Heart Failure in Children: Clinical Aspect and Management

Symposium on Advances in Cardiology - II
Heart Failure in Children: Clinical Aspect and
Vivek Chaturvedi and Anita Saxena
Department of Cardiology, All India Institute of Medical Sciences, New Delhi, India
Heart failure (HF) is a complex syndrome, with several definitions, the commonest being “an abnormality of cardiac function
whereby heart in unable to pump at a rate commensurate with the requirement of the metabolizing tissues, or does so only
at elevated filling pressures”. In case of children, this requirement includes growth and development. Unlike as seen in
adults, HF in children is commonly due to structural heart disease and reversible conditions. Thus the treatment for HF is
commonly required for short term only. The overall outcome with HF is better in children than in adults. While the general
principles on management are similar to those in adults, the evidence for the use of drugs in children is less convincing. It
requires a judicious balance of extrapolation from adult medicine, keeping in mind that children are not small adults. [Indian
J Pediatr 2009; 76(2) : 195-205] E-mail: [email protected]
Key words : Heart failure; Congenital heart disease; Drug therapy
Heart failure (HF) is a complex syndrome, with sinister
implications once diagnosed, the mystery of which is
still being unraveled despite decades of benchside and
clinical research. Several definitions have been
proposed for heart failure1, which again reflects our less
than complete understanding of this enigma. A
common definition used is 2 : ‘HF is a pathophysiological state in which an abnormality of cardiac
function is responsible for the failure of the heart to
pump blood at a rate commensurate with the
requirements of the metabolizing tissues, or does so
only at elevated filling pressures’. In case of children,
this requirement includes growth and development.
The current American College of Cardiology (ACC)/
American Heart Association (AHA) guidelines define
HF as a ‘complex clinical syndrome that can result from
any structural or functional cardiac disorder that
impairs the ability of the ventricle to fill with or eject
blood’.3 Our current understanding of HF is that of not
just a case of cardiac dysfunction but that of a multiorgan syndrome which explains the myriad pathophysiological and clinical features that accompany it.
While HF in adults has been the subject of voluminous
research and generation of evidence-base, it has
Correspondence and Reprint requests : Dr Anita Saxena, Prof.
Department of Cardiology, All India Institute of Medical
Sciences, New Delhi-110029, India. Phone: 91-11- 26594861, Fax:
[Received January 15, 2009; Accepted January 15, 2009]
Indian Journal of Pediatrics, Volume 76—February, 2009
– Angiotensin converting enzyme
– Acute decompensated heart failure
ALCAPA – Anomalous left coronary artery from
pulmonary artery
– Aortic regurgitation
– Aortic stenosis
– Atrial septal defect
– Angiotensin receptor blocker
– Arteriovenous
– Atrio-ventricular septal defect
– Coronary artery disease
– Chronic constrictive pericarditis
– Congenital heart disease
– Congestive heart failure
– Coarctation of aorta
– Corrected transposition of great arteries
– Dilated cardiomyopathy
– Hypoplastic left heart syndrome
– Human immunodeficiency virus
– Hypertension
– Ischaemic heart disease
– Intra-cardiac defibrillator
– Intact ventricular septum
– Jugular venous pressure
– Mitral valve
– Mitral regurgitation
– Nonspecific aorto-arteritis
– Pulmonary arterial hypertension
– Patent ductus arteriosus
– Persistent pulmonary hypertension of the
– Pulmonary stenosis
Vivek Chaturvedi and Anita Saxena
Rheumatic fever
Rheumatic heart disease
Restrictive cardiomyopathy
Single ventricle
Tricuspid atresia
Total anomalous pulmonary
Transposition of great arteries
Tetrology of fallot
Tricuspid regurgitation
Ventricular septal defect
TABLE 1. Causes of Heart Failure
received much less attention in children because of
several difficulties.
The patho-physiology and etiologies for heart failure
in children are very different from those in adults.
Common causes of adult heart failure including
ischemia, hypertension and valvular inflammation are
less common in children. Infants and children develop
heart failure more commonly due to volume overload
secondary to shunt lesions, and obstructive lesions of
the heart. Less common causes include homeostatic
abnormalities and cardiomyocyte dysfunction
secondary to myocarditis / cardiomyopathies. Palliated
congenital heart disease (CHD) leading to heart failure
is increasingly being recognized.
With the aim of keeping this review focused on
clinical aspects and management of heart failure, the
pathophysiology of heart failure shall not be discussed
in detail. Briefly, the manifestations of heart failure arise
due to mismatch of the circulatory load and the ability
of heart, or its components, to pump it in an adequate
fashion. These can be accompanied or followed by
compensatory changes in the regional perfusions and
renal, muscular and endocrine physiology (notable
among changes in virtually every organ). The outcome
of this, manifesting as symptoms, is excess extra
cellular volume in lungs and periphery and decreased
perfusion of vital organs like kidneys and brain as well
as the muscles.
Causes of Heart Failure in Infants and Children
Children can have diverse causes of heart failure
depending on the age, geographical location, and many
other factors. Hence a descriptive epidemiology of heart
failure in children is not possible. The causes of HF can
be broadly classified into two groups; one due to
volume overload of ventricle with preserved systolic
function of the ventricle, the common example is heart
failure secondary to a large left to right shunt. The
second group consists of pressure overload, persistent
arrhythmias, dilated cardiomyopathy and certain
systemic disorders where the contractile function of
ventricle is reduced (Table 1).
