Transcript heart failure in neonate and infant

HEART FAILURE IN
NEONATE AND INFANT
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Congestive heart failure (CHF) refers to a clinical state of
systemic and pulmonary congestion resulting from inability of
the heart to pump as much blood as required for the
adequate metabolism of the body.
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Clinical picture of CHF results from a combination of
“relatively low output” and compensatory responses to
increase it
PATHOPHYSIOLOGY
Unmet tissue demands for cardiac output result in activation of
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Renin-aldosterone angiotensin system
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Sympathetic nervous system
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Cytokine-induced inflammation
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“signaling” cascades that trigger cachexia.
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Longstanding increases in myocardial work and myocardial
oxygen consumption (MVO2) ultimately worsen HF
symptoms and lead to a chronic phase that involves cardiac
remodeling
CARDIAC REMODELING?
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Maladaptive cardiac hypertrophy
Expansion of the myofibrillar components of individual
myocytes (new cells rarely form)
An increase in the myocyte/capillary ratio
Activation and proliferation of abundant nonmyocyte cardiac
cells, some of which produce cardiac scarring
Produce a poorly contractile and less compliant heart
Endogenous mechanisms defend progressive HF
 Stimulation of insulin like growth factor and GH
 ANP and BNP are hormones secreted by the heart in
response to volume and pressure overload that increase
vasodilation and diuresis acutely and chronically prevent
inflammation, cardiac fibrosis and hypertrophy.
CLINICAL MANIFESTATIONS IN INFANTS
WITH HF
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Variety of age dependent clinical presentations
In neonates, the earliest clinical manifestations may be subtle
CLINICAL MANIFESTATIONS IN INFANTS
WITH HF
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Feeding difficulties
Rapid respirations
Tachycardia
Cardiac enlargement
Gallop rhythm (S3)
Hepatomegaly
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Pulmonary rales
Peripheral edema
Easy fatigability.
Sweating
Irritability
failure to thrive.
Feeding difficulties & increased fatigability
Important clue in detecting CHF in infants
 Often it is noticed by mother
 Interrupted feeding (suck- rest -suck cycles)
 Infant pauses frequently to rest during feedings
 Inability to finish the feed, taking longer to finish each feed
(> 30 minutes)
 Forehead sweating during feeds –due to activation of
sympathetic nervous system –a very useful sign
 Increasing symptoms during and after feedings
Rapid respirations
Tachypnea
> 60/min in 0-2mth
>50/mt in 2mth to 1yr
>40/mt 1-5 yr in calm child
 Happy tachypnea- tachypnea with out much retractions
 Grunting (a form of positive end-expiratory pressure)
 In cyanotic heart disease rapid respirations may be due to
associated brain anoxia and not CHF -treatment for these two
conditions is entirely different
 Fever especially with a pulmonary infection may produce
rapid respirations.
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Tachycardia
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Rate is difficult to evaluate in a crying or moving child
Tachycardia in the absence of fever or crying when
accompanied by rapid respirations and hepatomegaly is
indicative of HF
Persistently raised heart rate > 160 bpm in infants
> 100 bpm in older children.
Consider SVT if heart rate > 220 bpm in infants and > 180
bpm in older children.
Cardiomegaly
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Consistent sign of impaired cardiac function, secondary to
ventricular dilatation and/or hypertrophy.
May be absent in early stages, especially with myocarditis,
arrhythmias, restrictive disorders and pulmonary venous
obstruction(obstructed TAPVC)
Apex 4th space 1cm outside MCL in newborn
Hepatomegaly
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Lower edge of the liver is palpable 1 to 2 cms below right
costal margin normally in infancy
In the presence of respiratory infection increased expansion
of the lungs displace liver caudally
Usually in such circumstances the spleen is palpable
Hepatomegaly is a sign of CHF
Decrease in size is an excellent criterion of response to
therapy
Pulmonary rales
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Of not much use in detecting CHF in infants
Rales may be heard at both lung bases
When present are difficult to differentiate from those due to
the pulmonary infection which frequently accompanies
failure
Peripheral edema
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Edema is a very late sign of failure in infants and children
Presacral and posterior chest wall edema in young infants
It indicates a very severe degree of failure.
Daily wt monitoring is useful in neonates -- rapid increase in
wt > 30 gm /day may be a clue to CCF and is useful in
monitoring response to treatment.
