Heart failure in children: Management

Authors: Rakesh K Singh, MD, MS, TP Singh, MD, MSc

Section Editor: John K Triedman, MD
Deputy Editor: Carrie Armsby, MD, MPH

Contributor Disclosures

All topics are updated as new evidence becomes available and our peer review process is complete.

Literature review current through: Oct 2023. | This topic last updated: Nov 29, 2022.

INTRODUCTION

Heart failure (HF) results from structural or functional cardiac disorders that impair the ability of
the ventricle(s) to fill with and/or eject blood. The presentation of pediatric HF is diverse
because of the numerous underlying cardiac etiologies ( table 1) and varying clinical settings.

The management of HF in children will be presented here. The etiology, clinical manifestations,
and diagnostic evaluation of HF in children are discussed separately. (See "Heart failure in
children: Etiology, clinical manifestations, and diagnosis".)

GENERAL MEASURES

General measures that can be applied to all pediatric patients with HF include correcting
reversible conditions that may be causing or contributing the HF symptoms, ensuring adequate
nutrition, and promoting healthy and safe exercise.

Reversible contributors — Patients with HF may have comorbidities that can exacerbate or
contribute to cardiac dysfunction. A thorough evaluation should be performed in all patients to
identify such contributors and appropriate therapy should be provided if warranted. Examples
include:

● Iron deficiency and anemia – Iron deficiency, with or without anemia, is common in
patients with HF and is associated with worse symptoms and clinical outcomes [1-5]. Iron
studies and appropriate therapy should be considered in all children with HF even in
absence of anemia. Patients with HF and comorbid iron deficiency may not respond
adequately to oral iron supplementation [6]. Thus, some patients may require intravenous
iron therapy. (See "Iron deficiency in infants and children <12 years: Treatment" and
 "Evaluation and management of anemia and iron deficiency in adults with heart failure",
section on 'Iron supplementation'.)

● Hypertension. (See "Nonemergent treatment of hypertension in children and adolescents".)

● Renal failure. (See "Chronic kidney disease in children: Overview of management".)

● Acidosis. (See "Approach to the child with metabolic acidosis".).

● Obesity. (See "Prevention and management of childhood obesity in the primary care
setting".)

● Malnutrition. (See 'Nutritional support' below and "Poor weight gain in children younger
than two years in resource-abundant settings: Management" and "Poor weight gain in
children older than two years in resource-abundant settings".)

● Respiratory disorders (eg, asthma, obstructive sleep apnea, interstitial lung disease). (See
"An overview of asthma management" and "Management of obstructive sleep apnea in
children" and "Approach to the infant and child with diffuse lung disease (interstitial lung
disease)".)

● Thyroid disorders (hypo- or hyperthyroid state) or adrenal insufficiency. (See "Acquired

hypothyroidism in childhood and adolescence" and "Clinical manifestations and diagnosis
of Graves disease in children and adolescents" and "Clinical manifestations and diagnosis
of adrenal insufficiency in children".)

Nutritional support — Caloric intake and growth should be carefully assessed in infants and
children with HF. Children with HF often have increased caloric needs due to an increased
metabolic demand. In addition, patients with HF often tire with feeding and their intake may be
limited. Some children may need a daily intake >120 kcal/kg for optimal growth. In order to
provide adequate caloric intake, intermittent or continuous nasogastric or gastrostomy tube
feeds may be required. In addition, salt and fluid restriction is recommended in children with
severe HF to reduce volume overload. (See "Overview of enteral nutrition in infants and
children".)

Growth failure and poor weight gain are common in infants and children with HF and are
associated with poor outcome [7,8]. Approximately one-quarter of children with
cardiomyopathy manifest growth failure during the course of their illness [8,9]. In a registry
study that analyzed data from >900 children with dilated cardiomyopathy, children with poor
weight gain (defined as body mass index or weight-for-height <5th percentile for age) had an
increased risk of death compared with children of normal weight (hazard ratio 2.1; 95% CI 1.7-
3.6) [8].

Exercise and physical activity — Promoting healthy and safe physical activity in patients with
HF is an important part of management. The challenge is to balance routine daily physical
activity while minimizing any potential risks from exercise. Recommendations should be
tailored for each individual based on his or her specific diagnosis and a comprehensive
assessment of the child's exercise capacity. This should generally include formal
cardiopulmonary exercise testing if feasible (ie, if the child is old enough to cooperate, typically
beginning around age 6 or 7 years). (See "Exercise testing in children and adolescents:
Principles and clinical application", section on 'Limitations in young children'.)

Physical activity and exercise in patients with congenital heart disease (CHD) are discussed in
greater detail separately. (See "Physical activity and exercise in patients with congenital heart
disease".)

In adults with chronic HF, cardiovascular rehabilitation programs have been shown to improve
exercise performance, physical activity, and quality of life [10]; however, data in children are
limited. In a single-center study, 15 of 16 children with complex CHD demonstrated improved
exercise performance after participation in a cardiac rehabilitation program [11]. Furthermore,
a study of 20 hospitalized children awaiting heart transplantation demonstrated that even
those on inotropic support can safely participate in exercise training programs with relatively
moderate to high compliance [12]. Further studies are needed to evaluate the long-term
benefits of exercise rehabilitation in children with HF.

APPROACH TO HF MANAGEMENT

The management of pediatric HF is dependent on its etiology and severity [13-15].

Management begins with a thorough assessment of the underlying cause of HF. The causes of
pediatric HF can be divided into pathophysiologic categories ( table 1). This categorization
helps guide the approach to management. (See "Heart failure in children: Etiology, clinical
manifestations, and diagnosis", section on 'Etiology and pathophysiology'.)

Our management approach is generally consistent with the 2014 International Society of Heart
and Lung Transplantation (ISHLT) guidelines for the treatment of HF in children, which are
primarily based on the adult literature [13]. Modifications for specific pediatric diagnoses were
recommended based on expert consensus that was largely informed by clinical experience,
small case series, and physiologic studies.
Goals of therapy — Therapeutic goals for children with HF are to relieve symptoms, decrease
morbidity (including the risk of hospitalization), slow the progression of HF, and improve
patient survival and quality of life.

Unstable patients — In patients who present with severe cardiorespiratory compromise (ie,
shock or impending cardiac arrest), prompt initiation of treatment to restore adequate
perfusion should be provided even if the underlying etiology is uncertain. Guidance for
management of shock is summarized in the figures and discussed in detail separately:

● For neonates ( algorithm 1) (see "Neonatal shock: Management")

● For older infants and children ( algorithm 2) (see "Shock in children: Initial management
in resource-abundant settings")

Structural heart disease with preserved ventricular function — For patients with preserved
ventricular function who have HF symptoms due to structural heart defects causing volume
overload (eg, septal defects, patent ductus arteriosus) or pressure overload (eg, pulmonic
stenosis, aortic stenosis, other right or left ventricular outflow tract obstruction) ( table 1), the
mainstay of management involves surgical or catheter-based interventions to correct the
underlying defects. Medical therapy may be needed for stabilization or symptom relief while
awaiting a more definitive intervention. (See 'Pharmacologic therapy' below.)

