63 Two novel, unrelated probands with paediatric LVNC and the onset of heart failure were described with loss-of-function PRDM16 variants, one being the abovementioned PRDM16Q187X variant in exon 4 of the PRDM16 gene, another being a splice variant of PRDM16 exon 5. Here, PRDM16 is linked to left ventricular noncompaction.