Heart failure in children

Heart failure (HF) in children is rare and most often results from congenital heart disease or primary cardiomyopathies rather than acquired conditions. Symptoms are often nonspecific (e.g., feeding intolerance, growth faltering, respiratory distress in infants, and gastrointestinal symptoms in older children and adolescents). Diagnosis relies on echocardiography to define anatomy, ventricular function, and stage, and it is supported by natriuretic peptide levels and findings from basic laboratory studies, ECG, chest x-ray, as well as advanced testing performed by specialists. HF in children is classified based on stage (ACC/AHA staging adapted for children) and functional status (Ross classification in infants and young children, and NYHA in older children). Management of chronic HF focuses on treating the underlying cause, medication tailored to ventricular structure and function, and preventive care. Mechanical circulatory support or transplant may eventually be required. Acute heart failure (AHF) requires urgent stabilization, identification of reversible causes, and the targeted use of diuretics, inotropes, and/or mechanical support based on perfusion and congestion status.

HF in children is rare, with an annual incidence of 1 per 100,000. ^[1]

Epidemiological data refers to the US, unless otherwise specified.

ACC/AHA stages of heart failure ^[2]

• Similarly to adults, can be used to classify disease progression
• In children, certain congenital heart conditions are categorized as stage A HF, e.g.:
  • Single ventricle congenital heart disease
  • Congenitally corrected transposition of the great arteries

Functional classification ^[2]

Classification of patients by functional status is based on symptoms and age.

• Older children and adolescents: NYHA classification
• Infants and young children: Ross classification ^[3]
  • Class I: asymptomatic
  • Class II
    • Infants: mild tachypnea or diaphoresis with feeding
    • Children: exertional dyspnea
  • Class III
    • Infants: marked tachypnea or diaphoresis with feeding, prolonged duration of feeds, and subsequent growth faltering
    • Children: marked exertional dyspnea
  • Class IV: tachypnea, retractions, grunting, or diaphoresis at rest

Causes of chronic heart failure

The etiology of chronic HF in children is distinct from that of adults and predominantly involves structural defects or primary myocardial dysfunction. ^[4]

Congenital heart disease ^[4][5]

• Acyanotic congenital heart defects, e.g.:
  • Large ventricular septal defect
  • Coarctation of the aorta
• Cyanotic congenital heart defects, e.g.:
  • Tetralogy of Fallot
  • Ebstein anomaly

Inherited cardiomyopathy ^[2]

• Dilated cardiomyopathy (DCM)
• Hypertrophic cardiomyopathy (HCM)
• Restrictive cardiomyopathy
• Left ventricular noncompaction cardiomyopathy
• Arrhythmogenic right ventricular cardiomyopathy

In children with suspected inherited cardiomyopathy, consider inborn errors of metabolism, progressive muscular dystrophy, and genetic syndromes (e.g., Barth syndrome, Danon disease) as underlying causes. ^[6]

Acquired heart disease ^[6][7][8]

Acquired abnormalities in heart muscle, valves, and/or vasculature can cause HF in children.

• Inflammatory, e.g.:
  • Viral myocarditis
  • Kawasaki disease
  • Rheumatic heart disease
• Autoimmune, e.g.:
  • Systemic lupus erythematosus
  • Dermatomyositis
• Arrhythmia
• Toxins (e.g., anthracyclines, lead, iron) ^[6]
• Nutritional deficiencies, e.g.: ^[2]
  • Vitamin D deficiency
  • Thiamine deficiency
  • Selenium deficiency
  • Protein-energy malnutrition

Causes of acute heart failure ^[9]

Acute decompensated HF

Patients with preexisting heart disease may experience decompensation due to precipitating factors, e.g.:

• Infection
• Arrhythmia
• Anemia
• Uncontrolled hypertension
• Medication nonadherence
• Iatrogenic triggers (e.g., NSAIDs)

De novo HF

AHF can be the first manifestation of previously unrecognized cardiac disease or result from an acute, potentially reversible precipitating factor, e.g.:

