Innovations in Transplantation: UPMC Performs Heart-Liver Transplant

By David J. Kaczorowski, MD

Heart failure and hepatic dysfunction are intimately linked. When a combined heart-liver transplant is necessary, it is a remarkable undertaking requiring an extensive, multidisciplinary effort. Combined heart-liver transplantation is relatively rare, but in 2022, UPMC Transplant Services performed several heart-liver transplants at UPMC Presbyterian and UPMC Children’s Hospital of Pittsburgh.

Coexisting heart failure and advanced liver disease can be caused by systemic disorders and diseases that affect both organs or by cardio-hepatic interactions that can lead to failure of both organs. For example, acute heart failure decreases blood flow to the liver and triggers acute liver injury (cardiogenic ischemic hepatitis) in 20% to 30% of patients. Congestive hepatopathy develops in 15% to 65% of patients with severe heart failure due to impaired hepatic venous outflow secondary to right-sided heart failure. Liver disease can cause systolic dysfunction, impaired diastolic relaxation, and electrophysiological disturbances in the heart. Cirrhotic cardiomyopathy may be present in up to 50% of patients with cirrhosis. One mechanism for this is believed to be vasodilators produced in response to portal hypotension and other changes leading to myocardial hypo responsiveness and impaired ventricular ejection under stress, such as exercise.

Advances in the surgical treatment of congenital heart disease (CHD) in infants and children have also increased the prevalence of coexisting heart failure and advanced liver disease when they reach adulthood. These groundbreaking treatments can have long-term sequelae. These patients, particularly individuals with single-ventricle physiology corrected using a Fontan procedure, can develop heart failure and accompanying cirrhosis as they age. Over the last decade (2011–2020), approximately 30% of patients who underwent combined heart-liver transplant in the United States had CHD. As this population increases across the United States, UPMC is expanding care through a robust CHD program at UPMC Children’s Hospital of Pittsburgh. Patients who have received specialty care at UPMC since they were children must now be cared for in the context of long-term CHD and the natural course of the surgical corrections that, fortunately, have allowed us to care for them as adults.

Heart-liver transplants require organs from a single donor. The experts in the UPMC Department of Cardiothoracic Surgery Division of Adult Cardiac Surgery collaborate with Christopher Hughes, MD, surgical director of the UPMC Liver Transplant Program, and his team to care for these patients. Both the heart transplant committee and the liver transplant committee need to give their approval before listing patients for combined heart-liver transplantation. The two teams review every donor offer together. When considering organ donors, we use standard donation criteria, rather than extended criteria, and we aim to get the best donor organs possible for each patient. For these complex transplants, we have maintained the donated organs using normothermic extracorporeal perfusion prior to transplant.

In most cases, the heart is transplanted first and then the liver. This limits the length of time the patient spends on cardiopulmonary bypass and allows reversal of anticoagulation measures when the patient comes off cardiopulmonary bypass. The transplanted heart graft must immediately begin functioning well to support the patient while the liver transplant is being performed, and the surgeons must confirm this before moving forward with the second organ. From 1989 to 2010, sequential heart-then-liver transplant, which we have used in our patients, was performed almost exclusively, and over the last decade, 80% of heart-liver transplants have been sequential heart-first. A liver-before-heart approach, which may be advantageous in carefully selected patients with all sensitization, has been used in 14% of patients over the last decade, and a simultaneous approach has been used in 6%.

A recent review of combined heart-liver transplants using the United Network for Organ Sharing (UNOS) database found that only 369 adults in the United States received a combined heart-liver transplant from December 1989 through August 2020. In comparison, almost 3,000 adults in the United States received a heart transplant in 2022. When assessed by decade, the most common cardiac diagnosis of patients undergoing combined heart-liver transplant was restrictive or infiltrative cardiomyopathy from 1990 through 2010, but CHD was most common from 2011 to 2020. Extracorporeal organ perfusion prior to transplant has only been used in the most recent decade.

Survival outcomes after combined heart-liver transplant have been good, and survival after combined heart-liver transplant in carefully selected patients is similar to survival after heart or liver transplant alone. In the 369-patient cohort from the UNOS database, 1-year cumulative survival was 87%, 3-year survival was 80%, and 5-year survival was 78%. When 3-year and 5-year survival were examined conditional on 1-year survival, more than 90% survival was observed at both time points. Not surprisingly, the patients who underwent a combined transplant most recently (2011–2020) had a lower risk of mortality than the earliest transplanted patients (1989–2000). There is some evidence that heart-liver transplant results in less rejection and better survival than isolated heart transplant in patients with repaired CHD.

The first combined heart-liver transplant was performed at UPMC Children’s in 1984. Thomas Starzl, MD, the pioneering, world-renowned surgeon who is considered the father of modern transplantation, performed the procedure on a 6-year-old girl from Texas named Stormie Jones. UPMC transplant surgeons did 19 heart-liver transplants prior to 2022, with the last one in 2019. Continuing this tradition is particularly satisfying. At UPMC, we collaborate to do dual-organ transplants including combined heartkidney and heart-lung transplants in addition to heart-liver transplant. Appropriate patient and donor selection, coupled with careful collaborative surgical planning, have yielded excellent outcomes at UPMC.