What are the current guidelines for managing pediatric heart failure?

Current Guidelines for Pediatric Heart Failure Management

The management of pediatric heart failure requires a multi-disciplinary approach with medications, nutritional support, activity modifications, and potentially surgical intervention, as most evidence is extrapolated from adult studies due to limited pediatric-specific data. 1

Etiology and Presentation

• Pediatric heart failure differs from adults in two major ways: maturational differences in contractile function and congenital/structural/genetic causes that are either modified by adulthood or lead to early death 2
• The most common cause of heart failure in infants and children is ventricular septal defect, where myocardial function is frequently normal 2
• Clinical presentations in infants with chronic heart failure include difficulty feeding (>20 min to drink a bottle), diaphoresis (damp clothes upon waking), poor weight gain, and occasionally pneumonia 2

Diagnostic Approach

• After initial stabilization, diagnosis of idiopathic dilated cardiomyopathy must be established by exclusion 2
• If echocardiography has not demonstrated normal proximal coronary artery anatomy, cardiac catheterization must be performed 2
• If no anatomic cause is demonstrated, myocardial biopsy may be performed to diagnose myocarditis 2
• Metabolic and genetic familial causes of congestive cardiomyopathy (carnitine and selenium deficiency) are more common in children than adults 2

Acute Heart Failure Management

• In infants/children with acute heart failure and decreased ventricular function, follow principles similar to adults 2:
  • Administer diuretic drugs cautiously as acute decrease in preload may lead to hypotension 2
  • Intravenous inotropic agents (dobutamine, dopamine, epinephrine, isoproterenol) are generally similar to those used in adults 2
• For specific presentations:
  • Congenital heart disease with left-to-right shunt: Initially managed with intravenous furosemide 2
  • Systemic outflow obstruction (hypoplastic left heart syndrome, interrupted aortic arch, coarctation): Prostaglandin E1 is given to dilate the ductus arteriosus; oxygen is withheld due to its ductus-constricting properties 2
  • Dilated, poorly functioning heart: Rule out anomalous origin of left coronary artery from pulmonary artery 2

Chronic Heart Failure Management

Congestive Cardiomyopathy

• Management principles and drugs are similar to adults 2
• ACE inhibitors (enalapril, captopril) are used in infants and children with symptoms refractory to digoxin and furosemide 2
• For extremely poor function, heart transplantation should be considered 2

Congenital Heart Disease with Left-to-Right Shunt

• Medical management is weighed against severity of heart failure, natural history of defect, and expected success of operation 2
• For ventricular septal defects, surgical intervention is typically postponed if the infant is gaining weight and free of lower respiratory tract infection 2
• Medical management includes:
  • Furosemide (<2 mg/kg/day orally to avoid excess potassium loss) 2
  • At higher diuretic doses, spironolactone is added to avoid potassium supplementation 2
  • Use of digoxin remains controversial, especially in premature infants with patent ductus arteriosus 2

Advanced Therapies

• For patients with refractory heart failure, heart transplantation should be considered 2
• For diastolic dysfunction, the goal is to reduce ventricular filling pressure without reducing cardiac output 2
• Diuretic drugs and nitrates are drugs of choice for symptomatic patients with diastolic dysfunction 2
• Calcium channel blockers, beta-blockers, and ACE inhibitors may benefit diastolic dysfunction 2
• Positive inotropic agents are not indicated if systolic function is normal 2

Important Considerations

• The majority of infants with heart failure have a surgically correctable cause, and surgical correction is a Class I indication 2
• For medical management, there are no long-term prospective, randomized, controlled outcomes data available in children 2
• Treatment approaches have been proposed as both successful and unsuccessful based on uncontrolled trials 2
• Recent advances include introduction of newer medications like ivabradine and sacubitril/valsartan, though pediatric-specific data remain limited 3