When her twins were born, everything seemed to be going according to plan for Emily Duhe. “My husband and I wanted lots of kids, and we were so happy to start a big family,” she recalls.
Within a couple of months, though, it was clear that one of the twins, Allie, was in trouble. “We brought her to the hospital thinking she had pneumonia,” Emily says.
“That’s when they found multiple defects in her heart.”
It’s a familiar story. Margaret Narolis’ son Logan was also born with a major heart defect. “When Logan was born we were told he’d need to have multiple surgeries to reconstruct his heart,” she says.
The two families, separated by thousands of miles—the Duhes live in Louisiana, the Narolises in upstate New York—both came to Boston Children’s Hospital looking for better treatment options for their children’s damaged hearts.
Fortunately, they found what they were looking for.
Logan and Allie are now part of a small group whose hearts are beating with the help of a new expandable replacement valve—one that essentially can be made to grow as they do.
Both children had damaged mitral valves, a key part of the heart’s machinery. This one-way valve helps move oxygen-rich blood into the left ventricle; from there, it gets pushed out to the rest of the body. Surgeons need to repair malfunctioning mitral valve for proper health, but the surgery poses a unique set of challenges.
“In some ways, repair of the mitral valve is still more of an art than a science,” says Sitaram Emani, MD a pediatriccardiac surgeon at Boston Children’s. “It is structurally challenging from a surgical standpoint, and there are a lot of critical structures very close to it that restrict what you can do.”While surgeons prefer to fix a child’s existing mitral valve if they can, sometimes it’s just beyond repair, and replacing the valve becomes the only option. But replacement isn’t necessarily a one-shot deal.
“Children often outgrow a prosthetic valve within months to years after implantation, requiring multiple replacements over time,” Emani explains. And multiple replacements means more surgeries, both a huge expense and a huge strain on a child’s growing body. Furthermore, there are no commercially available valves small enough to use in infants and newborn children.
Emani wanted to find a solution for kids, particularly infants, who need a new mitral valve, but one that could be made to grow with the child, sparing them multiple surgeries. What he came up with was a modified version of what’s called the Melody valve. It’s an expandable prosthetic valve originally designed as a replacement for the pulmonary valve (the valve that controls blood flow from the heart to the lungs).
While the Melody is meant to be used at its full size, Emani noted that it still functions even when only partially expanded, opening the door to using it in infants and babies. By trimming the valve and cinching it down to its smallest size, Emani has been able to replace Logan and Allie’s mitral valves, as well as those of three other infants, with Melody valves—marking the first time this valve has been used for mitral valve replacement.
For both Allie and Logan, the modified valve helped them keep their whole hearts. And the change since their valve replacements has been remarkable.
“By Allie’s third surgery, her skin was grayish, she looked really unhealthy,” Emily says. “After Dr. Emani put the new valve in, she was pink and smiling. And a few weeks later, the team was able to expand her valve to fix a leak just by using a catheter, instead of having to carry out another surgery.
“When we first took her back to her cardiologists here, they didn’t recognize her because she looked so healthy,” she adds.
“Logan got his valve nine and a half months ago,” says Margaret. “It’s the longest he’s gone without having to go to the hospital or have surgery. He’s 25 months old now, and he’s doing the best he’s ever done.”
Neither child is completely out of the woods, but their futures look bright. “The doctors back home told us that she might only live to her teens,” Emily says of Allie, who is now nearly 2. “The doctors at Boston Children’s have never talked about mortality.” Both families are also relieved that by opting the modified Melody and not a mechanical replacement valve, they’ve avoided the need for blood thinners and the complications that come with them.
“By going this route, we could potentially leave the modified valve in place until a patient reaches adulthood, reducing the number of operations and the risk of lung swelling related to valve leakage,” Emani says. “And as these patients grow, we can expand the modified valve by cardiac catheterization It’s much less invasive than open-heart surgery and requires less recovery time.”