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Official reprint from UpToDate www.uptodate.com ©2015 UpToDate

AuthorsRakesh K Singh, MD, MSTP Singh, MD, MSc

Section EditorsJohn K Triedman, MDDavid R Fulton, MD

Deputy EditorCarrie Armsby, MD, MPH

Management of heart failure in infants and children

All topics are updated as new evidence becomes available and our peer review process is complete.Literature review current through: Jun 2015. | This topic last updated: Feb 11, 2015.

INTRODUCTION — Heart failure is estimated to affect 12,000 to 35,000 children below the age of 19 years in theUnited States each year [1]. It results from any structural or functional cardiac disorder that impairs the ability ofthe ventricle(s) to fill with or eject blood.

The management of children with heart failure will be presented here. The etiology, presentation, diagnosis, andinitial evaluation of the pediatric patient with heart failure are discussed separately. (See "Etiology and diagnosis ofheart failure in infants and children".)

OVERVIEW — Because heart failure is a common clinical condition in adults, there is a substantial amount ofevidence­based data based on large, placebo­controlled clinical trials that guide management. Over the past 20years, management of heart failure in adults has shifted based on observations that heart failure and leftventricular (LV) systolic dysfunction activate sympathetic nervous and renin­angiotensin systems. This responseis initially physiologic (and compensatory), but persistent activation is maladaptive and contributes to progressiveLV dilation and dysfunction (remodeling), and worsening heart failure. Data from clinical trials have shown thatdrugs targeted to block the effects of neuro­hormonal activation not only reverse LV remodeling but also improvesurvival in patients with heart failure. (See "Overview of the therapy of heart failure due to systolic dysfunction",section on 'Pharmacologic therapy of HF'.)

However, the ability to conduct similar trials in children is not possible because the much lower prevalence rate ofpediatric heart failure does not allow for a sufficient number of patients to replicate these studies. As a result,treatment of heart failure in children is based on results provided by adult studies. This approach is justifiablebecause children in heart failure have neuro­hormonal changes [2­4] and systemic ventricular remodeling similar tothat described in adults with heart failure [5].

In 2004, the International Society of Heart and Lung Transplantation (ISHLT) published guidelines for the treatmentof heart failure in children primarily based on the adult literature [6]. Modifications for specific pediatric diagnoseswere recommended based on expert consensus that was largely informed by clinical experience, small caseseries, and physiological studies. The management approach for this review is based on these guidelines andadvances in heart failure therapy since the publication of these guidelines.

Goals of therapy — Therapeutic goals for children with heart failure are to relieve symptoms, decrease morbidity(including the risk of hospitalization), slow the progression of heart failure, and improve patient survival.

Management approach — The management of pediatric heart failure is dependent on its etiology and severity [6­8].

Etiology and pathophysiology — Management begins with a thorough assessment of the underlying causeof heart failure. The causes of pediatric heart failure can be divided into pathophysiologic categories (table 1). Thiscategorization helps guide the approach to management. (See "Etiology and diagnosis of heart failure in infantsand children", section on 'Pathophysiology and etiology'.)

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Heart failure due to ventricular pump dysfunction – Ventricular pump dysfunction and reduced ventricularcontractility lead to impaired ejection of blood from the ventricle. Ventricular systolic dysfunction may occurin children with structurally normal hearts or in those with congenital heart disease. Medical pharmacologic

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Severity of heart failure — The management of pediatric heart failure is dependent on the severity of heartfailure, which is defined by a staging system (stages A to D) developed by a writing committee on behalf of theAmerican College of Cardiology (ACC) and the American Heart Association (AHA), and modified for children bythe ISHLT (table 2) [6]. This schema identifies asymptomatic patients in the early stages of heart failure (ie, stageB) in whom early intervention, such as ACE inhibitors, may prolong the symptom­free state, as well as determinewhich patients require more aggressive therapy. (See "Etiology and diagnosis of heart failure in infants andchildren", section on 'Staging of the progression of HF' and 'Our approach' below.)

COMPONENTS OF THERAPY — Components of therapy for children with heart failure include the following:

Surgical or catheter­based intervention — Surgical or catheter­based intervention can lead to resolution of heartfailure in patients with either volume or pressure overload, and preserved ventricular function (table 1). Volumeoverload is associated with lesions with significant left­to­right shunting (eg, large ventricular septal defects andatrioventricular canal defects), whereas pressure overload is seen in patients with ventricular outlet obstruction

therapy is generally the primary intervention. Drug choice is dependent on the severity of heart failure andmay include angiotensin­converting enzyme (ACE) inhibitors or angiotensin II receptor blockers (ARB),diuretics, aldosterone antagonists, digoxin, and beta­blockers. In patients with advanced heart failurerefractory to pharmacologic therapy, interventions such as positive pressure ventilation, mechanicalcirculatory support, and heart transplantation may be considered. (See 'Pharmacologic therapy' below.)

