The patient carrying LMNA p.A242V/LAMA4 p.A225 presented with ARVC/D and complex complications, including RVHF, atrial fibrillation, atrial standstill, multifocal ventricular premature, right bundle block and third-degree atrioventricular block, which were the high-risk factors for thromboembolism and sudden death. This evidence concerns the gene LMNA and atrial standstill.