A good Melody: mending the wounded heart of a dragon-slayer

George and Elisabeth

According to Christian legend, St. George saved the mystical town of Silene—along with a lovely princess—by slaying a dragon that plagued the city. As his namesake, William St. George Hunter (“George”), a 1-year-old heart patient at Boston Children’s Hospital, has shown a similar type of courage and spirit.

George’s battle against his heart condition began before he was born. At 25 weeks of pregnancy, his mother, Elisabeth Hunter, flew to Boston Children’s from South Carolina to undergo a fetal intervention with Wayne Tworetzky, MD, co-director of the Advanced Fetal Care Center and director of fetal cardiac imaging. George was born at Brigham & Women’s and went home with his family after several weeks of observation at Boston Children’s. But the Hunter family returned when George was five weeks old for the physicians to temporarily insert a balloon catheter in his aortic valve, a procedure called balloon valvuloplasty that seeks to dilate a narrowed valve.

“His condition improved after the balloon procedure,” says Elisabeth, “but we knew the treatment was unsustainable.”

George was scheduled for a check up visit this spring, but his condition suddenly deteriorated to the point that he was Medflighted up to Boston on April 20—two weeks earlier than planned. Through a series of diagnostic tests, Sitaram M. Emani, MD, cardiac surgeon, and his colleagues at Boston’s Children’s recognized that George’s mitral valve was leaking. To correct the leaky valve, the medical team and family decided it was appropriate to surgically replace it with a modified version of a valve (Melody, Medtronic, Inc.) originally designed for other positions of the heart. Unlike traditional prosthetic valves that have a fixed diameter, the Melody valve is housed in a stent that can be enlarged as a child grows, thus potentially avoiding the repeated valve replacement surgeries that are commonly required in a growing child.

Emani and George at Boston Children's

“George was a particularly good candidate to undergo this surgery, because his mitral valve was the predominant complication, so if we could fix the valve, there shouldn’t be many other problems,” says Emani. Also, his spirit gave the physicians confidence. “Even though he had quite severe heart disease prior to the procedure, his spirit never seemed to dampen—he was smiling and playing. It demonstrates how much of a fighter George is.”

The care of patients with disease of the mitral valve—which controls the flow of freshly oxygenated blood from the heart’s left atrium to its left ventricle—can be very complicated. If a child needs a mitral valve replacement, there are only a limited number of options available, all of which have one limitation in common: They have a fixed diameter and cannot grow with a child.

“Prior to this procedure, the current options for these patients have been disappointing. We were confined to either mechanical valves or prosthetic valves that didn’t grow with the pediatric patients,” Emani explains. “Now, with this modified Melody valve, we hope that George and other children who require a mitral valve replacement will not need to undergo multiple surgeries as they age and develop, because we can expand the valve as they grow.”

Emani and his colleagues have been performing the procedure for two years. “As we have gained more experience, we are more and more confident about the success of this novel procedure,” he says. “We fully anticipate that this valve could be dilated in order to prevent future surgeries.”

As George derives his name from the focus of legends and fairy tales, we are reminded that this child is a hero and why heroes are so important. In the words of the writer G.K. Chesterton, “Fairy tales do not give the child his first idea of bogey[man]. What fairy tales give the child is his first clear idea of the possible defeat of bogey[man]. The baby has known the dragon intimately ever since he had an imagination. What the fairy tale provides for him is a St. George to kill the dragon.”