Heart failure due to volume overload with preserved ventricular pump function – Volume overload (increasedpreload) may occur due to a significant left­to­right shunt from the systemic to the pulmonary circulation (eg,ventricular septal defect or atrioventricular canal defects) or, less commonly, due to valvular insufficiency.Surgical or catheter­based interventions to correct these defects lead to resolution of heart failure. Medicaltherapy may be needed for stabilization or symptom relief while awaiting a more definitive intervention.

Heart failure due to pressure overload with preserved ventricular pump function – Pressure overload(increased afterload) may lead to heart failure if severe ventricular outflow obstruction impedes ejection ofblood from the heart, resulting in inadequate cardiac output (eg, aortic stenosis), or if ventricular hypertrophydue to prolonged pressure overload results in high filling pressures and congestion (eg, hypertrophiccardiomyopathy). Surgical or catheter­based interventions to correct these defects lead to resolution of heartfailure. Medical therapy may be needed for stabilization or symptom relief while awaiting a more definitiveintervention.

Identification and correction of noncardiac factors that contribute to cardiac dysfunction. In the acute setting,these may include acidosis and sepsis. Other factors that may be associated with either acute or chronicheart failure include anemia, hypertension, and renal failure. (See "Approach to anemia in adults with heartfailure" and "Treatment of hypertension in patients with heart failure" and "Etiology and diagnosis of heartfailure in infants and children", section on 'Noncardiac causes' and "Etiology and diagnosis of heart failure ininfants and children", section on 'Structurally normal heart'.)

Surgical or catheter­based interventions to correct the underlying structural defect (eg, significant left­to­rightshunt or ventricular outflow obstruction).

Pharmacologic therapy to relieve symptoms, slow the progression of ventricular dysfunction, and improvepatient survival.

Additional therapeutic interventions for patients with advanced heart failure refractory to pharmacologictherapy include positive pressure ventilation, mechanical circulatory support, and heart transplantation.

Nonpharmacologic therapy for chronic heart failure includes optimal nutrition and exercise rehabilitation.

Therapeutic interventions to reduce the risk of and/or treat associated complications (eg, thromboembolism,arrhythmias, and ventricular dyssynchrony).

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(eg, critical aortic stenosis and coarctation of the aorta). (See "Etiology and diagnosis of heart failure in infants andchildren", section on 'Preserved ventricular pump function with volume overload' and "Etiology and diagnosis ofheart failure in infants and children", section on 'Preserved ventricular pump function with pressure overload'.)

Pharmacologic therapy

Overview — Pharmacologic therapy is primarily used in patients with ventricular pump dysfunction. Drugtherapy is also used initially to stabilize and relieve symptoms in patients with preserved ventricular function whoare awaiting correction of the underlying defect that results in either volume or pressure overload.

Data from adults with heart failure have shown that a number of drugs provide symptom relief, and improvement inpatient outcome and cardiac functions. (See "Overview of the therapy of heart failure due to systolic dysfunction",section on 'Pharmacologic therapy of HF' and "Treatment of acute decompensated heart failure: Components oftherapy".)

Based on this evidence in adults, these drugs are used in children with heart failure, if no contraindications to theiruse are identified. These drugs are discussed in detail in the following sections.

Diuretics — Diuretics decrease preload by promoting natriuresis, and provide relief of volume overloadsymptoms such as pulmonary and peripheral edema. Diuretics are used to treat children with stage C or D heartfailure (table 2).

Improvement in symptoms can be achieved by diuretics, digoxin, angiotensin­converting enzyme (ACE)inhibitors, and angiotensin II receptor blockers (ARBs).

Prolongation of patient survival has been documented with beta­blockers, ACE inhibitors, ARBs, andaldosterone antagonists.

Improvement in left ventricular (LV) function and reversal of LV dilation, described as reversal of LVremodeling, is seen with the chronic use of ACE inhibitors, ARBs, beta­blockers, and aldosteroneantagonists.

Hospitalized patients with end­stage heart failure awaiting heart transplant may require intravenous inotropicand/or diuretics because they are refractory to oral medical therapy. (See 'Drug therapy for advanced heartfailure' below.)

Loop diuretics – Loop diuretics inhibit sodium and chloride reabsorption in the thick ascending limb of the loopof Henle. Furosemide is the most commonly used loop diuretic. A study of 62 hospitalized children with heartfailure and fluid overload demonstrated the efficacy and safety of furosemide [9]. Bumetanide and torsemideare more potent drugs, which are used less frequently and reserved for more severe or furosemide­resistantfluid overload. Side effects of loop diuretics include electrolyte abnormalities (hyponatremia, hypochloremia,and hypokalemia), metabolic alkalosis, and renal insufficiency. Long­term therapy can lead tonephrocalcinosis and ototoxicity (usually with high intravenous dosages) [10].

Thiazide diuretics – Thiazide diuretics inhibit reabsorption of sodium and chloride ions from the distalconvoluted tubules of kidneys. They generally are used as second­line agents and often in combination witha loop diuretic. Commonly used thiazide diuretics are chlorothiazide, hydrochlorothiazide, and metolazone.

