At ten years old, Jahliah Parker likes to have fun with her siblings, play on her Nintendo Switch, and, along with thousands of other children across the country during the COVID-19 pandemic and lockdown, is ready to go back to in-person school. She is an ordinary kid except for one little thing, her congenital heart condition. Jahliah suffers from Ebstein’s Anomaly, a rare disease treated with world-class care at UNC Health.
Ebstein’s Anomaly: A Rare Disease Treated with World-class Care Here at Home
Jahliah Parker and her Expanding Heart
Jahliah is the youngest girl of six children; her brother, whom she is close to, is the youngest in the family, and none of them do anything without the others. Much like her siblings, she is an ordinary kid except for one little thing, her congenital heart condition. Throughout her entire life, Jahliah has had to deal with erratic heart rhythms, skipping of her heart, heart beating too fast, sometimes to the point of pain, and asthma. At the age of nine, she started to have fainting spells.
“We’ve been monitoring her carefully throughout the day, every day,” says mom, Takeisha Harrison. “Even her teachers at school would watch her for signs, making sure she didn’t do anything too strenuous that would make her heart race and pass out. However, around December of 2019, it started to get worse. She would start passing out for no reason. She would be sitting at her computer desk or hanging out on the couch watching television, and she would faint.”
Takeisha brought her to Pediatric Cardiologist James P. Loehr, MD, at New Hanover Regional Medical Center in Wilmington. Dr. Loehr had been monitoring Jahliah for a couple of years, and he felt it was time to start her on medication. He also coordinated with the UNC team in Chapel Hill to discuss the need for surgical intervention sooner rather than later.
How the Story Began
In her seventh month of pregnancy, Takeisha went to the doctor for a routine ultrasound appointment at Wilmington Maternal-Fetal Medicine. It was at this appointment that doctors noticed an anomaly in Jahliah’s heart in utero. She was referred to Dr. John Cotton, a Pediatric Cardiologist and Professor of Pediatrics at UNC Chapel Hill. He followed Takeisha throughout the rest of her pregnancy, monitoring Jahliah, her growth, and the progress of her heart condition.
The anomaly the doctors discovered is a rare congenital heart defect called Ebstein’s Anomaly. This anomaly affects about one in 200,000 live births, and it accounts for less than 1% of congenital heart disease in the United
States. “This congenital disorder is an abnormality of the tricuspid valve, which is the valve that separates the right upper and lower chambers of the heart,” explains Dr. Mahesh Sharma, Section Chief of Congenital Cardiac Surgery and Co-director of the NC Children’s Heart Center. “Imagine a divider between two rooms, an upstairs and downstairs. If you move that divider closer to one room, downstairs, then the room upstairs will be much larger than the other. Now imagine that the larger upstairs room is supposed to be pushing water into the smaller room, but because the divider is malformed and does not act as a back-stop, the water is instead leaking backward causing the upper chamber to continue to enlarge over time.”
“So that means that the atrioventricular junction, which is the division between the upper chamber, called the atria, and the lower chamber called the ventricle is displaced downward into the ventricular cavity. Because of that, the attachments to the valve are very abnormal, and the valve tends to leak. In addition, the muscle of the pumping chamber is replaced with muscle resembling the upper chamber. a phenomenon called atrialization of the right ventricle. Depending on the severity of the leakage and the arrhythmia burden, determines when a patient has symptoms and when something ought to be done.”
Takeisha was scheduled for a C-Section in Chapel Hill, so once Jahliah was born doctors could perform surgery to fix her condition. Around 38 weeks, Takeisha went in for her weekly ultrasound, where doctors were concerned by what they saw. “The baby wasn’t moving that much during the appointment, and the doctors didn’t like the sound of her heart,” recalls Takeisha. “They wanted to transport me by helicopter to Chapel Hill, but I was too scared to fly, so they rushed me there by ambulance instead. As soon as I got there, they got me checked in, prepped, and into surgery.”
Doctors planned to perform surgery on Jahliah that night, hours after birth, but once they examined her, they chose to delay surgery. Her heart was much better after birth, and she continued to improve during her eight days in the Neonatal Intensive Care Unit (NICU).
“The condition can be very severe at birth where blood is going backward so intensely that a baby doesn’t get enough oxygen or blood across the other valve, the pulmonary valve, into the lungs and back through to the left side of the heart,” says Dr. Sharma. “This is what the doctors were worried about when Jahliah was firstborn. Fortunately, for her, she had a less severe form of Ebstein’s Anomaly.”
