by Matt Batcheldor
When Liz Barnett was born in 1980 with congenital heart disease (CHD), her medical team initially thought she didn’t have much of a chance at a normal life.
“My mom was told … at birth to go home and have more kids to keep her company when I died,” Barnett said, “that there was no hope for me.”
Barnett found hope. As a child, she received a Fontan procedure, allowing her to live with a single ventricle, or pumping chamber in her heart, instead of the usual two. And in July, she became one of seven people at Vanderbilt University Medical Center to receive a combined heart/liver transplant in 2021. VUMC performed more combined heart/liver transplants in 2021 than any other center in the United States.
A generation ago, patients with CHD didn’t survive until adulthood. Due to advances in care, they now do, and they increasingly need heart transplants to continue to survive. Many such patients also need a liver transplant because the Fontan procedure places pressure on the liver, wearing it out faster.
Vanderbilt is one of only a few centers in the United States that have developed a multidisciplinary team that specializes in transplanting adults with CHD, and is increasingly becoming a destination for the rare dual heart/liver transplant, said Jonathan Menachem, MD, a transplant cardiologist and director of Advanced Congenital Cardiac Therapies (ACCT) at Vanderbilt Health. Most of Vanderbilt’s heart-liver transplants now go to CHD patients.
“In 2017, we made a decision that we were going to focus on transplanting more adults with congenital heart disease who were dying of heart failure,” Menachem said.
Between 2012 and 2017, VUMC had transplanted two patients with CHD, Menachem said. Since that time, 27 congenital patients have been transplanted, and five patients have undergone placement of a left ventricular assist device (LVAD).
Performing heart transplants on CHD patients is more complex than typical transplant patients, because such patients have already undergone multiple heart procedures. Add an additional major surgery to transplant a liver, and it’s a procedure that few centers have the expertise to undertake.
“These procedures require no less than 40 people to perform and can take as long as 16 hours to complete,” said Ashish Shah, MD, professor and chair of Cardiac Surgery. “They are technically complex, physically exhausting operations that demand a unique level of teamwork. They bring out the best in our operating room and ICU teams. Which is why we do it.”
To do so requires a vast, multidisciplinary care team. Vanderbilt has a team-based approach to evaluating transplant patients. Its pediatric and adult surgical teams work closely together, blending their expertise.
“This program works because of individuals and teams with singular expertise coming together in a unique, patient-centered manner. This allows VUMC to provide care possible at only a few centers in the world,” said Seth Karp, MD, H. William Scott Jr. Professor, chair of the Section of Surgical Sciences and director of the Vanderbilt Transplant Center.
For patients like Barnett and several others, Vanderbilt was a last resort when other centers couldn’t take care of their complex needs. Barnett had lived into her 40s with a mediocre quality of life, but her heart and liver were failing fast.
She recounted her local doctor “looked at me very clearly and said, ‘we’re never going to transplant you, you’re too complicated, we haven’t done Fontans, we haven’t done a combined heart/liver, you need to go to Vanderbilt. And don’t wait; go as soon as you can.’”
By the time Barnett came to Vanderbilt in January 2021, she was much sicker than she realized. She was approved for the transplant list not even a full week later, and five months after that she was transplanted.
Menachem is hoping that awareness of Vanderbilt’s growing reputation as a destination for congenital heart transplant will prompt other centers to refer patients here sooner, before patients are seriously ill.
Barnett was discharched from VUMC in August 2021 with a new heart and liver, having done things she never had before, like climbing three flights of stairs before she even left the hospital. She said she never realized what having a normal heart was like.
“I feel amazing,” she said. “I bought an exercise bike. I have plans to go hiking. I live in Missouri now and there’s all kinds of parks and stuff around here that I’ve always wanted to go to and explore, and I can do that now. I was a newlywed; my husband and I will celebrate two years at the end of February. So now I actually have the hope and the possibility of growing old with my husband.
“I’m honored to be on the receiving end of a gift like this.”
