“If you have a pediatric patient whose dysfunction is limited to one or two organs but is otherwise neurologically intact, never give up — even if that means taking risks that seem too big.” That’s the big lesson that pediatric cardiac surgeon Luca Vricella learned as he and his colleagues cared for Kamari, a 3-year-old boy born with hypoplastic right heart syndrome.

In the years following his birth, Kamari underwent two successive surgeries to support the function of his single cardiac ventricle. Then, in October 2016, his physicians at Johns Hopkins Children’s Center recognized the telltale signs of severe heart failure.

“We knew all along Kamari would need a heart transplant,” says Vricella, who directs pediatric cardiac surgery at Johns Hopkins Children’s Center. “The question was when — and how — would we get him there.”

With the scarcity of suitable donor hearts and the deteriorating condition of their young patient, Vricella and his colleagues decided to implant a ventricular assist device (VAD). However, they made an unusual move, selecting an intracorporeal adult VAD rather than the paracorporeal versions often used in children. 

Their reasons were twofold: First, the internal device would allow the boy to be more ambulatory, improving his rehabilitation and recovery leading up to transplant. Second, the internal VAD has a lower risk of thrombosis and therefore a lower risk of stroke.

Despite these benefits, the team’s choice posed some significant surgical challenges. “We had a patient who was failing — and who has very complicated physiology — and we were applying a device that is not only designed for adults but also for patients with two ventricles,” recalls Vricella.

Kamari was small for his age, at 14.2 kilograms and 38 inches, and he had significant scarring in his chest from past surgeries. To enable the device to fit and function properly, Vricella and his colleagues made some strategic concessions — removing part of the VAD, as well as a portion of the patient’s mitral valve.

In the end, the team was successful. Within a few days, their young patient was up and around — even riding a bicycle in the cardiac intensive care unit. Now, he stands as the smallest person on record to receive an internal VAD.

Even with this triumph, Kamari’s future remained uncertain. When would a donor heart become available? And would it be a suitable match?

Six months later, Vricella and his colleagues, including intensivist Kristen Nelson, pediatric cardiac surgeon Narutoshi Hibino and cardiologist William Ravekes, received notice of a compatible heart. Yet it wasn’t ideal. The donor had undergone CPR for a prolonged period and was on very high inotropic and respiratory support. Twenty-four other transplant centers had rejected the heart.

But the Johns Hopkins team saw a flicker of hope. Despite its highly resuscitated state, the donor heart had good function. “Ultimately, it was a team decision,” says Vricella. “We said to ourselves, ‘Why don’t we see where this heart goes over the next 24 hours?’”

Although Kamari was zipping around on his bike, the clock was ticking. The team had not received a suitable donor call for almost six months.

With Nelson’s dogged effort, painstakingly overseeing the care of the donor heart over the phone and weaning it from support, the Johns Hopkins team decided to accept the heart for transplant. They knew there would be some trade-offs, including managing Kamari postoperatively with extracorporeal membrane oxygenation (ECMO) to give the donor heart time to recover.

With a leap of faith, Hibino was dispatched to retrieve the heart several states away. To make things even more challenging, the procurement team’s jet was forced to make an emergency landing because of a leaking fuel tank.

In the end, the boy made a dramatic recovery after surgery. He did not bleed much from the surgical sites, despite being heparinized for the ECMO. Three days post-transplant, his heart function was normal, so the team took him back to the operating room, removed the ECMO and closed his chest.

Ultimately, the team’s big decision paid off. Kamari is now a healthy, energetic, 4-year-old, living at home with his six siblings.

“So many people came together for a single child,” says Vricella. “It was unbelievable. You get to save the world, one life at a time.”