New, Non-surgical Solution Repairs Heart Defect

The nearly 40,000 babies born each year with congenital heart disease (CHD), or birth defects of the heart, face a lifetime of surgeries and procedures to keep their hearts pumping properly. But a new, first-of-its-kind, non-surgical treatment now offered at Cook Children’s Medical Center is reducing the number of open-heart surgeries needed for some teenage and adult CHD patients.

Micah Rucker, 29, is one of them. He is the first patient at Cook Children’s to receive the Harmony™ Transcatheter Pulmonary Valve (TPV). It is the first catheter-based valve delivery system designed to treat a leaky native pulmonary valve approved for use in the United States by the Food and Drug Administration (FDA).

Transcatheter balloon expandable valves have been used in patients with previously placed surgical conduits, which is a prosthetic connection between the right ventricle and pulmonary artery. The Harmony TPV is the first valve designed specifically for those patients without a conduit who require pulmonary valve replacement.

In Micah's case, his pulmonary valve did not form correctly. It remained sealed and blocked the blood flow from the heart into the lungs. By the time he was 2 years old, he had already had two open-heart surgeries—one to open his sealed valve and another to expand the size of the right side of his heart. Doctors fully anticipated he’d need another by the time he was a teen. But Micah defied the odds, which bought enough time for technological advances to turn what could once only be done with open-heart surgery into a minimally invasive procedure.

“His father and I were just hoping that by the time he needed a new valve that the technology would get to the point where he wouldn't need open-heart surgery again,” Karen said. “Because we just really didn't want that for him, especially when he was old enough to remember it. He doesn’t really remember his surgeries when he was a baby.

A leaky pulmonary valve, also known as pulmonary regurgitation, allows blood to backflow into the heart’s right ventricle instead of into the pulmonary artery where it travels to the lungs for oxygen. It’s common for children with CHD to have a leaky valve as a result of their defect or previous surgeries.

“Leaking of a pulmonary valve in a child is not usually critical in the newborn or childhood years,” said Dennis VanLoozen, M.D., an interventional cardiologist at Cook Children’s. “They tolerate that quite well initially. So we often trade that early in life in order to relieve an obstruction. But over 10, 15, 20 years of that valve leaking, that’s when we start to see the effects on the heart where we know it is time to place a competent valve.”

Open heart surgery comes with a large incision in the chest wall and requires a multi-day stay in the hospital. Recovery can be long and painful. The Harmony TPV gives patients a welcome alternative.

The procedure is performed by an interventional cardiologist in the cardiac catheterization lab. During placement, a catheter encasing the artificial heart valve is inserted into a vein through a small incision in the leg or neck. It is guided through the vein into the heart’s right ventricle outflow tract where blood passes from the heart into the pulmonary artery. Once in position, the valve is deployed, expands to fit the size of the outflow tract and immediately begins directing blood flow from the heart into the blood vessels connected to the lungs. The catheter is removed through the same vein in which it was inserted.

Patients with right side heart defects typically have irregular outflow tracts that are larger in diameter. The self-expanding aspect of the valve can treat a much wider size range and provides a better fit than previous generations of valves.

The procedure takes about two hours. With just a small incision to insert the catheter, post-op pain is minimal. Most patients are able to go home the next day and can return to their normal daily activities within a week.

“How quickly people recover and bounce back is really the exciting part of doing these in the cath lab,” Dr. VanLoozen said.

Although Cook Children’s is a pediatric medical center, most of the candidates for this procedure are teenagers or adults, like Micah.

“The tricky part about CHD is just because you turn 18 doesn’t mean we are done with you,” Dr. VanLoozen said. “Congenital heart disease is a special thing where you stick with the pediatric side of care with physicians specialized in treating birth defects in adults. Cook Children’s has a program designed just for that.”

It’s called the Adult Congenital Heart Disease program and offers ongoing care for adults with CHD. Scott Pilgrim, M.D., is the program’s medical director.

“I am very excited about this new development in transcatheter pulmonary valve replacement technology and having the ability to do these procedures at Cook Children’s,” Dr. Pilgrim said. “The prospect of another open-heart surgery is sometimes very overwhelming for the adult with congenital heart disease, as many have had multiple prior surgeries already. The Harmony valve offers a relatively non-invasive option to a much wider group of patients who were previously determined to not be candidates for a transcatheter approach. With a recovery time of only one overnight stay, the Harmony valve is a very attractive option, with many patients returning to work within a few days.”

Today, Micah is home where he lives with his mother and grandmother. He still has a few weeks until he is cleared to exercise and ride his bike, but he’s back to doing other things he loves, like helping to care for his grandmother, playing video games, writing and cooking. Steak is his specialty, much to his mother’s delight.

“I have more energy than I used to have, and I’m breathing a little better than I used to,” Micah said.

He and his mom are happy to have avoided another painful surgery. Most of all, they’re grateful for the technological advances that made it possible.