The appropriate intervention depends upon the specific defect. Common examples include:

● Atrial septal defects – Management involves surgical or percutaneous closure of the defect.
(See "Isolated atrial septal defects (ASDs) in children: Management and outcome", section
on 'Closure procedures'.)

● Ventricular septal defects – Surgical closure is generally preferred. (See "Management of

isolated ventricular septal defects (VSDs) in infants and children", section on 'Closure
interventions'.)

● Patent ductus arteriosus – Surgical ligation is used in young infants; percutaneous

occlusion is more commonly used in older infants and children. (See "Management of
patent ductus arteriosus (PDA) in term infants, children, and adults", section on
'Management approach'.)

● Valvular aortic stenosis – Percutaneous balloon aortic valvotomy is the therapy of choice.
(See "Subvalvar aortic stenosis (subaortic stenosis)".)
 ● Pulmonic stenosis – Percutaneous balloon valvotomy is usually performed. (See "Pulmonic
stenosis in infants and children: Management and outcome".)

Interventions for other congenital heart defects are discussed in separate topic reviews.

Impaired ventricular function — For patients with ventricular systolic dysfunction or those
who require stabilization before surgical or catheter-based correction, therapy is provided
based on the stage of HF ( table 2):

● Stage A – For patients at risk for HF who have normal cardiac function and size, we
recommend not treating with HF-specific therapies. Predisposing conditions should be
treated if possible, as previously discussed. (See 'Reversible contributors' above.)

● Stage B – For asymptomatic patients with abnormal systemic ventricular function,

pharmacologic therapy consists of angiotensin-converting enzyme (ACE) inhibitors.
Angiotensin II receptor blockers (ARBs) can be used in patients who are intolerant of ACE
inhibitors. (See 'Renin-angiotensin-aldosterone system inhibition' below.)

Based on adult guidelines, beta blockers can be considered in children with asymptomatic
systolic LV dysfunction. It is important to note, however, that adult studies to support this
recommendation are limited to patients with ischemic heart disease. (See 'Beta blockers'
below.)

● Stage C – For patients with current or past symptoms and structural or functional heart
disease, we suggest initial treatment with an ACE inhibitor plus a mineralocorticoid
receptor antagonist. Oral diuretic therapy should be provided as needed for fluid overload.
After a few weeks of stability, a beta blocker is usually added in patients with persistent left
ventricle dilation and dysfunction. Low-dose digoxin can be added if needed for additional
symptom relief. (See 'Pharmacologic therapy' below.)

For patients with stage C HF that is associated with severe limitation of activity, significant
growth failure, intractable arrhythmias, or restrictive cardiomyopathy, early referral to a
pediatric transplant center should be considered to optimize medical therapy and the
timing of listing for heart transplant. (See 'Heart transplantation' below.)

● Stage D – Interventions for patients with end-stage HF who are refractory to oral medical
therapy may include intravenous administration of inotropes and diuretics and
nonpharmacologic interventions such as positive pressure ventilation, cardiac
resynchronization therapy (CRT), mechanical circulatory support, and heart transplantation.
 Some patients present in stage D (decompensated state) and after initial acute therapy
with inotropes may be able to transition to oral HF therapy as described above for patients
in stage C. (See 'Drug therapy for advanced HF' below and 'Nonpharmacologic
interventions for advanced HF' below.)

PHARMACOLOGIC THERAPY

Pharmacologic therapy is primarily used in patients with ventricular pump dysfunction. Drug
therapy is also used initially to stabilize and relieve symptoms in patients with either volume or
pressure overload with preserved ventricular function who are awaiting correction of the
underlying defect.

Evidence for efficacy — For most of the agents used in the management of children with HF,
the evidence supporting efficacy comes largely from adult studies. Because HF is common in
adults, there is a substantial amount of clinical trial data to guide management decisions.
Treatment strategies are based on observations that left ventricular (LV) systolic dysfunction
activates sympathetic nervous and renin-angiotensin systems. This response is initially
physiologic (and compensatory), but persistent activation is maladaptive and contributes to
progressive LV dilation and dysfunction (remodeling), and worsening HF. Data from clinical
trials have shown that drugs targeted to block the effects of neuro-hormonal activation (eg,
beta blockers, angiotensin-converting enzyme [ACE] inhibitors) not only reverse LV remodeling
but also improve survival in patients with HF. (See "Overview of the management of heart
failure with reduced ejection fraction in adults" and "Primary pharmacologic therapy for heart
failure with reduced ejection fraction", section on 'Management of specific agents'.)

However, the prevalence of HF in the pediatric population is substantially lower than in adults
which limits the ability to conduct similar trials in children. As a result, treatment of HF in
children is based in large part on indirect evidence from adult studies. This approach is
supported by observations that children with HF have neuro-hormonal changes and systemic
ventricular remodeling similar to that described in adults with HF [16-19].

Specific agents

Diuretics — Diuretics decrease preload by promoting natriuresis, and provide relief of volume
overload symptoms such as pulmonary and peripheral edema. Diuretics are used to treat
children with stage C or D HF ( table 2):
 ● Loop diuretics – Loop diuretics inhibit sodium and chloride reabsorption in the thick
ascending limb of the loop of Henle. Furosemide is the most commonly used loop diuretic.
A study of 62 hospitalized children with HF and fluid overload demonstrated the efficacy
and safety of furosemide [20]. Bumetanide and torsemide are more potent drugs, which
are used less frequently and reserved for more severe or furosemide-resistant fluid
overload. Side effects of loop diuretics include electrolyte abnormalities (hyponatremia,
hypochloremia, and hypokalemia), metabolic alkalosis, and renal insufficiency. Long-term
therapy can lead to nephrocalcinosis and ototoxicity. These complications occur most
commonly with prolonged high-dose intravenous (IV) therapy [21]. Increased risk of bone
fractures has also been reported [22].

● Thiazide diuretics – Thiazide diuretics inhibit reabsorption of sodium and chloride ions
from the distal convoluted tubules of kidneys. They generally are used as second-line
agents and often in combination with a loop diuretic. Commonly used thiazide diuretics are
chlorothiazide, hydrochlorothiazide, and metolazone.