Aldosterone antagonists – Aldosterone antagonists decrease sodium reabsorption and potassium excretion inthe collecting ducts of kidneys. Their potassium­sparing diuretic effect makes them particularly suitable foruse in conjunction with loop diuretics and thiazides. Both spironolactone and eplerenone have been shown toreduce mortality in adults with heart failure when added to standard therapy [11,12]. This effect isindependent of their diuretic effect and is mediated by inhibition of myocardial fibrosis, an importantcomponent of LV remodeling [13]. Side effects include hyperkalemia (with both) and gynecomastia (withspironolactone).

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Digoxin — Digoxin has a positive inotropic effect (mediated by Na+/K+ ATPase inhibition and increase inintracellular Ca+), a negative chronotropic effect that slows atrial conduction, and vagotonic properties that countersymptoms and signs mediated by the activation of the sympathetic nervous system in heart failure [14]. Digoxinused to be the mainstay of heart failure management before the 1990s, but its role became controversial after itwas found not to reduce mortality in adults with heart failure [15,16]. Although digoxin is no longer used in childrenwith asymptomatic ventricular dysfunction, it continues to be used in the treatment of infants and children withstage C heart failure because of its physiologic benefit and symptom relief. These benefits are generally seen witha smaller dose (trough level 0.5 to 1 ng/mL) than was used in the past. Potential adverse effects (arrhythmias) arerare with this lower level.

Renin­angiotensin­aldosterone system inhibition — Heart failure leads to activation of the renin­angiotensin­aldosterone system (RAAS) and increased sympathetic tone. ACE inhibitors and ARBs inhibit theRAAS, thereby decreasing afterload and promoting reversal of ventricular remodeling with long­term use.

Angiotensin­converting enzyme inhibitors — ACE inhibitors inhibit the formation of angiotensin II, apotent vasoconstrictor that also promotes myocyte hypertrophy, fibrosis, and aldosterone secretion [6]. Thus, ACEinhibitors benefit patients in heart failure first by reducing afterload, improving cardiac output, and, on chronic use,by mediating reversal of LV remodeling. Clinical trials in adults have shown that ACE inhibitors improve survival inpatients with symptomatic heart failure and reduce the rate at which asymptomatic patients with severe LVdysfunction develop symptomatic heart failure. (See "ACE inhibitors in heart failure due to systolic dysfunction:Therapeutic use", section on 'General efficacy' and "Angiotensin converting enzyme inhibitors and receptorblockers in heart failure: Mechanisms of action".)

Prospective studies with survival (or mortality) as their endpoint have not been possible in children with heartfailure. Nevertheless, ACE inhibitors are accepted as an important component of heart failure therapy in children.The mixed results in the following pediatric studies discussed should be cautiously interpreted because oflimitations in study design, including small sample size (power), length of follow­up, and surrogate endpoints.

Based on current evidence from adult trials and pediatric studies, most pediatric heart failure experts use ACEinhibitor therapy in children with ventricular pump dysfunction (stage B or C heart failure) (table 2). Blood pressureand renal function should be closely monitored, especially in neonates [25].

Angiotensin receptor blockers — In children with heart failure, there is a paucity of data on the use ofARBs, which block the angiotensin receptor. Thus, ACE inhibitors are the preferred class of drugs for inhibition of

In one small study of 16 patients, ACE inhibitors improved cardiac function in children with systemicventricular pump dysfunction [17].

A retrospective analysis of 81 children with dilated cardiomyopathy showed that treatment with captopril wasassociated with better survival during the first year of treatment [18].

A randomized, controlled study of enalapril in 230 infants with single ventricle anatomy and predominantlynormal systemic ventricular function did not show any difference between the enalapril and placebo groups insomatic growth, ventricular function, heart failure severity, or death after one year of therapy [19].

Children with mitral or aortic regurgitation treated with ACE inhibitors have shown inconsistent improvementin LV function, dilatation, and hypertrophy [20,21].

In a study of 17 children treated with anthracycline chemotherapy, enalapril was associated only withtransient early beneficial effects on LV function [22]. Despite continued enalapril therapy, deterioration in LVfunction and fractional shortening occurred between 6 and 10 years.

In a randomized, double­blinded trial in children with Duchenne muscular dystrophy (DMD), although groupstreated with perindopril and placebo had similar LV function after three years (when the trial stopped), thosetreated with perindopril had late beneficial effects on LV function at 5 and 10 years [23,24].

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the RAAS. ARBs are usually reserved for patients unable to tolerate ACE inhibitors due to cough or angioedema.

Beta­blockers — Beta­blockers counteract the maladaptive effects of chronic sympathic activation of themyocardium. In adults with heart failure, they improve patient survival, reverse LV remodeling, and decreasemyocardial fibrosis. (See "Overview of the therapy of heart failure due to systolic dysfunction", section on 'Betablockers'.)

