Approach Considerations

Appropriate laboratory testing includes assessment of the following: oxygen saturation, complete blood count (CBC), hemoglobin concentration, electrolyte levels, calcium level, cardiac biomarkers, blood urea nitrogen (BUN) level, creatinine level, and renal^[3]and hepatic function.

The CBC count can reveal signs of anemia or infection.

Brain natriuretic peptide (BNP) or N -terminal prohormone BNP (NT-proBNP) levels are elevated as a result of ventricular dilation. Elevation of serum BNP level is particularly useful in distinguishing patients with congestive heart failure from those with a primary respiratory process. BNP levels of more than 100 pg/mL are associated with congestive heart failure in adults and children. Normal levels may be slightly higher in neonates. Serial measurements of BNP levels in children with primary myocardial dysfunction and acute decompensated heart failure in which levels are persistently elevated and/or there is a lesser degree of decline in the first week of presentation are adverse prognostic factors.^[6]

Cardiac troponin may be elevated in cases of myocarditis or after ischemic injury due to coronary anomaly or cardiomyopathy, as well as in noncardiac conditions in which cardiac perfusion may be compromised (sepsis).

The evaluation of serum electrolyte levels in the patient with congestive heart failure may demonstrate hyponatremia secondary to water retention. Elevated potassium levels may represent renal compromise or even tissue destruction due to low cardiac output. Significant tissue hypoxia increases serum lactate concentration and depletes the serum bicarbonate level. In more chronic congestive heart failure states, reduced renal blood flow may be expressed as increased BUN and creatinine levels.

A 12-lead electrocardiogram (ECG) may reveal evidence of structural or coronary artery disease or a complete atrioventricular block or arrhythmia.

Pulse oximetry, as well as a hyperoxia test in newborns, may be useful. The systemic saturation on room air is a more reliable measure of oxygenation than are observations for cyanosis alone, which are often misleading. The partial pressure of arterial oxygen (PaO₂) when the patient is receiving 100% oxygen (hyperoxia test) may help in distinguishing intracardiac mixing malformations from pulmonary disease in the setting of hypoxia. Blood gas abnormalities may show respiratory alkalosis in mild forms of congestive heart failure or metabolic acidosis in patients with evidence of low cardiac output or ductal-dependent congenital heart disease.

Radiography and Echocardiography

In the presence of congestive heart failure, the cardiac silhouette is usually enlarged on the chest radiograph. As with BNP elevation, cardiac enlargement may help to distinguish patients with congestive heart failure from those with respiratory disease. However, exceptions may include restrictive cardiomyopathy, venous obstruction (total anomalous pulmonary venous obstruction), and diastolic dysfunction due to high ventilator mean airway pressures, displaying a normal cardiac size on chest radiographs. Increased pulmonary blood flow may be present, along with pulmonary edema or venous congestion. (See the image below.)

Chest radiograph shows signs of congestive heart failure (CHF).

Echocardiography is indicated in any child with unexplained congestive heart failure to assess cardiac function and identify potential cardiovascular causes, particularly anatomic lesions and cardiomyopathy. On the other hand, congestive heart failure itself is not an echocardiographic diagnosis; therefore, the underlying etiology is best identified by means of detailed history taking and physical examination and often by means of chest radiography. When oral sedation is performed for echocardiography, note that children with a low cardiac output can depend on endogenous catecholamine levels to maintain tissue perfusion. Sedation can cause withdrawal of the endogenous catecholamine drive, resulting in cardiac decompensation.

Treatment & Management

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Chest radiograph shows signs of congestive heart failure (CHF).

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Table. Pharmaceutical Agents Used in the Treatment of Congestive Heart Failure

Agent	Pediatric Dose	Comment
Preload Reduction
Furosemide	1 mg/kg/dose PO or IV	May increase to qid
Hydrochlorothiazide	2 mg/kg/d PO divided bid	May increase to qid
Metolazone	0.2 mg/kg/dose PO	Used with loop diuretic, may increase to bid
Inotropic
Digoxin	Preterm infants: 0.005 mg/kg/d PO divided bid or 75% of this dose IV; age 10 y: 0.005 mg/kg/d PO qd or 75% of this dose IV	...
Dopamine	5-10 mcg/kg/min IV (usual dosage; maximal dosage may be up to 28 mcg/kg/min)	Gradually titrate upward to desired effect
Dobutamine	5-10 mcg/kg/min IV	Gradually titrate upward to desired effect
Epinephrine	0.01-0.03 mcg/kg/min IV	Not to exceed 0.1-0.3 mcg/kg/min
Milrinone	0.3-1 mcg/kg/min IV	Typically used without loading dose, especially in unstable patients Load: 50 mcg/kg IV over 15 min
Afterload Reduction
Captopril	0.1-0.5 mg/kg/d PO divided q8h	...
Enalapril	0.1 mg/kg/d PO divided qd/bid, not to exceed 0.5 mg/kg/d	Adults: 2.5-5 mg/day PO qd/bid initially; titrate slowly at 1- to 2-wk intervals; target dose is 10-20 mg PO bid; not to exceed 40 mg/day
Lisinopril	Not established	Adults: Usual dosage is 10mg PO qd (range, 2.5-10 mg)
Losartan	Initial dose for hypertension is 0.1 mg/kg/day PO; dosage for treatment of CHF is not established in children	Adults: 25-100 mg/d PO qd or divided bid
Nitroprusside	0.5-10 mcg/kg/min IV	May need to monitor cyanide level
Nitroglycerin	0.1-0.5 mcg/kg/min IV	Vasodilator
Nesiritide	0.01-0.03 mcg/kg/min IV	Initiate with 0.01 mcg/kg/min May cause dose-related hypotension
Alprostadil*	0.03-0.1 mcg/kg/min IV	...
Beta-Blockade ^[7]
Carvedilol	Limited data suggest a therapeutic dosage range of 0.2-0.4 mg/kg/dose PO bid; initiate with lower dose and gradually increase dose q2-3wk to therapeutic range	Adults: 12.5-25 mg PO bid Initiate with 3.125 mg PO bid
Metoprolol	Not established	Adults: 25-100 mg PO qd
Selective Aldosterone Antagonists
Spironolactone	1-3.3 mg/kg/day PO in single or divided doses	Adults: 12.5-50 mg PO qd; reduce dose to 25 mg qod if hyperkalemia occurs
Eplerenone	Not established	25-50 mg PO qd
I(f) Current Inhibitor
Ivabradine	Initial >6 mo and < 40 kg: 0.05 mg/kg PO BID Maximum: 0.2 mg/kg BID (6 mo-1 y); 0.3 mg/kg BID (1 y or older)	Lowers heart rate
*Prostaglandin E1 (PGE₁).

