Novel Therapy Resolves Complications for Teen with Liver Transplant
A new intervention developed at the Cincinnati Children’s Heart Institute has resolved the problems of a teenager who was suffering from liver transplant complications. The transcatheter relief of hepatic venous outflow obstruction was so successful that the team has gone on to perform the procedure in other patients as well.
Leah’s Story
Leah is a teenager with cystic fibrosis who developed multiple complications of that diagnosis, including liver failure. She underwent liver transplant in 2015 but was extremely sick in the post-op period, requiring multiple admissions and multiple procedures for chronic and persistent ascites.
In late 2015 at age 14, Leah was referred to the Heart Institute’s Cardiac Catheterization Laboratory to rule out pulmonary hypertension or diastolic dysfunction as cardiac causes of her liver dysfunction. Pediatric interventional cardiologist Bryan H. Goldstein, MD, found that she didn’t have those problems, but she did have severe obstruction of her inferior vena cava and hepatic vein. This caused Leah’s liver to be massively congested and explained the chronic ascites, which was the source of her need for repeated hospitalization, drainage and overall difficulty following liver transplantation.
Creating a Solution
Once the problem was found, Goldstein convened a multidisciplinary meeting—with Interventional Radiology, Hepatology, Hematology and Liver Transplant Surgery—in which discussion resulted in the group’s willingness to try a novel therapy with complex vascular stent placement in the cath lab. He led the team in placing a vascular stent in Leah’s inferior vena cava. The stent was then customized in vivo so the hepatic vein did not have to cross stent material, thus reducing the risk of clot formation. Within days Leah had lost nearly 20 kg of abdominal fluid. Two years later she remains asymptomatic.
“All of Leah’s problems resolved entirely over the next few weeks. She has not had any recurrence or issues since,” says Goldstein, who is associate director of the Cardiac Catheterization and Intervention service. “The success of this intervention is exciting because it’s allowed us to create a program to address this problem in other patients.”
More Patients Benefiting
Since Leah’s case, the Heart Institute and Liver Care Center have worked together to develop a protocol they use for cases of hepatic venous outflow obstruction. This has led to similar modified stent placements in six other patients with this problem who previously didn’t have a good option available. The youngest patient treated to date was 1 year and 1 month of age; the oldest was 16.
The Journal of Pediatrics published the full case description in January 2017. Goldstein presented the work of the larger cohort at the International Pediatric Transplant Association meeting in Barcelona last spring, and the team is currently working on another manuscript covering the multiyear outcomes of the larger cohort.
The conversation that started all of this has led to further collaboration among these disciplines, a group effort that all are eager to be involved in. “We have developed relationships with other specialists, which leads to new ideas and new collaborations,” Goldstein explains. “Now all of us are meeting and exchanging information. We’re looking forward to finding out what else we can do together.”
