Results of Liver Retransplantation After Rescue Hepatectomy: A Single-Center Study.

BACKGROUND Liver retransplantation (reLT) is a well-accepted treatment for liver graft failure in selected patients. A rescue hepatectomy (RH), on the contrary, is a rare and controversial procedure in which a deteriorating liver graft causing failure of other organ systems is removed to stabilize the patient's condition before a new liver graft is available. MATERIAL AND METHODS In this retrospective cohort study, we evaluated the outcomes of the 104 patients who were listed for a first single-organ reLT in our center during the period 2000-2019, to compare the results after RH to other reLTs. RESULTS In the study population, RH was performed on 8 patients, while 7 of these received a new graft (8% of all first time reLTs) and 1 died before reLT. All RHs were performed within 1 week after the first transplantation. The median anhepatic time after RH was 36 hours (range 14-99). The 1-year patient survival rate was 57% for reLTs with RH and 69% for acute reLTs without RH that were performed within 14 days after the first transplantation (P=0.66). The 5-year survival rate was 50% in the RH and 47% in the non-RH group (P=1.0). CONCLUSIONS The use of RH prior to reLT results in a similar outcome to reLTs without RH. Therefore, RH should be considered in patients with a severe clinical instability caused by a deteriorating liver graft. However, further studies are needed to establish guidelines based on objective parameters for when RH should be performed.

Symptomatic Val122del mutated hereditary transthyretin amyloidosis: Need for early diagnosis and prioritization for heart and liver transplantation.

BACKGROUND: Hereditary transthyretin (ATTRv) amyloidosis is an autosomal dominant disease linked to transthyretin gene mutations which cause instability of the transthyretin tetramer. After dissociation and misfolding they reassemble as insoluble fibrils (i.e. amyloid). Apart from the common Val30Met mutation there is a very heterogeneous group of non-Val30Met mutations. In some cases, the clinical picture is dominated by a rapidly evolving restrictive and hypertrophic cardiomyopathy. METHODS: A case series of four liver recipients with the highly clinically relevant, rare and particularly aggressive Val122del mutation is presented. Medical and surgical therapeutic options, waiting list policy for ATTRv-amyloidosis, including the need for heart transplantation, and status of heart-liver transplantation are discussed. RESULTS: Three patients needed a staged (1 patient) or simultaneous (2 patients) heart-liver transplant due to rapidly progressing cardiac failure and/or neurologic disability. Domino liver transplantation was impossible in two due to fibrotic hepatic transformation caused by cardiomyopathy. After a follow-up ranging from 3.5 to 9.5 years, cardiac (allograft) function was maintained in all patients, but neuropathy progressed in three patients, one of whom died after 80 months. CONCLUSIONS: This is the first report in (liver) transplant literature about the rare Val122del ATTRv mutation. Due to its aggressiveness, symptomatic patients should be prioritized on the liver and, in cases with cardiomyopathy, heart waiting lists in order to avoid the irreversible neurological and cardiac damage that leads to a rapid lethal outcome.

Liver transplantation for acute liver failure - a 30-year single center experience.

OBJECTIVE: To determine overall long-term patient and graft survival rates among the recipients liver transplanted due to acute liver failure (ALF). Secondary aims included assessment of whether diagnosis, donor-recipient blood group compatibility and time-era of transplantation affected the outcome, and whether prescription-free availability of acetaminophen increased the need for liver transplantation (LTx). MATERIALS AND METHODS: A Retrospective cohort study of 78 patients who underwent LTx for ALF at Karolinska University Hospital 1984-2014. Patients were divided into two cohorts according to two 15-year periods: early cohort transplanted 1984-1999 (n = 40) and late cohort transplanted 2000-2014 (n = 38). Survival rates were established using Kaplan-Meier analyses. RESULTS: ALF patient survival rates for 1-year, 5-years, 10-years and 20-years were 71%, 63%, 52% and 40%, respectively. Survival for the late cohort at 1, 5 and 10 years was 82%, 76% and 71%, respectively. A high early mortality rate was noted during the first three months after transplantation when compared to LTx patients with chronic disease. Long-term survival rates were comparable between patients with ALF and chronic liver disease. Prescription-free access to acetaminophen did not increase the need for LTx. There was a strong trend towards improved survival in blood group identical donor-recipient pairs and blood group O recipients may have benefitted from this. CONCLUSIONS: The high early mortality rate most likely reflects the critical pre-transplant condition in these patients and the urgent need to sometimes accept a marginal donor liver. Long-term survival improved significantly over time and variation in patient access to acetaminophen did not influence the rate of LTx in our region.