Cardiological manifestations of mitochondrial respiratory chain disorders

摘要: Mitochondrial Respiratory Chain Disorders (MRCD) are a heterogeneous group of disorders that share the involvement cellular bioenergetic machinery due to molecular defects affecting mitochondrial oxidative phosphorylation system (OXPHOS). Clinically, they usually involve multiple tissues although tend mainly affect nervous and skeletal muscle. Cardiological manifestations frequent include hypertrophic or dilated cardiomyopathies heart conduction defects, being part adult infantile multisystemic or, less frequently, presenting as isolated clinical condition. The aim this review is update cardiological in both going briefly over genetics. Cardiac common feature associated with early late onset forms MRCD. In particular cases, these conditions should be considered into diagnostic algorithm idiopathic cardiomyopathies. Physicians strictly related need aware complications therefore periodical examinations performed such patients. Finally, therapeutic strategies suggested treat cardiac MRCD