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

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Introduction

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.

Each recommendation in this document is ranked with regard to the level of supporting evidence:

  • Level A recommendations are based upon multiple randomized clinical trials.

  • Level B are based upon a single randomized trial or multiple non-randomized trials.

  • Level C are based primarily upon expert consensus opinion.

The level of evidence upon which a recommendation is based, differs from the strength of the recommendation. A given recommendation may be based upon randomized trials yet still be controversial. Other forms of therapy, which are based solely upon expert consensus, may be strongly recommended.

Recommendations in this document adhere to the format of guidelines previously published by the American College of Cardiology (ACC) and American Heart Association (AHA).

  • Class I: Conditions for which there is general agreement that a given therapy is useful and effective.

  • Class II: Conditions for which there is conflicting evidence or a divergence of opinion concerning the usefulness and effectiveness of a therapy.

    • Class IIa: Weight of evidence/opinion favors usefulness/effectiveness.

    • Class IIb: Weight of evidence/opinion is less in favor of usefulness/effectiveness.

  • Class III: Conditions for which there is general agreement that a therapy is not useful and (in some cases) may be harmful.

Section snippets

Definition of heart failure

Heart failure is a complex clinical syndrome, with multiple etiologies and diverse clinical manifestations. Many definitions have been offered, but we prefer that set forth by Arnold Katz, which not only describes the clinical aspects of HF, but also reflects a growing understanding of the cellular processes which accompany this condition:4

“…heart failure is a clinical syndrome in which heart disease reduces cardiac output, increases venous pressures, and is accompanied by molecular

Overview

The morphologic right ventricle (RV) is connected to the aorta and is thus the systemic ventricle in 2 main groups of patients with biventricular circulation: patients born with d-transposition of the great arteries who were treated with atrial baffle surgery (Mustard or Senning repair) and patients born with congenitally-corrected transposition of the great arteries. RV dysfunction has been described in both these groups of patients and may lead to HF. Although RV performance at rest is often

Overview

For most patients with single ventricle anatomy, surgical management is the primary treatment path, and may include a variety of early palliative procedures followed by bi-directional Glenn (superior cavopulmonary connection, SCPC), and ultimately the Fontan procedure (total cavopulmonary connection, TCPC). Early palliation with complete mixing of systemic and pulmonary venous flow requires that ventricular output must be maintained at a level that is 2 to 3 times normal.100, 101 This chronic

Pharmacologic support

In adult studies, it has been recognized that inotropes may be required in the management of refractory acute HF exacerbations that are accompanied by hypoperfusion and hypotension.124 Currently available inotropic agents increase contractility through a common pathway of increasing intracellular levels of cyclic adenylate monophosphate (cAMP). Increased cytoplasmic levels of cAMP cause increased calcium release from the sarcoplasmic reticulum and increased contractile force generation by the

Overview

Arrhythmia is a major cause of morbidity and mortality in pediatric patients with end-stage HF.154, 155, 156, 157, 158, 159, 160, 161 Myocardial scars and stretching associated with pressure or volume overload, previous heart surgery or intrinsic myocardial disease provide a ripe substrate for arrhythmogenesis.162, 163 Multiple triggers for arrhythmia are prevalent in patients with HF and include electrolyte imbalance, blood gas and pH abnormalities, ischemia and administration of potentially

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