Thirteen pathogenic or likely pathogenic variants in genes previously associated with LVNC aetiology, including variants detected in ACTN2, HCCS, HCN4, LAMA4, MYH6, MYH7, PRDM16, TAFAZZIN and TTN—as well as three rare variants of uncertain significance in ACTC1 and RBM20 genes were identified. The gene discussed is ACTN2; the disease is left ventricular noncompaction.