After being released from the hospital, Takeisha and Jahliah settled back into Wilmington. Her doctors monitored her condition. In 2017, Dr. Loehr joined New Hanover Regional Medical Center in Wilmington after leaving his position as an Associate Professor at UNC in Chapel Hill. He took over the care for Jahliah. Once it became clear that Jahliah’s condition was worsening, he referred the family to Dr. Scott Buck, a Pediatric Cardiologist at UNC who specializes in the diagnosis and treating heart rhythm abnormalities, along with Dr. Mahesh Sharma one of the few surgeons in the country with experience in the surgical management of Ebstein’s anomaly.
Time to Find a Solution
“Patients can live with this anomaly, if it’s very mild, for their whole life without requiring treatment,” explains Dr. Sharma. “Some patients on the other extreme, born with such a severe form of the defect, require something done or they die. The vast majority of patients are in the middle, and they’ll come to develop symptoms like shortness of breath, palpitations, feeling less active, or getting short of breath with exertion at some point in their lifetime. As the amount of blood leaking backward from the tricuspid valve increases over time, as that valve leaks more and more, the upper chamber starts to dilate or balloon out because blood is going backward. When that happens, it sets the patient up for irregular heart rhythms. This is where Jahliah fell and why we felt it was finally time to correct the defect.”
In June 2018, Mahesh Sharma, MD, was recruited from the Children’s Hospital of Pittsburgh to lead the surgical program at UNC Children’s Heart Center. He brought with him expertise and training that have proven invaluable. When Jahliah was referred to him for a consult, Dr. Sharma called upon his previous training and connections to former colleagues to find the best solution for her.
During his time at Children’s Hospital of Pittsburg of UPMC, Dr. Sharma worked with Dr. Jose da Silva, the foremost authority in the world on Ebstein’s Anomaly. In the early 1990s, while trying to repair the tricuspid valve in patients with Ebstein’s Anomaly, Dr. da Silva was concerned about the limitations of multiple techniques that were available at the time. As such, Dr. da Silva developed a novel repair method called the “Cone” procedure pioneered in his native Brazil (Arq Bras Cardiol, volume 82 (no 3), 217-20, 2004). Worldwide experience has been limited to a handful of centers in North America until Dr. da Silva’s relocation to the United States. With the assistance of the State Medical Board of North Carolina, Dr. DaSilva was able to join Dr. Sharma in the operating room to assist in performing this rare and complex surgery.
In August 2020, Drs. Sharma and da Silva performed open-heart surgery on Jahliah. Doctors detached one of the leaflets of her valve and divided all of the abnormal attachments of the valve to the right ventricle. With the detached tissue, they recreated a functional tricuspid valve by creating a funnel or cone out of the valve tissue. As the conical valve was constructed, the surgeons reattached it inside the heart so the divider or separation is now at the correct location, referred to as the atrioventricular junction. This was the first such repair for Ebstein’s anomaly using the da Silva technique at UNC and very likely the first true Cone Procedure in the state of North Carolina.
“It’s our feeling that the Cone Procedure offers a good durable repair,” explains Dr. Sharma. “Other repair options include placing an artificial valve in a child, but this is not the best solution because the artificial valve doesn’t grow with them. In a child that’s still growing to replace the valve commits them to multiple operations, valve infections, or valve thromboses, which are clots in the valve. Eventually, the patient will need multiple valve replacements because the tissue valve won’t last their lifetime. Putting in the artificial mechanical valve on the right side of the heart can be dangerous because those valves tend to clot more and if the valve clots, it doesn’t work, and the patient can get very sick quickly. Those are the reasons why we selected the Cone technique, which uses their patient’s own valvular tissue, and is the most natural way to address the problem with the valve. Around 95% of the heart centers in the country don’t have experience or expertise with doing the Cone operation in children.”
Along with the Cone procedure, Dr. Sharma and his team performed arrhythmia surgery, also referred to as the “Maze” procedure. The Maze procedure interrupts the abnormal electrical circuits inside the heart that create an arrhythmia or abnormal heart rhythm. The Maze procedure establishes a way for electrical activity to follow the maze that the doctors created, and so the irregular heart rhythms are controlled without medicine. Jahliah showed promising results from both aspects of the operation, and her heart has maintained a normal rhythm since leaving the hospital.