Renin-angiotensin-aldosterone system inhibition — HF leads to activation of the renin-

angiotensin-aldosterone system (RAAS) and increased sympathetic tone. Agents that block
RAAS activation include ACE inhibitors, angiotensin II receptor blockers (ARBs), and angiotensin
receptor-neprilysin inhibitors (ARNIs). These agents decrease afterload and promote reversal of
ventricular remodeling.

ACE inhibitors — ACE inhibitors are an accepted first-line component of therapy for
children with stage B and C HF ( table 2).

ACE inhibitors are generally well tolerated in children and adverse effects are uncommon [23].
Blood pressure and renal function should be monitored when initiating therapy, especially in
neonates [24].

Clinical trials in adults have shown that ACE inhibitors improve survival in patients with
symptomatic HF and reduce the rate at which asymptomatic patients with severe LV
dysfunction develop symptomatic HF. (See "Primary pharmacologic therapy for heart failure
with reduced ejection fraction", section on 'Sacubitril-valsartan, ACE inhibitor, or ARB'.)

Clinical trial data on the use of ACE inhibitors in children are limited and have been difficult to
interpret due to healthier baseline cohorts, smaller sample size, and shorter duration of follow-
up compared with adult studies.
In a randomized, placebo-controlled, crossover trial of enalapril in 18 subjects (mean age 14
years old) who were 4 to 19 years post-Fontan operation, the use of enalapril for 10 weeks was
not associated with change in cardiac function or exercise performance [25]. In a subsequent
randomized, placebo-controlled study of enalapril in 230 infants with single ventricle anatomy
and predominantly normal systemic ventricular function, somatic growth, ventricular function,
HF severity, and one-year mortality were similar in the enalapril and placebo groups [26]. In
another randomized placebo-controlled trial of perindopril in 57 children with Duchenne
muscular dystrophy (DMD) with normal LV ejection fraction (LVEF), LV function was similar in
both groups at the trial's end (three years) [27]. However, fewer patients in the perindopril
group had LVEF <45 percent at five years (4 versus 28 percent) and fewer deaths were observed
in the perindopril group at 10 years (7 versus 34 percent) [27,28]. The small size of the study
limits drawing a firm conclusion regarding a possible mortality benefit.

Small nonrandomized trials and observational studies in other types of pediatric HF patients
have demonstrated physiologic benefits and improvements in short-term outcomes [29-31].
Other studies have found no benefit or only limited efficacy [32,33].

Despite the inconsistencies and limitations of the pediatric data, it is reasonable to believe that
the well-established benefits of ACE inhibitors in adult HF patients likely apply to pediatric
patients as well.

ACE inhibitors inhibit the formation of angiotensin II, a potent vasoconstrictor that also
promotes myocyte hypertrophy, fibrosis, and aldosterone secretion [13]. Thus, ACE inhibitors
benefit patients in HF first by reducing afterload, improving cardiac output, and, on chronic use,
by mediating reversal of LV remodeling. (See "Pharmacologic therapy of heart failure with
reduced ejection fraction: Mechanisms of action", section on 'ACE inhibitors'.)

Angiotensin II receptor blockers (ARBs) — In children with HF, there is a paucity of data
on the use of ARBs, which block the angiotensin receptor (eg, candesartan, losartan, valsartan).
Thus, ACE inhibitors are the preferred class of drugs for inhibition of the RAAS. ARBs are usually
reserved for patients unable to tolerate ACE inhibitors due to cough or angioedema. (See
"Pharmacologic therapy of heart failure with reduced ejection fraction: Mechanisms of action",
section on 'Angiotensin II receptor blockers' and "Primary pharmacologic therapy for heart
failure with reduced ejection fraction", section on 'Sacubitril-valsartan, ACE inhibitor, or ARB'.)

Angiotensin receptor-neprilysin inhibitor (ARNI) — The combination drug sacubitril-

valsartan (a neprilysin inhibitor plus an ARB) is the only agent in this class. Sacubitril-valsartan
has been shown to reduce mortality when compared with enalapril in adults with HF. The
PANORAMA-HF trial was designed to evaluate the efficacy and safety of sacubitril-valsartan in
pediatric patients with HF due to systolic dysfunction (LVEF ≤40 percent) [34,35]. The trial
enrolled 360 patients who were randomly assigned to sacubitril-valsartan or enalapril. Patients
are being followed for 52 weeks and the full results of the trial are not yet available. Based on
preliminary results from the PANORAMA-HF trial, the US Food and Drug Administration (FDA)
approved sacubitril-valsartan for use in children ≥1 year old in 2019 [36]. The FDA's approval of
this drug for pediatric use was based upon an unpublished analysis of 110 pediatric patients
enrolled in the PANORAMA-HF trial, the results of which are available only on the FDA label [36].
At 12 weeks, patients treated with sacubitril-valsartan had greater reduction in plasma N-
terminal pro-B-type natriuretic peptide (NT-proBNP) levels compared with those receiving
enalapril (44 versus 33 percent, respectively); however, the difference was not statistically
significant. According to the FDA label, adverse reactions observed in pediatric patients treated
with sacubitril-valsartan were similar to those observed in adult patients (hypotension and
hyperkalemia, being the most common). Additional data from the complete trial will be
important to fully assess the safety and efficacy of this agent in pediatric patients. (See
"Pharmacologic therapy of heart failure with reduced ejection fraction: Mechanisms of action",
section on 'Angiotensin receptor-neprilysin inhibitor (ARNI)' and "Primary pharmacologic
therapy for heart failure with reduced ejection fraction", section on 'Sacubitril-valsartan, ACE
inhibitor, or ARB'.)

Mineralocorticoid receptor antagonists — MRAs (eg, spironolactone, eplerenone) decrease

sodium reabsorption and potassium excretion in the collecting ducts of kidneys. Their
potassium-sparing diuretic effect makes them particularly suitable for use in conjunction with
loop diuretics and thiazides. Both spironolactone and eplerenone have been shown to reduce
mortality in adults with HF when added to standard therapy [37,38]. This effect is independent
of their diuretic effect and is mediated by inhibition of myocardial fibrosis, an important
component of LV remodeling [39]. A randomized trial in 42 boys with cardiomyopathy
secondary to Duchenne muscular dystrophy demonstrated that the addition of MRA therapy
(eplerenone in this trial) to ACE inhibitor or ARB therapy attenuated the decline in left
ventricular systolic function [40]. Side effects include hyperkalemia (with both drugs) and
gynecomastia (with spironolactone).