Although mixed results have been reported in the following pediatric studies, most experts in the field use beta­blockers for children with stage C heart failure (table 2). Results need to be cautiously interpreted because oflimitations in study design including small sample size (power), length of follow­up, and surrogate endpoints.

Based on the current evidence in adult and pediatric patients, most pediatric heart failure experts use carvedilol inchildren with a systemic LV and systolic dysfunction in stage C heart failure who are stable on other heart failuremedications (table 2). Beta­blockers are usually added to an established regimen of diuretics, digoxin, and an ACEinhibitor.

Carvedilol dosing is initiated at a low dose (approximately one­eighth of the eventual target dose, usually an oraldose of 0.05 mg/kg per dose given twice a day) and increased every two weeks to minimize side effects. Ingeneral, the dose is doubled after observing the response to the new higher test dose in clinic to a maximum oraldose of 0.4 mg/kg given twice a day. Side effects that may preclude dose increase include dizziness, fatigue,hypotension, bradycardia, bronchospasm, and hypoglycemia. Beta­blockers are discontinued in patients withdecompensated heart failure (stage D).

Pulmonary vasodilators — Sildenafil, a phosphodiesterase­5 inhibitor, is a pulmonary vasodilator. Its use hasbeen associated with improved LV function, functional capacity, and quality of life in adults with systolic LVdysfunction and secondary pulmonary hypertension [37]. Larger, controlled studies are underway in adults withheart failure and secondary pulmonary hypertension to assess the effect of these drugs on patient survival.Although sildenafil was shown to improve symptoms of heart failure in 13 children with failing Fontan physiology[38], it remains an investigational drug.

Drug therapy for advanced heart failure — Intravenous diuretics and inotropic agents are generally used inhospitalized patients with stage D heart failure (table 2).

In a retrospective multicenter study of 15 children with chronic heart failure, metoprolol was associated witha significant increase in ejection fraction from 27 to 41 percent [26].

In several small observational studies, carvedilol therapy has been associated with improved symptoms,improvement in ventricular function, and delay in time to transplant or death in children with heart failure [27­30]. Carvedilol therapy has also been shown to preserve LV function after exposure to anthracyclines at sixmonths follow­up [31], and to improve LV function when added to ACE inhibitor therapy in patients with DMDand dilated cardiomyopathy [32].

In a multicenter trial of 161 children with heart failure and ventricular pump dysfunction, there was nodifference in the composite endpoint between groups treated with carvedilol and placebo [33]. However, thestudy was thought to be underpowered as the clinical course of all children enrolled was better thanexpected. There was a trend towards clinical improvement in children with a systemic LV, but not in thosewith a systemic right ventricle (RV), suggesting that the response to carvedilol may be affected by themorphology of the child’s systemic ventricle [34].

In a large, retrospective multicenter review of the Pediatric Health Information System (PHIS) database, abeta­blocker was prescribed upon discharge in 37 percent of pediatric patients admitted with acutedecompensated heart failure [35].

A 2009 Cochrane review of beta­blocker therapy in children with heart failure concluded there were notenough data to recommend or discourage its use [36].

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Inotropes — Inotropic agents are used during acute exacerbations of heart failure to improve cardiac outputand to stabilize patients awaiting heart transplantation. Their effect is mediated through higher intracellular cyclicadenylate monophosphate (cAMP) levels, either by increased production (catecholamines) or by decreaseddegradation (phosphodiesterase III inhibition).

Catecholamines — Sympathomimetic stimulation by catecholamine agents improves myocardialcontractility and may have an additional beneficial effect on peripheral vascular beds [39]. Dopamine is thepreferred drug during decompensated heart failure (usually in combination with intravenous milrinone), althoughdobutamine has the additive effect of reducing afterload. Invasive monitoring of arterial blood pressure and centralvenous pressure may allow dose titration for optimal end­organ perfusion, as measured by urine output, serumlactate, and mixed venous saturations. (See "Use of vasopressors and inotropes", section on 'Dopamine' and"Use of vasopressors and inotropes", section on 'Dobutamine'.)

Milrinone — Intravenous milrinone, a phosphodiesterase III inhibitor, is the preferred drug indecompensated heart failure, as it increases contractility and reduces afterload without a significant increase inmyocardial oxygen consumption [40]. A randomized, double­blind, placebo­controlled trial in pediatric postoperativecardiac surgery patients demonstrated that children treated with high­dose milrinone infusion (0.75 mcg/kg/min)were at a lower risk for the development of low cardiac output syndrome (LCOS) compared with children treatedwith placebo (12 versus 26 percent) [41].

To avoid hypotension, milrinone is initially administered as an infusion starting at a dose of 0.25 mcg/kg/min(without a pre­infusion bolus) and titrated upwards slowly as needed to a maximum dose of 1 mcg/kg/min.

