Cardiac complications of childhood myopathies

Abstract

The management of individuals with a neuromuscular disorder is usually focused on the skeletal muscle weakness and resulting complications, such as respiratory failure. Long-term prognosis of a number of neuromuscular conditions is, however, also determined by the type and severity of cardiac involvement. Early recognition and treatment of the cardiovascular complications are part of the task of the multidisciplinary team involved in the care of these patients. Although for several of the common conditions, there is general consensus on the cardiac investigations and treatments, in the rarer disorders, evidence-based recommendations are not available, and suggestions from experts provide an acceptable solution. This review summarizes the recent advances in our understanding of the pathogenesis and phenotypic diversity of cardiac complications associated with pediatric myopathies and provides a rational framework for planning the monitoring and therapeutic intervention in individual conditions.