Chest Pain Leads to a Diagnosis

Two years ago, chest pain sent Mrs. Frank to an Ocean County cardiologist, who diagnosed the 4.8 centimeter aneurysm ballooning in a section of her ascending aorta. This is the major blood vessel through which blood leaves the heart and then travels to the rest of the body. 

Even though Mrs. Frank was told her aneurysm should be evaluated every year and did not require immediate treatment based on its size, she decided a second opinion was in order. At the recommendation of a Bergen County family friend and Valley Hospital cardiac rehabilitation nurse, Mrs. Frank met with Valley Heart and Vascular Institute cardiac surgeon Jason Sperling, M.D.

“I am married and have a 13-year-old son, who is everything to me,” says Mrs. Frank. “I did not like the idea of having this aneurysm checked every year to see if it grew. I felt as if I were a ticking time bomb.”

Expertise and Technology Define the Aneurysm

At The Valley Heart and Vascular Institute, Mrs. Frank underwent a series of diagnostic tests through the Thoracic Aneurysm and Bicuspid Aortic Valve (TABAV) program, created and directed by Dr. Sperling. This comprehensive program combines sophisticated diagnostic evaluation, surveillance, and both surgical and non-surgical management of thoracic aneurysms. The ultimate goal: to prevent deadly aortic dissections (tearing) and aortic ruptures. 

“With Mrs. Frank, imaging tests that are unique to our program in this region revealed a BAV, which may never have given her trouble, but when combined with a thoracic aortic aneurysm put her at higher risk than usual for a serious aneurysm-related event,” says Dr. Sperling.

Two Leaflets Instead of Three

A normal aortic valve is tricuspid; it contains three thin leaflets that open to allow blood to flow from the heart into the aorta and then close to prevent the backflow of blood. While most doctors think about BAV having an ‘obvious’ appearance with two fully formed leaflets, this represents the minority of BAVs. About 90 percent have only limited fusion of two of the three leaflets, and they can deceptively look a lot like ‘normal’ valves. According to Dr. Sperling, “it’s not ‘how’ bicuspid the valve looks- they all still carry the genes that portend a higher risk aneurysm when present”.  It is estimated that about 1 in 50 people have a congenital BAV, with at least one in three of these patients requiring surgery in their lifetime related to the valve itself or aneurysm.

“It would be easy for any physician to miss Mrs. Frank’s BAV because they can be tricky to diagnose on an echocardiogram,” explains Dr. Sperling. “But with our unique imaging package, developed with Dr. Edward Lubat and his partners in Valley’s Department of Diagnostic Imaging, we were able to diagnose this ‘occult’ BAV, which upgraded her risk. It is my impression that BAV is present in the setting of aneurysm more commonly than we think it is.”

According to the 2010 AHA/ACC Guidelines for Thoracic Aortic Diseases, when a patient is identified as having both BAV and aneurysm, it is a Class 2a indication to compare the aneurysm size with the patient’s height, and a ratio greater than 10 (like Mrs. Frank’s) indicates that elective surgery could be of benefit.  Though experts argue this point (and use the Class 1 indication of 5 cm in the guidelines for BAV-associated aneurysm), studies have clearly shown that certain aneurysms smaller than 5 cm can dissect or rupture. After discussing her options with Dr. Sperling at length, Mrs. Frank chose to have elective surgery to remove the aneurysm.

Surgery Goes Well

On February 1, 2011, Dr. Sperling removed “zones 2 and 3” (just above the aortic valve to roughly the mid-point of the aortic arch) of Mrs. Frank’s ascending aorta containing the aneurysm and replaced the missing tissue with a tube of Dacron (polyester). Her aortic valve was confirmed to be bicuspid, and it was healthy and functioning well and therefore did not require repair or replacement. Citing data from the Mayo Clinic, Dr. Sperling points out that 60 to 70 percent of well-functioning BAVs continue to perform well throughout patients’ lifetimes. .

To protect Mrs. Frank’s brain during this surgery, which involved her aortic arch, Dr. Sperling used a technique that has been shown to be associated with the best neurologic outcomes. “Using this neuro-protective protocol when indicated, we have completed approximately 200 consecutive aortic surgeries with no strokes, and in addition achieved a 99 percent survival rate for elective aortic surgery over the past five years.”

The Valley Heart and Vascular Institute’s cardiac surgery team perform a higher percentage of valvular surgery and other complex procedures than the national average of the Society of Thoracic Surgeons, which has recognized the Institute six consecutive years and ranks Valley’s cardiac surgery program in the top 12 percent of programs in the United States.

Life Moves Forward

Mrs. Frank spent just five days in The Valley Hospital and was able to return on March 28 to her position at Lanoka Harbor Elementary School as a paraprofessional teacher for third and fourth graders with special needs.

“Traveling to Valley was absolutely the right choice for me,” she says. “The nurses were exceptional, and I can’t say enough about Dr. Sperling’s surgical skills or his attention to his patients.”

Enrolled in the TABAV program’s surveillance registry, Mrs. Frank will continue to be monitored with regular phone calls and echocardiograms, MRIs, and specialty CT scans for the rest of her life. Because aortic aneurysms and BAVs have a familial risk, her son, Will, must also be monitored periodically by a pediatric cardiologist.

She is looking forward to boating on Barnegat Bay with Will and her husband, Bill, and to a yearly family trip to Aruba in November.

“Last year, I was very nervous during the flight, but I won’t be this year,” concludes Mrs. Frank. “I no longer have day-to-day worries about my heart. The TABAV program at The Valley Hospital gave me the freedom to enjoy the rest of my life.”