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  • New-onset heart failure may be difficult to diagnose in children, as symptoms closely mimic common respiratory and gastrointestinal illnesses, or even sepsis, diverting attention from the true primary cause.

  • A directed history and physical examination can provide clues to the presence and possible etiologies of heart failure.

  • Abnormal vital signs with elevated core temperature compared to peripheral extremities may suggest cardiac disease.

  • The clinical diagnosis of congestive heart failure in infants rests upon identification of cardinal signs: labored breathing and diaphoresis with feeding, failure to thrive, and hepatomegaly. A gallop may also be present.

  • Acute management in the emergency room is aimed at improving oxygenation and oxygen delivery as well as reducing metabolic energy and oxygen demands.

  • Medications to consider include diuretics, inotropes, vasodilators, and neurohumoral modulators (i.e., angiotensin-converting enzyme [ACE] inhibitors, β-blockers).

The term “heart failure” (HF) describes a state of cardiac dysfunction whereby the heart fails to generate sufficient forward output to meet the body’s metabolic demands, or meets those needs only in the setting of abnormally elevated filling pressures.1 The clinical appearance of a patient in HF depends on how well cardiovascular adaptations maintain cardiac output in the face of varying physiologic demands. With progressive disease, these physiologic responses reach a threshold limit after which they fail to prevent fluid from accumulating in body organs or to preserve adequate tissue perfusion, and a state of circulatory dysfunction is reached known as decompensated congestive heart failure.

In the United States, the incidence of pediatric heart failure has been estimated as high as 12,000 to 35,000 per year.2–4 Among children who develop cardiac failure, 70% to 80% do so in the first year of life, most commonly from congenital heart disease2,5,6; of the remainder who develop cardiac failure after 1 year of age, half are related to congenital anomalies and the other half due to acquired conditions.6 In general, outcomes of heart failure in children depend on the underlying cause. Overall mortality in pediatric heart failure is about 7%,3,7 with mortality associated with congenital heart disease declining significantly over the past two decades. However, up to 40% of children with cardiomyopathy progress to transplant or death.8


The most likely etiology of acute heart failure is age-dependent in children, and the timing of onset can provide important clues to underlying cause (Table 41-1). In the younger infant, heart failure is most likely related to structural heart disease including shunting and valvar lesions, yet inborn errors of metabolism, neuromuscular disease, arrhythmia or respiratory illnesses, anemia, and infection must be considered. In an older child, the etiology may relate to primary cardiomyopathy (dilated, hypertrophic, restrictive, non-compaction, arrhythmogenic right ventricular cardiomyopathy) or unrepaired structural defects as well as secondary cardiomyopathy related to metabolic, infectious, genetic, and/or environmental (toxins, chemotherapy) origins.2,4...

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