Table 2 enumerates the likely causes of heart failure
by age at presentation. This is important, as the
Volume overload with preserved systolic ventricular
a) Large left to right shunt: VSD, AVSD, PDA
b) Admixture lesions with high PBF: TGA, TAPVC,
c) Regurgitant lesions: MR, AR (Rheumatic/Congenital)
II Myocyte dysfunction with abnormal ventricular
contractile function
a) Pressure overload: Severe AS, PS
b) Muscular dystrophy, DCM
c) Inflammatory: Myocarditis, Chaga’s, HIV
d) Tachycardiomyopathies secondary to SVT
e) Abnormal morphology: single ventricular (pre and
post op)
f) Ischemic: ALCAPA
g) Others: Sepsis, post CPB, hypocalcaemia etc.
symptoms and signs of heart failure can be confusing
or fairly non-specific in children. The lists are not allexhaustive and the reader is referred to specialized
books for complete enumeration of heart failure
HF presenting on the first day of life are commonly
due to metabolic abnormalities. Structural diseases that
cause HF in neonates usually do not manifest on 1st day
of life; rather it is the causes of fetal HF like Ebstein’s or
abnormal heart rate/rhythm that predominate.
About 90% of all cases of HF in children occur before
the end of first year of life and reflect the preponderance
of CHD as a cause of HF. In the first week of life,
obstructive and duct-dependent lesions can present
with HF or acute circulatory shock. Development of HF
due to left-to right shunts usually waits the fall in
pulmonary vascular resistance at 4-6 weeks, though
large VSD, PDA, AVSD and aorto-pulmonary window
can cause HF by 2 nd week of life. Isolated ASD are
mostly asymptomatic in children and if an infant is
diagnosed to have ASD and is in failure, the likely
diagnosis is TAPVC.
The myocardium perse is normal in most CHD and
the heart failure, if not presenting in the first year, is
unlikely to develop for the next 10 years unless
complicated by infective endocarditis, anemia,
infections or arrhythmias. Thus older children (usually
beyond two years) are likely to have other causes for HF
like acute rheumatic fever with carditis, decompensated
chronic rheumatic heart disease, myocarditis,
cardiomyopathies and palliated CHD (post Senning
operation for transposition of great arteries or Fontan
group of surgeries for univentricular hearts).
Epidemiology of Heart failure in Children
Epidemiology of heart failure in children is a difficult
science given the fact that symptoms, etiology,
Indian Journal of Pediatrics, Volume 76—February, 2009
Heart Failure in Children: Clinical Aspect and Management
TABLE 2. Common Causes of Heart Failure by Age at Presentation
Day 1 of life/fetal
Systemic AV fistula
1 st week of life (after Day 1)
Critical AS/PS
1 to 2 months
Transposition and
malposition complexes
2 to 6 months
Day 1 causes
Causes at 1-2
Large VSD
Large PDA
AV Septal defect
CHD with complications
(i.e. endocarditis)
Adrenal insufficiency
TGA with IVS
Aorto-pulmonary window
2 nd week of life
Aortic stenosis
Older children
diagnostic criteria, and outcomes are quite
heterogeneous. In Germany, a hospital based study at
University Children’s Hospital at Essen studied the
epidemiology of heart failure between 1989 and 1998.
Heart failure occurred in 40% of all admissions for
CHD and one-third of all admissions for all heart
disease (congenital and acquired, n=1755); if postoperative congestive heart failure was excluded, HF
accounted for a quarter of all CHD admissions.
Incidence of HF was 289/1000 heart disease patients
and 20.1/1000 of al pediatric inward admissions. In
70%, it occurred in the first year of life. Overall mortality
in children with HF was 14%, more than double when
compared to mortality in all heart disease patients.4 One
large database from US found out that the heart failure
in children (<18 years of age) was complicated by more
frequent procedures, longer stay, but similar mortality as
adults (7.5%). The cause was predominantly congenital
heart disease in infants (<1 year of age) at 83% while it
was present in only 34% in children older than 1year of
age. This study however was based on the findings of a
large financial database that was exhaustive, but had
limited clinical information available.5
In developed countries, the annual incidence of CHD
is about 8 per 1000 (0.8%) of live births, of which onethird to on-half are severe enough to warrant attention.
Of these, about half result in CHF; thus due to CHD, the
incidence of CHF is about 0.1-0.2% of all live births.6
Ninety percent of all cardiomyopathies in children
are of the dilated variety, others being hypertrophic and
restrictive type. The reported population incidence of
idiopathic DCM in children is 0.6/100, 000 children7
with recent studies showing 5 year rates of death or
transplantation of 46%.8 More than 50% present within
the first year of life. Children with myocarditis as a
Indian Journal of Pediatrics, Volume 76—February, 2009
Palliated CHD /postoperative
cause of DCM have a favorable prognosis, with 50-80%
showing resolution within 2 years of presentation. The
population based studies on childhood cardiomyopathies systematically excluded cardiomyopathies
secondary to cancer drug therapy. At least in the past,
anthracycline toxicity has accounted for 50% of
admissions due to congestive cardiomyopathy in
Boston Children’s Hospital. 6
Prevalence of heart failure in palliated or operated
CHD cases is unknown. It has been estimated that 1020% of operated cases with Mustard/Senning surgery
for transposition of vessels and those with Fontan-type
of operation have symptoms of heart failure.