• Thyroid disorders
• Myocarditis
• Arrhythmia-induced cardiomyopathy

• All children
  • Symptoms depend on underlying physiology.
  • Similarly to adults, children may present with features of right-sided HF and/or features of left-sided HF.
  • Clinical features of AHF are similar in children and adults.
• Young children: Symptoms are often nonspecific. ^[2][8]
  • Irritability or lethargy ^[10]
  • Feeding difficulties (e.g., poor intake, diaphoresis with feeding, prolonged feeding times)
  • Growth faltering
  • Signs of increased work of breathing (e.g., grunting, tachypnea, retractions)
• Older children and adolescents: Gastrointestinal symptoms (e.g., abdominal pain, nausea, vomiting, anorexia) are common. ^[2][8]

Heart failure in children often manifests with vague symptoms and requires a high degree of clinical suspicion. ^[2]

Approach ^[2]

• Suspected AHF: Perform diagnostics in tandem with management of AHF in children.
• Perform a clinical evaluation to assess:
  • Symptom severity
  • Personal and family history of cardiac disease
  • Volume status (e.g., presence of edema, jugular venous distention, hepatomegaly)
• Obtain initial diagnostic studies.
  • Echocardiography: to confirm diagnosis and assess cardiac function
  • Laboratory studies, ECG, and chest x-ray: to support the diagnosis, determine severity, and guide management
• Perform additional diagnostic testing to identify the underlying cause and assess for complications.

Initial diagnostics ^[2]

The following tests are typically obtained in all children with a suspected new diagnosis of HF or acute decompensation of HF.

Echocardiography ^[2]

• Assesses cardiac structure and function to:
  • Confirm the diagnosis of HF and determine severity.
  • Identify underlying causes of HF in children.
  • Monitor disease progression.
• Findings include:
  • Structural abnormalities (e.g., valvular disease, ventricular hypertrophy, chamber enlargement)
  • Hemodynamic abnormalities (e.g., increased filling pressures, wall motion abnormalities)
  • Decreased ejection fraction

ECG

• May show arrhythmia, Q waves, and abnormalities of the ST segment and/or T waves that suggest a strain pattern or abnormal repolarization ^[2]
• Normal in some patients ^[8]

Chest x-ray ^[2]

• Cardiac findings may include:
  • Chest x-ray findings in cardiogenic pulmonary edema
  • Features of underlying congenital heart disease
• Can identify additional or alternative pulmonary causes of symptoms (e.g., pneumonia)

Laboratory studies

• Natriuretic peptides (BNP or NT-proBNP): to aid diagnosis and staging, and assess the response to therapy ^[2]
• CMP ^[8]
  • To assess for end-organ dysfunction (e.g., elevated creatinine, elevated transaminases)
  • Some patients with AHF have hyponatremia. ^[11]
• CBC and iron studies
  • Indicated at the time of HF diagnosis to assess for anemia and/or iron deficiency ^[2]
  • Some patients with cyanotic congenital heart disease have polycythemia. ^[12][13]

Additional diagnostics ^[2]

Testing to identify underlying causes and complications

• AHF and/or signs of cardiogenic shock: ABG and lactate to assess for hypoperfusion ^[2][14]
• Cardiomyopathy of unknown etiology
  • Testing for nutritional deficiency (e.g., vitamin D testing)
  • Inflammatory markers
  • Thyroid function testing
  • Metabolic testing (e.g., lactate, pyruvate, ammonia, plasma amino acids, urine organic acids): to evaluate for inborn errors of metabolism
  • Genetic testing
• Respiratory symptoms: pulmonary function testing
• Symptoms of obstructive sleep apnea: pulmonary function testing and polysomnography

Advanced cardiac testing ^[2][15]

The following diagnostics may be ordered by specialists as needed.

