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97]

Review Article

Heart Failure in Children

N. Jayaprasad
Department of Cardiology, Government Medical College, Kottayam, Kerala, India

ABSTRACT

Heart failure (HF) in children differs from that in adults in many respects. The causes and clinical presentations may differ
considerably among children of different age groups and between children and adults. The time of onset of HF holds the key
to the etiological diagnosis. Clinical presentation of HF in younger children can be nonspecific requiring heightened degree of
suspicion. The overall outcome with HF is better in children than in adults as HF in children is commonly due to structural heart
disease and reversible conditions which are amenable to therapy. The principles of management include treatment of the cause,
correction of any precipitating event, and treatment of systemic or pulmonary congestion. Though HF in adults has been the
subject of extensive research and generation of evidence‑based guidelines, there is a scarcity of evidence base in pediatric HF.

Key words: Cardiomyopathy, congenital heart disease, heart failure, heart transplantation

INTRODUCTION including edema, respiratory distress, growth failure, and

exercise intolerance, and accompanied by circulatory,

H
eart failure (HF) has been defined as an
abnormality of cardiac structure or function
leading to failure of the heart to deliver oxygen
at a rate commensurate with the requirements of the
metabolizing tissues, despite normal filling pressures (or
only at the expense of increased filling pressures).[1]
HF in adults has been the subject of extensive
research and generation of evidence‑based guidelines;
it has received much less attention in children because
of several difficulties. The causes of HF in children are
significantly different from those usually responsible for
the condition in adults, which include coronary artery
disease and hypertension. In children, cardiac failure is
most often caused by congenital heart disease (CHD)
and cardiomyopathy. Hsu and Pearson have given a
good working definition of HF in children as a progressive
clinical and pathophysiological syndrome caused by
cardiovascular and noncardiovascular abnormalities
that result in characteristic signs and symptoms
Address for correspondence: Dr. N. Jayaprasad,
Department of Cardiology, Government Medical College, Kottayam ‑ 686 008,
Kerala, India.
E‑mail: jayaprasadn@gmail.com
neurohormonal, and molecular derangements.[2]
There is a large amount of research published on the
management of HF in adults, whereas there is minimal
research on pediatric HF and those which do exist are often
small, retrospective studies. As a result, the management
of cardiac failure in children has largely evolved based
on clinical experience and the extrapolation of adult data,
supported by the more limited pediatric literature. Given
the significant differences in etiology of HF between the
adult and pediatric populations, this may not be ideal. This
review aims at providing a concise picture of pediatric HF
with special emphasis on diagnosis and management.
EPIDEMIOLOGY
In children, the causes of HF are significantly different
from adults and many cases are due to congenital
malformations which usually result in high output cardiac
failure. Some children suffer from low output cardiac
failure such as cardiomyopathy. CHD occurs in around
8/1000 live births. HF associated with CHD occurs in
approximately 20% of all patients.

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DOI: How to cite this article: Jayaprasad N. Heart failure in children. Heart
Views 2016;17:92-9.
10.4103/1995-705X.192556 © Gulf Heart Association 2016.

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Jayaprasad: Pediatric heart failure
Many of the children with CHD receive early
surgical intervention and it has been estimated that the
yearly incidence of HF as a result of congenital defects
is between 1 and 2 per 1000 live births.[3] The outcome of
HF related to CHD has changed dramatically following
the introduction of early surgical interventions. The
incidence of symptomatic HF has also declined in the
“early surgical era.” Massin et al. reported that only 10%
of their patients in a tertiary care pediatric cardiology
care setting developed symptomatic HF.[4]
Cardiomyopathy also contributes significantly to
the number of pediatric patients who present with the
symptoms of cardiac failure. Rossano et al. from the
United States report that 10,000–14,000 children are
hospitalized every year with HF as one of their diagnoses
and of those approximately 27% (approximately 3000)
have abnormalities of the heart muscle as an underlying
cause.[5] The incidence of cardiomyopathies in developed
countries is about 0.8–1.3 cases per 100,000 children
in the 0–18 years age group but is ten times higher in
the 0‑ to 1‑year old age group.[6,7] Ninety percent of all
cardiomyopathies in children are of the dilated variety.