Beta blockers — Beta blocker therapy (eg, carvedilol or metoprolol) is usually added to an
established regimen of diuretics and an ACE inhibitor. Beta blockers are used in children who
are stable on other HF medications, have systolic dysfunction with stage C HF ( table 2), and
have a systemic LV (as opposed to certain congenital heart defects with a systemic right
ventricle [RV]). Beta blockers are discontinued in patients with decompensated HF.
Beta blockers commonly used for management of HF in children include carvedilol and
metoprolol; metoprolol may be preferred for patients with frequent ventricular ectopy:

● Carvedilol is initiated at a low dose (approximately one-eighth of the eventual target dose,
usually a dose of 0.05 mg/kg per dose given orally twice a day) and increased every two
weeks to minimize side effects. In general, the dose is doubled after observing the
response to the new higher test dose in clinic to a maximum dose of 0.4 mg/kg given orally
twice a day. Side effects that may preclude dose increase include dizziness, fatigue,
hypotension, bradycardia, bronchospasm, and hypoglycemia.

● Metoprolol is initiated at 0.1 mg/kg per dose orally twice daily and increased slowly (usually
every two weeks) as needed up to 1 mg/kg/day (maximum daily dose in adults: 2
mg/kg/day or 200 mg/day, whichever is less). Side effects are similar to those of carvedilol.

Beta blockers counteract the maladaptive effects of chronic sympathetic activation of the
myocardium. In adults with HF, they improve patient survival, reverse LV remodeling, and
decrease myocardial fibrosis. (See "Primary pharmacologic therapy for heart failure with
reduced ejection fraction", section on 'Beta blocker'.)

As is the case with ACE inhibitors, studies of beta blockers in children with HF have been limited
by small sample size, relatively short follow-up, and the use of surrogate endpoints. A 2009
systematic review of beta blocker therapy in children with HF concluded there were not enough
data to recommend or discourage their use [41].

In a multicenter randomized trial of 161 children with HF and ventricular pump dysfunction,
there were no differences in the number of patients who improved (56 percent in both groups),
worsened (24 percent with carvedilol versus 30 percent with placebo), or were unchanged (19
versus 15 percent) [42]. However, the study was thought to be underpowered as the clinical
course of all children enrolled was better than expected. There was a trend towards clinical
improvement in children with a systemic LV, but not in those with a systemic RV, suggesting
that the response to carvedilol may be affected by the morphology of the child's systemic
ventricle [43].

A subsequent clinical trial in 89 pediatric HF patients also found no difference in clinical

improvement with carvedilol compared with conventional treatment; however, improvements
in echocardiographic parameters and serum BNP levels were noted with carvedilol [44].

In several small observational studies, beta blocker therapy has been associated with improved
symptoms, improvement in ventricular function, and delay in time to transplant or death in
children with HF [45-49]. Carvedilol therapy has also been shown to preserve LV function after
exposure to anthracyclines at six months follow-up [50], and to improve LV function when
added to ACE inhibitor therapy in patients with DMD and dilated cardiomyopathy [51]. In a
large, retrospective multicenter review of the Pediatric Health Information System (PHIS)
database, a beta blocker was prescribed upon discharge in 37 percent of pediatric patients
admitted with acute decompensated HF [52].

Digoxin — Digoxin is most commonly used in the treatment of infants and children with stage
C HF who have persistent symptoms despite treatment with other agents (eg, diuretics and ACE
inhibitors). In this setting, digoxin may provide physiologic benefit and symptom relief. These
benefits are generally seen with a low dose (trough level 0.5 to 0.9 ng/mL). Potential adverse
effects (arrhythmias) are rare with this low level.

Digoxin was previously the mainstay of HF management until the 1990s, but its role diminished
after it was found not to reduce mortality in adults with HF although it consistently reduced
hospitalizations in these studies [53,54]. (See "Secondary pharmacologic therapy for heart
failure with reduced ejection fraction", section on 'Digoxin'.)

Digoxin has a positive inotropic effect (mediated by Na+/K+ ATPase inhibition and increase in
intracellular Ca+), a negative chronotropic effect that slows atrial conduction, and vagotonic
properties that counter symptoms and signs mediated by the activation of the sympathetic
nervous system in HF [55].

Other agents

● Sodium-glucose cotransporter 2 (SGLT2) inhibitors – SGLT2 inhibitors (eg, dapagliflozin,

empagliflozin) are emerging as an important component of HF therapy in adult patients
based upon clinical trial data demonstrating that these agents improve survival, reduce HF
hospitalizations, and improve HF symptoms. (See "Primary pharmacologic therapy for
heart failure with reduced ejection fraction", section on 'Sodium-glucose co-transporter 2
inhibitors'.)

Data on use of SGLT2 inhibitors in pediatric patients with HF are extremely limited. None of
the available SGLT2 inhibitors are approved by the FDA for this indication in children.

In a retrospective single-center study of 38 pediatric patients with HF receiving standard

medical therapy, the addition of dapagliflozin was associated with modest improvements in
BNP levels and ventricular EF measurements [56]. The drug was generally well tolerated;
 however, six patients (16 percent) experienced a symptomatic urinary tract infection
requiring antibiotic treatment.

● Ivabradine – Ivabradine is not a routine component of pediatric HF management.

However, it is an option for patients with symptomatic stable chronic HF with reduced
ejection fraction who either continue to have a high resting heart rate despite beta blocker
and/or digoxin therapy or who have a contraindication or intolerance to beta blocker or
digoxin use.

Ivabradine is a selective inhibitor of the sinoatrial pacemaker modulating "f-current" [57].

Ivabradine slows the sinus rate by prolonging the slow depolarization phase.

The efficacy of ivabradine in reducing resting heart rate and its safety were demonstrated
in a clinical trial involving 116 children with dilated cardiomyopathy (DCM) and
symptomatic chronic HF receiving stable HF therapy who were randomly assigned to
treatment with ivabradine or placebo [58]. The ivabradine dose was adjusted to achieve a
20 percent reduction in resting heart rate, which was achieved in 70 percent of children on
ivabradine versus 12 percent of those on placebo. Among secondary endpoints analyzed at
one year, the degree of improvement in left ventricular ejection fraction from baseline was
greater in ivabradine-treated patients compared with placebo (13.5 versus 6.9 percent).
However, the degree of reduction in N-terminal pro–B-type natriuretic peptide levels from
baseline was similar in both groups. The proportion of patients with stable or improved
New York Heart Association or Ross functional class was also similar in both groups.
Bradycardia occurred more frequently in the ivabradine group (11 versus 2.4 percent);
approximately 5 percent of patients in the ivabradine group experienced symptomatic
bradycardia. Other adverse events were similar in both groups.

Clinical trials in adult patients have demonstrated that ivabradine reduces HF

hospitalizations, but an effect on mortality has not been demonstrated. For this reason, its
use is limited to patients with chronic HF who do not achieve adequate heart rate reduction
despite standard HF therapy, including maximum tolerated dose of beta blocker.

