Filamin C variants are associated with a distinctive clinical and immunohistochemical arrhythmogenic cardiomyopathy phenotype.

•FLNC-linked ACM presents with LV involvement, ventricular arrhythmia, sudden death.•Late gadolinium enhancement on cardiac MRI may be the only structural abnormality.•Mutation carriers have abnormalities on ECG or cardiac MRI non-diagnostic for ARVC.•FLNC disease mechanism different to classic ARVC caused by desmosomal mutations.