Rheumatic fever/rheumatic heart disease is an
important cause of HF in children in developing
countries like India. While the incidence and
prevalence of RF/RHD are well documented, there are
no data on presentation with HF in this group, though
a significant majority of acute rheumatic carditis and
established juvenile mitral stenosis will present with
features of HF. The true incident of HF in children for
India is not available but is likely to be enormous
considering the huge burden of the problem of critical
congenital heart disease in newborns and rheumatic
heart disease in older children.
Clinical features
The clinical features of HF in children vary according to
the cause and the age of the child. The presentation of
HF in fetus is that of hydrops fetalis and fetal wastage.
An important point to remember is that raised
jugular venous pressure, peripheral edema, effusions
and chest crepitations are not seen in neonates and are
unlikely in young children as a sign of HF. Chest
Vivek Chaturvedi and Anita Saxena
T ABLE 3. Clinical Features of Heart Failure by Age and Associated Findings
Tachypnea Hepatomegaly
Feeding difficulties
Subcostal recession
Tachycardia Cardiomegaly Bounding pulses in AV malformations, PDA, Truncus
Excessive sweating
Asymmetric upper and lower limb blood pressure in aortic arch
Cyanosis and wheeze
Central cyanosis in TGA, TAPVC, Truncus , TA with no PS
Differential cyanosis in PPHN and R-L shunt through patent ductus
Multiple heart sounds in Ebstein’s
Ejection systolic murmur in AS/PS
Syndromic anomalies (Down’s, Noonan)
Think of HLHS, COA, Interrupted aortic arch, critical AS,
tachyarrhythmias, myocarditis
Poor feeding
Excessive sweating
Slow weight gain
Tachypnea, tachycardia
Precordial bulge, signs of PAH and less impressive systolic murmurs
suggest larger L-R shunts
Crepitations should alert to possibility of chest infection
Cyanosis in TAPVC, TGA with VSD, AVSD, Truncus
Findings of CHF and cyanosis in suspected ASD suggest TAPVC
Later onset of HF in infancy can be due to certain forms of TAPVC
Older children
Poor weight gain
Effort intolerance, orthopnea
Peripheral edema
Raised JVP
Gallop rhythm, murmurs
Basilar crepitations
crepitations in fact suggest the possibility of underlying
chest infection, which so often accompanies HF in
children especially in high pulmonary flow situations.
Common clinical features of HF in children are given in
table 3. Certain features deserve mention:
The clinical features of HF in a newborn can be
fairly non-specific; sometimes the clinical picture
resembles that of septicemia. Thus a high index of
suspicion is required.
Unequal upper and lower limb pulses, peripheral
bruits or raised/asymmetric blood pressure
indicating aortic obstruction (including non-specific
aortoarteritis, NSAA) , should always be looked for
in a child with unexplained HF at any age.
An interrupted aortic arch or coarctation of aorta
(COA) in neonates can have normal femoral
pulsations in presence of patent ductus arteriosus
(PDA); when the ductus closes, these babies may
present with acute shock.
COA usually does not cause HF after one year of
age, when sufficient collaterals have developed.
Central cyanosis, even if mild, associated with HF
and soft or no murmurs in a newborn, should
always be taken seriously (suggests transposition of
great arteries with intact septum, obstructed total
anomalous pulmonary venous connection etc)
Diastolic murmur in a child with known VSD suggests
associated AR
Pericardial rub in appropriate settings suggests acute RF
Hypertension and unequal pulses or bruits suggest NSAA
Findings of raised JVP, ascites and anasarca should lead to
suspicion of CCP or RCMP
An ASD or VSD does not cause HF in first 2 weeks
of life; their presence with HF should prompt
evaluation for associated TAPVC or COA
A premature newborn with significant respiratory
distress and a systolic murmur should be evaluated
for patent ductus arteriosus causing HF.
Heart rates above 220/mt are unusual in a neonate
even with HF and should always be investigated to
rule out tachyarrhythmias as a cause of HF
Several children with CHD or cardiomyopathies
have associated chromosomal anomalies or extracardiac manifestations which provide clues for
Older children with TOF physiology can have HF
due to complicated course (anemia, infective
endocarditis, bicuspid aortic valve with aortic
regurgitation) or overshunting from aortopulmonary shunts.
