Evaluation of Myocardial Changes in Familial Amyloid Polyneuropathy after Liver Transplantation

Objective The progression of cardiac amyloidosis is a prognostic factor after liver transplantation (LT) in familial amyloid polyneuropathy (FAP). The aim of this study was to assess myocardial changes in FAP amyloidgenic transthyretin (ATTR) Val30Met after LT. Patients and Methods Twelve Japanese FAP ATTR Val30Met patients who underwent LT and were followed for more than 2 years, were examined with serial echocardiography after LT. Serum BNP levels were measured in 9 patients. Results A significant increase in mean left atrial diameter and interventricular septal thickness was observed after LT. The increase in left atrial diameter was correlated with the presence of granular sparkling echo (GSE) at preoperative examination. Serum brain natriuretic peptide (BNP) levels in patients with left atrial diameter dilation (152.0 +/- 157.6 pg/mL) were higher than in those without left atrial diameter dilation (32.0 +/- 30.0 pg/mL). Conclusion LAD and IVS were significantly increased after LT compared with preoperative examinations in Japanese FAP ATTR Val30Met patients. BNP is an important biochemical indicator of myocardiac dysfunction in FAP patients. GSE is a useful echocardiographic marker to predict cardiac amyloidosis after LT.