Children’s Hospital Colorado Performs Its First Pediatric Heart-Liver Transplant

More than two years ago, the cardiology and hepatology teams at Children’s Hospital Colorado began planning for a surgery they had never completed before. In fact, only roughly 30 pediatric dual heart-liver surgeries had been performed across the entire country. That careful preparation would ultimately be lifesaving for Gracie, an 11-year-old with single ventricle heart disease, whose dual transplant was performed earlier this year.

Gracie was born with her condition and has been in the care of Children’s Colorado experts, including pediatric hepatologist Dania Brigham, MD, and pediatric cardiologist Kathleen Simpson, MD, for years. Children with Gracie’s condition, in which the heart only has one functional ventricle, typically have three procedures (Norwood, Glenn and Fontan) to improve their condition. These surgeries are generally completed by the time a child reaches age 5. Because Gracie’s condition was accompanied by lymphatic complications, Children’s Colorado experts referred her to specialists at Children’s Hospital of Philadelphia for some of her surgical procedures. Throughout her medical journey, she has been cared for through the multidisciplinary Single Ventricle Care Program at Children’s Colorado.

Because care for Gracie’s condition is not curative, the team knew Gracie would one day need a heart transplant. And while the strain that abnormal heart anatomy puts on the rest of the body’s organs often causes patients some degree of liver damage, Gracie’s liver was more impacted than most children. For some kids, a heart transplant alone helps them improve enough that the liver can recover on its own. But in Gracie’s case, previous surgeries impacted her liver so greatly that it would need to be replaced as well — and soon.

This year, after much discussion, the team convened with Gracie’s family and decided it was time to put its years of preparation for a dual transplant into action. Because research shows that getting both grafts from the same donor yields better outcomes and a lower chance of graft rejection, Gracie was placed on the organ donor waiting list in the hopes of getting a heart and liver from the same donor.

Planning a complex surgery

The first step — even before organs became available — was to bring together every person responsible for completing the surgery and managing postoperative care.

“There's not a lot of time when you have an organ available, and it's not predictable,” Dr. Simpson says. “So we really had to have most of it planned beforehand, and then we had a final meeting to make sure everyone was on the same page prior to the actual OR time.”

That final meeting saw dozens of providers pushing the capacity limits of a conference room with just as many joining virtually. They planned each moment of surgery, from keeping the donor organs healthy until transplant to teasing out and creating contingency plans for every single possible complication.

The first major decision was whether to transplant both organs simultaneously or to focus on the heart first. Because of its shorter ischemic time and Gracie’s cardiac complexity, the team decided to start with the heart and complete the liver transplant after. To ensure the donor liver remained in the best possible condition during the heart operation, Children’s Colorado acquired a TransMedics Organ Care System which mimics the conditions of the human body to keep a donor organ in good condition far longer than usual. The pump allowed the cardiac surgery team to complete its work without the pressure of a ticking clock.

“It does everything that the body would do,” Dr. Brigham says. “It has a hookup for the venous flow, the arterial flow and for the bile — it was still making bile while it was on this pump. It gets nutrition filtered in so that it keeps a good electrolyte balance, and it essentially keeps the liver alive and functioning like it was in someone’s body.”

As a group, the team considered the many different elements that would need to come together both before and after the surgery to create the best possible outcomes for Gracie. What operating room would they use? Should the team use extracorporeal membrane oxygenation (ECMO) during the surgery? What level of anti- and pro-coagulants should doctors employ? How could anesthesiologists monitor volume loads, and how can everyone manage dialysis and electrolytes? Where would Gracie recover? What nutritional considerations needed to be taken into account? How could the team balance optimizing aftercare for both organs given their different support needs?

Dr. Brigham credits this level of detail and preparation to the surgery’s success.

“Because we had so much preparation leading up to it, I feel like everyone really knew exactly how it was going to roll out,” she says. “And then it just went beautifully.”

A successful operation

On the day of the surgery, the team readied a cardiac OR, which everyone felt would be best equipped for managing possible surgical complications. Matthew Stone, MD and Emily Downs, MD, were the cardiac surgeons performing the heart transplant. Previous MRIs and CT scans had given them a clear look into Gracie’s particular anatomy, allowing for a smooth operation.

After the heart was successfully transplanted, the team took her off bypass and used ECMO to support Gracie during the liver portion of the surgery.

“The goal of using ECMO was to minimize the stress on the heart first off, and secondly, to support her physiology throughout, particularly with a liver transplant,” Dr. Stone explains. “It's very common for there to be electrolyte derangements and changes in the acid-base status of the patient, even after a heart reperfusion, but with a dual organ, that is further exacerbated. This gave us the opportunity also to dialyze her so that we could optimize her electrolytes during the reperfusion period.”

From there, the liver transplant team took over, led by Megan Adams, MD. The most complex part of Dr. Adams’ job was removing Gracie’s liver and preparing for a new one. A previous surgery had required the use of an occlusive glue that, over time, made its way to Gracie’s portal vein and created a clot. Dr. Adams and Kendra Conzen, MD, had the difficult job of removing every bit of the glue, before successfully transplanting the donor liver.

“Having her on ECMO allowed us to be able to clamp vessels to get the dissection done and then to hook it back up,” Dr. Adams says. “Once we got the old liver out, everything was straightforward. All the vessels were fine, and it was very smooth.”

Once her liver was reperfused, Gracie was taken to the cardiac intensive care unit (CICU) for recovery. In total, the operation lasted 17 hours.

Recovery and beyond

During recovery in the CICU, the team continued to monitor Gracie from every perspective, carefully considering the differing needs of the heart and liver.

“That's where the organs need two totally different environments, and so we had to make very calculated moves every day as to what we thought she could tolerate and what each perspective graft could tolerate,” Dr. Adams says.

For example, she explains, the liver prefers a dehydrated environment during resuscitation and after a liver transplant, the care team generally uses anticoagulation to help the liver recover. However, hearts prefer a less dry environment, and anticoagulation can create new risks.

This required close, constant communication on what little tweaks could be made to optimize healing for both grafts.

“Fortunately, the post-operative management was pretty straightforward because we had two grafts that functioned really well,” Dr. Stone adds. “When one of the grafts isn't functioning as well, that's where you do have competing interests and it becomes very challenging. But she came out on minimal support.”

Gracie was discharged from Children’s Colorado after just one month and was able to get home to her school, friends and beloved dogs. Though she has required frequent clinic appointments and ongoing care, Gracie has been healing better than the team expected.

And while the team anticipates that she will need another heart transplant later in life due to her age, Gracie’s case has still laid the groundwork for other kids who may benefit from the same procedure in the future. The team says the surgery has opened the door to more collaboration, and other patients are already being evaluated for a potential dual transplant.

“Even though it was a lot of work for us to be able to combine our protocols and combine the way we do things, we were able to do this because everyone was so experienced and open, and we have such strong programs,” Dr. Brigham says.

Ultimately though, advancements like this one are only possible when care teams come together with families and build relationships based on trust and expertise. Experts like Dr. Stone note that in cases like these, providers are often walking alongside families on a difficult journey. 

“I think the trust families put in us is a big thing that we don't take lightly,” Dr. Stone says. “For her to undergo such a huge operation was a testament to their trust in us. Through this, we hope we can establish that trust with other patients too.”