Fiona D’Souza remembers the intense fear that swept over her when she learned that her newborn, Clyde, had a hole between the lower two chambers of his heart.
“It was overwhelming, and I was already overwhelmed just by having my first baby,” she says. “The fact that my husband is an internist actually made me more anxious, because he was running through the worst-case scenarios.”
Clyde’s condition, tetralogy of Fallot, was discovered shortly after his birth in a British hospital. It is a rare disease, with fewer than 20,000 cases in the United States each year. Clyde actually had four problems: a hole between the lower chambers of the heart, an obstruction from the heart to the lungs, the aorta lying over the hole in the lower chambers and overly thickened muscle surrounding the lower right chamber.
When Clyde was just 3 months old, doctors in Britain performed open-heart surgery to close the hole and relieve the obstruction to the lungs. While this operation is lifesaving, it leaves patients such as Clyde with no valve between the heart and the lung arteries. Without this valve, much of the blood going to the lungs can flow backward from the lungs to the heart. Over time this can lead to problems with the heart’s function and rhythm—and in older patients can increase the risk of sudden death.
By the time Clyde was 5, in 2010, his heart was struggling. His right ventricle had become severely dilated and was functioning at only 30 percent of normal. He needed a valve between his lungs and his heart.
Keeping up with Clyde
At Yale Medicine, Clyde underwent a second open-heart surgery, and a bioprosthetic pulmonary valve (manufactured from a cow’s heart tissue and a metal frame) was placed in his heart.
Although the operation went well, enduring another open-heart surgery was difficult for the family. And this time, Clyde was old enough to be aware of the procedure.
Clyde’s parents knew that yet another surgery would be necessary when their son outgrew his new valve. Last year, Clyde seemed fine—a happy, active 11-year-old boy who played the trumpet, piano and recorder and ran around his Connecticut home with a younger brother and sister.
But during a checkup at Yale Medicine, doctors determined that his bioprosthetic valve had degenerated and was no longer working. It was leaking severely, causing his heart to expand.
The nonsurgical fix
This time, though, there would not be an open-heart surgery. Clyde was eligible for an innovative treatment called a transcatheter heart valve implantation, performed by Jeremy Asnes, MD, director of the Congenital Cardiac Catheterization Laboratory at Yale Medicine. This procedure became commercially available a few years ago, and Yale Medicine is the only place in Connecticut to offer it.
“We put a catheter into a vein in the groin area,” Dr. Asnes says. “The valve, which compresses to just seven millimeters, can go through the vein. Once we get it into the correct position, we can expand it with a balloon to 22 millimeters in diameter. Rather than taking out the old valve, the new valve is put inside the old one, kind of like a chimney liner is inserted into a chimney.”
The procedure entails far less risk and recovery time than an open-heart surgery, says Dr. Asnes, who has performed about 100 of them.
The big day
Clyde weighed 83 pounds on the day of the procedure in November 2015. Dr. Asnes has performed it on children who weigh as little as 33 pounds. “The technology is terrific and cutting-edge,” he says.
Clyde quizzed the medical team about the procedure beforehand. His favorite subject is science, and he wants to be a pediatrician. “Some are scared, but a lot of kids are fascinated by what we’re doing,” Dr. Asnes says. “We go over everything in detail with the children and their parents.”
Clyde remembers going to the hospital on the morning of his surgery. “I was lying on the bed in the lab, and they let me put the mask on myself so it would be comfortable,” he says. The day before, he says, his team let him decide how his anesthesia would smell. “There was a vivid smell of green apples,” Clyde says.
He also remembers what it felt like to let go and fall asleep. “Everything started to look like the static on TV screens,” he says. “When I woke up, I wondered if I had to have the procedure again, since it was so fast. I didn’t feel any pain.”
An easy recovery
“Two days later, I was back at school and racing my brother,” Clyde says. “My friends were happy for me. I feel quite lucky to have been born in this century.”
Eventually—probably in eight or nine years—Clyde will have to have an open-heart surgery to take out the defective valve, but transcatheter heart valve implantation avoids surgeries along the way, Dr. Asnes says.
“Clyde is doing great,” his mom says. “We are filled with gratitude toward the team at Yale Medicine. It was a phenomenal experience.”
Dr. Asnes says he was touched when he received a handwritten letter from Clyde soon after the procedure.
“I came out of the hospital on Wednesday the 11th, one day after you placed the valve in my body,” Clyde wrote in his letter. “It went so great I was begging my parents to let me go to school! Of course, they would not let me but the next day I went to school and the day after I was playing sports! Thank you for giving me the best care on the planet."