technologies and data collection industry, the last 15 of them as the head of North American health care sales for one of the world’s largest hardware manufacturers. In a typical year, he travels 100,000 miles for his work.

Jerry still manages to maintain an ambitious exercise regimen. A veteran of many marathons and triathlons, he also lifts weights and plays golf every chance he gets. In 2006, a close friend, who also handles his life insurance, dangled a free game of golf at his club to get Jerry to finally agree to an EKG, a prerequisite to extend his term policy. He even arranged to have the nurse come to his house beforehand.

The test done, Jerry was taking some practice swings at the first tee when his friend nonchalantly dropped a bombshell: “By the way,” he said, “don’t worry about extending your life insurance. You’re uninsurable. You have a heart problem.”

Jerry was shocked. As soon as he returned home, he scheduled an appointment with his family doctor, who then referred him to a cardiologist. The eventual diagnosis: bicuspid aortic valve. Bicuspid aortic valve is a birth defect in which the aortic valve only has two small parts, called leaflets, instead of three. Those leaflets may be thicker and stiffer than normal, which can lead to aortic stenosis, where the valve doesn’t open as easily as it should when the heart contracts. A bicuspid aortic valve can also allow blood to flow backwards into the heart when the heart relaxes, a process known as aortic regurgitation. Both conditions can force the heart to work harder than it should. If a dysfunctional bicuspid aortic valve is left untreated, it can cause severe damage to the heart.

Tempting the inevitable

In 2007, soon after his diagnosis, Jerry began seeing Joseph Bavaria, MD, Co-Director of Penn’s Center for Bicuspid Aortic Valve Diseases (CBAVD), which opened last winter.

“Jerry actually had what we refer to as bicuspid aortic valve syndrome,” says Melanie Freas, DNP, CRNP, Clinical Program Manager of the CBAVD. “Patients with that diagnosis generally fall into one of three categories: They either have a bicuspid aortic valve, an aortic aneurysm, or a combination of both. Jerry had a combination of both.”

Up to 50 percent of patients with a bicuspid aortic valve may also have ascending aortic aneurysm disease, where an abnormal enlargement of a blood vessel puts the patient at risk for severe complications, like an aortic dissection or rupture.

Just before Jerry arrived at Dr. Bavaria’s office, a CT scan revealed his ascending aorta measured 5.1cm. Normal is around 3cm. Surgery is necessary at 5.5cm, though newer guidelines now recommend even earlier intervention, at 5cm.

“My youngest son played Division III football, and I tried to stay within 100 pounds of him on the bench press,” Jerry says. “When my cardiologist noticed my hands were callused, I told him that and he said, ‘If you keep lifting like that, I won’t see you in six months.’ ”

Dr. Bavaria brought Jerry in every six months for a CT scan and an echocardiogram to monitor his bicuspid valve and ascending aorta. 

“He became like my family doctor, asking lots of questions, looking for very subtle signs,” Jerry says. “With all the flying I was doing, he felt we were pushing it. He told me, ‘If it goes, you won’t see the ground. It’s not a heart attack. It’s a blowout.’

“I don’t know why, but we got into a stare-down,” Jerry says. “I never had any symptoms. And I didn’t want the surgery.”

The total lack of symptoms for someone living with a bicuspid aortic valve is common, Freas says. Relatively little is known about bicuspid aortic valve syndrome. The cause is unclear, and most people don’t learn that they have it until their family doctor detects a murmur during a routine physical and subsequent testing. 

Another potential indicator: First-degree relatives (siblings and children) of someone with bicuspid aortic valve syndrome have a 20 percent higher risk of having it themselves. The CBAVD began offering screenings for first-degree relatives earlier this year.

Jerry says he had his two sons screened immediately after he was diagnosed. They don’t have it, nor do his siblings.

Coming out on the other side

Jerry finally underwent surgery to replace his bicuspid valve on March 18, 2013, six years after he started seeing Dr. Bavaria. His ascending aorta measured 5.4cm at that point. He was in and out of the hospital in three days. 

In hindsight, Jerry has a hard time understanding why he held off as long as he did. He had his rotator cuff and labrum surgically repaired last year—“My shoulder was beat up from doing healthy stuff”—and he says that was a more trying experience than having his valve replaced.

Jerry retired in April, though he still has a hard time sitting still. He rattles off a list of charitable causes he’s supporting in Cape May, where he and his wife now reside. He’s also become an advocate for early screening.

“This is so preventable. All it takes is an ultrasound. It’s inexpensive and fast,” he says. “I could have died twice. The first time because I didn’t know I had it and it could have blown. The second time because I wanted to wait to have the surgery. I was living on borrowed time.”

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