Perioperative Predictors of Survival After Liver Transplantation for Familial Amyloid Polyneuropathy in a Portuguese Center

Transplant Proc. 2016 Jul-Aug;48(6):2098-101. doi: 10.1016/j.transproceed.2016.04.020.

Abstract

Background: Liver transplantation (LT) has been the treatment of choice to halt the progression of familial amyloid polyneuropathy (FAP). Few studies have identified prognostic factors for post-LT survival in FAP. Our aim was to assess survival rate and to identify independent factors for survival after LT.

Methods: This retrospective cohort study of FAP patients transplanted for the first time analyzed 116 transplantations from 2006 to 2014. The median follow-up period was 45.5 months.

Results: The overall survival rates at 1 month, 1 year, and 5 years were 89%, 82% and 79%, respectively. On multivariate analysis, only number of red blood cell (RBC) units transfused during surgery, operation time, and body mass index were independent prognostic factors for patient survival. Only 30% of patients were transfused during surgery, and, in these, each RBC unit transfused increased mortality by 53%. The operation time increased mortality by 20% for every 15 minutes of surgery.

Conclusions: This study suggests that operation time and RBC transfused are predominant factors affecting post-LT survival in our FAP patients.

MeSH terms

  • Adult
  • Amyloid Neuropathies, Familial / mortality*
  • Disease Progression
  • Female
  • Follow-Up Studies
  • Humans
  • Kaplan-Meier Estimate
  • Liver Transplantation / mortality*
  • Male
  • Middle Aged
  • Multivariate Analysis
  • Operative Time
  • Portugal / epidemiology
  • Retrospective Studies
  • Survival Rate

Supplementary concepts

  • Amyloidosis, Hereditary, Transthyretin-Related