For Cleveland Clinic Children’s heart transplant program, 2014 was in many ways a year of two numbers:
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- 12: The number of heart transplants performed in patients with congenital heart disease — 10 of them in pediatric patients, the most in a single year at Cleveland Clinic. All patients were faring well as of spring 2015.
- 5: The number of ventricular-assist devices (VADs) placed in pediatric patients (two Berlin Heart Excor® and three HeartWare® devices), continuing the growth in pediatric VAD placement over the past several years.
But for Robert Stewart, MD, Surgical Director of Congenital Heart Transplantation, the year’s most noteworthy success may lie in another number related to the two above: 7, the number of patients who were able to be transplanted without need for bridge therapy with a VAD.
“Although VADs are a great technology and expertise in their use in children and adolescents is invaluable, they are nevertheless invasive and risky,” explains Dr. Stewart. “So it’s probably a bigger distinction to be able to successfully manage pediatric patients on the heart transplant waitlist without needing to resort to a VAD.”
Case study: H1N1 triggers rejection of a longtime graft
Consider the case of 12-year-old Maxine Frere, who received her second heart transplant at Cleveland Clinic Children’s in December 2014.
Maxine was born with a complex congenital heart defect — a double inlet left ventricle with coarctation of the aorta. She underwent several heart surgeries to treat the defect before ultimately receiving her first heart transplant at Cleveland Clinic Children’s as a toddler in 2005. Her new heart served her well for many years until she contracted the H1N1 flu virus in 2012 — a potentially life-threatening development for a transplant patient.
Maxine’s immunosuppressant therapy had to be reduced to fight the infection, and although she recovered from the H1N1 virus, she developed graft rejection, almost certainly due to the reduced immune suppression. The rejection was successfully treated by her longtime pediatric cardiologist, Gerard Boyle, MD, but it left her with transplant coronary artery disease, the leading threat to long-term survival after heart transplant.
Months of meticulous medical management
“In Maxine’s case it was primarily small vessel disease, which resulted in a restrictive cardiomyopathy,” says Dr. Boyle, Medical Director of Pediatric Heart Transplantation. “So we listed her for retransplantation and watched her carefully on an outpatient basis.”
Monitoring in early 2014 showed worsening of her cardiomyopathy and the start of dilation. “That signaled that we didn’t have much time,” says Dr. Boyle. He admitted her to the hospital in April 2014 and started her on a continuous milrinone infusion to improve her ejection fraction.
Thus began a hospital stay of more than 200 days until a donor heart could be found for Maxine’s second transplant. The milrinone clearly improved Maxine’s heart function and energy level for a while, allowing her to walk throughout Cleveland Clinic’s vast campus almost daily. Eventually her heart function worsened, requiring escalation of the milrinone to nearly the maximal dose. Further deterioration later required another dosage increase.
“I was concerned we would need to admit her to the ICU for breathing assistance and consider a VAD,” says Dr. Boyle, “but then a donor heart became available.”
Transplanted at last
Dr. Stewart led Maxine’s transplant surgery, which was complicated by extensive tissue scarring from her prior surgeries and the fact that her failing heart was densely stuck to the back of the sternal bone. This prompted placement of a bypass catheter in the left femoral artery to avoid catastrophic bleeding. Once the bypass was in place, the transplant was completed smoothly.
Maxine’s post-transplant course proceeded largely as expected, and she was faring well several months afterward, with no signs of rejection. “She’s back to being herself again,” says Dr. Boyle, who has cared for Maxine since her first transplant. (Dr. Boyle is shown with Maxine in the photo above, taken at a checkup visit four months after her December 2014 transplant.)
Growing retransplant trend: A good problem to have
As a retransplant case, Maxine reflects a growing trend in pediatric heart transplantation. “The indication for transplanting a heart increasingly is becoming retransplantation,” says Dr. Stewart, who notes that Cleveland Clinic Children’s has performed several second transplants and one third transplant to date.
The trend is largely a sign of success, he adds: “In the past, by the time the transplanted heart had become compromised by endothelial change from slow, chronic rejection over a decade or more, the patient was often so diseased in other ways that he or she couldn’t withstand retransplantation. Now we’re much better at keeping these patients well enough to be retransplanted — sometimes more than once.”
With VADs, judiciousness is key
Dr. Boyle says similar progress has been made in managing young patients while they wait for a donor organ. “It takes exceptional dedication and coordination among a large team to manage a patient like Maxine on an outpatient basis as long as we did,” he notes, “and then keep her free from infection for over 200 days with a continuous IV line. That comes from Cleveland Clinic Children’s 30 years of institutional experience performing pediatric heart transplants.”
While Dr. Boyle says his task is made easier by having an expert VAD team to fall back on when needed, Dr. Stewart is committed to using the devices judiciously. “Of course you don’t want to lose a patient because you didn’t put a VAD in them,” he says. “But if you can use fewer VADs and still keep everybody well enough to reach transplant, that’s strong medical care.”