Chondroid And Osseous Metaplasia Of The Central Fibrous Body In Adolescent Hearts With Mutations Intnni3andtnnt2genes

The histological spectrum of the central fibrous body (CFB) of the heart, particularly in humans, is not fully characterized. Herein, we describe the presence of cartilage and bone within the CFB of 2 explanted heart specimens from patients with known mutation-driven cardiomyopathy involving theTNNI3andTNNT2genes, review the existing literature on the identified variants particularlyTNNI3(p.Asn185Thrfs*14) andTNNT2(p.Arg141Trp), and provide insights into the plausible nature of such histopathological observation based on animal studies and the few reported cases in humans.