Development of cardiomyopathy after liver transplantation in Swedish hereditary transthyretin amyloidosis (ATTR) patients

Abstract

Background: Recent studies of liver transplanted (LTx) familial amyloidotic polyneuropathy (FAP) patients have shown a progression of cardiomyopathy in some patients after LTx, but knowledge of the underlying factors remains limited. Methods: Seventy-five patients, who had undergone LTx from 1996 to 2008, were included. They had all been examined by echocardiography 1–16 months before LTx. Fifty-four had been re-examined 7–34 months, and forty-two 36–137 months after LTx. Results: A significant increase in interventricular septum (IVS) thickness occurred after LTx (p < 0.01), particularly in males (p = 0.002) and late onset patients (p = 0.003). The development of post-LTx cardiomyopathy was related to patient’s age at onset of the disease, male gender and pre-LTx IVS thickness. On multivariate regression analysis, however, age at onset was the only significant predictor for the development of cardiomyopathy (odds ratio = 1.14, 95% confident interval 1.01–1.30, p = 0.04). Conclusion: An increase of IVS thickness can be observed in FAP patients after LTx. Age at onset of the disease is the main predictor for increased IVS thickness and for the development of cardiomyopathy after liver transplantation.

Keywords:

• Amyloidosis-hereditary-neuropathic
• cardiomyopathy-hypertrophic
• senescence
• transplantation-liver
• transthyretin

Declaration of interest: This work was supported by grants from the patient organization FAMY Västerbotten, FAMY/AMYL Norrbotten, the Swedish Heart and Lung Foundation, a Spearhead grant from Västerbotten’s County Council and a grant from the regional agreement on clinical research (ALF) from Umeå University and University Hospital.