BACKGROUND: The amyloidogenic transthyretin (ATTR) mutation Leu111Met causes a primarily cardiac amyloidosis: Familial amyloidotic cardiomyopathy (FAC). Combined heart-liver transplantation (CHLTx) is the preferred treatment for patients with heart failure due to familial amyloidosis, but information on outcome of patients with Leu111Met mutation is limited. The aim of this study was to evaluate the long-term outcome of CHLTx in patients with FAC. METHODS AND MATERIALS: Between 1998 and 2009, CHLTx was performed in 7 FAC patients (four men). Six patients underwent simultaneous transplantation. All patients suffered from severe cardiomyopathy. RESULTS: Mean recipient age at transplantation was 48.3 ± 4.2 yr. Mean follow-up was 55 months. No peroperative mortality occured. Two patients died within the first year (infection, multi-organ failure) of transplantation. Cumulative survival at 4.5 yr was 71%. No significant liver rejections occurred. One patient experienced an episode of cardiac rejection requiring treatment (H2R). For the surviving five patients, most recent left ventricular ejection fraction was 0.61 ± 0.02, and plasma creatinine was 129 ± 47 μM. None developed significant allograft vasculopathy or neuropathy after transplantation. No recurrence of cardiac amyloid was found. CONCLUSIONS: CHLTx in selected patients with FAC due to Leu111Met mutation offers acceptable long-term survival, almost comparable with isolated cardiac transplantation. Allograft rejection was rare.