The cornerstones for rapid clinical diagnosis of HF in
Chest radiograph: Should be done in all patients with
suspected HF; an echocardiogram is not a substitute for
Indian Journal of Pediatrics, Volume 76—February, 2009
Heart Failure in Children: Clinical Aspect and Management
radiograph. It enables diagnosis of cardiomegaly,
quantification of pulmonary blood flow, presence of
associated chest infection, pleural effusion etc., as well
as being pathognomonic in certain disease states. A
cardiothoracic ratio of >60% in neonates and >55% in
older children suggest cardiomegaly though expiratory
films should be interpreted with caution. A large
thymus can also give false impression of cardiomegaly
in neonates and infants (Fig. 1). Cardiomegaly with
increased pulmonary blood flow (pulmonary plethora),
prominent main and branch pulmonary arteries, left
atrial enlargement etc. are signs of significantly
increased pulmonary blood flow (a finding not
appreciable on echocardiogram!) which could cause
HF (Fig. 2). Typical radiographs strongly suggestive of
certain diagnosis include those with transposition of
great arteries (egg-on-side, Fig. 3), obstructed TAPVC
(snowstorm appearance, Figure 4), unobstructed
TAPVC (Fig. of 8 appearance, Fig. 5), Truncus arteriosus
(waterfall appearance of hila, Fig. 6), Ebstein’s anomaly
(globular cardiomegaly with decreased pulmonary
flow), CCP (calcification in RV/AV groove), juvenile
mitral stenosis (left atrial appendage enlargement), etc.
Fig. 3. Chest radiograph of a newborn with transposition of
great arteries (egg-on-side)
Fig. 4. Chest radiograph of a newborn with obstructed TAPVC
showing the ‘snow-storm’ appearance
Fig. 1. X-ray chest of a normal neonate with a large thymus.
Fig. 5. Chest radiograph in an older child with uobstructed
TAPVC showing the typical ‘figure of 8’ appearance of
Fig. 2. Chest radiograph showing cardiomegaly, prominent
pulmonary artery segment and increased pulmonary
blood flow in a case of large VSD.
Indian Journal of Pediatrics, Volume 76—February, 2009
Electrocardiogram: An electrocardiogram is very useful
in heart failure for elucidation of cardiac diagnosis. It
shows biventricular hypertrophy with volume overload
of the left ventricle in the commonest cause of HF in the
infant i.e. a large VSD. Tachycardiomyopathy, a
Vivek Chaturvedi and Anita Saxena
potentially reversible cause of HF, due to incessant
supraventricular tachycardias (like ectopic atrial
tachycardia) can only be picked up by ECG (Fig. 7).
Similarly bradyarrhythmias due to congenital complete
heart block are detected on ECG (Fig. 8). Certain
patterns on ECG are also virtually diagnostic of specific
Fig. 6. Chest radiograph in an infant with persistent truncus
Fig. 7. Electrocardiogram of a child presenting with heart failure
due to atrioventricular re-entrant tachycardia at the rate
of 200 beats per minute. Arrows point to P waves. Note
that RP interval is shorter than PR interval.
Fig. 8. Holter trace of an infant with bradycardia (ventricular
rate of 40 beats per minute) due to congenital complete
heart block. Note that there is no relationship between P
waves and QRS complexes.
cardiac pathologies. Thus ALCAPA can present with
pathognomonic pathologic q waves in anterolateral
leads (Fig. 9). A superior or northwest axis with BVH
suggests atrioventrcular septal defect as a cause of HF
(Fig. 10). In a neonate with unexplained HF, a
prolonged QTc interval with terminal T wave inversion
Fig. 9. Electrocardiogram of a child with ALCAPA showing
presence of pathologic q waves and ST – T changes
(arrows) in lateral leads.
Fig. 10. Electrocardiogram of a child with atrioventricular septal
defect showing left axis deviation and right ventricular
Fig. 11. Electrocardiogram from a child with hypocalcemia
showing long QTc interval and bizarre, inverted T
waves (white arrow).
Indian Journal of Pediatrics, Volume 76—February, 2009
Heart Failure in Children: Clinical Aspect and Management
are suggestive of hypocalcemia as the cause of left
ventricular dysfunction (Fig. 11).
Echocardiogram: An echocardiogram is invaluable in
the diagnosis of heart failure. It confirms the presence
of structural heart disease and great vessel anomalies
and aids in the acute and long term management
strategy. While an echocardiogram is essential for
diagnosis of heart disease in HF, as mentioned above, it
should always be interpreted in an integrated fashion
with clinical, radiographic and ECG findings. Owing to
its dependence on operator skills and inherent
problems of imaging small children, an echo can miss
findings such as TAPVC and aortic arch anomalies.
However echo by a skilled echocardiographer is
adequate for diagnosis and initial management of
practically all diseases causing HF.
Other investigations
B-type natriuretic peptide (BNP): BNP, a cardiac
natriuretic hormone, secreted in escalating fashion in
ventricular dysfunction and progressive HF, is
increasingly used in acute settings for differentiation of
HF from pulmonary causes of respiratory distress.
While its utility in adults is established, its incremental
value in pediatric patients is still investigational.
Plasma BNP elevation is a reliable test however for
recognizing ventricular dysfunction in children with a
variety of CHD.9
Hemoglobin is important in diagnosis of HF in
children; while protracted values around 5 gm/dl can
cause HF even with a normal heart, hemoglobin of 7-8
gm/dl can cause decompensation in cases with
underlying heart disease but no HF. Electrolytes like
serum calcium, phosphorous and blood glucose should
be routinely measured in all children with HF,
especially neonates, where their abnormalities are an
uncommon but reversible cause of ventricular
dysfunction. Similarly screening for hypoxia and sepsis
also constitute evaluation of HF in a newborn. Work-up
for ascertainment of etiology of myocarditis and
cardiomyopathy is exhaustive and detailed elsewhere10.