In contrast to HF secondary to CHD, the outcome
of children with cardiomyopathy remains poor, with a
5‑year risk for death or cardiac transplantation of around
50% for patients with dilated cardiomyopathy (DCM).[8]
Another major group of diseases causing HF in
children in developing countries is rheumatic fever
and rheumatic heart disease. While the incidence and
prevalence of rheumatic fever and chronic rheumatic
heart disease are well documented, there are scanty
data on presentation with HF in this group. A significant
number of acute rheumatic carditis and established
juvenile mitral stenosis present with features of HF.[9]
CAUSES OF HEART FAILURE IN INFANTS
AND CHILDREN
HF in children can be divided into two groups.
Over‑circulation failure [Table 1] and pump failure [Table 2].
Over‑circulation includes conditions that result in volume
overload of cardiac chambers. The left ventricular (LV)
function is either normal or LV is hypercontractile in
them. Pulmonary venous or arterial hypertension may
be present to a variable degree. Causes of pump failure
include both congenital and acquired conditions. LV or
systemic ventricle function is abnormal and most patients
have pulmonary venous hypertension in that group.
DIAGNOSIS OF HEART FAILURE IN
CHILDREN
History and examination
The time of onset of CHF holds the key to the
etiological diagnosis. Causes of HF in the fetus include
93
Table 1: Causes of over circulation heart failure
Conditions associated with increased pulmonary blood flow
Left to right shunts like ventricular septal defects, patent ductus
arteriosus, aortopulmonary window, and atrioventricular defect
Admixture lesions like total anomalous pulmonary venous
connection, truncus arteriosus, single ventricle, etc.,
Parallel circulation as in transposition of great arteries
Conditions causing increased cardiac output
Anemia, systemic arteriovenous fistula, beriberi, etc.,
Regurgitant valvular lesions
Mitral and aortic regurgitation‑ congenital , rheumatic, and
infective endocarditis
Table 2: Causes of pump failure
Congenital causes: Obstructive lesions like LV outflow tract
obstructive lesions ‑ aortic stenosis, coarctation of aorta, right
ventricular outflow tract obstruction in pulmonary stenosis,
anomalous origin of left coronary artery from pulmonary artery,
postoperative congenital heart disease with ventricular dysfunction
Inflammatory: Viral myocarditis, HIV‑related, and Chaga’s disease
Dilated cardiomyopathies: Idiopathic, familial, neuromuscular
disease, and metabolic
Rhythm disturbances: Tachycardiomyopathy and complete heart
block
Others: LV noncompaction, anthracycline toxicity, etc.,
LV: Left ventricular
supraventricular tachycardia, severe bradycardia due
to complete heart block, severe tricuspid regurgitation
due to Ebstein’s anomaly of the tricuspid valve,
mitral regurgitation from atrioventricular canal defect,
systemic arteriovenous fistula, myocarditis, etc., HF
presenting on the 1 st day of life are commonly due
to metabolic abnormalities such as hypoglycemia,
hypocalcemia, asphyxia, or sepsis.
Structural diseases that produce fetal cardiac
failure can present on the 1st day. Conditions which
present in the 1st week of life include critical obstructive
lesions such as severe aortic stenosis, coarctation of
the aorta (COA), obstructed total anomalous pulmonary
venous connection (TAPVC), the great arteries (TGA)
with intact ventricular septum (IVS), and hypoplastic
left heart syndrome.
Development of HF due to left‑ to right‑shunts
usually occurs with the fall in pulmonary vascular
resistance at 4–6 weeks, though large ventricular
septal defect (VSD), patent ductus arteriosus (PDA),
atrio‑VSD and aortopulmonary window can cause
HF in the 2nd week of life. Other conditions such as
truncus arteriosus, unobstructed TAPVC also present
in the 2 nd week of life. As premature infants have a
poor myocardial reserve and their pulmonary vascular
resistance falls faster PDA may result in HF in the
1st week in them.
