Heart failure in the young

eart failure is an enormous clinical burden in adult medicine, largely because of the prevalence of atheromatous coronary disease.In children, where coronary disease is not the leading cause of heart failure, it is less common.It is, however, an important disease, accounting for 10% of paediatric cardiac transplants in children.Cardiac symptoms in children are usually the result of congenital lesions.Most of these lesions, such as septal defects, are amenable to surgical intervention.It is not appropriate to expand on the management of congenital heart lesions in this review.There is a small subgroup of children that have diastolic failure from cardiomyopathic restriction to flow.The remaining patients, which will be focused on below, have heart failure that is principally related to poor myocardial function and largely comprise those children with dilated poorly contracting ventricles, which can be related to specific aetiologies in some cases.Particular topics of debate in paediatric heart failure concern: c the diagnosis and management of myocarditis versus dilated cardiomyopathy c the most appropriate investigations for new onset heart failure c cellular responses to heart failure c the increasing population of anthracycline treated survivors of childhood malignant disease c treatment strategies. c DILATED CARDIOMYOPATHYIndications for transplantation are a guide to the spectrum of causes of severe heart failure.Dilated cardiomyopathy remains the principal indication for cardiac transplantation in children worldwide throughout childhood, apart from infancy when congenital heart disease is a more common indication.The prognosis for dilated cardiomyopathy is around 60% at five years from presentation (fig 1), with a high attrition within six months of presentation. 1 The genetics of dilated cardiomyopathy have been described as a "molecular maze".w1 Linkage analysis for autosomal dominant dilated cardiomyopathy has proved difficult and direct candidate gene analysis has been used instead, although this is more difficult to use as proof for causation.A variety of lesions have been described including mutations in the cytoskeleton, troponin T, w2 and other sarcomere protein genes. 2 For the short term molecular genetic analysis is largely a research tool in dilated cardiomyopathy, but it is likely to enter into clinical practice in the foreseeable future.New onset heart failure in children should be investigated for specific causes and these are discussed below.One particular problem is whether the child has myocarditis or cardiomyopathy as this currently alters management in many centres.