ASO (anti-streptolysin O) and CRP (C-reactive protein)
are invaluable in work up for diagnosis of suspected
primary attack of rheumatic fever (RF) or recurrence of
RF in cases with rheumatic heart disease.
Staging the severity of Heart Failure
While several systems exist for grading severity of HF in
adults, including universally known New York Heart
Association Class, it is difficult to grade HF or apply
these classifications in children especially infants. A
common system followed is that advocated by Ross11 for
classification of heart failure (Table 4) and scoring its
severity (not shown).
Indian Journal of Pediatrics, Volume 76—February, 2009
T ABLE 4. Ross Classification of Heart Failure in Infants
Class I
Class II
Class III
Class IV
No limitations or symptoms
Mild tachypnea or diaphoresis with feeding in infants
Dyspnea on exertion in older children
No growth failure
Marked tachypnea or diaphoresis with feeds or
Prolonged feeding times
Growth failure from CHF
Symptoms at rest with tachypnea, retractions,
grunting, or diaphoresis
Management of Heart Failure
The management of HF in children can be divided into
following categories:
1. CHD presenting with acute shock, where
immediate treatment (pharmacologic, percutaneous,
or surgical) is required
In neonatal period several causes of HF can present
with acute circulatory collapse or progress to shock if
not recognized early. These can be due to
A closing ductus where antegrade systemic flow is
compromised, for e.g., tight COA, interruption of
aortic arch, critical AS, HLHS, and TGA with intact
septum and restrictive inter-atrial communication.
These disorders require maintenance of duct
patency with prostaglandin infusion till the time
more definitive treatment can be employed. This
consists of percutaneous procedures for critical AS
(valvuloplasty), TGA (balloon atrial septostomy) as
well as surgical procedures for neonatal COA,
HLHS etc. In cases where surgery for COA is not
possible due to severe comorbid conditions, a
balloon dilatation is performed, although the
restenosis rates are likely to be higher.
Conditions like mitral atresia (requiring emergency
atrial septostomy) and obstructed TAPVC (requires
emergency surgery) can cause severe elevations in
pulmonary venous pressure and have similar
Non-cardiac cause of neonatal HF, tachyarrhythmias and neonatal myocarditis can also rapidly
progress to shock if not managed early.
Lesions like AS, PS and COA, if associated with
corresponding ventricular dysfunction or heart
failure should undergo urgent relief of obstruction,
irrespective of magnitude of gradient at baseline.
Because these children are very sick, they should be
transferred to tertiary centers with expertise in their
care, after initial resuscitation and prostaglandin
infusion (if required). They require intensive monitoring
because of frequent co-morbidities and likely
Vivek Chaturvedi and Anita Saxena
requirement of ventilation (due to pulmonary edema,
chest infections or due to apnea as an adverse effect of
prostaglandin therapy)
2. CHD awaiting surgery where medical treatment is
applied for stabilization and alleviation of
symptoms- short-term medical therapy
This is a very common group because except some
VSDs and PDA in premature babies (which may close
spontaneously), most of CHDs causing HF require
surgical intervention. These children present with HF
and frequently have co-morbidities like sepsis or chest
infection. They tolerate repeated bouts of HF and chest
infection (which enters into a vicious cycle with HF)
poorly and should undergo surgery or nonsurgical
catheter intervention promptly after stabilization of
medical condition12. These conditions include
Large VSD/PDA/AVSD/Persistent truncus
arteriosus with uncontrolled CHF or history of life
threatening infection
Severe AS or COA
TGA with IVS and prepared LV
Unobstructed TAPVC.
3. CHD requiring long term medical therapy
Several causes of HF in children require prolonged
medical therapy because of tendency for spontaneous
resolution or a cure for the condition in long term or due
to the fact that the surgical treatment is problematic.
Ventricular septal defect is one of the commonest
causes for HF after the neonatal period, in infancy.
About 10% of non-restrictive VSD die in 1st year of
life, primarily due to CHF. However up to 30-40% of
small or moderate sized defects close spontaneously
(mostly by 3-5 years of age) and 25% decrease in
size 13. A minority of VSD with large L-R flow can
also close spontaneously14. Thus at least some VSD
presenting in infancy with less than severe HF can
be judiciously followed on medical therapy and
watched for spontaneous closure. Similarly, small
PDAs in term babies uptil 3 months of age, and those
in premature babies not in HF (with or without the
use of indomethacin) may be observed for
spontaneous closure.
Myocarditis in children is a potentially reversible
cause of HF provided the acute phase is cared for
with the best available medical care (ventricular
assist devices, if necessary). Similarly some
uncommon causes of cardiomyopathies (e.g.
carnitine deficiency associated) can be treated
effectively with supplementation.
Some conditions like congenital mitral stenosis are
problematic to manage in infancy and it is prudent to
defer surgery till later if the child is growing
4. Long-term therapy in cases with irreversible
myocardial dysfunction or where no other definitive
therapy can be offered.
Finally, there is the group of conditions causing HF
where there is established myocardial dysfunction. This
can be due to cardiomyopathies (primary and
secondary), decompensated systemic ventricle (SV
physiology, cTGA), and following palliative surgeries.
This group displays the whole spectrum from
asymptomatic ventricular dysfunction to decompensated
HF and requires long term medical therapy. HF therapy
is also required long term in residual defects following
surgery and in valvular diseases where the risk-benefit
ratio is not in favour of surgery.