• Cardiac MRI: to further assess cardiac function and structure (e.g., diagnostic uncertainty after echocardiogram) ^[2]
• Cardiac CT angiogram: to assess coronary anatomy, if echocardiogram findings are equivocal
• Cardiac catheterization: rarely indicated
  • Assessment of hemodynamics
  • Visualization of coronary anatomy, if noninvasive imaging findings are equivocal ^[2]
  • To perform endomyocardial biopsy for investigating underlying causes (e.g., myocarditis) ^[6]
• Ambulatory ECG monitoring and electrophysiology testing: recommended for patients with palpitations and/or syncope, DCM, and/or a high risk of arrhythmia or heart block
• Cardiopulmonary exercise test (preferred) or 6-minute walk test: to assess exercise tolerance and prognosis ^[2]

Management is usually multidisciplinary and based on the duration (acute vs. chronic) and severity of symptoms, HF stage, and individual anatomy and physiology. ^[2]

• Consult a pediatric cardiologist urgently for guidance and consider the need for transfer. ^[2]
• Initiate acute stabilization measures (e.g., pediatric respiratory support) and continuous cardiac monitoring with pulse oximetry. ^[2][16][17]
• Determine the classification of AHF based on perfusion status and degree of congestion. ^[2]
  • End-organ hypoperfusion ("cold"): Initiate management of cardiogenic shock in children. ^[18]
  • Congestion ("wet")
    • Loop diuretics (e.g., furosemide): first-line therapy ^[2]
    • Vasodilators (e.g., nitroprusside): useful for symptom relief when combined with diuretics in patients without hypotension ^[2]
• Identify and treat reversible causes (e.g., adrenal insufficiency, thyroid dysfunction, anemia); see "Diagnostics for HF in children." ^[2]
• Admit to pediatric ICU or general pediatric floor as appropriate for further management and monitoring.
• Continuously monitor fluid status, electrolytes, hemoglobin levels, and end-organ perfusion to assess response to therapy. ^[2]
• Consider discharge for patients with:
  • Symptomatic improvement and hemodynamic stability
  • Stable medication regimen
  • Adequate nutritional intake
  • Close follow-up in place for management of chronic heart failure in children ^[2]

Although fluid and sodium restriction are commonly included in the management of AHF with congestion, there is insufficient evidence to support their use in children. ^[2]

Positive pressure ventilation may precipitate cardiovascular collapse in children with preload-dependent cardiac defects; ensure senior clinician support is available before attempting intubation and mechanical ventilation. ^[16][17]

Management of cardiogenic shock in children

Cardiogenic shock is rare in children but associated with poor outcomes. ^{[2] [14]}

• Manage in an ICU setting under specialist guidance.
• Establish central venous access for medication administration and monitoring. ^[2]
• Monitor closely. ^[2]
  • Continuous ECG and pulse oximetry
  • Central venous line for measurement of central venous pressure and mixed venous saturation
  • Arterial catheter for continuous BP monitoring in children requiring inotropes
  • Consider near‐infrared spectroscopy for continuous monitoring of organ perfusion.
• Initiate IV inotropes, e.g.: ^[2]
  • Initial therapy
    • Milrinone ^[2]
    • Dobutamine ^[2]
  • Refractory hypotension: epinephrine
  • Levosimendan: adjunctive therapy for AHF refractory to traditional inotropic agents ^[2]
• Initiate mechanical circulatory support (e.g., ECMO or ventricular assist devices) for children who do not respond promptly to IV inotropes. ^[2][19]
• Consider cautious fluid resuscitation in consultation with a specialist. ^[16][20]
• Regularly monitor clinical status and laboratory values (e.g., lactate, arterial pH, NT-proBNP) for signs of poor end-organ perfusion (e.g., lactic acidosis, decreased urine output). ^[14]

For children with rapidly deteriorating chronic HF, see "Management of AHF in children."

Approach

• Refer to cardiology to oversee management, e.g.:
  • Pharmacological treatment for chronic HF in children
  • Consideration of advanced cardiac therapies ^[21]
• Identify and treat the underlying cause.
• Provide preventive health care to facilitate early identification and management of comorbidities.
• Educate patients and caregivers on signs of deterioration and when to seek immediate medical assistance.

Pharmacological treatment for chronic heart failure in children

Treatment is specialist-directed and tailored to ejection fraction and physiology. ^[2][5]

HFrEF (EF ≤ 40%) ^[2]

The choice of agent(s) is based on ACC/AHA staging and is largely extrapolated from adult studies.

