Outcomes of Liver Transplantation for Hereditary Hemorrhagic Telangiectasia (HHT).

Abstract

Introduction: Hereditary Hemorrhagic Telangiectasia (HHT) can result in visceral arteriovenous malformations (AVM) including the liver. There is paucity of data on the characteristics/outcome of patients who undergo LTx for HHT with liver involvement. Methods: Two sources of data were utilized for this study: (1) UNOS database (95-2012). (2) Single center transplant database (Mayo Clinic Rochester). Aims: The aims of this study were (1) Define the frequency of liver transplantation in the United States for HHT with liver involvement (2) Determine the patient survival after LTx (3) Use single center data to evaluate more specific data on individual characteristics, indications, and outcomes for LTx in this population. Results: In the UNOS data set, 12 patients with HHT and liver involvement underwent LTx. Median age at transplant was 50 and the majority (83%) were female. Median MELD score at LTx was 8.5 with bilirubin, INR, and creatinine of 0.9, 1.1 and 0.9 respectively.5 patients had ascites at LTx, 2 (17%) slight and 3 (25%) moderate, while 2 (17%) patients had grade 1-2 and only 1 (8%) had grade 3-4 HE. Median waitlist time was 112 days (IQR 54-229). Two patients received LTX as status 1 and died at the time of transplant. The remaining 10 patients underwent successful LTx. Nine (75%) patients were alive. Median post LTx follow-up was 560 days (229-1109) while one patient was lost to follow-up. Using single center data at Mayo Clinic, five patients were identified who underwent LTx between years 2009-2013. Median age was 57 years. The patients were all female. The primary indication for LTx was high output cardiac failure in 4 patients and biliary necrosis in one patient. All patients were alive at the time of follow-up with good graft function. Heart failure resolved with transplant in all four cases. Conclusion:Outcomes following LTx for HHT with liver involvement are excellent with 82% survival in 12 patients in the UNOS database with median follow-up of 560 days. At a single center, the main indication for LTx in our 5 cases was high output heart failure which resolved after transplant. All five patients are alive with similar excellent outcomes.Table: No Caption available.