In this particular section, we will focus on pediatric cardiomyopathy associated with the rare genetic disorder, BTHS, which is caused by mutations in the TAZ gene, encoding for tafazzin, a mitochondrial transacylase critical for catalyzing the final step in cardiolipin remodeling (Jefferies, 2013; Dudek and Maack, 2017). Here, TAFAZZIN is linked to Barth syndrome.