Although milrinone is usually used in hospitalized children with heart failure refractory to oral drug therapy awaitingheart transplantation, a small study of 14 patients reported the safe administration of intravenous milrinone athome in children awaiting heart transplants [42]. Several centers (including ours) use home milrinone in selectedchildren awaiting heart transplantation. In our practice, home milrinone infusion therapy is used in children who areclinically stable without end­organ dysfunction, with no history of arrhythmias, who generally are on a milrinonedose ≤0.5 mcg/kg/min and a stable regimen of oral diuretic therapy, and who are under continuous adultsupervision.

Nesiritide — Nesiritide is a recombinant B­type natriuretic peptide that reduces preload and afterload bypromoting diuresis, natriuresis, and arterial­ and veno­dilation, thereby improving cardiac output without a directinotropic effect on the myocardium. It has been reported to be well tolerated and efficacious in children asillustrated in a prospective, open­label study in 63 children with refractory heart failure that showed nesiritide wasassociated with improved urine output, serum creatinine, and cardiac function [43,44]. However, trials in adultswith acute decompensated heart failure have failed to show that nesiritide is associated with lower mortality orrehospitalization rate, or that it improves dyspnea. In addition, there is an associated increased risk ofhypotension. As a result, nesiritide is not recommended for general use in acute heart failure. (See "Nesiritide inthe treatment of acute decompensated heart failure".)

Nonpharmacologic interventions for advanced heart failure — Therapeutic interventions for selected patientswith advanced heart failure refractory to pharmacologic therapy (stage D) may include:

Positive pressure ventilation — Noninvasive positive pressure ventilation (NPPV), such as continuouspositive airway pressure (CPAP) or bilevel positive airway pressure (BiPAP) ventilation, is effective in alleviatingrespiratory distress from cardiogenic pulmonary edema. It promotes alveolar recruitment, improves lungcompliance, and leads to decreased left ventricular preload and afterload [45]. Although there is high qualityevidence of the benefit of NPPV in adult patients with cardiogenic pulmonary edema, there are no pediatric studiesevaluating its use in heart failure. (See "Noninvasive positive pressure ventilation in acute respiratory failure in

Positive pressure ventilationMechanical circulatory support in patients with end­stage heart failureHeart transplantation

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adults".)

Mechanical circulatory support — In a child with decompensated heart failure with low cardiac outputsyndrome unresponsive to medical therapy, mechanical circulatory support (MCS) can be life­saving. MCSmaintains end­organ function and reduces myocardial oxygen requirements. It is used as a bridge to recovery(extracorporeal membrane oxygenation [ECMO]) in patients with secondary cardiomyopathy or to hearttransplantation (ECMO or ventricular assist device [VAD]). In a report from the International Society for Heart andLung Transplantation (ISHLT), 25 percent of pediatric heart transplant recipients received mechanical circulatorysupport as a bridge to transplantation [46].

The options include the following:

The choice of device depends on the etiology of heart failure, the patient’s cardiac anatomy, the expected length ofsupport, the availability of devices, and the expertise of the center’s clinicians. Serious complications associatedwith ECMO and VAD include bleeding (eg, gastrointestinal and intracranial hemorrhage), thromboembolism (eg,stroke), and infection. (See "Circulatory assist devices: Cardiopulmonary assist device and short­term leftventricular assist devices".)

Heart transplantation — Heart transplantation is recommended for end­stage heart failure refractory tomedical therapy (stage D). It may also be considered for less severe heart failure (stage C) associated with severelimitation of activity, significant growth failure, intractable arrhythmias, or restrictive cardiomyopathy [52]. Thecurrent survival in pediatric recipients 1, 5, and 10 years after transplantation is approximately 90, 80, and 60percent, respectively [46]. Early referral to a pediatric heart failure and transplant center should be considered tooptimize medical therapy and the timing of listing for heart transplant. Decision to pursue heart transplantation isbased upon the expected survival with medical therapy, quality of life, alternative options for treatment, andestimation of survival post­transplantation. (See "Indications and contraindications for cardiac transplantation".)

Nonpharmacologic therapy for chronic heart failure — Nonpharmacologic therapy for chronic heart failureincludes optimal nutrition and exercise rehabilitation.

Nutrition — Growth failure is common in infants and children with heart failure. Nearly one­third of childrenwith cardiomyopathy manifest growth failure during the course of their illness [53]. A retrospective analysis of 165children with idiopathic dilated cardiomyopathy showed that nutritional status positively and independentlycorrelated with survival [54].

Caloric intake and growth should be carefully assessed in infants and children with heart failure. Some childrenmay need a daily intake greater than 120 kcal/kg for optimal growth because of increased metabolic demands of

Extracorporeal membrane oxygenation (ECMO) is a total heart­lung bypass device and is used in the settingof imminent or actual cardiac arrest, such as postcardiotomy shock following cardiac surgery and acutemyocarditis. Cannulation can be performed percutaneously, and ECMO can provide full cardiopulmonarysupport for days to weeks. A multicenter registry review of 3416 neonatal and 4181 pediatric cardiac ECMOcases showed a survival to discharge rate of 38 and 45 percent, respectively [47]. If myocardial recoverydoes not occur or is not expected to occur within two to three weeks, ECMO may be used as a bridge to amore durable ventricular assist device placement and subsequent heart transplantation.