DCM is also a common cause of HF in infants.
Causes of DCM in infancy include idiopathic, inborn
errors of metabolism, and malformation syndromes.
Older children (usually beyond 2 years) are likely to
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Jayaprasad: Pediatric heart failure
have other causes for HF like acute rheumatic fever
with carditis, decompensated chronic rheumatic
heart disease, myocarditis, cardiomyopathies, rhythm
disturbances, and palliated CHD.
Clinical features suggestive of HF in infants include
tachypnea, feeding difficulty, diaphoresis, etc., Feeding
difficulty ranges from prolonged feeding time (>20 min)
with decreased volume intake to frank intolerance and
vomiting after feeds. Irritability with feeding, sweating,
and even refusal of feeds are also common.
Established HF presents with poor weight gain
and in the longer term, failure in linear growth can also
result. Edema of face and limbs is very uncommon in
infants and young children. The clinical features of HF in
a newborn can be fairly nonspecific and a high index of
suspicion is required. Tachycardia > 150/min, respiratory
rate >50/min, gallop rhythm, and hepatomegaly are
features of HF in infants. Primary cardiac arrhythmia
should be considered if heart rate is more than 220/
min. Duct dependent pulmonary circulation present with
severe cyanosis and acidosis, whereas duct dependent
systemic circulation present with HF and shock.
Features of HF in older children and adolescents
include fatigue, effort intolerance, dyspnea, orthopnea,
abdominal pain, dependent edema, ascites, etc.
Unequal upper and lower limb pulses, peripheral
bruits, or raised/asymmetric blood pressure indicating
aortic obstruction should always be looked for in a child
with unexplained HF at any age. COA in neonates can
have normal femoral pulsations in the presence of PDA.
COA usually does not cause HF after 1 year of age,
when sufficient collaterals have developed. Central
cyanosis, even if mild, associated with HF and soft or
no murmurs in a newborn suggests TGA with intact IVS,
obstructed TAPVC, etc.
An atrial septal defect or VSD does not lead to CHF
in the first 2 weeks of life and therefore an additional
cause like TAPVC or COA should be ruled out. Older
children with tetralogy of Fallot physiology can develop
HF due to complications such as anemia, infective
endocarditis, aortic regurgitation, or overshunting from
aortopulmonary shunts.
The well‑established New York Heart
Association (NYHA) HF classification is not applicable
to most of the pediatric population. The Ross HF
classification was developed to assess severity in
infants and has subsequently been modified to apply to
all pediatric ages. The modified Ross classification for
children [Table 3] provides a numeric score comparable
with the NYHA classification for adults.[10]
Investigations
Basic investigations such as chest radiography (CXR),
electrocardiography (ECG), and echocardiography are
indicated in all patients with suspected HF.
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Table 3: Modified ross heart failure classification for
children
Class I: Asymptomatic
Class II: Mild tachypnea or diaphoresis with feeding in infants,
dyspnea on exertion in older children
Class III: Marked tachypnea or diaphoresis with feeding in infants,
marked dyspnea on exertion, prolonged feeding times with growth
failure
Class IV: Symptoms such as tachypnea, retractions, grunting, or
diaphoresis at rest
Chest radiography
Cardiomegaly on pediatric CXR is suggested by a
cardiothoracic ratio of >60% in neonates and >55%
in older children. Cardiomegaly is highly predictive
of ventricular dilation on echocardiography, with high
specificity and negative predictive value, but low
sensitivity and positive predictive value.[11] Cardiomegaly
on CXR indicates poor prognosis in children with
DCM.[12] A large thymus can mimic cardiomegaly in CXR
of infants and neonates. Left to right shunts usually
present with cardiomegaly, enlarged main and branch
pulmonary arteries, and pulmonary plethora. CXR is
useful in certain cyanotic CHD that presents with typical
radiographic features such as egg‑on‑side appearance
in transposition of great arteries, snowstorm appearance
in obstructed TAPVC, and figure of eight appearances
in unobstructed TAPVC.
