Furthermore, several mutations in MYH7 have been associated with congenital heart defects (CHDs), including Ebstein’s anomaly [6], bicuspid aortic valve (BAV) [7], and ventricular septal defect (VSD) [8], but thus far always in combination with cardiomyopathy (typically NCCM). Here, MYH7 is linked to ventricular septal defect.