Hope for Kevin’s heart: Five-year-old shines after novel treatment for Ebstein’s anomaly

Kevin, who was born with Ebstein's anomaly, practices his dance moves.As the lights dimmed and Pharrell Williams’ “Happy” blasted from loudspeakers, Kevin Nolan III took to the stage for his very first dance recital. Sporting striped pants, a turquoise bow tie and a black top hat, Kevin joined his class in performing two hip-hop jazz routines to a packed house. Kevin’s mood was perfectly in step with the song’s lyrics.

“He had so much fun,” says Kevin’s mom, Laura. “He said he can’t wait to get on stage again.”

While a first dance recital is a big deal for any 5-year-old, it’s especially poignant for Kevin, who was diagnosed prenatally with Ebstein’s anomaly, a rare heart condition that causes leakage of the tricuspid valve and backup of blood flow into the heart. Kevin also had pulmonary valve regurgitation, which was stealing blood flow away from his essential organs. His condition was so severe that when it was first discovered during a prenatal ultrasound, doctors at a hospital in Boston said he might not survive.

“We met with a heart specialist who told us we should just say goodbye,” says Kevin’s dad, Kevin Jr. “He said nothing could be done.”

But the expectant parents weren’t ready to give up. They pressed the doctor for a second opinion.

Luckily, that doctor referred the Nolans to Dr. Wayne Tworetzky, director of the Boston Children’s Hospital Fetal Cardiology Program. He offered them another option.

An experimental treatment offers hope

“Dr. Tworetzky suggested an experimental medication I could take during my pregnancy,” says Laura. “It was so new that I would be one of the first to try it.”

The medication, indomethacin, which is similar to ibuprofen, is not traditionally recommended during pregnancy. Although it is used prenatally for other conditions, it had not been used to treat this particular heart defect.

Dr. Tworetzky examines Kevin, who was born with Ebstein's anomaly.
Dr. Tworetzky examines Kevin

“The indomethacin would help Kevin’s heart by narrowing his ductus arteriosus, the connection between the aorta and pulmonary artery,” explains Tworetzky. “This would reduce the amount of pulmonary regurgitation and improve blood flow to Kevin’s body.”

The hope was that the more robust blood flow would allow Kevin to be delivered at term, instead of prematurely, giving him a better chance of survival.

“Dr. Tworetzky was really good at explaining everything to us,” says Kevin Jr. “We knew there were no guarantees, but it was a chance for our son and we decided to take it.”

Laura started the treatment at about 25 weeks. After a few days of indomethacin, the medication was switched to ibuprofen.

“I was a little nervous at first,” says Laura. “But once we saw the medication was doing what it was supposed to do, we felt reassured.”

Kevin, born with Ebstein's anomaly, smiles during a checkup.

Throughout her pregnancy, Laura had frequent ultrasounds to monitor Kevin’s progress. Tworetzky worked closely with her and her obstetrician to adjust the dose of medication as needed.

“It was a bit of a balancing act. We needed to keep an eye on Kevin’s amniotic fluid because the medication caused it to get really low,” says Laura. “So I would stop the medication for a few days, his fluids would increase again, and then I could continue taking it.”

A Mother’s Day birth, a Father’s Day homecoming

On Mother’s Day 2012, at 38 weeks, Kevin was born weighing 7 pounds, 13 ounces. Tworetzky and his team were on hand to make sure Kevin received immediate care. The Nolans were prepared for a rocky start.

“Dr. Tworetzky had warned us that his lungs might not have room to develop properly because his heart was so big,” says Laura. “Although they had developed, he still needed to be intubated right away because he was having trouble breathing.”

Kevin remained in the Cardiac Intensive Care Unit (CICU) for a few weeks receiving breathing support and medication for his heart. After five weeks, on Father’s Day, he was able to go home.

Kevin, born with Ebstein's anomaly, poses recently with Dr. Tworetzky.

“His ductus arteriosus eventually closed completely and his pulmonary regurgitation switched directions,” says Tworetzky. “As his lungs expanded, there was normal forward flow of the blood. This is what we wanted.”

Surgery opens new doors

For the next four years, as Kevin grew and developed, Tworetzky kept watch on his heart.

“He was doing well and developing normally, but he would get tired easily,” says Laura. “He’d run around like other kids, but then he’d have to rest and be mellow. We knew he’d eventually need to have surgery, but we weren’t sure exactly when.”

Last year, Tworetzky and Dr. Pedro del Nido, chief of cardiac surgery at Boston Children’s, decided it was time for Kevin to have a cone procedure to repair his tricuspid valve. Del Nido performed the procedure last July.

Since that time, Kevin has blossomed.

“This past year has been amazing,” says Laura. “He has so much energy now; he never stops moving. He’s grown three inches since the surgery and gained about seven pounds. Now he’s taller than all his friends.”

Kevin puts that excess energy to good use on the dance floor. He also started swim lessons and is excited to begin kindergarten this month. At a recent checkup, the Nolans got the good news that Kevin can now move to yearly visits with Tworetzky.

Kevin, born with Ebstein's anomaly, poses with his mom and dad.
Kevin poses with mom and dad

“He was born with heart failure and now his heart is functioning normally,” says Laura. “It’s really incredible to think that if we had gone with the first doctor’s opinion, he might not be here at all. All we needed was a little bit of hope and that’s what we got from Dr. Tworetzky and Boston Children’s.”

Explore bostonchildrens.org/fetalheart for more information and resources.