PLEKHM2 and left ventricular noncompaction: One patient with severe left ventricular dysfunction (LVEF <20%) had a diagnostic variant in PLEKHM2. To our knowledge this patient represents the third family with homozygous/compound heterozygous truncation of PLEKHM2. In previous studies the patients with PLEKHM2 variants presented with DCM and features of LVNC, and a family history of SCD (31, 32).