Mortality of young biliary atresia patients listed for liver trans­plan­ta­tion: results from the Eurotransplant registry

H.P.J. van der Doef, P.F. van Rheenen, M. van Rosmalen, X. Rogiers, H.J. Verkade

Chair(s): dr. Marion J. Siebelink, programmamanager Transplantatie Centrum, UMC Groningen & dr. Raechel J. Toorop, transplantatiechirurg, UMC Utrecht

Thursday 10 march 2016

9:50 - 10:00h at Zaal 14

Categories: Parallelsessie (donatie)

Parallel session: Parallelsessie IX - Donatie en Allocatie

Liver transplantation has become the standard treatment for children with biliary atresia (BA) who develop end stage liver disease despite Kasai portoenterostomy. Up to 50% of BA patients need a liver transplantation before the age of 5 years. The prognosis after transplantation has steadily improved, but pre-transplant mortality is also important for the overall prognosis of BA patients. The primary objectives of this study were to assess the magnitude of pre-transplant (in particular, waiting list) mortality and to identify possible risk factors.

We retrospectively studied 642 patients with BA aged <5 years listed for liver transplantation in the Eurotransplant region between 2001 and 2014, and with a waiting list duration of < 1 year. In a subcohort of 365 children (84% from the period 2007-2014) we evaluated the association of pretransplant variables [dichotomous variables: age at listing ( 0.5 years), blood group, gender, MELD score ( 20) and renal replacement therapy; continuous variables: albumin, bilirubin, creatinin, and international normalized ratio (INR)] with waiting list mortality by Cox regression analysis.

The waiting list mortality was 4.5%, 7.5% and 8.4%, at 3, 6 and 12 months after listing for liver transplantation, respectively. Age at listing below 0.5 years [n=141, P=0.001, hazard ratio (HR) 4.1, 95% confidence interval (CI) 1.8-9.0] and MELD score above 20 (n=131, P<0.001, HR 10.2, 95% CI 4.4-23.8) were independently associated with the waiting list mortality. Other factors were not significantly associated. In 36 patients (9.9%) both risk factors were present, which coincided with waiting list mortality of 25% already at 6 months. This subgroup accounted for 32% of all pretransplant mortality in this cohort.

Waiting list mortality of BA patients below 5 years is 8.4%, based on a large cohort in the Eurotransplant region. Age at listing below 0.5 years and a MELD at listing above 20 are associated with a strongly increased waiting list mortality. Identification of risk factors for waiting list mortality could be helpful for optimizing the allocation of donor organs.