Several clinical studies previously reported that OBSCN missense variants or mutations are associated with left ventricular non-compaction (LVNC) (Rowland et al., 2016), hypertrophic myocardiopathy (HCM) (Arimura et al., 2007) and dilated cardiomyopathy (DCM) (Marston et al., 2015). Here, OBSCN is linked to left ventricular noncompaction.