Drugs for Treatment of Heart Failure
Management of CHF in children is complex because of
frequent presence of structural heart disease (on which
medical treatment has little effect) and variable
presentation and spontaneous resolution of some
diseases. On the other hand, advances in medical
science have ensured a wide variety of evidence-based
and emerging therapies for treatment or palliation of
HF (Table 5). There are several newer agents, the roles
of which are still investigational, in adults and in
children. These include natriuretic peptides (e.g.,
nesiritide), calcium sensitizers (e.g., levosimendan),
vasopressin antagonists (e.g., tolvaptan), renin
inhibitors (e.g., aliskiren), endothelin antagonists (e.g.,
sitoxentan) etc. Some like oral phosphodiesterase
inhibitors, anti-inflammatory molecules, nitric oxide
agonists, and neuropeptidase antagonists have not
proven useful or found to have excess side-effects.
Most of this evidence base has been generated in
adults in whom randomized trials usually happen
TABLE 5. Treatment Options for Chronic Heart Failure
ACE Inhibitors
Aldosterone antagonists
Anticoagulants (with
severe ventricular
Cardiac transplantation
(for structural disease)
Ventricular assist devices
Extracorporeal membrane
Intermittent ionotrope infusion
(weekend pulsed dobutamine)
Angiotensin receptor blocker
Ventricular remodeling
Cardiac resynchronization
Stem cell therapy
Indian Journal of Pediatrics, Volume 76—February, 2009
Heart Failure in Children: Clinical Aspect and Management
earlier. Conducting such trials in children has been
difficult on account of ethical issues and logistical
problems. While treatment of HF in children is often
extrapolated from that in adults, this may not be accurate
owing to different pathophysiology and etiology of HF in
children. These dilemmas are exemplified by the recently
published randomized controlled trial for carvedilol in
children15 with HF, where beta-blockade did not improve
outcomes over those with placebo. While smaller
uncontrolled studies earlier had shown significant
benefit with carvedilol, the neutral results of this trial
were presumably due to inadequate sample size (due to
high rate of spontaneous improvement in placebo group)
and heterogeneous effect of drug on type of systemic
ventricle (beneficial in systemic left ventricle only). Tables
6-8 outline the treatment of heart failure in children and
dosages of common drugs used for treatment in acute
and chronic settings.
TABLE 7. Oral Dosages of Common Drugs Used to Treat
Chronic Heart Failure
10mcg/kg/day (in two divided doses for
children <5 years)
1-4 mg/kg/day (1-2 doses)
Spironolactone 2-4 mg/kg/day (2 doses)
Neonates: (0.4-1.6 mg/kg/day) in 3 divided
Infants and children : 0.5-4 mg/kg/day in 3
divided doses
0.1-0.5 mg/kg/day (2 doses)- avoid in
0.5 mg/kg/day once daily
0.1-0.2 mg/kg/dose (2 doses) and increase to
1 mg/kg/dose or maximally tolerated dose
over weeks or months
0.05 mg/kg/dose (twice daily) and increase to
0.4-0.5 mg/kg/dose (twice daily) or
maximally tolerated dose
T ABLE 8. Stepwise Guide to Management of Heart Failure
TABLE 6. Treatment Algorithm for Acute Heart Failure in
Step 1
Supportive measures
Avoid hypothermia and hypoglycemia, check for hypocalcemia
Step 2
Maintenance of
Monitoring of blood gases if perfusion
adequate oxygenation is poor Ventilate, if required, with
modest PEEP to achieve PaO2 of 5060 mmHg and SaO2 of 75-85% to
avoid pulmonary congestion
Adequate hydration
Stop oral feeds if severe tachypnea
Intravenous access
I/V and CVP lines (umblical vein
Intravenous ionotropes Isoproterenol 0.5-2 mcg/kg/min
for shock
Dopamine 5-20 mcg/kg/min
Dobutamine 5-20 mcg/kg/min
Avoid digoxin or use cautiously
Ionotrope and vasodilator
Load with 25-50 mcg/kg/min.
maintain at 0.25-1 mcg/kg/min
Furosemide 2-4 mcg/kg PO/IV
Captopril 0.1-1 mg/kg/day PO q 8hrly
Sodium nitroprusside 0.5-4 mcg/kg/
min IV
Careful monitoring of blood pressure
Prostaglandin infusion Start at 0.1 mcg/kg/min (uptil 0.4
for ductus-dependent mcg/kg/min if no response), taper to
lowest dose possible (0.005 mcg/kg/
min) Monitor for apnea, keep minimum
required dose
Management of Heart Failure: Important Issues in
Treatment of HF in children, like in adults, should
consist of treatment of the cause, precipitating
factors (like anemia, infective endocarditis,
infections, acute rheumatic fever, non-compliance
with drug or diet, arrhythmias, etc), and treatment
of the congested state.
Digoxin has a very narrow safety window in
Indian Journal of Pediatrics, Volume 76—February, 2009
Step 3
Step 4
Step 5
Step 6
In acute decompensation : bed rest, propped up
position, humidified oxygen Sodium and, if required,
volume restriction.
