Thus, in this cohort, a female patient, without any skeletal myopathy but exhibiting pure apical hypertrophy and a likely pathogenic variant in ACTA1, as well as a VUS in TNNI3, suggests that despite α-actin being one of the most expressed proteins in skeletal muscle, it might produce myocardial involvement alone. This evidence concerns the gene ACTA1 and Skeletal myopathy.