Ventricular assist device (VAD), a cardiac­only support device, can offer either univentricular or biventricularsupport. Multiple devices currently exist and differ by flow design (pulsatile, centrifugal, or axial), pumplocation relative to patient (implantable, paracorporeal, or extracorporeal), and delivery system (percutaneousor central) [48]. They are primarily used in patients awaiting heart transplantation and have yielded favorableresults [49]. A retrospective, multicenter analysis of 99 children supported by VAD as a bridge totransplantation showed a 77 percent survival to transplant [50]. VAD options are limited in small childrenawaiting heart transplantation due to body size and anatomic considerations, though new devices arecurrently under development [51].

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heart failure. In order to provide adequate caloric intake, intermittent or continuous nasogastric or gastrostomy tubefeeds may be required.

In addition, salt and fluid restriction is recommended in children with severe heart failure to reduce volumeoverload.

Exercise rehabilitation — Although cardiovascular rehabilitation programs have been shown to improveexercise performance, physical activity, and quality of life in adults with chronic heart failure [55], data in childrenare limited. In a single­center study, 15 of 16 children with complex congenital heart disease demonstratedimproved exercise performance after participation in a cardiac rehabilitation program [56]. Furthermore, a study of20 hospitalized children awaiting heart transplantation demonstrated that even those on inotropic support cansafely participate in exercise training programs with relatively moderate to high compliance [57]. Further studiesare needed to evaluate the long­term benefits of exercise rehabilitation in children with heart failure.

Therapy to reduce complications — Patients with heart failure are at risk for complications, which increasemorbidity and mortality. Complications include thromboembolism, arrhythmias, and ventricular dyssynchrony.

Thrombi formation — Children with heart failure due to systemic ventricular dysfunction are at risk for theformation of intracardiac thrombi, which may result in pulmonary embolus, cerebral embolic strokes, and, in somecases, death.

Although there are no controlled trials in children, most experts recommend using aspirin in children with moderateLV dysfunction, and warfarin or enoxaparin for children with severe LV dysfunction. Anticoagulation with aspirinshould also be considered in the presence of marked atrial dilation in children with restrictive cardiomyopathy. (See"Antithrombotic therapy in patients with heart failure", section on 'Role of antithrombotic therapy'.)

Arrhythmias — In patients with decreased ventricular function, sustained atrial and ventriculartachyarrhythmias can rapidly impair hemodynamics. In these patients, cardioversion or defibrillation may beneeded along with antiarrhythmic therapy. Ablation therapy also has a potential role in the setting of chronic atrialtachyarrhythmias. (See "Management and evaluation of wide QRS complex tachycardia in children" and "Atrialarrhythmias (including AV block) in congenital heart disease" and "Management of supraventricular tachycardia inchildren", section on 'Radiofrequency ablation'.)

Implantable cardioverter defibrillatory (ICD) therapy is used in select patients who are at risk for sudden cardiacdeath due to ventricular tachycardia and fibrillation. The indications for ICD placement are aborted sudden cardiacdeath, unexplained syncope, and recurrent, sustained ventricular dysrhythmias [58]. However, the risk of suddendeath in children with end­stage heart failure awaiting transplantation is only about 1 percent [59]. Unlike adults,there are no data to support when an ICD should be placed in children with ventricular dysfunction. Riskstratification for sudden cardiac death (SCD) in children with dilated cardiomyopathy may be useful in identifyingchildren who may benefit from this therapy [60]. (See "Primary prevention of sudden cardiac death in heart failureand cardiomyopathy", section on 'Risk stratification strategies'.)

Ventricular dyssynchrony — Intraventricular conduction delay or left bundle branch block (LBBB) may worsenheart failure by causing ventricular dyssynchrony. Cardiac resynchronization therapy (CRT) uses biventricularpacing to minimize ventricular dyssynchrony seen in patients with heart failure who usually have a prolonged QRSon electrocardiogram. In adult patients with LV dysfunction, heart failure, and LBBB, CRT has been shown toimprove hemodynamics and symptoms. Pediatric data are limited and include a multicenter, retrospective analysisof 103 children and young adults with congenital heart disease and prolonged QRS on electrocardiogram thatshowed CRT was associated with improvement of ventricular ejection fraction from 26 to 40 percent [61]. WhenCRT is considered for a pediatric patient, guidelines for use of CRT in adults are a useful reference [58]. (See"Cardiac resynchronization therapy in heart failure: Indications".)

OUR APPROACH — Our management approach for treating pediatric heart failure is consistent with the 2004International Society of Heart and Lung Transplantation (ISHLT) published guidelines [6]. (See 'Overview' above.)

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It is based on the etiology of the heart failure and the severity of heart disease, as follows:

SUMMARY AND RECOMMENDATIONS

For patients with structural heart disease with preserved ventricular pump function, surgical or catheter­basedinterventions are used to correct the underlying defects causing heart failure (ie, left­to­right heart shunts orventricular outflow obstruction). (See 'Etiology and pathophysiology' above and 'Surgical or catheter­basedintervention' above.)