Electrocardiography
Most common ECG findings in pediatric HF patients
are sinus tachycardia, LV hypertrophy, ST‑T changes,
myocardial infarction patterns, and conduction blocks.
In idiopathic DCM, ECG findings of the left bundle
branch block and left atrial enlargement correlated
with mortality. [13] Myocardial infarction pattern with
inferolateral Q waves indicates anomalous left coronary
artery from the pulmonary artery. ECG is particularly
useful in the diagnosis of tachycardiomyopathy and
other arrhythmic causes of HF like an atrioventricular
block. Ambulatory ECG monitoring is useful in the
diagnosis of tachycardiomyopathy as well as risk
stratification of sudden death in HF resulting from
primary cardiomyopathy.[13]
Echocardiography
Transthoracic echocardiography is indicated in all
cases of pediatric HF to exclude possible structural
disease. Baseline echocardiography will be required
for future comparison. LV systolic dysfunction in
children is currently defined by an ejection fraction (EF)
<55%. Echocardiography is also useful for the
screening of cancer patients undergoing treatment
with anthracycline chemotherapy, patients with storage
disorders, neuromuscular diseases, etc., Periodic
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echocardiographic evaluation is required in the
first‑degree relatives of patients with various genetic
forms of cardiomyopathy. Periodic echocardiography
follow‑up is useful in HF patients for the surveillance
of disease progression and to assess the response to
therapy.[14]
Biomarkers
The natriuretic peptides (brain natriuretic peptide [BNP]
or amino terminal [NT]‑proBNP) are useful in the
acute settings for differentiation of HF from pulmonary
causes of respiratory distress.[15] Elevated natriuretic
peptide levels might be associated with worse outcome
in HF.[16] Plasma BNP elevation is a reliable test for
recognizing ventricular dysfunction in children with a
variety of CHD.[17] In chemotherapy patients, higher
NT‑proBNP levels are associated with higher treatment
doses of doxorubicin and abnormal echocardiographic
parameters including ventricular dysfunction.[18,19] The
use of serial BNP or NT‑proBNP measurements in
children with HF to guide therapeutic intervention or to
monitor HF status shows some promise.
Blood glucose and serum electrolytes like calcium,
phosphorous should be measured in all children with HF
as their abnormalities can cause reversible ventricular
dysfunction. Screening for hypoxia and sepsis should
be done in newborn with HF.
Antistreptolysin O and C‑reactive protein
measurement should be done in cases of HF with
suspected acute rheumatic fever or reactivation of
chronic rheumatic heart disease. Metabolic and genetic
testing may be considered in primary cardiomyopathy
as recent reports suggest a genetic cause for more than
50% of patients with DCM.[20]
Special investigations
Endomyocardial biopsy (EMB) may be considered
for the diagnosis of myocarditis in selected patients
presenting with HF when a specific diagnosis is
suspected that would influence therapy. A diagnosis of
acute myocarditis should be suspected in all children
with new‑onset HF without a history of decreased
functional capacity, especially if ventricular dilation
is less than expected for the degree of systolic
dysfunction. The prognosis in pediatric myocarditis is
significantly better than DCM with a higher probability of
recovery in children. However, the diagnostic accuracy
of RV EMB for suspected myocarditis tends to be low,
and there is limited evidence to support the use of
immunosuppressive or immune‑modulating therapy
in children.
In patients with a clinical diagnosis of myocarditis
typical viral pathogen screening using polymerase
chain reaction may be considered. Cardiac magnetic
resonance imaging (CMRI) is a less invasive alternative
95
to EMB in myocarditis to identify inflammation by showing
myocardial edema on T2‑weighted images.[21] CMRI
might provide additional information in cardiomyopathy
by tissue and scar characterization though the prognostic
value is uncertain in children.
Treatment
The principles of management include treatment
of the cause, correction of any precipitating event,
and treatment of systemic or pulmonary congestion.
Wherever possible, the cause of CHF should be
identified and treated.
