Juan Acevedo was born with situs inversus, a condition in which his internal organs are located on the opposite side of their usual position. He also had a hole in his heart, known as a ventricular septal defect (VSD). Two successful childhood surgeries at the University of Minnesota—one at birth, another at 11 months—gave him a normal, healthy childhood.

But as usually happens with people who have congenital heart disease, complications from those early surgeries eventually emerged. In late adolescence, Acevedo started experiencing weakness, fatigue, and shortness of breath as scar tissue began interfering with his heart function. Another surgery—in Texas, where Acevedo’s family lived at the time—fixed the problem for a few years.

Acevedo moved back to Minnesota in his early 20s. After a couple of years, the fatigue and shortness of breath returned. Unfortunately, the doctors in Texas had been unable to remove all of the scar tissue due to Acevedo’s unique heart anatomy.

His physician sent him to the University of Minnesota Health Adult Congenital and Cardiovascular Genetics Center, where he met electrophysiologist Henri Roukoz, M.D., and cardiologist Cindy Martin, M.D.

“They realized my heart function was really weak,” Acevedo says. “They started me on meds and tried to figure out how to put off a transplant.”

Roukoz and Martin wanted to get at the remaining scar tissue to fix his heart rhythm problem and install a special pacemaker to improve its function, but his anatomy complicated the plan.

Ingenuity, uncommon expertise, and leading-edge technology enabled them to pull it off: with a robot helper, they took an unconventional route to Acevedo’s heart. They used ablation, the application of radiofrequency energy, to destroy scar tissue in his heart and correct the rhythm problem.

“Since then, I’ve felt perfectly normal,” he says. “I’ve gone to concerts. I’ve played basketball with my friends. Dr. Roukoz says there’s no reason to look at a transplant anytime in the next few years.”

A growing need

The number of children born with heart defects who live to adulthood is increasing all the time—at the rate of 5 percent a year, according to the National Institutes of Health. In 2005, for the first time, adults with congenital heart disease (CHD) outnumbered children with the disorder; today, about 85 percent of infants with CHD are expected to reach adulthood.

Roukoz, Martin, and their University of Minnesota colleagues are committed to ensuring that more of them live longer, avoid the need for a transplant, and enjoy a better quality of life than ever before.

That’s owing to a host of factors, including improved fetal diagnoses and better neonatal intensive care. It’s also due to the skill and creativity of physicians like Roukoz, who have made the M Health Adult Congenital and Cardiovascular Genetics Center a national leader in caring for adults with CHD.

The center treats people who have many types of congenital conditions, including d-transposition, or transposition of the great arteries, in which the two arteries carrying blood away from the heart are reversed; single-ventricle heart defects, in which the heart has only one ventricle that’s able to pump effectively; and atrial septal defect, a hole in the wall that separates the heart’s two chambers. Such conditions can be corrected surgically in childhood, but the patients need lifelong monitoring—and they often experience secondary complications, like arrhythmias, later in life.

Set up for success

That’s where Roukoz and his colleagues come in. “A valve that was repaired when the patient was a kid—it will maybe last until their 30s, and then it needs to be replaced again,” says Roukoz, an assistant professor of cardiology in the Medical School. “Or when you do surgery on a pediatric patient, sometimes the scar tissue that builds up can cause problems 10, 20 years later—problems like ‘short circuits’ in the heart, the most common pathology we treat in my subspecialty.”

About half the adult CHD patients in the M Health clinic have been followed by University doctors their whole lives, Roukoz says, starting in infancy with the pediatric heart team. Others are referred later in life; a few are diagnosed unexpectedly during unrelated procedures.

“We’ve had people who simply were not diagnosed as kids, and we discovered them decades later,” he says. “Those are exceptions—they simply weren’t supposed to live that long.”

The patients come from across Minnesota, around the Midwest, and beyond. 

Imagination, an exquisitely steady hand, and an unparalleled knowledge of heart anatomy in adult CHD patients are Roukoz’s best tools, says Martin, an associate professor of cardiology who codirects the Adult Congenital and Cardiovascular Genetics Center. The combination prompts Martin to refer to him as the LeBron James of his field.

“You have to have an extremely strong understanding of the congenital anatomy to be able to interpret some of these electrical conditions. And he’s probably one of the most gifted procedurists anywhere,” Martin says. “He’s also just incredibly thoughtful: just because he can do a procedure doesn’t mean he’s going to. He’s always asking, ‘Does it give the greatest benefit for the lowest risk?’”

The team’s success relies on the expertise of innovators in other disciplines at the U, too. Assistant professor of cardiology Chetan Shenoy, M.B.B.S., for example, is using state-of-the-art magnetic resonance imaging to help Roukoz pinpoint precisely where problems are occurring and how to get at them as safely and directly as possible.

“The imaging people give me exact anatomy details before a procedure,” Roukoz says. “And working with our interventionists, we often combine procedures so the patient doesn’t have to travel multiple times, and to [lessen] the risk of infection.”

Newfound hope

Among the pleasures of his job, Roukoz says, is letting adults with congenital heart defects know that their heart problems can be managed or even fixed completely—in some cases extending their lives into old age.

“One misconception patients have, if they haven’t had a specialized team caring for them, is that their problem isn’t fixable,” he says. “Sometimes they’ve been told, ‘Nobody should touch you. Nobody can fix this.’ I’ve told many patients, ‘No, we can actually fix this.’ And we did.”

Acevedo is one of those patients, and he’s grateful that he found the right team to keep him healthy.

“I’m going to start working again soon,” Acevedo says. “Dr. Roukoz and Dr. Martin are amazed at how well I’m progressing. I’m just so thankful to have them as my doctors.”