Abstract 14674: Cardiac Infiltration is an Independent Risk Factor of Poor Survival after Liver Transplantation in Transthyretin Amyloidosis
Background: Hereditary transthyretin amyloidosis (HA-TTR) is an autosomic dominant disease caused by mutated TTR, leading to both polyneuropathy and infiltrative cardiomyopathy. By removing the main source of mutated TTR, liver transplantation (LT) improves patient's prognosis. Our aim was to document the influence of cardiac and neurological status on survival after LT.
Method: In HA-TTR patients having been liver-transplanted since 1993 in our French reference center, survival was analyzed by using a multivariate Cox model including age, sex, mutation type and preoperative characteristics including neurological Norris score, relative wall thickness (RWT) measured by echocardiography and invasive intraventricular pressures . ROC curves (5-year survival) were also obtained.
Results: The present interim analysis included 155 HA-TTR patients (46±13 yrs, 67% Val30Met mutation). During a mean follow-up of 77±56 months, 43 patients died (1-year survival 92±2%, 5-year survival 79±4%, 10-year survival 66±5%). Non survivors were older, mostly males, with less frequent Val30Met mutation, lower Norris score, thicker cardiac walls and higher LV and RV pressures. Multivariate analysis indicated that RWT and Norris score were independent predictors of death (RWT; HR=87, 95%IC: 8-900, P=0.0002; Norris score: HR=0.94, 95%IC: 0.9-0.98, P=0.002) while age, sex and mutation type were not. The 5-year survival following LT was 23±12% for patients with RWT>0.526 and Norris score<86% vs. 98±2% for patients with RWT86% (cf. Figure, Logrank test P<0.0001).
Conclusion: Cardiac infiltration and the severity of polyneuropathy were independent risk factors of poor survival in liver-transplanted patients with HA-TTR.
- © 2012 by American Heart Association, Inc.