Cardiological manifestations of mitochondrial respiratory chain disorders.

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