New Criteria and Surgeon’s Unique Expertise Offer Hope for Some Congenital Heart Disease Patients

In September 2012, 24-year-old Brooke Stone had her second lifesaving heart surgery, this time at Lucile Packard Children’s Hospital Stanford. When people ask about her chest scar, she gives them the simplest answer she can: “I tell them I was born with my heart attached backwards.”

As a newborn in 1988, Stone was diagnosed with Transposition of the Great Arteries and underwent a complex surgery to correct her blood flow. In TGA, the two main arteries that come out of the heart—the pulmonary artery and aorta—are connected to the wrong chambers of the heart. As a result, blood from the lungs flows back to the lungs and blood from the body flows back to the body without ever getting the oxygen it needs.

“Transposition of the Great Arteries was almost 100 percent fatal before the late 1950s,” said Frank Hanley, MD, director of the Children’s Heart Center at Lucile Packard Children’s Hospital Stanford.

Brooke’s first surgery—a Senning procedure, one of two similar procedures known as Mustard or Senning after the two doctors who developed them—was considered the most trusted repair for TGA at the time. These procedures, which reroute the heart’s blood flow without correcting its congenital defect, were a major breakthrough in their day. They saved the lives of countless children who did well through their 20s and 30s before anyone realized a problem: After a lifetime of pumping blood at a higher pressure to the body, the right ventricle, designed to pump blood gently to the lungs, would eventually fail.

To remedy this problem, surgeons in the late 1980s began taking down patients’ old Mustard/Senning repairs and doing a new surgery—the “arterial switch”—to completely repair the heart’s anatomy. While the arterial switch had become standard procedure for newborns with TGA, it was much riskier for adults with a weakened heart. Many patients required months of left-ventricle training to strengthen the heart in preparation for the arterial switch.

“Like a lot of new things,” said Hanley, “there was a flurry of interest at first and a whole bunch of cutting-edge institutions jumped on board to try it. A lot of those patients didn’t do well, and many surgeons were discouraged by the bad results. So they abandoned it.”

But not Hanley. Instead of backing away from the procedure, he looked closer to understand its limitations. “The idea that everyone who needed the procedure could just be slam-dunked into the arterial switch was wrong,” he said. “We focused on setting rigid criteria for accepting people into the program, and setting up a five-point report card after left-ventricle training to ensure that we were selecting appropriate patients who would have good outcomes.”

Today, Hanley may be the only surgeon in the United States doing the procedure. A careful process of lifelong monitoring, patient selection and rigorous evaluation is key to his successful approach. Over the past 15 years, as the criteria for selection and the procedure have evolved, the survival rate for Hanley’s patients has grown to exceed 90 percent. So far, Hanley has managed 36 patients with a failing Mustard/Senning, and estimates that thousands more in the United States may still need lifesaving intervention of some kind.

Congenital heart disease is the most common birth defect, killing more kids than all childhood cancers combined. “Many parents of children with congenital heart disease don’t recognize that their kids need lifelong cardiac care,” said Susan Fernandes, program director of the Adult Congenital Heart Program at Stanford, and lead author of a 2011 study on the topic. “And it is estimated that more than 50 percent of adults with congenital heart disease are not receiving specialized adult congenital cardiac care or are lost to follow up, with most falling out of appropriate care before mid-adolescence.”

Those who are monitored are often followed by a primary care doctor who may not know about the potential complications of this condition. This unseen risk points to the larger problem of how to provide the best monitoring and care of survivors of congenital heart surgery.

“There’s a big push in this country to organize adult survivors of congenital heart surgery and bring them into clinics where they can be treated effectively,” said Hanley. Packard Children’s Hospital is one of those, with the multidisciplinary Adult Congenital Heart Program at Stanford. This program is a major priority for these institutions. Stanford recently recruited George Lui, MD, as medical director of its Adult Congenital Heart Program.

Because catching patients before they suffer irreversible cardiac damage could improve their ability to qualify for novel therapies that may increase their chances of survival, the push for more organized care can’t happen soon enough. With resources and clinic directories for patients across the country, the Adult Congenital Heart Association is an invaluable driver in this effort.

It’s especially important because, as Brooke experienced, the heart of a person with congenital heart disease may be weakening and experiencing potentially lethal rhythms without any outward signs or symptoms. During the summer that Brooke’s arrhythmia was first discovered, she said, “I felt the best I’ve ever felt in my whole life.” Staying on top of the disease allowed Brooke and her family to plan ahead, to have treatment choices, and, most importantly, to avoid a heart transplant.

Dr. Hanley told Brooke she would need surgery within 3 to 5 months, but let her pick the date. Her 8-hour surgery took place on September 27. She was told to expect a 3-week-long hospital stay. After seven days recovering in the cardiovascular intensive care unit and one night on the cardiac step-down unit, Brooke was released.

“She came out of the hospital and smiled,” said Brooke’s mother, Barb. “She looked up at the blue sky and she started crying. That was a moment I will never forget, because I could see the feeling in her, like, ‘I am alive.’”

“I’m looking forward to running,” Brooke said, “and just being able to not have any limitations—living to live instead of living to survive.”