Characteristic Imaging Phenotype of Arrhythmogenic Cardiomyopathy With Filamin C Gene Variant

cardiomyopathy (DCM) and mild LV systolic dysfunction, together with ring-like late gadolinium enhancement (LGE) on CMR imaging.A novel FLNC truncating variant (p.Gln937Ter) was identified, and she was diagnosed as FLNC-related ALVC.The authors clearly demonstrate the characteristic imaging phenotype of FLNC-related ACM.FLNC encodes filamin C, a large actin-cross-linking structural protein located in the intercalated discs.Filamin C is also one of the largest Z-disc proteins.Its main function is the binding of actin rods in the adherens junctions, G enetic abnormalities affect the phenotype and clinical prognosis in cardiomyopathies, including arrhythmogenic cardiomyopathy (ACM).Arrhythmogenic left ventricular cardiomyopathy (ALVC) is a subtype of ACM, defined as a disease with fibrofatty replacement predominantly in the left ventricle (LV), impaired LV systolic function and ventricular arrhythmias originating from the LV. 1 In this issue of the Journal, Yoshimura et al 2 present a case of ALVC harboring filamin C gene (FLNC) truncating variant and showing a characteristic imaging phenotype on multiparametric cardiac magnetic resonance (CMR) imaging.A 28-year-old woman had a family history of dilated