Newswise — Patients with a form of congenital heart disease — having only one ventricle (pumping chamber) — are now living longer lives due to the successful surgical and medical treatments they receive as children.
However, as these patients survive to adulthood some of them face a new problem — heart failure, for which they will require heart transplantation.
Transplant in these adults with congenital heart disease (CHD), which remains rare, is more frequently required and becoming more common at Vanderbilt University Medical Center, the only location in Tennessee and one of a few in the South where such transplants are possible, said Jonathan Menachem, MD, a transplant cardiologist and director of Advanced Congenital Cardiac Therapies (ACCT) at Vanderbilt Health.
Menachem, assistant professor of Medicine and Pediatrics, said four single-ventricle patients who have been palliated with a Fontan procedure have been transplanted in the last 12 months, out of a total of nine transplant recipients with congenital heart disease. Fontan is the name of the procedure given to pediatric patients with one ventricle.
Menachem points to two remarkable patient outcomes in the last six months involving Fontan patients. In January, Shawn Land of Clarksville, Tennessee, a Fontan patient with liver cirrhosis (a common complication in these patients), successfully underwent a combined heart/liver transplant. Then, in October 2018, Deja Baynard-Watson of Delaware became Vanderbilt’s first documented case of using a heart from a hepatitis C-positive donor in a patient with CHD.
“These two cases demonstrate the ability of Vanderbilt to take the sickest single-ventricle patients and successfully transplant them using innovative techniques,” Menachem said. “A lot of these patients and also providers don’t realize that transplantation is an option.”
Vanderbilt Health has the second largest heart transplant program by volume nationwide.
“As one of the largest heart transplant programs in the country, we need to leverage our true strengths: an uncommon depth of experience and teams that can work collaboratively when faced with difficult problems,” said Ashish Shah, MD, professor and chair of Cardiac Surgery.
A normal heart has two pumping chambers, called ventricles, said David Bichell, MD, William S. Stoney Jr. professor of Cardiac and Thoracic Surgery, professor of Clinical Cardiac Surgery and chief of Pediatric Cardiac Surgery.
“Patients with only a single working ventricle can suffer progressively inefficient circulation, resulting in fatigue,” he said. “The condition can be fatal if untreated. For some, additional surgeries can improve the situation, but not completely repair it.”
Single ventricle heart defects occur in about five out of 100,000 live births, according to the Adult Congenital Heart Association.
With more advanced treatment, these patients are living into adulthood, having jobs, families and children of their own and are becoming candidates for heart transplants in increasing numbers.
One of them is Land, who was born in 1989 with hypoplastic left heart syndrome. He had three open heart surgeries as a young child.
“They only gave me to age 9 or 10 for survival time,” he said. “I’ve lived to be 29 years old.”
In June 2018, Land found himself on the transplant list at Vanderbilt. On Nov. 6, 2018, he was in for “just a normal checkup” when doctors noticed his liver function was greatly deteriorating. He checked into the hospital that day and waited for a matching heart and liver to become available. His double transplant occurred on Jan. 17.
He says he feels much better after the transplant. “I’m glad that technology has come so far,” he said.
Land’s mother, Teresa, said her son is motivated to fight. He now has a son of his own, Mason, 7. And he fights in memory of his brother Dakota, who died in 2013 with cystic fibrosis at age 17.
“To have this second chance to extend his life is just so amazing to us,” she said. “We’re very, very happy with the outcome and really looking forward to a happy, healthy future. “It’s going to be exciting to see the things he can do now."
Baynard-Watson, 38, remembers two surgeries when she was 8 years old. Then, as a young adult, she passed out one day when she was walking from her home to her high school to pick up some papers for college. She said a woman revived her and called 911. “From that point on I kept passing out,” she said.
After several years of procedures, including receiving a pacemaker and a defibrillator, Baynard-Watson ended up on a transplant list at another hospital in Pennsylvania. Deemed too risky to transplant, she was placed in hospice care.
But Baynard-Watson wasn’t ready to give up, and she came to Vanderbilt. “I was really glad that they accepted my case because I didn’t know where I was going to go,” she said.
Her initial course at Vanderbilt was quite bumpy as she nearly died on multiple occasions, Menachem said. However, due to her internal strength and the collaborative team effort to get her to transplant, she was able to undergo transplantation on Oct. 25.
Now she is looking forward to returning home, where she loves being an aunt to her brother’s three children. “I feel back to normal,” she said. “I feel energized and I’m just ready to get back to going back to work.”
Menachem said that as recently as 20 years ago, a 38-year-old single-ventricle patient like Baynard-Watson would be unexpected. But not today. “It’s because they’ve done so well as kids,” he said. “They’re surviving these surgeries, but now they’re having heart failure and needing transplants.”
Vanderbilt has the right elements in place for excellent outcomes for such patients, Menachem said. “A dedicated and collaborative multi-disciplinary team is the key to success,” he said. “In order to transplant these highly complicated and risky patients, you have to have a high-volume transplant program, adult congenital program, and pediatric and adult surgical teams, amongst others. Furthermore, as in the case of Land, there is a need for a dual-organ transplant program as well. It takes a lot of pieces at the institution to make it go well.”
Menachem’s team recently published data in the Journal of Heart and Lung Transplantation that shows that adults with CHD benefit from undergoing transplantation at high-volume centers such as Vanderbilt.
Patients who survive the first year after their transplant have outcomes equivalent, if not better than, non-congenital patients, he said.
“We have a group in place that is focused on taking care of these patients, and while it’s not perfect, we continue to grow and improve due to the dedication of our team and the needs of our patients,” Menachem said. “And with the growth in volume, we expect that our outcomes will continue to improve so that we can positively impact the lives of these patients.”