AKRON, Ohio — Atrioventricular (AV) heart block is a rare condition that causes the heart to beat too slowly and pause several seconds between beats.
Previously, the only treatment for this condition was implanting a pacemaker. Now Akron Children’s is one of a handful of centers in the nation doing cardioneuroablation, or CNA.
Five-year-old Evelyn Rice loves the rare and mysterious unicorns. So it seems fitting that not only would she have a very rare condition, but also a very rare procedure that’s only being done in a few places in the United States.
Fortunately for her, one was only 40 minutes from her home.
Earlier this month Evelyn passed out in her kindergarten classroom at Sandy Valley Elementary School in Stark County.
Her mother, Chelsea Mack, rushed her to Akron Children’s Hospital, where she had another episode in the emergency department.
Doctors ruled out seizures and Lyme disease, but they noticed that Evelyn’s heartbeat was not only out of rhythm, but also pausing up to five seconds between beats.
Doctors determined Evelyn has a rare congenital condition known as atrioventricular heart block and the treatment is a pacemaker.
“It was a little overwhelming at first because I was like, 'what is this going to like, be like for the rest of her life? How are we going to manage this?'” Evelyn’s mother, Chelsea said.
Basically, Evelyn’s problem was a miscommunication from her brain to her heart.
“We all have a nerve in our brain that tells our heart to slow down, but that nerve sometimes can send an inappropriate signal and the heart rate just stops. If it stops for more than a few seconds, you’re going to pass out. Where that nerve enters the heart is at very defined points in the heart and, in our cath lab, we can find those points and we can cauterize that area. That modifies the heart’s response to that nerve so the heart doesn’t stop. It just slows down.” Said Akron Children’s pediatric cardiologist, Dr. John Clark, who is also the Director of the Arrhythmia Center.
Dr. Clark is one of a handful of pediatric cardiologists skilled at cardioneuroablation.
“That is a procedure that allows rewiring of the heart to let those signals that are telling the heart to communicate down to the lower chambers and create a pulse, as it should,” Dr. Clark said.
Evelyn’s procedure took place in the catheterization lab and Dr. Clark used 3-D imaging to help find the problem areas, instead of exposing her to radiation from an x-ray.
The entire time they’re recording Evelyn’s heartbeat. When it finally starts to beat normally, they know they’ve fixed the problem.
“It's a great time to be alive and I love what technology has allowed me to do in the cath lab because I’m getting to do things I never dreamed would be possible in my professional life. And I’m late in my career and just launching something brand new in the United States and it’s exciting,” Dr. Clark said.
For Evelyn’s parents, it’s a gift they were grateful to receive.
“I don’t even think I can really put it into words just how lucky we are that he was in our backyard, so close we didn’t have to go to another state or anything for this,” Chelsea said.
CNA procedures typically take between three to five hours. A catheter runs through the leg up to the neck where Dr. Clark stimulates the vagus nerve so he can watch the heart’s response in order to find the places that need to be cauterized.
What’s even more novel about this approach is that it may help those who’ve had pacemakers have the opportunity to have them removed.
Dr. Clark says that procedure would take up to two years to complete. The CNA would be done and the pacemaker would be turned down to a lower setting and monitored. If it doesn’t activate in a year, then it would be turned off for a year before it would be removed.
“The ideal patient for this would be between 20 and 40, so it could be done on adults, but there’s less optimism for those over age 60,” Dr. Clark said.
Dr. Clark is sure this will be the standard of care in the next decade.
Watch | Dr. Clark explains how CNA works below:
Read more about Dr. Clark's work on this procedure at Akron Children's here.
To learn more about CNA, Dr. Clark has made several trips to Brazil to train with Dr. Jose Carlos Pachon, a pioneer in the field.
This past October, the two doctors hosted a virtual global medical conference on CNA that became one of the most successful medical conferences ever for Akron Children’s with doctors from 39 different countries participating.
But for Evelyn’s family, they have much to be thankful for this season.
“I try my best to never take a single day for granted. Every moment that I have my kids is a blessing. But this Thanksgiving is something special that I'm forever thankful for, especially Dr. Clark and his team,” Evelyn’s father, Anthony Rice said.