While Stephen A. Barnes, MD, JD, was being prepped for surgery, he couldn’t resist joking with his surgeon and longtime friend Jorge D. Salazar, MD: “You’re so accustomed to repairing tiny newborn hearts that my colossal 51-year-old adult heart should be a walk in the park.” Although Salazar is a pediatric cardiothoracic surgeon, he is a leading expert in repairing the most challenging congenital heart defects in children and adults.

More than two decades ago, Barnes oversaw Salazar’s training in general surgery at Johns Hopkins Hospital. But in October 2017, he found himself on a stainless steel operating table waiting for Salazar to repair a hole in his heart that had been quietly sapping his vitality his entire life.

Approximately one percent of all babies in the United States are born with congenital heart disease, which involves a defect in the structure of the heart that can eventually lead to heart failure or other issues such as arrhythmia. These defects are usually found and treated during infancy or childhood, but some people, like Barnes, evade diagnosis until dire symptoms appear in adulthood.

Barnes, who specialized in surgical oncology before adding a law practice to his career, leads an exceptionally healthy life, avoiding hazards like smoking that would increase his risk for heart disease. That’s why the extreme fatigue he began experiencing in 2016 confounded him. “I had just turned 51, so I figured it was finally time for me to accept that I was getting older,” he says.

He ignored his fatigue for months, chalking it up to his active lifestyle and late nights working on cases. Then, in summer 2017, Barnes started experiencing severe heart palpitations where he felt his racing heart trying to escape his chest. It was time to see a specialist.

Barnes’ cardiologist ordered a battery of tests on his heart, and the results from the echocardiogram noted a possible rare heart defect that can lead to heart failure — a sinus venosus atrial septal defect and partial anomalous pulmonary venous return, a combination defect where the blood incorrectly returns from the lungs to the right atrium instead of the left. However, because the other tests indicated his heart was functioning correctly with no other symptoms, his cardiologist believed an MRI was unnecessary. 

Barnes symptoms continued over the next couple of months, and his surgical instinct took over. When he read that 90 percent of adult patients with his potential heart defect die before age 60, he scheduled his own cardiac MRI, which confirmed his echocardiogram’s warning: He had a congenital heart defect that needed immediate attention.

As fate would have it, Salazar had just joined McGovern Medical School at UTHealth as Professor and Chief of the Division of Pediatric Cardiovascular Surgery and was less than two miles from Barnes law office. When Barnes called his old friend and colleague, Salazar immediately scheduled an appointment for him with Poyee “Pansy” Tung, MD, a cardiologist at the UT Physicians Adult Congenital Heart Disease Clinic.

Tung promptly confirmed Barnes heart defect and coordinated with Salazar to determine the surgical approach to repair it. Although up to 80 percent of patients with congenital heart defects are diagnosed in childhood, she wasn’t surprised that Barnes slipped through the cracks and still led a productive, active life.

“Stephen’s heart defect forced his body to work overtime, sending two to three times the normal amount of blood to his lungs,” she explains. “His heart was sliding into failure his whole life, and it finally caught up with him.”

Three hours after Barnes joked that Salazar should have no problem repairing his adult heart, he awoke with a properly functioning heart and renewed vigor.

“I was so fortunate to have Dr. Salazar and Dr. Tung by my side, but so many other adults with congenital heart disease don’t find out they have a problem until it’s too late,” Barnes says. “My hope is that UTHealth continues raising awareness for adult congenital heart disease to advance care for both adults and children.”

Salazar echoes Barnes hope. “We are integrating our pediatric and adult congenital heart disease programs to provide a continuum of care from the womb to adulthood,” says Salazar. “Children and adults with heart defects require interventions and continuous monitoring to lead a normal life, and we have the experts and tools to help them take control of their condition and live life to the fullest.”