Table. Recommendations for Pharmacologic Therapy for Chronic Heart Failure

Drug Class	Recommendation	Reduced EF (systolic HF)	Preserved EF (diastolic HF)
Diuretics	Class I	Patients with fluid retention associated with ventricular dysfunction (HF Stage C) to achieve a euvolemic state	All patients to establish a euvolemic state
	Class I		Perform close monitoring of renal function and blood pressure during initiation and up-titration of diuretic therapy
	Class IIa		Consider diuretics to treatment systemic hypertension to prevent disease progression.
Ace Inhibitors	Class I (LoE B)	For symptomatic left ventricular dysfunction (HF Stage C), use ACE inhibitors routinely unless there is a specific contraindication. Start at low doses and up-titrate to a maximum tolerated safe dose
	Class IIa (LoE B)	For asymptomatic left ventricular dysfunction (HF Stage B), use ACE inhibitors routinely unless there is a specific contraindication.
	Class III (LoE B)	Do not routinely use for patients with single-ventricle CHD, but could be considered in specific cases such as in situations of valve regurgitation or ventricular dysfunction
Angiotensin antagonists	Class IIa	Generally reserved for systemic ventricular systolic dysfunction in patients who are intolerant of ACE inhibitors
	Class IIb		Routine use is not recommended unless there is an additional indication such as hypertension.
	Class IIb		May be used for control of hypertension but careful monitoring of hemodynamics and renal function is required due to the enhanced risk of hypotension and renal toxicity.
Beta- Blockers	Class IIa (LoE B)	Consider beta-blockers in symptomatic children with systemic LV systolic dysfunction, particularly if the systemic ventricle has a LV morphology. Therapy should start at a small dose and slowly up-titrate
	Class IIa (LoE B)	Consider beta-blockers in asymptomatic children with systemic LV systolic dysfunction. Therapy should start at a small dose and slowly up-titrate
Calcium Channel Blockers	Class III		Not recommended unless there is an additional indication.
Inotropic Agents	Class III	Use of intermittent or chronic inotropic therapy, other than as a bridge to transplant, is not recommended.
Nesiritide	Class IIb	Not recommended for routine use although it may be considered in select patients where other interventions to lower central venous pressure have been unsuccessful.

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Author

Gary M Satou, MD, FASE Director, Pediatric Echocardiography, Co-Director, Fetal Cardiology Program, Mattel Children's Hospital; Associate Clinical Professor, Department of Pediatrics, University of California, Los Angeles, David Geffen School of Medicine

Gary M Satou, MD, FASE is a member of the following medical societies: American Academy of Pediatrics, Society of Pediatric Echocardiography, American College of Cardiology, American Heart Association, American Society of Echocardiography

Disclosure: Nothing to disclose.

Coauthor(s)

Nancy J Halnon, MD Assistant Professor, Division of Pediatric Cardiology (Heart Transplantation and Pediatric Cardiology), Mattel Children's Hospital, University of California, Los Angeles, David Geffen School of Medicine

Nancy J Halnon, MD is a member of the following medical societies: American Academy of Pediatrics, American Heart Association, American Society of Transplantation, Society for Pediatric Research

Disclosure: Nothing to disclose.

Chief Editor

Stuart Berger, MD Executive Director of The Heart Center, Interim Division Chief of Pediatric Cardiology, Lurie Childrens Hospital; Professor, Department of Pediatrics, Northwestern University, The Feinberg School of Medicine

Stuart Berger, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American College of Chest Physicians, American Heart Association, Society for Cardiovascular Angiography and Interventions

Disclosure: Nothing to disclose.

Acknowledgements

Lars C Erickson, MD, MPH Associate Professor of Pediatrics, University of Massachusetts Medical School; Consulting Staff, Department of Pediatrics, Division of Pediatric Cardiology, University of Massachusetts Medical Center

Lars C Erickson, MD, MPH is a member of the following medical societies: American Heart Assocation and Sigma Xi