For patients with ventricular pump dysfunction or those who require stabilization before surgical or catheter­based correction, therapy is provided based on the severity of heart failure (table 2).

Stage A – Patients at risk for heart failure with normal cardiac function and size: No therapeuticintervention.

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Stage B – Asymptomatic patients with abnormal systemic ventricular function: Angiotensin­convertingenzyme (ACE) inhibitors. (See 'Angiotensin­converting enzyme inhibitors' above.)

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Stage C – Patients with current or past symptoms and structural or functional heart disease: ACEinhibitors, aldosterone antagonists, beta­blockers, low dose digoxin, and oral diuretic therapy. (See'Pharmacologic therapy' above.)

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Stage D – Patients with end­stage heart failure who are refractory to oral medical therapy: Intravenousadministration of inotropes and diuretics. In addition, nonpharmacologic interventions include positivepressure ventilation, mechanical circulatory support, and subsequent heart transplantation. (See 'Drugtherapy for advanced heart failure' above and 'Nonpharmacologic interventions for advanced heartfailure' above.)

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Identification and correction of noncardiac factors that contribute to cardiac dysfunction. These includeanemia, hypertension, acidosis, renal failure, and sepsis.

For children with chronic heart failure, nonpharmacologic interventions include provision of adequate caloricintake for growth (which may entail nasogastric or gastrostomy tube feedings), and an exercise program toimprove physical activity level. (See 'Nonpharmacologic therapy for chronic heart failure' above.)

Patients with heart failure are at risk for thromboembolic disease, arrhythmia, and ventricular dyssynchrony.

To decrease the risk of intracardiac thrombi, we administer prophylactic aspirin to children withmoderate left ventricular (LV) dysfunction, and warfarin or enoxaparin for children with severe LVdysfunction. (See 'Thrombi formation' above.)

•

In patients with sustained atrial or ventricular tachyarrhythmias, interventions include cardioversionand/or antiarrhythmic therapy followed by ablation therapy in patients deemed suitable. Implantablecardioverter defibrillatory therapy may be considered in patients who remain at risk for sudden cardiacdeath (SCD) due to ventricular arrhythmias. (See 'Arrhythmias' above.)

•

In patients with ventricular dyssynchrony, cardiac resynchronization therapy may be used to improveventricular function. (See 'Ventricular dyssynchrony' above.)

•

Because of limited pediatric data, the management of children with heart failure is primarily based onevidence from clinical trials of adults with heart failure. (See 'Overview' above.)

The goals of therapeutic interventions for children with heart failure are to relieve symptoms, decreasemorbidity (including the risk of hospitalization), slow the progression of heart failure, and improve patientsurvival. (See 'Goals of therapy' above.)

Our management approach is based on the etiology and severity of heart failure, and is consistent with the2004 International Society of Heart and Lung Transplantation (ISHLT) published guidelines. (See

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REFERENCES

1. Hsu DT, Pearson GD. Heart failure in children: part I: history, etiology, and pathophysiology. Circ Heart Fail2009; 2:63.

2. Baylen BG, Johnson G, Tsang R, et al. The occurrence of hyperaldosteronism in infants with congestiveheart failure. Am J Cardiol 1980; 45:305.

3. Ross RD, Daniels SR, Schwartz DC, et al. Plasma norepinephrine levels in infants and children with

'Management approach' above and 'Our approach' above.)

For patients with structural heart disease and preserved ventricular pump function, we recommend surgical orcatheter­based interventions to correct the underlying defects responsible for heart failure (ie, left­to­rightheart shunts or ventricular outflow obstruction) (Grade 1A). (See 'Etiology and pathophysiology' above and'Surgical or catheter­based intervention' above.)

Patients with ventricular pump dysfunction or those who require stabilization before surgical or catheter­based intervention are treated with a combination of medications that alter afterload, preload, and myocardialcontractility, and may be effective with chronic use in reverse­remodeling of the systemic ventricle. For thesepatients, we suggest the following approach based on the severity of heart failure (table 2) (Grade 2B):

Stage A – Patients at risk for heart failure with normal cardiac function and size: No therapeuticintervention.

•

Stage B – Asymptomatic patients with abnormal systemic ventricular function: Angiotensin­convertingenzyme (ACE) inhibitors, which decrease afterload by blocking formation of angiotensin II and promotereversal of ventricular remodeling on long­term use. (See 'Renin­angiotensin­aldosterone systeminhibition' above.)

•

Stage C – Patients with current or past symptoms and structural or functional heart disease: ACEinhibitors, aldosterone antagonists, beta­blockers (counteract the maladaptive effects of chronicsympathic activation on the myocardium), low dose digoxin (improves symptoms with its positiveinotropic effect and vagotonic properties), and oral diuretic therapy (decreases preload by promotingnatriuresis). (See 'Renin­angiotensin­aldosterone system inhibition' above and 'Beta­blockers' aboveand 'Digoxin' above and 'Diuretics' above.)