Start digoxin (not in myocarditis)
Assess reversible causes and precipitating causes
Assess the need for surgery/ interventional
procedure in case of structural heart disease
Add ACE Inhibitor. In case of ACEI induced cough,
switch to ARB like losartan
Switch to nitrates if ACE Inhibitor/ARB not
Add carvedilol in compensated HF especially in
cases with tachycardia
Once or twice weekly dobutamine therapy
Consider stem cell coronary infusion
Cardiac transplantation
Ventricular assist device as bridge in case of delays
or even ?destination
children and adults alike. It should be avoided in
premature babies, those with renal compromised
state and cases with acute myocarditis. Electrolytes
(K+, Ca++, Mg ++) should be carefully monitored to
avoid potentiation of toxicity and development of
bradyarrhythmias in children)
Generally initial total digitalization is not
performed. One can start directly to oral
maintenance dose at 10 mcg/kg/day (The
available digoxin elixir has 50 mcg/ml, hence the
dose is 0.1 ml/kg twice daily)
Continuous infusion of diuretics is recommended
in cases of acute decompensated heart failure
(ADHF). Monitoring and supplementation of K+ is
necessary at higher doses, as deficiency is
associated with increased arrhythmic death.
During early infancy supplementation of
potassium is usually not required uptil 2mg/kg of
dose for furosemide or equivalent. In these cases we
Vivek Chaturvedi and Anita Saxena
give the parentral preparation of furosemide orally
for logistic reasons as furosemide syrup is not
available in our country. In other cases requiring
higher doses and in older children, we usually give
a combination of loop diuretic and spironolactone
(or other potassium sparing diuretics). In cases
requiring chronic therapy, development of diuretic
resistance is quite common. Addition of low dose
dopamine may help in this situation by increasing
renal blood flow.
ACE Inhibitors should be avoided in HF caused by
lesions having pressure overload physiology e.g. in
aortic stenosis, as they might interfere with
compensatory hypertrophy. The incidence of ACE
Inhibitor induced cough is much less in children
as compared to adults.
Beta blockers should not be administered in acute
decompensated heart failure. They should be
started once child is stable at low dose, and slow
up-titrations (once every two weeks through 4
levels according to pediatric carvedilol study
group trial15) should be done as this determines the
occurrence and degree of side effects. In case uptitration is not tolerated, lower doses should be
continued rather than discontinuing the drug. In
the carvedilol trial, overall about 20% children had
worsening of HF in both carvedilol and placebo
population, of which 11% each withdrew from the
study due to this.
Persistently high heart rates (>180/min in older
children) with absence of normal variability during
sleep or exercise should always be investigated to
rule out tachycardiomyopathy as the cause of HF.
Acute myocarditis and pediatric cardiomyopathy
deserve a special mention as despite intensive research
they still are an enigma and a challenge to clinicians.
Many clinicians view acute myocarditis and
cardiomyopathy in children as a continuum; indeed a
large study in North America found myocarditis as the
most common cause of cardiomyopathy (46%) 8. This
however increases the ambiguities in the diagnosis of
myocarditis especially the relevance and indications of
tissue diagnosis by endomyocardial biopsy. Several
small studies have been conducted with
immunoglobulin and immunosuppressive therapy in
children with acute myocarditis. However robust trials
are few, with the outcome that there is still no
consensus on use of these therapies16, 17. Of note, studies
in myocarditis indicate a high prevalence of resolution
of cardiomyopathy in 2 years to the tune of 50-80%18.
Children with fulminant myocarditis with a high
chance of recovery, do well when put on extracorporeal
membrane oxygenation (ECMO) and ventricular assist
devices 19, 20. These findings suggest that a diagnosis of
myocarditis is a positive prognostic factor in children
with HF even if requiring mechanical support.
Therefore circulatory assist devices, if available, should
be used aggressively in these children.
Cardiac transplantation
Heart transplantation has been used for treatment of
end-stage heart disease in children for nearly 4 decades
with first infant transplant done in late 1960s. Around
350 pediatric cardiac transplantations are done
annually, representing about 10% of total cardiac
transplantations. Majority of the transplantations are
carried out for end-stage heart disease due to
cardiomyopathies. Other causes include congenital
heart diseases like hypoplastic heart syndrome and
other complex CHD, single ventricle, palliated heart
disease, etc. One year survival has approached 90% and
estimated conditional graft half-life is about 17.5 yeas
in younger children (in comparison immediate waiting
list mortality is about 20 %.)18. However, given the fact
that the surgery is done in few centers globally and the
available donor hearts have remained static over last
many years to few hundreds, it is clear that heart
transplantation can be a solution for a minority only.
Stem Cell Therapy
A heightened interest has developed in stem cell
therapy for heart failure. Several trials have been
completed, or are ongoing in adults with HF,
predominantly due to ischemic heart disease. Stem cell
therapy has been also used for non ischemic
cardiomyopathy at our center 21, and is being
investigated worldwide under experimental settings,
including our center, for children with refractory heart
failure who are not candidates for transplantation.
