Hypertrophic cardiomyopathy, heart transplant
When Kaila and Vincent Ellinwood took their son, Gray, to see the ophthalmologist, the only expected outcome from the visit was a new prescription. Yet through a series of unexpected test results, it was revealed Gray had a serious heart condition.
The ophthalmologist discovered some inflammation in Gray’s optic nerve, which led to the scheduling of an MRI. Before performing the MRI, doctors first wanted to get an EKG since Gray’s heart rate was abnormally high—since Gray was only 4 years old at the time, he was very nervous at the hospital—and it was discovered that Gray was suffering from hypertrophic cardiomyopathy, a disease that causes the heart muscle to become thickened, making it harder for the heart to pump blood.
Doctors at the time recommended that Gray go home wearing a monitor that would record his heart’s rhythm. “So that’s what we did,” Kaila shared. “We went back to the normal routine of Gray going to daycare, but within days, it was clear something was really wrong because he had two episodes at daycare when he fell down without an obvious reason.”
The family quickly took Gray back to their local hospital and then transferred to USA Children’s and Women’s Hospital, where he spent the next two nights. During that time, he had a sporadic heart rate and bouts of atrial tachycardia. “It was nothing normal for a 4 year old,” Vincent added.
The team at USA Children’s and Women’s Hospital consulted with F. Bennett Pearce, M.D., at Children’s of Alabama and transferred Gray to the Children’s of Alabama Bruno Pediatric Heart Center. After Gray was flown to Birmingham, doctors found that the monitor had recorded two times that his had heart failed to contract. “They said people who exhibit that will exhibit it again, and they most likely die from it if it’s not caught in time,” Vincent said. “That’s when it was decided the only protection against it would be a pacemaker and implantable cardioverter-defibrillator (ICD).”
While the pacemaker is used to prevent heartbeats that are too slow, ICDs deliver electric shocks, when needed, to restore a regular heart rhythm. Gray underwent surgery to have a dual capability device implanted, and the procedure and early recovery went well. Subsequently, he began to retain fluid and show signs of congestive heart failure which is rare in hypertrophic cardiomyopathy. His response to anti-congestive medications was suboptimal, Pearce said, and the medical team began to talk with family about heart transplantation. “After a discussion with Dr. Pearce, we began the heart transplant evaluation, which is necessary to be placed on the transplant list, in hopes that we would never need to use it,” Vincent explained.
The patient and family began going through the process of testing and evaluation as prerequisites to getting on the transplant waiting list while Gray continued to redeveloping gross motor skills through physical and occupational therapy. Over the course of three months in the hospital waiting for a heart, Gray’s body began showing signs that his heart just wasn’t able to adequately respond to conventional guideline-based anti-congestive therapy. “He’d lose all color and become very lethargic,” Vincent said. “His heart just couldn’t keep up with the oxygen demands of his body. Those spells became more and more prevalent, before one day his body just couldn’t come out of it.”
Thankfully, the pacemaker and ICD did prevent Gray from experiencing a fatal arrhythmia, but due to low cardiac output, he required extracorporeal membrane oxygenation (ECMO). He eventually had surgery to implant a ventricular assist device (VAD). “Neither were great solutions," Vincent shared. “They have their own potential side effects, but they were the stopgap we needed while waiting for a transplant.” Pearce said that Gray’s family participated in shared decision-making for all of these difficult steps.
While Gray was on the VAD, the family finally received the news from the heart transplant coordinators: A heart was available. Gray went into surgery just a couple days later, and the surgery was a success. “Then all of a sudden his blood pressure and arterial pressures weren’t where they should have been,” Vincent added. “His new heart was not acclimating to the pressures his old heart set up, which we were worried would lead to rejection of his new heart.”
Doctors made the decision to put Gray back on ECMO to support the transplanted heart, and this was continued for three days. They weaned him off ECMO after the pulmonary pressures in Gray’s heart had finally stabilized. He began physical and occupational therapies again and was soon able to leave the hospital and move into a nearby apartment with his family—which by then included his new baby sister, Skarlett.
After being discharged, clinic visits happened twice a week, before reducing to once a week, which allowed the family to finally go back home to Daphne. Gray is now down to biweekly visits, and the latest heart biopsy revealed his rejection level was zero. “He’s doing so much better,” Vincent said. “From the minute he wakes up and gets his medication, you have to do your best to keep up with him. He’s constantly moving and into everything he can be. We still have to be careful—he’s immune suppressed, so he can’t go quite everywhere—but he’s doing great. We couldn’t be more thankful for the care he received at Children’s. They took such great care of him and did everything possible to make sure he left there happy and healthy.”
Pearce said Gray’s case was a rare and difficult one and that the multidisciplinary approach at Children’s of Alabama’s Bruno Pediatric Heart Center was essential to his timely evaluation and survival. Pearce believes an ongoing care partnership with Gray’s family will help provide Gray with long term survival and an excellent quality of life.