The hope for Jahliah is that she is not going to require lifelong medication for the heart rhythm problem because doctors solved that issue with the Maze procedure. Her heart function has improved with the repair of her heart valve, the amount of dilation or stretching of the heart has decreased, and her heart size is now approaching normal. The heart valve that was repaired, the tricuspid valve with the Cone operation, seems to be working very well with no valve narrowing and minimal or no regurgitation or backflow of the valve anymore.
Timing and Recovery
Jahliah underwent surgery at UNC Children’s hospital in the middle of the COVID-19 pandemic. Doctors weighed the timing of her procedure since they didn’t want to put her at risk. Her heart condition also affects the lungs, as does going on the heart-lung machine during the operation. They didn’t want to subject her to any kind of threat of COVID infection on top of the open-heart surgery. This is how doctors came to the decision to schedule her surgery over the summer. Not only was Jahliah out of school, but the hospital didn’t have any pediatric patients with COVID in the ICU at the time.
“It was very stressful and scary knowing that my nine-year-old was about to have open-heart surgery,” says Takeisha. “I have to say she had an awesome, amazing team of doctors and nurses that worked with her. They reassured me that they were going to do everything they could for her while she was in surgery. It gave me a little peace of mind, but I was still worried, still scared, still praying as a mom. Jahliah, on the other hand, didn’t have any fear about the surgery at first. I think I was more scared than her.”
After her surgery, Jahliah was able to recover in a COVID-free zone in the pediatric cardiac ICU. They would call Jahliah’s siblings multiple times a day to check in via Facetime so the family could see her progress and hear her voice.
“Dr. Sharma was the best,” says Takeisha. “The best I ever ran across! He was awesome. He really was. He reassured me, gave me peace of mind. The words of encouragement that he gave were a relief for my worried soul. It helped me along the process. The doctors made sure they did everything right. And in the end, it was successful, better than they expected.”
Care in Her Community, with Support from NC Children’s Heart Center, Quaternary Care Provider
Since her surgery in August, Jahliah celebrated her 10th birthday. She still has a loop monitor under her skin, which allows UNC Health to monitor her heart rhythm. The hospital sends Takeisha reports periodically, letting her know how Jahliah is doing, no more irregular heartbeats, no more racing heart moments. As part of her recovery, she also has follow-up care with Dr. Loehr. All of Jahliah’s doctors stay in contact to discuss her case and make sure she progresses well in her recovery.
The new partnership between UNC Health, Novant Health, and New Hanover Regional Medical Center has helped cement those relationships and open up care for additional members of the community across the state of North Carolina. This partnership will expand the recently approved relationship between these two organizations in New Hanover County to include Mecklenburg, Forsyth, and other counties, with an additional focus to find innovative solutions to enhance care in rural areas. “This partnership is very exciting for us because we are providing regional pediatric subspecialty services both in their communities as well as quaternary care at the medical center here,” explains Dr. Sharma. “The partnership allows patients to get the full spectrum of care either at home or here in Chapel Hill depending on the level of care that’s required.”
This allows for a seamless relationship between Jahliah’s cardiologist in Wilmington, Dr. Loehr, to work with the team at UNC Children’s Hospital in Chapel Hill to make sure she and other patients like her receive the care they deserve and don’t fall through the cracks.
Advice for Parents
When a child is sick, it can be overwhelming for parents when it comes to any disease they face, because they face it as a family. For a child with a congenital heart defect, the vigilance of parents is paramount when monitoring their child. Kids want to play, they want to hang out with friends, ride their bike, catch a ball or play on a playground and UNC Health wants that too, helping children be kids and enjoy this time in their life. It is why the UNC Health team appreciates the work our parents do to ensure the safety and watch for warning signs of kids diagnosed with congenital heart conditions.
“I think the most important thing I would want other parents to know is that many times your child is not going to tell you something is wrong,” says Takeisha. “Jahliah would never tell me her heart was beating too fast unless it started beating to the point where it was hurting her chest. I had to look at her for warning signs. I knew something was wrong when I looked at her face and her eyes would get real little, she’d get a weak look and she might start getting hot or throwing up. You have to know the signs with the kids because they don’t want to stop playing or being a kids. But as the parent you need to be ready to know if they need to be rushed to the emergency room or you need to call an ambulance.”