• Stage A HF: Treatment is recommended for selected patients (e.g., children with dystrophinopathies) to reduce progression. ^[22]
• Stage B HF: Treatment is recommended for patients with LV dysfunction. ^[5]
  • RAAS inhibitors: angiotensin-converting enzyme inhibitors (ACEIs; preferred) or angiotensin receptor blockers (ARBs) ^[2]
  • Beta blockers ^[2]
• Stage C HF ^[5]
  • LV dysfunction
    • Mineralocorticoid receptor antagonists (MRAs) ^[2][5]
    • RAAS inhibitors: ACEIs, ARBs, or angiotensin receptor-neprilysin inhibitors (ARNIs) ^[2]
    • Beta blockers
    • SGLT-2 inhibitors may be considered. ^[2]
  • Symptomatic congestion: loop diuretics ^[2][5]
  • Heart rate control: ivabradine ^[2]
  • Persistent symptoms ^[2]
    • Digoxin
    • Vericiguat
• Stage D HF: IV milrinone (continuous infusion) may be used as palliative or bridge therapy. ^[2][22]

HFpEF (EF ≥ 50%)

Evidence on treating HFpEF in children is limited; medical therapy may include: ^[2]

• Diuretics: for symptomatic congestion
• SGLT-2 inhibitors

In children with HFpEF, routine use of ACEIs, ARBs, MRAs, ARNIs, beta blockers, or digoxin is not recommended. ^[2]

Single ventricle conditions or systemic RV with ventricular dysfunction

• Refer early to advanced HF and transplant specialists. ^[2]
• Medical therapy may include ACEIs, ARBs, and/or digoxin. ^[2][5]

Beta blockers should be used with caution in patients with systemic RV or single ventricle lesions because of evidence suggesting poorer outcomes. ^[5]

Procedural therapies ^[2]

• Implantable cardiac devices ^[21]
  • Pacemaker placement: may be indicated for ventricular dyssynchrony
  • Implantable cardiac defibrillators: Consider for children at increased risk for sudden cardiac death.
• Ablation: selected patients with arrhythmia-associated ventricular dysfunction
• Mechanical circulatory support devices (e.g., left ventricular assist devices): may be used as a bridge to transplant, a bridge to recovery, or destination therapy ^[19]
• Heart transplant: patients with end-stage HF

Preventive health care

Activity considerations

• Perform a pre-participation physical examination to identify children at risk for adverse outcomes with physical activity.
• Consider cardiopulmonary exercise testing to help assess capacity and guide sports clearance.
• Consider the need for interventions (e.g., implantable cardioverter defibrillator).
• Only restrict sports if there is a high risk of adverse events or disease progression.
• For ambulatory patients with current or previous symptoms: Encourage supervised exercise, if deemed safe. ^[2]

Avoid blanket activity restrictions, as exercise helps improve functional status. ^[2]

Screening and management of comorbidities ^[2]

• Follow routine well-child visit recommendations, including screening for neurodevelopmental disorders and mental health conditions.
• Screen all children regularly for:
  • Iron-deficiency anemia in children: If present, treat with IV iron supplementation. ^[2]
  • Renal and liver dysfunction ^[5]
  • Growth faltering: If present, refer to a dietician for nutritional support (e.g., hypercaloric feedings, nasogastric tube feedings). ^[2]
• Evaluate children with advanced HF for frailty based on: ^[2]
  • 6-minute walk test
  • Handgrip strength
  • Assessment for unintentional weight loss
  • Assessment for fatigue and/or declining levels of physical activity
• Children with obesity: Screen for metabolic syndrome and other cardiovascular risk factors. ^[2]
  • Refer to a multidisciplinary team for management of weight and related risk factors; see "Obesity in children."
  • Initiate treatment as needed; see:
    • "Management of hypertension in children"
    • "Management of pediatric dyslipidemia"

Counseling and psychosocial support

• Provide psychological support for children and caregivers. ^[23]
• Address barriers to accessing health care and risk factors for poor adherence. ^[2]
• Refer to palliative care to set goals of care for patients with advanced HF.
• For adolescents ^[2]
  • Discuss the effects of recreational drugs and alcohol on their condition and potential interactions with medications.
  • For patients of reproductive age, discuss the teratogenicity of medications.
    • Provide highly effective contraception as needed.
    • Adolescents who are pregnant or trying to conceive may need medication changes; see "HF medications in pregnancy."
  • Support the transition from pediatric to adult health care.