International Society for Heart and Lung Transplantation: Practice guidelines for management of heart failure in children

Abstract

Heart failure (HF) in the United States is well recognized as a major public health problem, with over 900,000 hospital admissions annually in the United States, and greater than 250,000 deaths per year. The great majority of heart failure occurs in adults. In children, the scope of the problem is less well defined, but recent data from the Pediatric Cardiomyopathy Registry suggest an annual incidence of 1.13 cases of cardiomyopathy per 100,000 children. 1 While some of this represents asymptomatic disease, the burden of disease overall is nonetheless quite high. In the Pediatric Cardiomyopathy Registry, the majority of children with cardiomyopathy also had HF, with mortality rates of 13.6% at 2 years in dilated forms of cardiomyopathy. The etiology of heart failure differs greatly between children and adults. Children in the Pediatric Cardiomyopathy Registry had a recognizable syndrome or genetic diagnosis in 27% of cases, with an additional 5% of cases due to myocarditis. Furthermore, a large percentage of children with end-stage HF (between 25% and 75%, depending upon the age group) have an underlying diagnosis of congenital heart disease. 2 In contrast to adult patients, ischemic heart disease is rare in children. There is a large, and rapidly growing literature addressing HF treatment for adult patients, with a much smaller literature concerning HF therapy in children. Excellent guidelines for adult patients have recently been published, but given the significant differences between adult and pediatric patients with HF, there is little reason to believe that these guidelines are directly applicable to children. 3 Accordingly, in this document we have attempted to summarize the relevant literature and synthesize management guidelines for children with HF. The document that follows has been prepared in a consensus fashion, with input from pediatric cardiologists at multiple sites throughout the United States and Canada.