When Laura Jones was 33 weeks pregnant, her belly swelled to three times the normal size.
An ultrasound showed what appeared to be excess fluid around her baby’s lungs. A week later, an echocardiogram done in utero found the baby had critical aortic stenosis. Because of this, the baby wasn’t processing the amniotic fluid, which in turn caused the fluid to build up in Laura.
Laura was sent from her home in Albany, Georgia, to an Atlanta hospital where she gave birth at 38 weeks to baby boy named Bennett. He weighed just 5 pounds, 2 ounces and was already in respiratory distress.
Bennett was quickly transferred to an Atlanta children’s hospital. The team there performed a balloon catheterization on Bennett’s aortic valve to buy him some time because he was too small for surgery or any other procedure.
By 2 months old, Bennett was in heart failure and had his first open heart surgery, a pulmonary autograft, also known as the Ross procedure. This surgery replaces the aortic valve and part of the aorta. The child's own pulmonary valve and part of the pulmonary artery are used to replace the damaged aortic valve. A pulmonary valve and part of the pulmonary artery from a donor organ are used to replace the transplanted valve and artery. The aortic valve grows with the child, but the donor pulmonary valve doesn’t.
At age 4, Bennett needed a second open heart surgery to repair his mitral valve. For the next two years, Bennett enjoyed an active life. “He played T-ball and was non-stop running,” says his mom, Laura. “It was the best couple of years of his whole life.”
A Turning Point Toward Transplant
In December 2017, when Bennett was 6, his echocardiograms began changing. Doctors were concerned that the right side of Bennett’s heart was enlarged. A catheterization showed his pulmonary pressure was triple what it should be. The family was advised to wait it out.
Two months later, on Super Bowl Sunday 2018, Bennett went into cardiac arrest at home. “We thought he had pneumonia but the antibiotics weren’t working,” Laura says. “He seemed to be compensating well. We later learned heart failure can mimic pneumonia. One minute he was sitting up, the next minute he felt dizzy and laid down. I knew something wasn’t right. I rolled him over and he was blue and had no pulse.”
Laura’s husband, Todd, began chest compressions, which briefly brought back a pulse. Bennett was whisked away by ambulance and then transferred to Atlanta where he was first evaluated for a heart transplant.
Because of the many blood products Bennett had received over the years, his antibodies were high, which made him an unsuitable transplant candidate. He took desensitizing medications for a month to lower the antibodies but they were still too high to qualify to be a transplant candidate at the Georgia hospital.
“I was not stopping,” Laura says. “I knew if I didn’t pursue every avenue, I would regret it and couldn’t live with myself. I knew transplant is a huge surgery but there was no alternative.”
Fighting for Hope
Laura sent Bennett’s records to other transplant centers, including St. Louis Children’s Hospital. In October 2018, Bennett came to St. Louis for a transplant evaluation.
Janet Scheel, MD, a Washington University pediatric cardiologist who specializes in heart failure and heart transplantation, saw Bennett soon after his cardiac catheterization as he was being evaluated. “He was quite sick and fragile after his cardiac catheterization,” she says. “He had a mighty big hill to climb. His pulmonary pressure was high and he was highly sensitized. He was rapidly adding challenges as we went along. But we’re a big transplant center and have seen a lot and done a lot of transplants. About 40 percent of our transplant patients have been turned down elsewhere. We push the bar if we can safely do so. It’s about being willing to try.”
That willingness to try was the life raft Laura was looking for. “When we came to our evaluation, everyone was advocating for Bennett,” she says. She is especially grateful to Pirooz Eghtesady, MD, PhD, a Washington University pediatric cardiothoracic surgeon at St. Louis Children’s Hospital.
“I can’t thank Dr. Eghtesady enough. He opened my eyes and made me see that Bennett was very sick and in severe heart failure. After pushing Dr. Eghtesady, he told me Bennett may only have two months to live as he was. He gave us two options: take Bennett home and provide comfort care or try a heart assist device as a bridge to transplant. I was not going home and doing nothing. Dr. Eghtesady said he was willing to fight if we were. He gave us hope.”
Bridge to Transplant Leads to Better Quality of Life
Modifying the way a left ventricular assist device (LVAD) is typically implanted, Dr. Eghtesady placed the device in Bennett’s left atria to lower his pulmonary pressure and improve heart function. This unique configuration of implanting the device in the upper chamber is rarely done.
The mechanical device also allows Bennett to be mobile again. He carries the external equipment in a backpack.
After one month on the assist device, Bennett’s pulmonary pressures were normal. Six weeks after being on the device, Bennett was running and riding his bike, Laura says. “Before he would be exhausted just walking to the car and had to sleep for hours. Now he has energy, an appetite and has started growing and gaining weight. He never really knew what it felt like to feel good until now.”
“Bennett is an extraordinary kid with an amazing, supportive family,” Dr. Scheel says. “He is a trouper and tries hard. Our concern now is to get an organ in time with the right match. The assist device can work a long time but it’s not in the typical position and Bennett is growing.”
Part of Bennett’s preparation for transplant included having plasma pheresis and a drug to lower his antibodies. With his lower antibodies, he can now accept a donor heart from 100 percent of the population. Since January 2019, Bennett has been 1A status—at the top of the transplant list.
“We were told the average wait time for a heart is six to nine months so we’re waiting in St. Louis,” Laura says. “We’re excited about the transplant but nervous. We live each day and try not to think about it. We’re so thankful to everyone at St. Louis Children’s Hospital for believing in Bennett when no one else did.”