Ivabradine is approved by the FDA for treatment of stable symptomatic HF due to DCM in
pediatric patients ≥6 months old [59]. Ivabradine should not be used in patients with acute
decompensated HF, hypotension, sinus node dysfunction, heart block, pacemaker
dependence (heart rate maintained exclusively by pacemaker), or severe hepatic
impairment. In addition, it should be avoided in patients taking medications that are strong
 cytochrome CYP34A inhibitors ( table 3) since these would increase ivabradine plasma
concentrations.

● Pulmonary vasodilators — Pulmonary vasodilators are used in children with right HF due
to pulmonary hypertension. This is discussed in detail separately. (See "Pulmonary
hypertension in children: Management and prognosis", section on 'Targeted pulmonary
hypertension therapy'.)

● Nesiritide – We suggest not routinely using nesiritide for management of pediatric acute
HF. Use of this agent (where available) is generally limited to carefully selected patients
with acute decompensated HF who have not achieved adequate reduction in filling
pressures with other interventions and who have acceptable hemodynamics (ie, no
hypotension or shock). Nesiritide is no longer available in the United States.

Nesiritide is a recombinant B-type natriuretic peptide that reduces preload and afterload by
promoting diuresis, natriuresis, and arterial and venous dilation, thereby improving cardiac
output without a direct inotropic effect on the myocardium. In a prospective, open-label
study in 63 children with refractory HF, nesiritide was associated with improved urine
output, serum creatinine, and cardiac function [60,61]. However, trials in adults with acute
decompensated HF have failed to demonstrate improvement in mortality, HF
hospitalization rate, or symptoms with nesiritide use. In addition, there is an associated
increased risk of hypotension. (See "Treatment of acute decompensated heart failure:
Specific therapies", section on 'Approach to vasodilator therapy'.)

Drug therapy for advanced HF — IV diuretics and inotropic agents are generally used in
hospitalized patients with stage D HF ( table 2).

Inotropes — Inotropic agents are used in the setting of low cardiac output (eg, during acute
exacerbations of HF to improve cardiac output and to stabilize patients awaiting heart
transplantation). Their effect is mediated through higher intracellular cyclic adenylate
monophosphate (cAMP) levels, either by increased production (catecholamines) or by
decreased degradation (phosphodiesterase III inhibition).

Catecholamines — Sympathomimetic stimulation by catecholamine agents improves

myocardial contractility and may have an additional beneficial effect on peripheral vascular
beds [62]. Dopamine is the preferred drug during decompensated HF (usually in combination
with intravenous milrinone). Dobutamine has the additive effect of reducing afterload. Low-
dose epinephrine is used in the setting of refractory hypotension and/or poor end-organ
perfusion. (See "Use of vasopressors and inotropes", section on 'Dopamine' and "Use of
vasopressors and inotropes", section on 'Dobutamine' and "Use of vasopressors and
inotropes", section on 'Epinephrine'.)

An arterial catheter and central venous catheter facilitate safe administration, close monitoring,
and careful titration of these medications targeting optimal end-organ perfusion, as measured
by urine output, serum lactate, and mixed venous saturations.

Milrinone — IV milrinone, a phosphodiesterase III inhibitor, is the preferred drug for

decompensated HF at most institutions. Milrinone increases contractility and reduces afterload
without a significant increase in myocardial oxygen consumption [63]. A randomized, double-
blind, placebo-controlled trial in pediatric postoperative cardiac surgery patients demonstrated
that children treated with high-dose milrinone infusion (0.75 mcg/kg/min) were at a lower risk
for the development of low cardiac output syndrome (LCOS) compared with children treated
with placebo (12 versus 26 percent) [64].

To avoid hypotension, milrinone is initially administered as an IV infusion starting at a dose of

0.25 mcg/kg/min (without a pre-infusion bolus) and titrated upwards slowly as needed to a
maximum dose of 1 mcg/kg/min.

Milrinone therapy is generally provided in the hospital setting. However, several centers
(including ours) use home milrinone in selected patients awaiting heart transplantation. In our
practice, home milrinone infusion therapy is used in children who are clinically stable without
end-organ dysfunction, with no history of arrhythmias, who generally are on a milrinone dose
≤0.5 mcg/kg/min and a stable regimen of oral diuretic therapy, and who are under continuous
adult supervision. Small case series support the safe use of milrinone in this setting [65,66].

NONPHARMACOLOGIC INTERVENTIONS FOR ADVANCED HF

Therapeutic interventions for selected patients with advanced HF refractory to pharmacologic

therapy (stage D) may include:

● Positive pressure ventilation

● Mechanical circulatory support in patients with end-stage HF
● Heart transplantation

Noninvasive ventilation — Noninvasive ventilation (NIV), such as high-flow nasal cannula

(HFNC), continuous positive airway pressure (CPAP), or bilevel positive airway pressure (BiPAP)
ventilation, can be effective in alleviating respiratory distress from cardiogenic pulmonary
edema. NIV promotes alveolar recruitment, improves lung compliance, and leads to decreased
LV preload and afterload [67]. Although there is high-quality evidence of the benefit of NIV in
adult patients with cardiogenic pulmonary edema, pediatric data are limited [68,69]. (See
"Noninvasive ventilation for acute and impending respiratory failure in children".)

Cardiac resynchronization therapy — Cardiac resynchronization therapy (CRT) may be an

option for some children with stage C and D HF ( table 2) who do not respond adequately to
optimal medical therapy, particularly patients with reduced EF (ie, <35 percent) and a left
bundle branch block (LBBB) pattern on electrocardiogram (ECG).

Intraventricular conduction delay or LBBB may worsen HF by causing ventricular dyssynchrony.

CRT uses biventricular pacing to minimize ventricular dyssynchrony. In adult patients with LV
dysfunction, HF, and LBBB, CRT has been shown to improve hemodynamics and symptoms.
(See "Cardiac resynchronization therapy in heart failure: Indications and choice of system" and
"Cardiac resynchronization therapy in heart failure: Indications and choice of system", section
on 'Rationale for CRT'.)

The effectiveness of CRT in the pediatric population is difficult to evaluate because of the
complex anatomic substrates of congenital heart disease (CHD), scar formation from multiple
cardiac surgeries, and a higher proportion of right bundle-branch block (RBBB) and RV failure
than in the adult population [70]. Guidelines used in adult patients provide a useful reference
[71]; however, the typical adult HF scenario of an LVEF ≤35 percent with LBBB is relatively
uncommon in children [72].