•

Stage D – Patients with end­stage heart failure who are refractory to medical therapy: Intravenousadministration of inotropes (improve myocardial contractility) and diuretics. In addition,nonpharmacologic interventions include positive pressure ventilation, mechanical circulatory support,and subsequent heart transplantation. (See 'Drug therapy for advanced heart failure' above and'Nonpharmacologic interventions for advanced heart failure' above.)

•

In addition, medical care for children with heart failure includes identification and correction of noncardiacfactors that contribute to cardiac dysfunction such as anemia, hypertension, renal failure, and sepsis. (See'Components of therapy' above.)

For children with chronic heart failure, we recommend provision of adequate caloric intake for growth (whichmay entail nasogastric feedings) (Grade 1B). We also suggest an exercise program to improve physicalactivity level (Grade 2C). (See 'Nonpharmacologic therapy for chronic heart failure' above.)

Additional therapeutic interventions are used to prevent or treat complications associated with heart failuresuch as intracardiac thrombi, arrhythmias, and ventricular dyssynchrony. (See 'Therapy to reducecomplications' above.)

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GRAPHICS

Causes of heart failure in infants and children

Ventricular pump dysfunction

Structurally normal heart

Primary cardiomyopathy

Dilated

Hypertrophic

Restrictive

Non­compaction

Arrhythmogenic right ventricular dysplasia (ARVD)

Secondary cardiomyopathy

Myocarditis

Myocardial infarction/ischemia

Anomalous left coronary artery arising off the pulmonary artery (ALCAPA)

Arrhythmogenic

Complete heart block with bradycardia

Supraventricular or ventricular tachycardia

Drug/toxin exposure

Anthracycline

Noncardiac causes

Sepsis

Renal failure

Congenital heart disease

Complex congenital heart defect with concurrent ventricular dysfunction

Complex congenital heart defect, surgically corrected with late ventricular dysfunction("burnt­out" congenital heart disease)

Preserved ventricular pump function

Volume overload (increased preload)

Left­to­right shunting

Ventricular septal defect

Patent ductus arteriosus

Atrial septal defect (rare)

Aortopulmonary window

Atrioventricular septal defect

Single ventricle physiology with unobstructed pulmonary blood flow

Valvular insufficiency

Aortic regurgitation

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Mitral regurgitation

Pulmonary regurgitation

Pressure overload (increased afterload)

Left­sided lesions

Aortic stenosis

Aortic coarctation

Right­sided lesions

Pulmonary stenosis

Adapted from: Hsu DT, Pearson GD. Heart failure in children: part I: history, etiology, andpathophysiology. Circ Heart Fail 2009; 2:65.

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Stages of heart failure in infants and children and recommendedtherapy

Stage Definition Example Therapy

A Patients with increased riskof developing HF, but withnormal cardiac functionand chamber size

Exposure to cardiotoxicagents, family history ofheritable cardiomyopathy,univentricular heart,congenitally correctedtransposition of the greatarteries

None

B Patients with abnormalcardiac morphology orfunction, with nosymptoms of HF, past orpresent

Aortic insufficiency with LVenlargement, history ofanthracycline exposurewith decreased LV systolicfunction

ACE inhibitors for patientswith systemic ventriculardysfunction

C Patients with structural orfunctional heart disease,and past or currentsymptoms of HF

Symptomaticcardiomyopathy orcongenital heart defect withventricular pumpdysfunction

ACE inhibitors, aldosteroneantagonists and betablockers for remodelingreversal; low dose digoxinand diuretics for symptomcontrol

D Patients with end­stage HFrequiring specializedinterventions

Marked symptoms at restdespite maximal medicaltherapy

Intravenous diureticsand/or inotropes, positivepressure ventilation,mechanical circulatorysupport, hearttransplantation

HF: heart failure; LV: left ventricular; ACE inhibitors: angiotensin­converting enzyme inhibitors.

From: Rosenthal D, Chrisant MR, Edens E, et al. International Society for Heart and LungTransplantation: Practice guidelines for management of heart failure in children. J Heart Lung Transplant2004; 23:1313. Table used with the permission of Elsevier Inc. All rights reserved.

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Disclosures: Rakesh K Singh, MD, MS Nothing to disclose. TP Singh, MD, MSc Nothing to disclose.John K Triedman, MD Consultant/Advisory Boards: Biosense Webster [Ablation (EP mapping andablation devices)]; Boehringer Ingelheim [Anticoagulation (Dabigatran)]. David R Fulton, MD Nothing todisclose. Carrie Armsby, MD, MPH Nothing to disclose.Contributor disclosures are reviewed for conflicts of interest by the editorial group. When found, these areaddressed by vetting through a multi­level review process, and through requirements for references to beprovided to support the content. Appropriately referenced content is required of all authors and mustconform to UpToDate standards of evidence.Conflict of interest policy

Disclosures