HF in children is a complex syndrome with
heterogeneous etiology and presentation. Unlike adults,
HF in children is commonly due to structural heart
disease and reversible conditions, thus lending it
amenable to definitive therapy or short term aggressive
therapy. Thus the overall outcome with HF is better in
children than that in adults. Clinical presentation of HF
in younger children can be non-specific requiring
heightened degree of suspicion. In particular, some
conditions that can present with acute shock are
important to recognize, as they can be effectively treated
or palliated on an urgent basis. While the general
principles of management are similar to those in adults,
there is a dearth of evidence base in pediatric heart
failure. It would require a judicious balance of
extrapolation from adult medicine (thus avoiding
Indian Journal of Pediatrics, Volume 76—February, 2009
Heart Failure in Children: Clinical Aspect and Management
generation of redundant evidence) and development of
children specific treatments ( thus recognizing the
inherent differences in HF of children and adults) to
optimize the outcomes in this challenging field.
1. Ramakrishnan S, Kothari SS, Bahl VK. Heart FailureDefinition and Diagnosis. Indian Heart J 2005; 57:13-20
2. Braunwald E, Grossman W. Clinical aspects of heart failure.
In Braunwald E ed. Heart Disease: A Textbook of
Cardiovascular Medicine, 4th ed. Philadelphia; Saunders,
1992; 444–463
3. Hunt SA, Baker DW, Chin MH, Cinquegrani MP, Feldman
AM, Francis GS et al. ACC/AHA Guidelines for the
Evaluation and Management of Chronic Heart Failure in the
Adult: Executive Summary, A Report of the American
College of Cardiology/American Heart Association Task
Force on Practice Guidelines. Circulation 2001; 104: 2996–
4. Sommers C, Nagel BH, Neudorf U, Schmaltz AA.
Congestive heart failure in childhood. An epidemiologic
study. Herz 2005;30:652-662.
5. Webster G, Zhang J, Rosenthal D. Comparison of the
epidemiology and co-morbidities of heart failure in the
pediatric and adult populations: a retrospective crosssectional study. BMC Cardiovascular Disorders 2006;6: 23
6. Kay JD, Colan SD, Graham TP. Congestive heart failure in
pediatric patients. Am Heart J 2001; 42:923-928
7. Lipshultz SE, Sleeper LA, Towbin JA et al. The incidence of
pediatric cardiomyopathy in two regions of the United
States. N Engl J Med 2003; 348:1647–1655
8. Towbin JA, Lowe AM, Colan SD et al. Incidence, causes,
and outcomes of dilated cardiomyopathy in children.
JAMA 2006;296:1867-1876
9. Law YM, Keller BB, Feingold BM, Boyle GJ. Usefulness of
plasma B-type natriuretic peptide to identify ventricular
dysfunction in pediatric and adult patients with congenital
heart disease. Am J Cardiol 2005; 95: 474-478
10. Ross RD, Bollinger RO, Pinsky WW. Grading the severity of
congestive heart failure in infants. Pediatr Cardiol 1992;
Indian Journal of Pediatrics, Volume 76—February, 2009
11. Burch M. Heart failure in the Young. Heart 2002;88:198-202
12. Saxena A for ‘Working Group on Management of Congenital
Heart Diseases in India’. Consensus on timing of
intervention for common congenital heart disease. Indian
Pediatr 2008;45:117-126
13. Keith JD, Rose V, Collins G, Kidd BS. Ventricular septal
defect: incidence, morbidity, and mortality in various age
groups. Br Heart J 1971; 33:81.
14. Saxena A, Tandon R, Shrivastava S. Clinical course of
isolated ventricular sepal defect: an Indian experience.
Indian J Paediatr 1993; 60:777-782
15. Shaddy RE, Boucek MM, Hsu DT et al. Carvedilol for
children and adolescents with heart failure: a randomized
controlled trial. JAMA 2007;298:1171-1179
16. Robinson JL, Hartling L, Crumley E, Vandermeer B, Klassen
TP. A systematic review of intravenous gamma globulin for
therapy of acute myocarditis. BMC Cardiovasc Disord
17. Hia CP, Yip WC, Tai BC, Quek SC. Immunosuppressive
therapy in acute myocarditis: an 18 year systematic review.
Arch Dis Child 2004;89:580-584
18. Canter CE, Shaddy RE, Bernstein D et al. Indications for
Heart Transplantation in Pediatric Heart Disease: A
Scientific Statement from the American Heart Association
Council on Cardiovascular Disease in the Young; the
Councils on Clinical Cardiology, Cardiovascular Nursing,
and Cardiovascular Surgery and Anesthesia; and the
Quality of Care and Outcomes Research Interdisciplinary
Working Group. Circulation 2007;115;658-676
19. Duncan BW, Bohn DJ, Atz AM et al. Mechanical circulatory
support for the treatment of children with acute fulminant
myocarditis. J Thorac Cardiovasc Surg 2001; 122:440–448.
20. Stiller B, Dahnert I, Weng YG, Hennig E, Hetzer R, Lange PE.
Children may survive severe myocarditis with prolonged
use of biventricular assist devices. Heart 1999;82: 237–240.
21. Seth S, Narang R, Bhargava B, for the AIIMS
Cardiovascular Stem Cell Study Group. Percutaneous
intracoronary cellular cardiomyoplasty for nonischemic
cardiomyopathy: clinical and histopathological results: the
first-in-man ABCD (Autologous Bone Marrow Cells in
Dilated Cardiomyopathy) trial. J Am Coll Cardiol 2006;