There are no randomized controlled trials evaluating CRT in pediatric HF; data on the outcomes
of pediatric CRT are limited to case reports and several retrospective single-center and
multicenter studies with heterogeneous CHD populations [70,73]. In a multicenter,
retrospective analysis of 103 children and young adults with CHD and prolonged QRS on ECG,
CRT was associated with improvement of ventricular EF from 26 to 40 percent [74]. In another
single-center retrospective case-control study of 63 patients with symptomatic HF with reduced
EF and prolonged QRS on ECG, CRT was associated with improved heart transplant-free survival
[73].

The placement of transvenous, endocardial pacing systems is limited in pediatric patients due
to patient size, and, in CHD patients, due to surgically altered venous anatomy. Although
transvenous leads have been successfully placed in patients who are <50 kg, doing so may not
be in the best long-term interest of the patient because of the lifelong risk of venous
thrombosis, infection, and lead failure necessitating lead extraction. In patients <50 kg,
epicardial lead placement is often necessary which involves a sternotomy and/or a
thoracotomy.

Mechanical circulatory support — In children with decompensated HF with low cardiac

output syndrome unresponsive to medical therapy, mechanical circulatory support (MCS) can
be life-saving. MCS maintains end-organ 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 heart transplantation (ECMO or
ventricular assist device [VAD]). In a report from the International Society for Heart and Lung
Transplantation (ISHLT), one-third of pediatric heart transplant recipients received MCS as a
bridge to transplantation [75].

The options include the following:

● Extracorporeal membrane oxygenation – ECMO is a total heart-lung bypass device and is

used in the setting of imminent or actual cardiac arrest, such as postcardiotomy shock
following cardiac surgery and acute myocarditis. Cannulation can be performed
percutaneously, and ECMO can provide full cardiopulmonary support for days to weeks. A
multicenter registry review of 3416 neonatal and 4181 pediatric cardiac ECMO cases
showed a survival to discharge rate of 38 and 45 percent, respectively [76]. If myocardial
recovery does not occur or is not expected to occur within two to three weeks, ECMO may
be used as a bridge to a more durable VAD placement and subsequent heart
transplantation.

● Ventricular assist device – VAD, a cardiac-only support device, can offer either
univentricular or biventricular support. Multiple devices exist and differ by flow design
(pulsatile, centrifugal, or axial), pump location relative to patient (implantable,
paracorporeal, or extracorporeal), and delivery system (percutaneous or central) [77]. They
are primarily used in patients awaiting heart transplantation and have yielded favorable
results [78,79]. In a national registry study of 364 pediatric patients who underwent
durable VAD implants between 2012 and 2016, 72 percent were alive at six months and
nearly 50 percent had undergone transplant [80]. Adolescent patients (age 11 to 19 years)
had the highest survival (81 percent survival), whereas survival among infants (age <1 year)
was only 47 percent. Serious adverse events were common and included infection,
bleeding, and stroke [80]. VAD options are limited in small children awaiting heart
transplantation due to body size and anatomic considerations, though new devices are
under development [81,82].
The choice of device depends on the etiology of HF, the patient's cardiac anatomy, the expected
length of support, the availability of devices, and the expertise of the center's clinicians. Serious
complications associated with ECMO and VAD include bleeding (eg, gastrointestinal and
intracranial hemorrhage), thromboembolism (eg, stroke), and infection. (See "Short-term
mechanical circulatory assist devices".)

Heart transplantation — Heart transplantation is recommended for end-stage HF refractory

to medical therapy (stage D). It may also be considered for less severe HF (stage C) associated
with severe limitation of activity, significant growth failure, intractable arrhythmias, or
restrictive cardiomyopathy [75,83]. Early referral to a pediatric transplant center should be
considered to optimize medical therapy and the timing of listing for heart transplant. The
decision to pursue heart transplantation is based upon the expected survival with medical
therapy, quality of life, alternative options for treatment, and estimation of survival post-
transplantation. (See 'Outcome' below and "Heart transplantation in adults: Indications and
contraindications".)

MANAGEMENT AND PREVENTION OF HF COMPLICATIONS

Patients with HF are at risk for complications, including thromboembolism, arrhythmias, and
sudden cardiac death.

Thromboembolism — Children with HF due to systemic ventricular dysfunction are at risk for
the formation of intracardiac thrombi, which may result in pulmonary embolus, cerebral
embolic strokes, and, in some cases, death.

The optimal strategy for prevention of thromboembolism in children with HF is uncertain. There
are no controlled trials in children. Many experts (including the authors of this topic review) use
aspirin for prevention of thromboembolism in children with moderate left ventricular (LV)
dysfunction, and warfarin or low molecular weight heparin (LMWH) for children with severe LV
dysfunction, particularly in the presence of an indwelling catheter and/or a history of prior
thrombus. Aspirin can also be considered in children with restrictive cardiomyopathy if there is
marked atrial dilation. (See "Antithrombotic therapy in patients with heart failure", section on
'Role of antithrombotic therapy'.)

Children who develop intracardiac thrombi, or other clinically significant thromboembolic

events, are managed with anticoagulation therapy (initially with unfractionated heparin or
LMWH, and subsequently with either LMWH or warfarin). Management of thrombosis in
children is discussed in greater detail separately. (See "Venous thrombosis and
thromboembolism (VTE) in children: Treatment, prevention, and outcome".)

Arrhythmias — In patients with decreased ventricular function, sustained atrial and ventricular
tachyarrhythmias can rapidly impair hemodynamics. In these patients, management of the
arrhythmia is essential. This may include:

● Cardioversion or defibrillation if necessary (eg, if the patient is acutely unstable). (See

"Management of supraventricular tachycardia (SVT) in children", section on 'Cardioversion'
and "Atrial tachyarrhythmias in children", section on 'Acute management' and
"Management and evaluation of wide QRS complex tachycardia in children", section on
'Unstable patient'.)

● Antiarrhythmic therapy – Antiarrhythmic therapy is warranted if the arrhythmia is

persistent (ie, does not resolve with correction of electrolyte abnormalities or other
possible triggers) and poorly tolerated. Antiarrhythmic medications should not be used
routinely for prevention of arrhythmia in children with HF who have not previously had
arrhythmia.

● Ablation therapy, particularly in the setting of chronic atrial tachyarrhythmias. (See

"Management of supraventricular tachycardia (SVT) in children", section on 'Catheter
ablation' and "Atrial tachyarrhythmias in children", section on 'Focal atrial tachycardia'.)

The management (both acute and chronic) of specific arrhythmias is discussed in separate topic
reviews:

● Supraventricular tachycardia (including atrioventricular reentrant tachycardia [AVRT] and

atrioventricular nodal reentrant tachycardia [AVNRT]). (See "Management of
supraventricular tachycardia (SVT) in children", section on 'Acute management'.)

