Cardiomyopathy in childhood

Cardiomyopathies are a heterogeneous group of disorders of both genetic and non-genetic aetiology. Their importance lies in the fact that although the mortality for structural congenital heart disease has been reduced from around 40% to 5% in the past 40 years there has been little change in the prognosis for paediatric patients with cardiomyopathies. Around one-third of children with a cardiomyopathy will die or require transplantation. Advances in genetics over the past decade have resulted in the recognition that a large proportion of cardiomyopathies are genetic in origin. Management of these patients is best in centres offering cardiology, genetic and metabolic input. This article aims to briefly review the broad categories of cardiomyopathies encountered in childhood.