In this cohort of 107 genetically evaluated children with DCM, 38 children (36%) carried a LP/P variant in a DCM-related gene, most often in MYH7. Children with DCM who carried a LP/P variant had a 2.8-times increased risk of death or HTx compared with children without such a variant, but clinical characteristics at time of diagnosis did not differ between the 2 groups. Here, MYH7 is linked to familial dilated cardiomyopathy.