Heart transplants are now an established mode of treatment for heart failure in adults. Are they justified in children? We believe they are.

The aim is to provide a normal quality of life. However, for some, particularly those with an acute cardiomyopathy, it can be a question of life or death. Long term survival is limited by the development of coronary artery disease (probably as a result of chronic rejection) and post-transplant lymphoproliferative disease (PTLD). However, data from the International Society of Heart and Lung Transplantation (URL <www.ISHLT.org/regist>) shows a one year survival of 80%, five year survival of 70%, and a “half life” of 12.2 years.

So who should be considered? The ISHLT database shows roughly equal numbers of children with congenital heart disease and some form of cardiomyopathy, though this depends on the age group. In those under 1 year of age, over two thirds have congenital heart disease. In the older children (11–17 years of age) the ratio is reversed with most having a cardiomyopathy.

In patients with congenital heart disease, the indication is usually progressive decline in ventricular function although in some it may be intractable arrhythmias. Often it is a subjective decision by the child and their parents based on a poor quality of life. In contrast, patients with a cardiomyopathy may present with an acute episode. Although in most cases it is a dilated cardiomyopathy, patients with hypertrophic or indeed restrictive cardiomyopathies are seen. Bonnet and colleagues1 in a recent issue ofHeart highlighted the rare but important cases which are caused by metabolic disorders—the screening of a child with cardiomyopathy is a very complex issue and is reviewed well by Schwartz and colleagues.2

Of the children presenting with a dilated cardiomyopathy, who should …