The following are key points to remember from an American Heart Association scientific statement on the evaluation and management of chronic heart failure (HF) in children and adolescents with congenital heart disease (CHD):

1. Up to 40% of patients with congenital heart disease (CHD) have been reported to have HF within 20 years after congenital heart surgery. Patients with CHD account for the greatest proportion of pediatric HF admissions.
2. HF with preserved ejection fraction is relatively common in patients with CHD. This may be related to chronic exposure to increased preload or afterload, or secondary to the effects of hemodynamic stressors, or myocardial fibrosis/scarring related to the underlying CHD or previous interventions.
3. Patients with CHD are at increased risk for end-organ dysfunction, particularly renal, hepatic, and pulmonary disease.
4. This scientific statement proposes a framework for characterizing the severity of HF in children with CHD and chronic HF. This ranges from grade 1 to grade 4, and includes HF class, growth percentile, B-type natriuretic peptide (BNP) or NT-proBNP, maximal myocardial oxygen consumption on exercise stress test, invasive measures of cardiac output or ventricular end-diastolic pressure, and need for hospitalization for HF within the last year.
5. This statement also proposed as a framework to standardized HF stages for the CHD population. Stages range from stage A in which patients are asymptomatic with normal ventricular size and function, but have risk factors for development of HF, to stage D in which patients are highly symptomatic with frequent HF hospitalizations, or uncontrolled ventricular arrhythmias.
6. Optimization of nutrition is an important component of the management of children with CHD and chronic HF.
7. There are little data to support the use of adult guideline-directed therapies for HF in patients with CHD. Only two randomized, controlled studies have been performed: one for carvedilol and the other for enalapril.
8. A randomized, placebo-controlled trial of enalapril in infants with single ventricle and normal to mildly reduced ejection fraction did not improve somatic growth, ventricular function, or HF severity.
9. A randomized, placebo-controlled trial for carvedilol failed to demonstrate a benefit. Patients with systemic left ventricular dysfunction trend towards improvement, although patients with CHD and systemic right ventricle or single ventricle treated with carvedilol trended towards a worse outcome than the placebo group.
10. Timing for heart transplantation is critical. To ensure success after heart transplantation, multiple issues need to be mitigated while patients are awaiting transplant as well as in the post-transplant period. These include identifying and treating nutritional and physical deconditioning, minimizing and addressing end-organ dysfunction, addressing aortopulmonary collateral flow, identifying and treating allosensitization, and addressing anatomic abnormalities that require surgical reconstruction at the time of transplantation.

Clinical Topics: Congenital Heart Disease and Pediatric Cardiology, Heart Failure and Cardiomyopathies, Congenital Heart Disease, Acute Heart Failure

Keywords: Heart Defects, Congenital, Heart Failure

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