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Cold extremity, low blood pressure, skin mottling are signs of
impending shock
Pulsus alternans (alternate strong and weak contractions of a
failing myocardium),or pulsus paradoxus (decrease in pulse
volume and blood pressure with inspiration) are frequently
observed in infants with severe CHF
CLASSIFICATION
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NYHA Heart Failure Classification is not applicable
Ross Heart Failure Classification was developed for global
assessment of heart failure severity in infants
Modified to apply to all pediatric ages
Modified Ross Classification incorporates
Feeding difficulties
Growth problems
Symptoms of exercise intolerance
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
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The time of onset of CHF holds the key to the etiological
diagnosis in this age group
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Parallel circulation becomes series at birth
Cardiac anomalies present at that point are
Critical AS
HLHS
Mitral atresia
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Functional closure PDA 1 to 2weeks
PDA dependent lesions ,depend on patent duct for either
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pulmonary blood flow- Fallots with pulmonary atresia
systemic blood flow-IAA/COA
mixing of systemic and pulmonary blood-TGA
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Present at 1 to 2weeks
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Anatomic closure of PDA by 2to4 weeks
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Coarctation of aorta
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Pulmonary vascular resistance falls 4to 6weeks
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Congestive heart failure due to L-R shunt
Large VSD
PDA
ALCAPA
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CHF in the fetus
Disorders that are fatal in the immediate neonatal period are
often well tolerated in the fetus due to the pattern of fetal
blood flow (e.g. TGA)
Causes of CHF in the fetus
 SVT
 Severe bradycardia due to CHB
 Anemia
 Severe TR due to Ebstein’s anomaly or MR from AV canal
defect
 Myocarditis
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FETAL BLOOD FLOW
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Most of these are recognized by fetal echo
Severe CHF in the fetus produces hydrops fetalis with ascites,
pleural and pericardial effusions and anasarca.
Digoxin or sympathomimetics to the mother may be helpful
in cases of fetal tachyarrhythmia or CHB respectively.
Premature neonates
PDA
 poor myocardial reserve
 Fluid overload
CHF on first day of life
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Myocardial dysfunction secondary to asphyxia, hypoglycemia,
hypocalcaemia or sepsis are usually responsible for CHF on
first day
Few structural heart defects cause CHF within hours of birth
HLHS, severe TR or PR, Large AV fistula
TR secondary to hypoxia induced papillary muscle
dysfunction or Ebstein’s anomaly of the valve
Improves as the pulmonary artery pressure falls over the next
few days
CHF in first week of life
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Serious cardiac disorders which are potentially curable but
carry a high mortality if untreated often present with CHF in
the first week of life
A sense of urgency should always accompany evaluation of
the patient with CHF in the first week
Closure of the ductus arteriosus is often the precipitating
event
Prostaglandins E1 should be utilised
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Peripheral pulses and oxygen saturation (pulse oximeter)
should be checked in both the upper and lower extremities
A lower saturation in the lower limbs means right to left
ductal shunting due to PAH or AAI
ASD or VSD does not lead to CHF in the first two weeks of life,
an additional cause must be sought (eg.COA or TAPVC).
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TGA
no VSD -1ST week
VSD and no PS-6-8 weeks
Critical AS or PS
Obstructive TAPVC
Adrenal insufficiency due to enzyme deficiencies or neonatal
thyrotoxicosis could present with CHF in the first few days of
life
ALPROSTODIL
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Prostaglandins E1
Maintain patency of ductus
Cyanotic lesions TGA
LT sided obstructive lesions HLHS, critical AS,COA,IAA
Available as inj 500microgm/ml
IV 0.05 to0.1microgm /kg/min
0.01 to 0.05 microgm /kg/min maintainance
Vasodilation of all arteries including ductus
Monitor spo2,RR, HR,BP,ECG,temp
Complications
 apnea,
 Seizure
 Hypotension
 Bradycardia
 Tachycardia
 cardiac arrest
 fever
 Extravasation may cause sloughing and necrosis
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CHF beyond second week of life
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Most common cause of CHF in infants is VSD
Presents around 6-8 weeks of age.
Left to right shunt increases as the PVR falls
Murmur of VSD is apparent by one week
Full blown picture of CHF occurs around 6-8 weeks.
