Patients with Fontan circulation are at elevated risk for liver complications, particularly congestive hepatopathy. Transplantation can help patients with severe disease, but what should be transplanted? Heart? Liver? Both? Neither?
According to John C. Magee, MD, Professor of Surgery and Director of the Transplant Center at the University of Michigan, the short answer is “it depends.”
“A high central venous pressure is the death of livers,” he said. “It is a sign that the right ventricle has given up. A liver transplant alone isn’t likely to help the patient long term.”
Dr. Magee discussed transplant options and decision making for patients with Fontan associated liver disease (FALD) during the Pediatric Liver Disease Special Interest Group session on Friday.
The longer answer, he said, is that the optimal use of heart transplant versus liver transplant in FALD is unsettled. Patients with FALD should be evaluated by the liver transplant team, but not every patient with FALD needs a new liver. More and longer term data are needed, especially in patients receiving a heart transplant only.
“The reality is that the heart is the basic problem,” Dr. Magee said. “Fixing the heart may make the liver better and, at the very least, should not make additional liver complications.”
The liver will not be happy with a heart transplant, he continued, but the insult is relatively minor. Complications such as delay in getting off bypass or the need for extracorporeal membrane oxygenation are not likely to lead to additional liver damage.
The basic question is whether the patient actually needs a new liver. First impressions are a strong indicator, Dr. Magee noted. Patients who don’t look like they need a liver transplant probably don’t. Lab tests and liver biopsy are unlikely to help because impaired liver function is a given and cirrhosis is expected.
Hepatic venous pressure gradient (HVPG) measurement is similarly unhelpful.
“What HVPG tells me is that the heart is bad and the patient has portal hypertension,” Dr. Magee said. “Those are already known factors. I would fall back on the clinical scenario.”
Patients with refractory ascites versus uncomplicated ascites are more likely to benefit from a heart/liver transplant, he added. A history of bleeding varices also suggests a combined transplant.
Portal vein Doppler may be the most helpful test. Hepatopetal flow is a good sign, Dr. Magee noted, while hepatofugal flow is a problem.
Liver size is the clearest clinical sign pointing to liver transplantation. Loss of significant hepatic volume is a clear, long-term problem, Dr. Magee said.
If the patient is to receive a heart/liver transplant, is it better to perform an en block procedure or sequential transplants? Dr. Magee suggested sequential is more practical.
“Even the biggest OR only has room for one attending surgeon,” he said. “Two surgeons, one for heart, one for liver, just get in each other’s way.”
Transplanting the heart first allows hemodynamics to stabilize without having to worry about the potential impact on a new liver graft. And optimal post heart transplant management may affect the perceived need for liver transplantation.