The clinical and histological phenotype reported in Ahrens-Nicklas et al. [6], is highly concordant with the two infant siblings presented herein and is mirrored by two Lmod2-deficient mouse models; onset at birth leading to cardiac ventricular dilatation and a non-contractile heart leading to heart failure, with associated shortening of cardiac thin filaments [6–8]. The gene discussed is LMOD2; the disease is Ventriculomegaly.