Other left to right shunts like PDA present similarly
Fall in PVR is delayed in presence of hypoxic lung disease and
at high altitude and can alter the time course
Spontaneous improvement in CHF -development of
obstructive pulmonary arterial hypertension even in early
childhood
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ALCAPA a rare disease in this age group
It is curable
As the pulmonary artery pressure decreases in the neonatal
period, these babies suffer from episodes of excessive crying
with sweating (angina) and myocardial infarction.
ECG shows pathologic q waves
Often misdiagnosed as having “dilated cardiomyopathy”
CAUSES OF HF IN CHILDREN
CARDIAC
Congenital structural malformations
● Excessive Preload
● Excessive Afterload
● Complex congenital heart disease
No structural anomalies
● Cardiomyopathy
● Myocarditis
● Myocardial infarction
● Acquired valve disorders
● Hypertension
● Kawasaki syndrome
● Arrhythmia
(bradycardia or tachycardia)
NONCARDIAC
● Anemia
● Sepsis
● Hypoglycemia
● Diabetic ketoacidosis
● Hypothyroidism
● Other endocrinopathies
● Arteriovenous fistula
● Renal failure
● Muscular dystrophies
CONGENITAL STRUCTURAL
MALFORMATIONS
VOLUME OVERLOAD (EXCESSIVE PRELOAD)
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Left-to-right shunting
VSD
PDA
AP window
AVSD
ASD(rare)
Total/Partial Anomalous Pulmonary Venous Connection
AV or semilunar valve insufficiency
AR in bicommissural aortic valve/after valvotomy
MR after repair of AVSD
PR after repair of TOF
Severe TR in Ebstein anomaly
Right-sided volume loading
 Large ASD or anomalous pulmonary vein connections
 Congenital or surgically acquired PR especially if downstream
pulmonary arterial narrowing
 Highly compliant RV accepts significant volume -without
increasing filling pressure
 Rarely causes HF early in life
PRESSURE OVERLOAD (EXCESSIVE AFTERLOAD)
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Left sided obstruction
Congenital AS
Aortic coarctation
Lethal arrhythmias - severe afterload stress?
?HTN
Right-sided obstruction
Severe PS
Left heart obstructive lesions
 First postnatal week-ductus arteriosus closes
 Increased LVEDP and a decreased pressure gradient between
the aorta and ventricle at end-diastole produce
subendocardial ischemia due to inadequate coronary flow
 Increased afterload and subendocardial ischemia result in
HF syndrome
COMPLEX CONGENITAL HEART DISEASE
Abnormal RV
 CCTGA
 D TGA
Single ventricle physiology
 HLHS
 Unbalanced AVSD
 Post Fontan procedure
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Often combined volume and pressure overload
Both systemic and pulmonary circulations can be affected
Cyanosis in CCHD-risk of subendocardial ischemia
contributing to impaired ventricular performance
Molecular abnormalities in transcription factors that lead to
congenital structural abnormalities – also associated with
abnormal myocardial performance and arrhythmias
ABNORMAL RV
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In pediatric heart disease much of the pathology is due to an
abnormal RV
RV myocytes appear to be structurally identical to LV
myocytes
Differences in contraction compared to the LV are due to the
shape of the RV and myocardial organization
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Gene expression patterns are different in the RV and the LV,
which may affect function.
Genes that affect angiotensin and adrenergic receptor
signaling showed lower expression in the RV than the LV
Genes that contribute to maladaptive signaling showed
higher expression in the RV
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Hypoplastic right heart syndromes -3 parts of the RV do not
form normally or may be missing entirely.
Defects in the IVS or abnormal LV function- Adversely affect
the third phase of normal RV contraction through its
interdependence on normal septal function
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Volume overload of the RV
Can arise through significant PR or TR
Compensatory dilation to decompensated dilation occur
slowly
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Increased RV afterload
RVOT obstruction
RV serving as the systemic ventricle
Usually can adapt if present at birth
Once the RV assumes a mature, thin-walled configuration, it
cannot always mount a hypertrophic response
RV is able to support the systemic circulation for many years
but function often deteriorates over time
SINGLE VENTRICLE PHYSIOLOGY
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Ventricular morphology (left, right, indeterminate, or
unbalanced) results in a single functional pumping chamber
At birth presentation depends on the morphology
Range from well-tolerated cyanosis to decompensated heart
failure and cardiogenic shock
double inlet ventricle(SV), HLHS , Tricuspid atresia, isomerism
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Pathophysiological factors associated with heart failure in SV
physiology in the newborn period are
Unobstructed pulmonary blood flow
Obstruction to systemic flow
Obstruction to pulmonary venous return
Insufficiency of the atrioventricular valve
Myocardial abnormalities or dysfunction
Coronary hypoperfusion.