In large left to right shunts, prompt surgical therapy
should be considered after initiating medical therapy.
Other conditions requiring prompt surgery or catheter
intervention include severe AS or COA, TGA with IVS,
obstructed TAPVC, etc.
A precipitating event such as intercurrent infections,
anemia, electrolyte imbalances, arrhythmia, rheumatic
reactivation, infective endocarditis, drug interactions,
drug toxicity, or drug noncompliance should be identified
and corrected if present. Acute HF patients can have
symptoms related to fluid overload, underperfusion, or
both. The early management of children with HF should
address these problems.
In neonates, several causes of HF can present
with acute circulatory collapse or progress to shock if
not recognized early. Many of these conditions require
maintenance of duct patency with prostaglandin infusion
or emergency procedures such as ductal stenting and
balloon atrial septostomy. Indiscriminate administration
of intravenous fluid resuscitation is contra‑indicated and
will worsen the condition of children with HF. In acute
decompensation general measures such as bed rest,
propped up position, humidified oxygen sodium, and
if required, volume restriction are followed routinely.
Infants with CHF require 120–150 Kcal/kg/day of caloric
intake and 2–3 mEq/kg/day of sodium.
PHARMACOLOGICAL THERAPY
Drug therapy is aimed at reducing the pulmonary
or systemic congestion by the use of diuretics,
increasing contractility by inotropes, and reducing the
disproportionately elevated afterload by vasodilators
and other measures. Routinely used drugs in the
management of cardiac failure in children include
diuretics, digoxin, angiotensin‑converting enzyme
inhibitors (ACEIs), spironolactone, beta-blockers, and
inotropes. The drugs which are still investigational
include natriuretic peptides, vasopressin antagonists,
renin inhibitors, endothelin antagonists, oral
phosphodiesterase inhibitors, anti‑inflammatory
molecules, nitric oxide agonists, and neuropeptidase
antagonists, etc.
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Jayaprasad: Pediatric heart failure
Diuretics
Diuretics are the first line agents to reduce systemic
and pulmonary congestion. Loop diuretics have been
the most widely used in HF although in some situations
other types of diuretics have a role. Frusemide is given
intravenously at a dose of 1–2 mg/kg or 1–2 mg/h
infusion. For chronic use 1–4 mg/kg of frusemide or
20–40 mg/kg of chlorothiazide in divided doses are
used. Patients who are unresponsive to loop diuretic
agents alone might benefit from the addition of a thiazide
agent like metolazone. Continuous infusion of diuretics
is recommended in cases of acute decompensated HF.
Diuretic‑induced hypokalemia and hypontremia are
rare in children. Secondary hyperaldosteronism does
occur in children with HF and addition of spironolactone
1 mg/kg single dose to other diuretics conserves
potassium.
Digoxin
In the setting of chronic HF, digoxin use decreased the
rate of hospitalization and improved the quality of life
but not survival in adults.[22] Digoxin is widely used in
pediatric cardiac failure despite the lack of trial evidence.
Digoxin has a very narrow safety window and it should
be avoided in premature babies, those with renal failure
and those with acute myocarditis. Electrolyte imbalance
like hypokalemia and hypomagnesemia should be
promptly corrected to avoid potentiation of toxicity and
development of arrhythmias. Rapid digitalization is
generally not required. In most circumstances, starting
with an oral maintenance dose (8–10 µg/kg/day) with no
loading dose is adequate. Dose reduction is required in
HF patients on carvedilol and amiodarone targeting lower
serum digoxin concentrations (e.g., 0.5–0.9 ng/ml).[23]
Angiotensin‑converting enzyme inhibitors and
angiotensin II receptor blockers
In adult HF patients, ACEI therapy reduces symptoms and
improves survival. In children, survival benefit remains
unvalidated by any randomized controlled trial. For the
treatment of symptomatic LV dysfunction in children,
ACEIs are routinely used unless contra‑indicated. In
children with cardiac failure, the ACEIs which have been
most studied are captopril and enalapril.[24,25] Clinical
improvement is demonstrated with these agents in left to
right shunts with HF as well.[26,27] They should be started
at low doses and should be up‑titrated to a maximum
tolerated, safe dose. ACEIs should be avoided in HF
caused by pressure overload lesions as they might
interfere with compensatory hypertrophy. Captopril is
preferred in neonates (0.4–1.6 mg/kg/day in 3 divided
doses) and infants (0.5–4 mg/kg/day in three divided
doses). Enalapril is the first choice for those older than
2 years of age (0.1–0.5 mg/kg/day in two divided doses).
