Heart Failure in Children: Indian Academy of Pediatrics Guidelines

Classification of Functional Status of Heart Failure:

The modified Ross classification is most widely accepted and used among the many available classifications for pediatric heart failure (Table1).

Etiology:

The most important step in managing heart failure in a child is to determine the exact cause (Table 2).

(ALCAPA: anomalous coronary artery from a pulmonary artery; AP: Aortopulmonary; ASD: atrial septal defect; PDA: patent ductus arteriosus; VSD: ventricular septal defect)

Diagnostic Approach:

It is essential to identify co-morbidities and differentiate cardiac from noncardiac causes of heart failure. The tests help that guide the diagnosis and management are listed in Table 3.

(ALCAPA: anomalous coronary artery from pulmonary artery)

Management:

The main goals of HF therapy are to correct the underlying cause, reduce associated morbidity and mortality, improve functional status and improve quality of life.

Fluid Management:

Fluid management: Fluid intake is restricted in acute HF where there is lung congestion, peripheral edema despite diuretics, or when there is hyponatremia.

Rest and restriction of activity: Nasogastric feeding is helpful for infants in acute severe HF. Mild-to-moderate aerobic activity should be encouraged in older children with chronic compensated HF.

Correction of anemia: Blood transfusion is generally reserved for severe anemia (Hb 7 g/dL or less).

Nutrition: In infants calorie intake of 120–150 kcal/kg/day is recommended with a fluid intake of 100 mL/kg/day. This is achieved by thickening of feeds or by adding fat (coconut oil/medium chain triglyceride). In older children increase the protein content of the diet while optimizing the fat and carbohydrate intake. Dietary supplementation of calcium and vitamin D3 should be encouraged. Dietary restriction of sodium is generally not recommended in children unless there is severe edema unresponsive to diuretic therapy. Supplementary oxygen may be necessary when there is respiratory distress.

Drug Therapy Goals:

Reduce congestion in lungs and other organs

Increase cardiac output with improved end-organ perfusion:

• Decrease systemic vascular resistance without decreasing tissue perfusion whenever physiologically appropriate.

• Reduction in systemic vascular resistance is not safe when there is dynamic or fixed left ventricular outflow obstruction.

Optimize the preload without causing intra-arterial volume depletion.

Potentially delay disease progression through improved ventricular remodeling.

Diuretics:

• It is the mainstay of therapy and reduces systemic and pulmonary venous congestion. Furosemide—usual dose is 1–2 mg/kg q 6–12 hours. In acute heart failure, it can be used as an infusion at a dose of 1–3 mg/kg/day. In case of refractoriness addition of thiazide diuretics such as metolazone may be done. Spironolactone (Aldosterone antagonist) 0.5–1.5 mg/kg q 12 hours decreases potassium loss and may prevent myocardial fibrosis.

• It is essential to monitor for hypovolemia, hypotension, hypokalemia, hyponatremia metabolic alkalosis, and renal failure.

Vasodilators:

• These are administered in HF only when the altered physiology is likely to benefit from a reduction in vascular resistance such as in systolic ventricular dysfunction with no outflow tract obstruction and acute left-sided valve regurgitation.

• Angiotensin-converting enzyme inhibitor: Enalapril—most widely used— 0.1–0.5 mg/kg/day in 2–3 divided doses. Side effects include cough, hypotension, and renal failure. The first dose of 0.1 mg serves as a 'test dose' and helps avoid serious hypotension.

• Angiotensin receptor blocker: Losartan 0.75–1.4 mg/kg/day for those patients who do not tolerate Enalapril.

• Nitroglycerine/nitroprusside: Acute setting for afterload reduction. This has largely been replaced by milrinone and levosimendan which are essentially vasodilators with an additional inotropic effect (inodilator).

Management:

• Inotropes:

Digoxin is an orally administered cardiac glycoside—10 µg/kg/day orally, serum levels to be maintained between 0.5 and 0.9 nmol/L. Intravenous Dobutamine, Dopamine, and Epinephrine are common choices of inotropes, while Milrinone and Levosimendan are used as inodilators.

• Beta Blockers:

Reduces tachycardia and improves diastolic filling of the part. It is useful in mitral stenosis, hypertrophic obstructive cardiomyopathy, and tachyarrhythmia-related heart failure. Commonly used agents are non-selective beta-blockers such as propranolol or selective agents such as metoprolol, atenolol, or bisoprolol. Carvedilol (0.1–0.8 mg/kg/day in three divided doses) has additional vasodilatory properties and is most commonly used for the elected case of cardiomyopathy and fersistent ventricular dysfunction after cardiac surgery (such as after ALCAPA repair).

• Newer Drugs:

Ivabradine—used to control heart rate with no negative inotropic effect. A combination of Neprilysin inhibitor (Sacubitril) and Valsartan is now increasingly used in children and results of clinical trials are awaited.

A suggested management algorithm for acute heart failure is shown in Flowchart 1.

• Treatment of Advanced Pediatric Heart Failure and End-stage Heart Disease:

Advanced therapies for chronic heart failure include cardiac resynchronization therapy (CRT), ventricular assist devices (typically as a bridge to cardiac transplantation), and heart transplants. These therapies are expensive and there are significant logistic challenges in the Indian setting.

Reference:

• Hinton RB, Ware SM. Heart failure in pediatric patients with congenital heart disease. Circ Res. 2017;120:978-94.

• Shaddy RE, George AT, Jacklin T, Lochlainn EN, Thakur L, Agrawal R, Solar-Yohay S, Chen F, Rossano JW, Severin T, Burch M. Systematic literature review on the incidence and prevalence of heart failure in children and adolescents. Pediatr Cardiol. 2018;39:415-36.

• Venkatesh S, Kumar RK, Heart Failure in Children. IAP Specialty Series on Pediatric Cardiology, 3rd edition. Jaypee Brothers Medical Publishers, New Delhi; 2022. . pp. 351-76.

• The guidelines can be accessed on the official site of IAP:https://iapindia.org/standard-treatment-guidelines/