● Focal atrial tachycardia and atrial ectopic tachycardia. (See "Atrial tachyarrhythmias in
children", section on 'Focal atrial tachycardia'.)

● Ventricular tachycardia. (See "Management and evaluation of wide QRS complex

tachycardia in children", section on 'Initial management' and "Management and evaluation
of wide QRS complex tachycardia in children", section on 'Chronic management'.)

Sudden cardiac death — Implantable cardioverter defibrillator (ICD) placement is

recommended for patients with HF who have survived sudden cardiac arrest (ie, aborted
sudden cardiac death [SCD]). (See "Secondary prevention of sudden cardiac death in heart
failure and cardiomyopathy" and "Sudden cardiac arrest (SCA) and sudden cardiac death (SCD)
in children", section on 'Survivors of SCA'.)

In addition, ICD placement is generally indicated for patients who are at high risk for SCD due
to ventricular arrhythmia, including patients with HF or cardiomyopathy who have a history of
unexplained syncope or recurrent, sustained ventricular dysrhythmias [71].

The general principles of ICD use and efficacy in children are similar in many respects to those
in adults and application of adult guidelines for ICD implantation is generally the approach in
the older adolescent (ie, ≥16 years). (See "Implantable cardioverter-defibrillators: Overview of
indications, components, and functions", section on 'Indications'.)

There are some unique considerations for ICD placement in pediatric patients, including the
longevity of the device and lead, the size of the patient relative to the device, and the increased
physical activity, particularly in young children. In addition, many children with ICDs outlive
their devices and leads, necessitating complex extraction and multiple replacement procedures.
These issues need to be carefully considered when evaluating therapeutic options in children
with HF. The risks and benefits of each approach differ by age, size, and overall assessment of
risk for SCD.

The risk of sudden death in children with end-stage HF awaiting transplantation is

approximately 1 percent [84]. In contrast to adults, there is little pediatric evidence to guide
decision-making regarding ICD placement for primary prevention of SCD in children with
cardiomyopathy. (See "Primary prevention of sudden cardiac death in patients with
cardiomyopathy and heart failure with reduced LVEF".)

ICD placement in children with hypertrophic cardiomyopathy is discussed in greater detail

separately. (See "Hypertrophic cardiomyopathy in children: Management and prognosis",
section on 'Prevention of sudden cardiac death (ICD placement)'.)

LONG-TERM HEALTH MAINTENANCE

Longitudinal care for children with HF should be closely coordinated with the child's
cardiologist. Important aspects of long-term health care maintenance in children with HF
include:

● Immunizations – Infants and children with HF should receive all routine childhood
vaccinations, including pneumococcal vaccine, yearly influenza vaccine, COVID-19 vaccine,
 and respiratory syncytial virus (RSV) immunoprophylaxis for eligible infants. (See "Standard
immunizations for children and adolescents: Overview" and "Pneumococcal vaccination in
children" and "Seasonal influenza in children: Prevention with vaccines" and "COVID-19:
Vaccines", section on 'Children' and "Respiratory syncytial virus infection: Prevention in
infants and children".)

● Monitoring of growth parameters – It is important to monitor growth and development

in children with HF, as it is in all children. Poor weight gain may be the main clinical sign of
HF in young infants and children. (See "Normal growth patterns in infants and prepubertal
children".)

● Monitoring for cardiac symptoms – Between visits with the cardiac specialist, the primary
care provider should monitor for symptoms related to HF (eg, poor weight gain, tachypnea,
dyspnea, syncope). If the patient develops new or worsening HF symptoms, the patient
should be promptly referred to the specialist for cardiac evaluation. (See "Heart failure in
children: Etiology, clinical manifestations, and diagnosis", section on 'Clinical
manifestations'.)

● Treatment of respiratory illnesses – Respiratory illnesses can be associated with

considerable morbidity and mortality in children with HF. It is important to promptly
recognize acute respiratory illnesses and to provide appropriate treatment if warranted.
(See "Community-acquired pneumonia in children: Outpatient treatment".)

● Exercise and sports participation – Promoting healthy and safe physical activity in
patients with HF is an important part of management. The challenge is to balance routine
daily physical activity and limiting inactivity while minimizing any potential risks from
exercise. Recommendations should be tailored for each individual based on his or her
specific diagnosis and a comprehensive assessment of the child's exercise capacity. (See
'Exercise and physical activity' above and "Physical activity and exercise in patients with
congenital heart disease".)

● Antibiotic prophylaxis – Antibiotic prophylaxis for the prevention of bacterial endocarditis

should be provided to cyanotic patients and those with indwelling central lines. (See
"Prevention of endocarditis: Antibiotic prophylaxis and other measures".)

● Planning of non-cardiac surgery – Children with HF are at increased risk for adverse
events when undergoing surgery and other procedures under anesthesia. Careful
perioperative planning (including consultation with cardiac anesthesia, coordination with
 the cardiologist, and appropriate postprocedural monitoring) are important for pediatric
patients with HF undergoing surgery or other procedures requiring anesthesia/sedation.

● Airplane travel – Airplane travel should be avoided in children with HF who are in an
unstable or decompensated condition. Supplemental oxygen may be warranted in select
patients during airplane travel. (See "Approach to patients with heart disease who wish to
travel by air or to high altitude".)

OUTCOME

Outcomes for pediatric patients with HF vary considerably depending on the underlying
etiology and severity of HF.

● Outcomes for children with HF – Among patients hospitalized for management of HF,
mortality ranges from 6 to 15 percent [52,85-90]. In a study using data from the
Nationwide Emergency Department Sample database of 5971 pediatric heart failure-
related emergency department visits in 2010, 60 percent required hospital admission [88].
The median duration of hospitalization was 6 days. Among admitted patients, the mortality
rate was 5.9 percent. Independent risk factors for mortality included comorbid renal failure
and respiratory failure.

For patients with New York Heart Association (NYHA)/Ross class II and III HF symptoms
( table 4), medical management appears to improve outcomes. In the Pediatric Carvedilol
Study, a prospective clinical trial involving 161 children with predominantly class II and III
HF, medical management included angiotensin-converting enzyme (ACE) inhibitors in
nearly all children, diuretics and digoxin in approximately 85 percent, beta blocker therapy
(carvedilol) in two-thirds, and spironolactone in approximately one-third [42]. Over the
eight-month study period, 56 percent of patients demonstrated clinical improvement
(defined as improvement in HF class and/or moderate to marked improvement in global
assessment score), 18 percent remained stable, and 26 percent worsened, including 11
deaths (7 percent) and 18 cardiac transplantations (11 percent).

