What is the treatment for congestive heart failure in children with rheumatic heart disease?

Treatment of Congestive Heart Failure in Children with Rheumatic Heart Disease

For children with rheumatic heart disease and heart failure, initiate standard medical therapy with diuretics (furosemide), ACE inhibitors, and digoxin, while recognizing that definitive catheter-based or surgical intervention is the only treatment that can improve outcomes in moderate to severe disease. 1

Initial Medical Management

The cornerstone of acute and chronic heart failure management in children with rheumatic heart disease follows standard pediatric heart failure protocols:

Diuretic Therapy

• Start furosemide at 1 mg/kg/dose orally or IV every 12-24 hours for initial management of congestion and pulmonary edema 2
• At doses ≤2 mg/kg/day, potassium supplementation is generally not required 2
• If inadequate diuresis occurs, increase by 1 mg/kg increments up to a maximum of 10 mg/kg/day, though use the lower end of dosing (1-2 mg/kg/day total) for mild heart failure 2
• Never exceed 6 mg/kg/day for longer than 1 week due to significant ototoxicity risk 2
• Administer IV furosemide over 5-30 minutes to minimize ototoxicity 2

ACE Inhibitor Therapy

• ACE inhibitors (enalapril or captopril) should be used in children with symptoms refractory to digoxin and furosemide 3
• These agents improve function and survival in children with systemic ventricular dysfunction 4
• Monitor closely for renal insufficiency or renal failure, particularly in infants 4

Digoxin Therapy

• Digoxin may be beneficial if ventricular function is reduced or symptomatic heart failure persists despite other therapies 2
• The combination of digoxin and furosemide provides increased contractility over baseline 2
• Use usual age and weight dosing schedule: 5 μg/kg orally twice daily up to 10 years, then 5 μg/kg once daily, with maximum dose of 0.125 mg/day 3
• Monitor for bradycardia, which is dose-limiting 3

Combination Therapy

• If furosemide alone is inadequate at 2 mg/kg/day, add spironolactone rather than increasing furosemide dose 2
• Spironolactone decreases potassium excretion and may eliminate the need for potassium supplementation 2
• Monitor for hyperkalemia when using spironolactone, especially with concurrent potassium supplementation 2

Critical Monitoring Parameters

Monitor the following parameters closely:

• Fluid status and urine output (target >1 mL/kg/hour) 2
• Electrolytes (potassium, sodium, chloride) periodically 2
• Renal function, blood pressure, and weight 2
• Watch for hypercalciuria leading to nephrocalcinosis, hypokalemia, metabolic alkalosis, and transient deafness with furosemide 2
• Signs of dehydration: decreased skin turgor, sunken fontanelle, lethargy 2

Beta-Blocker Considerations

• Carvedilol is NOT recommended for pediatric heart failure based on the only randomized controlled trial 5
• In a double-blind trial of 161 children with chronic heart failure, there was no significant effect on clinical outcomes after 8 months 5
• Adverse reactions in children included chest pain (17%), dizziness (13%), and dyspnea (11%) at rates significantly higher than placebo 5

Definitive Management

The most important consideration is that medical therapy is temporizing only:

• Definitive catheter-based or surgical intervention is the only treatment that can improve outcomes in patients with moderate or severe rheumatic heart disease 1
• The majority of children with structural heart disease causing heart failure have surgically correctable causes, and surgical correction is a Class I indication 6
• Heart transplantation should be considered for patients with refractory heart failure and extremely poor function 6

Important Caveats

• There are no long-term prospective, randomized, controlled outcomes data available for medical management in children 6
• Rheumatic heart disease remains the most common cause of cardiovascular morbidity and mortality globally in children and young adults 1
• Access to definitive intervention remains very limited in rheumatic heart disease endemic regions 1
• Medical management principles are extrapolated from adult medicine, but children are not small adults and require careful dose adjustments 7
• The overall outcome with heart failure is better in children than adults because it is commonly due to structural heart disease and reversible conditions amenable to therapy 8, 7