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Children treated with ACEIs should be
watched for deterioration in renal function and
hypotension. Other adverse effects include cough
and angioedema. Angiotensin receptor blockers are
generally reserved for those children with systemic
ventricular systolic dysfunction who would benefit from
renin‑angiotensin‑aldosterone system blockade but are
intolerant of ACEIs.
Aldosterone antagonists
Aldosterone blockade is a well‑established therapy
in adults with systolic HF with reduction of mortality
in selected patients with HF. The literature supporting
the role of spironolactone in pediatric HF is however
limited. Aldosterone antagonist therapy is reasonable in
children with chronic systolic HF. It is contra‑indicated
in patients with renal dysfunction and hyperkalemia.
Spironolactone is used most often in children because
experience with eplerenone in children is limited.
Usual starting dose of spironolactone is 1 mg/kg/day
and the target maximum dose is 2 mg/kg/day. Male
gynecomastia can occur with spironolactone requiring
replacement with eplerenone. Monitoring of renal
function and serum potassium is required when
coadministered with ACEIs.
Beta‑blockers
The benefits of beta‑blocking agents in HF are well
established in adults whereas published experience
of their use in children with HF is much less robust
and has not yet proven conclusive. According to the
guidelines, it is reasonable to consider β‑blockers in
children with moderate to severe systolic dysfunction
of the systemic ventricle, particularly if the systemic
ventricle has an LV morphology.[28,29]
In children with HF and related conditions, carvedilol
has been the most widely studied beta-blocker.
Carvedilol is started at 0.05 mg/kg/dose (twice daily)
and increased to 0.4–0.5 mg/kg/dose (twice daily) by
doubling the dose every 2 weeks. In many small scale
and retrospective studies, carvedilol was found to be
effective in improving clinical and echocardiographic
parameters and preventing transplantation. [30,31]
However, a multicenter, randomized, double‑blind,
placebo‑controlled study of 161 children and
adolescents with symptomatic systolic HF did not
show an improvement in their composite clinical status
after 8 months of treatment with carvedilol compared
to placebo.[32] These findings may have been due to an
unexpected level of improvement in the placebo arm,
inclusion of children with the systemic right ventricle and
single ventricle physiology, the low dose of carvedilol
used, and the study being underpowered.[33]
Metoprolol (0.1–0.2 mg/kg/dose twice daily and
increased to 1 mg/kg/dose twice daily) or bisoprolol may
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Jayaprasad: Pediatric heart failure
be used as an alternative to carvedilol. Beta‑blockers
should not be administered in acute decompensated
HF. Therapy should be started at a small dose and
slowly up‑titrated.
Inotropes
A number of inotropes have been used in acute HF; the
most common among these are the catecholaminergic
drugs such as dopamine and dobutamine, and the
phosphodiesterase inhibitors such as milrinone and
amrinone.
There are no controlled clinical trial data to guide
the use of inotropic agents in the setting of acute HF
in children. Even in adults a long‑term survival benefit
was not demonstrated by trials. However, many cases
of acute cardiovascular collapse in childhood result
from reversible disease states, including acute severe
myocarditis and inotropes are a lifesaving temporary
measure.
On the basis of a lack of any pediatric data and
lack of data supporting improved outcomes in adults,
the use of intermittent or chronic inotropic therapy for
chronic HF, other than as a bridge to transplant, is not
recommended.[28] Catecholaminergic drugs commonly
used are dopamine 5–20 mcg/kg/min and dobutamine
5–20 mcg/kg/min. Epinephrine and norepinephrine
are more commonly associated with arrhythmias and
increased myocardial oxygen demand.
