History

Thorough history taking and physical examination, including an assessment of the upper-extremity and lower-extremity blood pressures, are crucial in the evaluation of an infant or child with congestive heart failure.

Regardless of the etiology, the first manifestation of congestive heart failure is usually tachycardia. An obvious exception to this finding occurs in congestive heart failure due to a primary bradyarrhythmia or complete heart block.

As the severity of congestive heart failure increases, signs of venous congestion usually ensue. Left-sided heart failure is generally associated with signs of pulmonary venous congestion, whereas right-sided heart failure is associated with signs of systemic venous congestion. Marked failure of either ventricle, however, can affect the function of the other, leading to systemic and pulmonary venous congestion.

Later stages of congestive heart failure are characterized by signs and symptoms of low cardiac output. Generally, congestive heart failure with normal cardiac output is called compensated congestive heart failure, and congestive heart failure with inadequate cardiac output is considered decompensated.

Signs of congestive heart failure vary with the age of the child.^[5]Signs of pulmonary venous congestion in an infant generally include tachypnea, respiratory distress (retractions), grunting, and difficulty with feeding. Often, children with congestive heart failure have diaphoresis during feedings, which is possibly related to a catecholamine surge that occurs when they are challenged with eating while in respiratory distress.

Right-sided venous congestion is characterized by hepatosplenomegaly and, less frequently, by edema or ascites. Jugular venous distention is not a reliable indicator of systemic venous congestion in infants, because the jugular veins are difficult to observe. In addition, the distance from the right atrium to the angle of the jaw may be no more than 8-10 cm, even when the individual is sitting upright.

Uncompensated congestive heart failure in an infant primarily manifests as a failure to thrive. In severe cases, failure to thrive may be followed by signs of renal and hepatic failure.

In older children, left-sided venous congestion causes tachypnea, respiratory distress, and wheezing (cardiac asthma). Right-sided congestion may result in hepatosplenomegaly, jugular venous distention, edema, ascites, and/or pleural effusions.

Older children with uncompensated congestive heart failure may have fatigue or lower-than-usual energy levels. Patients may complain of cool extremities, abdominal pain, nausea/vomiting, exercise intolerance, dizziness, or syncope.

Physical Examination

Clinical findings may include hypotension, cool extremities with poor peripheral perfusion, a thready pulse, and decreased urine output. Chemical evidence of renal and liver dysfunction may be present, as well as a diminished level of consciousness. Children with uncompensated congestive heart failure, particularly older children, generally have a lower cardiac output than that which most experienced clinicians would estimate on the basis of the clinical signs.

Signs and symptoms of congestive heart failure include the following:

Tachycardia
Venous congestion - Right-sided (hepatomegaly, ascites, abdominal pain, pleural effusion, edema, jugular venous distention); left-sided (tachypnea, retractions, nasal flaring or grunting, rales, pulmonary edema)
Low cardiac output - Fatigue or low energy, pallor, sweating, cool extremities, nausea/vomiting, poor growth, dizziness, altered consciousness, and syncope

Workup

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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