For children with dilated cardiomyopathy (DCM), outcomes depend on the etiology, the
degree of left ventricular (LV) dysfunction, and the severity of symptoms [91]. In a registry
study of 549 children with DCM who were diagnosed between 2000 and 2009 and followed
for a median of one year, 27 percent recovered normal LV function and size, 24 percent
underwent heart transplantation, and 9 percent died (median time from diagnosis to death
was 3.2 months) [91]. The most frequent diagnoses in this cohort included idiopathic DCM
 (63 percent), myocarditis (17 percent), familial DCM (12 percent), and neuromuscular
disease (eg, Duchenne muscular dystrophy; 5 percent); most patients (>70 percent) had
heart failure symptoms at diagnosis. Independent risk factors for mortality included
presence of heart failure symptoms at diagnosis, neuromuscular disease, and lower LV
shortening fraction; myocarditis was associated with better survival. Mortality in this cohort
was considerably lower than in an earlier cohort diagnosed with DCM from 1990 to 1999
and followed for a median of 1.6 years (9 versus 18 percent, respectively), though rates of
heart transplantation were similar (24 percent in both cohorts).

● Outcomes after heart transplantation – Outcomes following pediatric cardiac

transplantation are described in reports from the Registry of the International Society for
Heart and Lung Transplantation (ISHLT), which includes data on >15,000 pediatric heart
transplantations performed at >100 centers around the world from 1982 to 2018 [92-95].
Overall median survival following heart transplantation ranges from 13 years for
adolescents recipients to 22 years for infant transplant recipients [75]. Mortality is highest
in the first year following transplant. Survival has improved considerably over time, with
estimated five-year post-transplant survival of 82 percent for patients transplanted in the
era from 2009 to 2014 compared with 60 percent for those transplanted in 1982 to 1989
[75].

Risk factors for mortality following heart transplantation include [75]:

• Age of the recipient – The risk of mortality in the first year after transplant is higher in
infants compared with older children. However, among one-year conditional survivors,
infants generally have a better long-term prognosis compared with adolescents.

• Age of the donor – Older age is associated with higher mortality.

• Longer allograft ischemic time.

• Poor pretransplant renal function.

• Requiring ECMO or ventilator support prior to transplant.

• Type of cardiac disease – Mortality is higher among children with congenital heart
disease compared with dilated cardiomyopathy.

• Retransplant.
 Among survivors of pediatric heart transplantation, functional status at up to three years
post-transplant is generally good, with >80 percent of patients reporting normal activity or
only minor limitations in strenuous activity. Hospitalizations for the treatment of rejection
and/or infection are common, occurring in 30 to 40 percent of patients in the first three
years after transplantation [75].

SOCIETY GUIDELINE LINKS

Links to society and government-sponsored guidelines from selected countries and regions
around the world are provided separately. (See "Society guideline links: Heart failure in
children".)

SUMMARY AND RECOMMENDATIONS

● Goals of therapy – Therapeutic goals for children with heart failure (HF) are to relieve
symptoms, decrease morbidity (including the risk of hospitalization), slow the progression
of HF, and improve patient survival and quality of life. (See 'Goals of therapy' above.)

● General measures – General measures that can be applied to all pediatric HF patients
include correcting reversible conditions that may be causing or contributing to the HF
symptoms (eg, anemia, hypertension, renal failure, obesity, malnutrition, respiratory
disorders), ensuring adequate nutrition, and promoting healthy and safe exercise. (See
'General measures' above.)

● Patients with structural heart disease – For patients with preserved ventricular function
who have HF symptoms due to structural heart defects causing volume overload (eg, septal
defects, patent ductus arteriosus) or pressure overload (eg, pulmonic stenosis, aortic
stenosis, other right or left ventricular outflow tract obstruction) ( table 1), the mainstay
of management involves surgical or catheter-based interventions to correct the underlying
defects. Medical therapy may be needed for stabilization or symptom relief while awaiting
a more definitive intervention. (See 'Structural heart disease with preserved ventricular
function' above.)

● Heart failure management – Pharmacologic therapy is generally warranted for patients

with ventricular pump dysfunction and those who require stabilization before surgical or
catheter-based intervention. Because pediatric data are limited, pharmacologic treatment
 for children with HF is largely based on evidence from clinical trials involving adults with HF.
(See 'Impaired ventricular function' above and 'Evidence for efficacy' above.)

HF therapy is provided based on the stage of HF ( table 2). Treatment is not necessary for
stage A (those at risk for HF who have normal cardiac function) (see 'Pharmacologic
therapy' above):

• Stage B – For asymptomatic patients with abnormal ventricular function, we suggest

pharmacologic therapy with an angiotensin-converting enzyme (ACE) inhibitor (Grade
2B). Angiotensin II receptor blockers (ARBs) can be used in patients who are intolerant of
ACE inhibitors. (See 'Renin-angiotensin-aldosterone system inhibition' above.)

• Stage C – For patients with current or past HF symptoms due to structural or functional
heart disease, we suggest initial treatment with an ACE inhibitor plus a mineralocorticoid
receptor antagonist (Grade 2B). In addition, oral diuretic therapy is provided as needed
to treat fluid overload. After a few weeks of stability, if there is no improvement in LV
dilation and dysfunction, we suggest adding a beta blocker (Grade 2B). Low-dose
digoxin may be added if needed for symptom relief. (See 'Pharmacologic therapy'
above.)

• Stage D – Interventions for patients with end-stage HF who are refractory to oral medical
therapy may include intravenous administration of inotropes and diuretics and
nonpharmacologic interventions such as positive pressure ventilation, cardiac
resynchronization therapy, mechanical circulatory support, and heart transplantation.
(See 'Drug therapy for advanced HF' above and 'Nonpharmacologic interventions for
advanced HF' above.)

● Complications of HF – Management of pediatric patients with HF includes assessment of

risk for complications, including thromboembolism, arrhythmias, and sudden cardiac
death. (See 'Management and prevention of HF complications' above.)

● Long-term health maintenance – Longitudinal care for children with HF should be closely
coordinated with the child's cardiologist. Important aspects of long-term health care
maintenance in children with HF include routine immunizations, monitoring of growth
parameters, prompt recognition and treatment of respiratory illnesses, antibiotic
prophylaxis if warranted, counseling regarding exercise, planning of non-cardiac surgery,
and advice regarding air travel. (See 'Long-term health maintenance' above.)
 ● Outcome – Outcomes for pediatric patients with HF vary considerably depending on the
underlying etiology and severity of HF. Among patients hospitalized for management of HF,
mortality is approximately 5 to 15 percent. (See 'Outcome' above.)

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