Milrinone, a phosphodiesterase inhibitor is an
inotrope and vasodilator that has been shown to
prevent low cardiac output syndrome after cardiac
surgery in infants and children.[34] The loading dose of
milrinone is 25–50 mcg/kg/min and maintenance dose
is 0.25–1 mcg/kg/min. Milrinone might cause peripheral
vasodilation and should be used with caution in
hypotensive patients. Levosimendan is another inotrope
with vasodilatory property by a calcium‑sensitizing
effect and opening up of vascular ATP‑dependent
K+ channels. Initial reports of the use of levosimendan
in children are promising.[35,36]
Device therapy
Device therapy in HF predominantly includes
pacemaker therapy, cardiac resynchronization
therapy (CRT), and mechanical circulatory support.
Permanent pacemaker implantation is recommended
for advanced second‑ or third‑degree atrioventricular
block associated with ventricular dysfunction.
Implantable cardioverter defibrillator (ICD) implantation
is indicated for patients with a history of cardiac arrest
or symptomatic sustained ventricular tachycardia in
association with CHD.[37]
ICD is reasonable for patients with CHD with
recurrent syncope in the presence of ventricular
97
dysfunction or inducible ventricular arrhythmia at
electrophysiological study. CRT can be useful for
pediatric patients with a systemic LV with an EF < 35%,
complete left bundle branch block pattern, QRS duration
more than the upper limit of normal for age, NYHA
Class II‑IV on guideline‑directed medical therapy.[28]
Extracorporeal membrane oxygenation (ECMO)
has been widely used in the setting of cardiopulmonary
arrest. For a child with isolated cardiac failure that is
believed to be reversible, ECMO or a ventricular assist
device (VAD) may be considered as a temporizing
measure as a bridge to recovery of function. ECMO
is also used in emergency perioperative salvage.
Children with fulminant myocarditis with a high chance
of recovery, do well when put on ECMO and VADs.[38,39]
For those patients requiring a long‑term mechanical
support to bridge to cardiac transplantation, VADs are
being used with increasing frequency. These devices
can offer univentricular or biventricular circuit support.
Cardiac transplantation
Heart transplantation remains the therapy of choice
for end‑stage HF in children refractory to surgical and
medical therapy. There were 9,566 pediatric heart
transplants reported to the international society for
heart and lung transplantation from 1982 to 2009.[40]
The most common indication is the end‑stage heart
disease due to cardiomyopathies.
Other causes include CHDs such as hypoplastic
heart syndrome and other complex CHD, single
ventricle, and palliated heart disease. The data show
an improved early (1 year) survival for all age groups
in the more recent era of transplantation, averaging
80% in children and 90% in infants. Systemic viral
infections such as cytomegalovirus, Epstein–Barr
virus, and adenovirus, acute cellular rejection, allograft
vasculopathy with graft failure, renal dysfunction,
hypertension, and malignancy like lymphoproliferative
disorders are the major complications in children.
Ringewald et al. reported a high rate of rejection
in nonadherent adolescent pediatric heart transplant
recipients, which led to a high rate of death in this
population.[41] The advances in immunosuppression
coupled with a better understanding of rejection have
resulted in improved survival, quality of life, and fewer
adverse effects after transplantation. However, heart
transplantation can be a solution for only a minority of
end‑stage HF patients owing to the scarcity of donor
hearts and expert centers.
CONCLUSION
The causes and clinical presentation of HF are different
from adults. The overall outcome with HF is better in
children than that in adults. There has been a significant
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advance in the evidence base for the management of HF
in adults. While the general principles of management
are similar to those in adults, there is a compelling need
for larger and higher quality studies on the treatment
of cardiac failure in children to provide a more robust
evidence base.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
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Ponikowski P, Poole‑Wilson PA, et al. ESC guidelines for the
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chronic heart failure 2008 of the European Society of Cardiology.
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