A novel therapy helps Amaryana

After returning from recess at Mack Benn, Jr. Elementary School in Suffolk, 7-year-old Amaryana Harden told her teacher that she didn’t feel well. “It hurts here,” she explained, pointing to her chest. 

When the discomfort didn’t subside, her teacher sent the second-grader to see the school nurse, Stacy Breneman. 

"Amaryana came into the clinic smiling and looking just fine, but when I listened to her heart, it sounded unusual, and her heart rate was alarmingly low,” says Stacy, a registered nurse who spent more than a decade working in cardiac care before accepting her position at the school. “A normal heart rate for a child her age is between 80 and 100 beats per minute and Amaryana’s was only 48. Something wasn’t right.” 

Stacy called the girl’s mother and recommended Amaryana be evaluated right away. Keysha Harden picked her daughter up from school and took her to the emergency department at Sentara BelleHarbour where physicians diagnosed her with bradycardia – a slow heart rate. They immediately called for a transport to CHKD where Amaryana could be further evaluated at the hospital’s heart center.

At CHKD, Dr. John Reed, a pediatric electrophysiologist who specializes in abnormal heart rhythms, examined Amaryana and ordered an EKG. “Chest pain in children is usually unrelated to heart problems. But Amaryana’s heart rate was exceptionally low, and the results from her EKG were very telling,” says Dr. Reed. “The test showed that the upper chambers of her heart were not working properly.”

A normal heartbeat begins in one of the upper chambers, the right atrium, of the heart. The electrical impulse then travels to the two lower chambers, called the ventricles, which do the majority of work pumping the blood into the lungs and out to the body. The right and left ventricles work simultaneously. Amaryana’s EKG showed that there was no electrical activity in the right or left atrium of her heart. Her blood was passively flowing through these upper chambers, and her lower chambers were doing all the work, resulting in a very slow heart rate. If left untreated, the effects of this condition could be life-threatening and would likely  damage her heart.

Dr. Reed explained that Amaryana would need a pacemaker to prevent dangerously slow heart rhythms and prevent long-term cardiac damage. “I was scared and shocked,” says Keysha. “You don’t think about a child needing a pacemaker.” 

The pacemaker, a small computer about the size of a short stack of silver dollars, sends electrical impulses to the heart muscle to maintain a proper heart rate and rhythm. Using the advanced imaging technology in CHKD’s new state-of-the-art cardiac catheterization lab, doctors can diagnose and treat heart conditions like Amaryana’s using catheters instead of surgery. Dr. Reed’s plan was to place the pacemaker through Amaryana’s armpit into the area behind her pectoralis muscle in the chest and then guide its tiny wires through her veins to the right atrium.

During the procedure, however, Dr. Reed found the upper chambers of Amaryana’s heart to be completely unresponsive to the pacemaker’s electrical pulses. ”She had a condition called atrial standstill,” says Dr. Reed.  

Atrial standstill occurs when there is complete absence of electrical or mechanical activity in the atria. “This is exceedingly rare, especially in children,” says Dr. Reed. “I’ve seen maybe two cases in my career.” 

After identifying the condition, Dr. Reed revised his treatment approach and used an innovative strategy to help Amaryana’s lower heart chambers beat efficiently and avoid long-term damage. He maneuvered the pacemaker wires to just below the node in the center of her heart, known as the AV node, which normally serves as the electrical gateway between the upper and lower chambers. Maintaining this natural path would allow the electrical pulse to travel to both lower chambers of the heart at the same time. 

“One of the long-term complications that can arise from pacemakers is ventricular dilation and dysfunction due to the lower heart chambers beating out of sync. By placing the wire from the pacemaker just below the AV node, the electrical impulse spreads to both sides of the heart at the same time and allows the lower chambers to achieve a more natural, synchronized beat,” says Dr. Reed. “This is a novel approach that will give Amaryana a much healthier long term outcome.”

Having her condition identified by the school nurse was a stroke of luck that gives Amaryana a very big advantage. “Her chest pain was not caused by her heart condition, so she is fortunate that her school nurse noticed her low heart rate,” says Dr. Reed. “With few other obvious symptoms, there is a chance this condition would have gone undiagnosed for many years – until a catastrophic event or permanent damage to her heart had occurred.”

Amaryana has a special place in her heart for Dr. Reed, and her mother is extremely grateful CHKD had the specially-trained expert her daughter needed. “I’m so grateful to her teacher, the school nurse and everyone at CHKD,” says Keysha. “Dr. Reed is one of the only experts of his kind in the area, and we are so lucky to have him close by. He was amazing.”

After the procedure, Amaryana spent only two days in the hospital and was able to resume normal activities after six weeks. At a follow-up visit in the future, she will receive a home transmitter that can read her pacemaker activity and transmit it remotely to CHKD if she is experiencing problems. But Dr. Reed doesn’t anticipate any issues. “Amaryana has very few restrictions,” he says. “I expect her to have an active and healthy life.”