Heather Hinton

A happy ending for patient
with a rare disorder

Heather Hinton stands more than six feet tall. She has long fingers and toes and a slender face. These features may not seem extraordinary, except in Heather's case, they're caused by a rare genetic disorder — Marfan syndrome.

One night in late January, after an ordinary day, Hinton and her husband, Jon, sat down at the table for their evening meal. But Hinton never got to eat. It wasn't that she didn't like Jon's cooking. Rather, Hinton immediately experienced excruciating pain down her back. Instead of enjoying a candlelight dinner, she took an ambulance ride to the nearest emergency room.

Hinton took her sudden bout of pain seriously, knowing that aortic dissection — which can cause severe back pain — is a complication often associated with Marfan syndrome, an inherited disorder of the connective tissue in the body. Born with Marfan syndrome, she became well-educated in living with the disorder and recognizing the signals of serious complications. She is one of an estimated 1 in 15,000 people in the United States with Marfans.

"My mother was diagnosed with Marfans during her first pregnancy," says Hinton, "and I was born with a prolapsed mitral heart valve." Her only sibling, an older brother, does not have Marfan syndrome. But doctors detected her valve abnormality (leakage) at birth. A diagnosis of Marfans followed shortly thereafter.

"As a child, I had restrictions and limitations, but didn't really have any serious problems associated with the disorder," recalls Hinton. "Strenuous activity and contact sports were strictly prohibited, but I could swim. I would have been a great basketball player, being 6'3" tall by age 14. But basketball was too dangerous for me, as the pressure exerted and potentially rough contact could cause my aorta to rupture."

A standard regimen for someone with Marfans is an annual echocardiogram. Hinton had her yearly echo just a month before her episode and everything appeared to be normal. "However, I was more tired than usual for a whole year prior to my dissection, but couldn't determine why," she recalls. "I just lacked a feeling of well-being and got tired easily."

From 'back of the mind,' to top priority

When Hinton hiked or swam, her limitations and risks always lurked in the back of her mind. "But I didn't want to be so cautious and safe that I couldn't enjoy life either ... so I hiked and swam and walked a lot." She likens this activity to trying to plan a trip with a continual weather advisory. "You never know when the storm is going to descend," she says.

Upon arrival at a Minneapolis hospital, Hinton learned that her entire descending aorta — the part running from the heart to the neck and down back to the abdomen — had dissected. Dissection occurs when the layers making up the aorta separate or tear, weakening its structure.

She remained in intensive care for one week, on blood pressure medication to reduce the chances of an aortic rupture. "When I was released from the hospital, things were not stabilizing the way doctors had hoped and I was at a very high risk for an aortic rupture," she recalls. "This is why John Ritter collapsed so suddenly," she explains. "It happens quickly and many people die before they can get help."

So, what was once just a consideration in the back of her mind became a constant source of anxiety and worry.

She and Jon believed they would need to travel from Minneapolis to somewhere on the East Coast or farther to have surgery to repair the aorta. Then, they learned about the cardiovascular surgery expertise and Marfan Clinic at Mayo Clinic in Rochester. "I would have gone anywhere in the world to address my situation," she says, "but I was thrilled that I might get help so close to home."

Repairing the damage

Hinton was initially seen by Thoralf Sundt III, M.D., one of the team of cardiovascular surgeons at Mayo Clinic in Rochester. Dr. Sundt's interest and expertise lies in aortic surgery.

"We made an appointment for Heather to be seen in the special Marfan Clinic at Mayo Clinic in Rochester," recalls Dr. Sundt. "Since Marfan syndrome can affect multiple organ systems, a multidisciplinary care approach is important so specialists can address the medical nuances of the disorder."

"As part of Heather's treatment in our Marfan Clinic, she received education and preparation for her surgery," says Naser Ammash, M.D., a specialist in cardiovascular diseases in the Marfan Clinic. "Our Marfan Clinic educates patients on what to expect with their disorder and treatment here, but it was obvious that Heather had already done her homework." To the contrary, Hinton says she's never learned so much about Marfan syndrome as she did during her education sessions at Mayo.

During her first procedure, the descending thoracic and abdominal portions of the aorta were repaired with a synthetic polyester fiber (Dacron). "In a subsequent operation, we went back and grafted the ascending and arch portions of the aorta and replaced Heather's aortic and mitral valves," explains Dr. Sundt. The latter surgery was an "elective" part of her treatment — a proactive measure to prevent further dissection in the future.

"Heather's body was giving us signals that she was at risk for rupture, so we replaced the entire aorta with the Dacron graft," explains Dr. Sundt, who performed both of her operations. "And yes, we're talking about the same Dacron material that's used in textiles."

Good to be back

Both procedures were successful. Hinton will remain on Coumadin — an anticoagulant, in addition to medication that controls her blood pressure, and she will need ongoing follow-up care in Mayo's Marfan Clinic. "I can't lift weights or play contact sports, but I never did anyway," she says. And, the black cloud that once cast a shadow on all her activities has lifted. "I no longer worry about my aorta dissecting," she says. "It's happened, I survived and it's been repaired."

Hinton returned to her position in relationship management for corporate banking with Wells Fargo in Minneapolis. After a six-month leave, she's happy to be back on the job.

"My treatment at Mayo Clinic has allowed me to return to my husband, my career and a fairly normal life," she says. "I have more wind and energy and far less fear."

"I'm thankful every night that I'm here to sit down for dinner."