Surgeon at Packard Children's Succeeds with a Heart Repair Believed so Risky that Others Avoid It

For Release: March 18, 2013

PALO ALTO, Calif. - “I’ve never run before,” said 24-year-old Brooke Stone, from Aptos, California. “I had to sit out of gym class my whole life because of my heart. But now I will be able to run and work out, thanks to Dr. Hanley.”

Brooke’s happy story is a lifesaving example of the pioneering work of cardiothoracic surgeon Frank Hanley, MD, at Lucile Packard Children’s Hospital Stanford. Hanley successfully corrected Brooke’s failing heart last September with a surgical procedure most hospitals avoid, thus saving her from a heart transplant.

Although many congenital heart defects are successfully repaired in infancy, the surgical fixes that work for babies sometimes produce deadly complications later in patients’ lives. That’s the situation Brooke was dealing with. She was born in 1988 with Transposition of the Great Arteries (TGA), one of the most complex congenital heart defects. But the surgical repair she received in infancy could lead to heart failure in the long run.

Brooke’s situation is increasingly common: Recent research confirms that the frequency of hospitalizations for adults with congenital heart disease, the No. 1 birth defect in the United States, has grown at a rate more than twice that for children. With an estimated 2,000,000 congenital heart disease survivors in the nation, there has been a rapid rise in congenital heart disease programs like the Adult Congenital Heart Program at Stanford. Now, although many people entering these programs had heart repairs when they were younger, some, like Brooke, are confronting deadly complications associated with those fixes.

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. Hanley was a cardiothoracic surgery fellow when Brooke was born, and a member of the team that provided Brooke with an atrial-switch repair when she was four weeks old.

The atrial-switch—which reroutes the heart’s blood flow without correcting its anatomical defect—was a major breakthrough in its day. It 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.

With Hanley’s approach, saving patients who are aging out of the atrial-switch repair they had as infants requires not only careful monitoring to catch them before right-ventricular failure, but ensuring that the left ventricle—a once-strong muscle weakened by years of doing the relatively easy job of pumping to the lungs instead of the body—is fit enough to endure the switch and pump blood to the whole body.

In the late 1980s, Hanley followed Australian surgeon Roger Mee in training a patient’s left ventricle back into shape by placing a band around the pulmonary artery. By tightening the band at intervals over the course of a year to 18 months, the left ventricle works harder to push blood through a smaller opening, increasing its ability to pump at high pressure. The heart is readied to have its old atrial-switch repair undone, and a newer procedure, the arterial-switch, performed to fully correct the dysfunctional anatomy. “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.” (In Brooke’s case, an obstruction had developed in her left-ventricular outflow-track, strengthening her left-ventricle naturally and making her an ideal candidate for the arterial switch procedure, with no left-ventricle training required.)

This careful process of ongoing monitoring, patient selection and rigorous evaluation is key to Hanley’s successful approach. Over the past 15 years, as the criteria for selection and the procedure have evolved, the survival rate has grown higher, and now exceeds 90 percent. So far, Hanley has managed 36 patients with failing atrial-switch, and estimates that thousands more in the U.S. may still need life-saving intervention of some kind. Like his pioneering unifocalization surgery, it’s another one of Dr. Hanley’s “against conventional wisdom” techniques that makes him stand apart from so many of his peers.

Now, as Brooke celebrates a future that once seemed very uncertain, her family is getting used to the promise of Brooke’s now-normal heart. “As of right now, it’s just hard to believe this is real,” mom Barb said. “Her whole life, she’d been told she couldn’t have children. Now she can have children—all because of one surgery. It’s the most amazing thing.” In the meantime, Brooke is easing into her newfound energy by going for walks, working out lightly and getting ready for that eventual first-time run. “I am so grateful,” said Brooke, who can now leave behind a life of being sick all the time. “I’m looking forward to living to live, instead of living just to survive.”

Authors

Robert Dicks
650-497-8364
rdicks@stanfordchildrens.org

Erin Digitale
650-724-9175
digitale@stanford.edu