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These factors can occur individually or in various
combinations
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Functional single ventricle heart is volume-loaded because of
the need to supply the pulmonary and systemic circulations,
until the creation of the cavo-pulmonary anastomosis at 6
months of age.
Elevated BNP levels before the surgery; afterward, they
return to normal
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After the Fontan procedure
Diastolic filling properties often remain abnormal for some
time
Ventricular function depend on morphology
Single RV has a lower mass: volume ratio which creates a
relative increase in wall stress -poorer performance
Single RV does not have the functional benefit of the
interdependence with the LV and interventricular septum
that the RV has in 2-ventricle physiology
Fontan procedure
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Conduction and rhythm abnormalities is relatively high after
Fontan procedure
Fontan procedure is often well-tolerated for many years
As increasing numbers of these patients survive to adulthood,
the prevalence of so-called Fontan failure is increasing
CHF WITH NO CARDIAC
MALFORMATIONS
PRIMARY CARDIAC
NONCARDIAC
Cardiomyopathy
 Myocarditis
 Cardiac ischemia
 Acquired valve disorders
 Hypertension
 Kawasaki syndrome
 Arrhythmia
(bradycardia or tachycardia)
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Anemia
 Sepsis
 Hypoglycemia
 Diabetic ketoacidosis
 Hypothyroidism
 Other endocrinopathies
 Arteriovenous fistula
 Renal failure
 Muscular dystrophies
DISORDERS OF CONTRACTILITY
Cardiomyopathy is a genetically triggered or acquired disease
 Occurs in approximately 1.13 in 100,000 children
 HF (less commonly, dysrhythmia) is the presenting feature
DCM
 Characterized by enlarged ventricular chambers and impaired systolic and
diastolic function
 Usually idiopathic
Infection (myocarditis viral-enterovirus)
Operative injury
Consequence of degenerative or metabolic diseases
Muscular dystrophies
Mitochondriopathy,
Hyperthyroidism
carnitine deficiency
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Restrictive cardiomyopathy
Idiopathic
Infiltrative or storage diseases
hemochromatosis
Pompe disease
Hypertrophic cardiomyopathy
 Idiopathic hypertrophic subaortic stenosis, rarely associated
with pediatric HF.
ARRHYTHMIAS
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Arrhythmias cause HF when the heart rate is too fast or too
slow to meet tissue metabolic demands
TACHYCARDIA
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Diastolic filling time shortens to and cardiac output is
decreased.
Most common childhood tachyarrhythmia is SVT
Often presents in the first few months of life
Rarely cause heart failure
Occasionally PJRT ,ectopic atrial tachycardia and VT
CHRONIC BRADYCARDIAS
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LV enlarges to accommodate larger stroke volumes
Chamber dilation reaches a limit that cannot be compensated
without increase in heart rate
Febrile states are particularly stressful
Congenital CHB may be well-tolerated in utero
Dysfunction cause hydrops and intrauterine demise
After birth, progression to HF depends on the ventricular rate
and the speed of diagnosis and intervention
Children with congenital CHB who are pacemaker dependent
are at risk of subsequent pacemaker-mediated
cardiomyopathy
CARDIAC ISCHEMIA
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Relatively rare in children
ALCAPA
Palliative surgery that requires reconstruction of or near the
coronary arteries
e.g. Ross procedure, arterial switch operation
HIGH OUTPUT HF +EXCESSIVE PRELOAD
Septic shock causes
 Volume load on both sides of the heart
 Increased SV associated with hyperdynamic systolic function
 Elaboration of vasoactive molecules such as endotoxin and
cytokines such as TNF-alpha leads to decreased SVR
 Cardiac output is increased
 Precapillary shunting
 Decreased tissue perfusion and lactic acid production
 Increased vascular permeability -increased total body fluid volume
 Toxin or direct microbial actions -negative inotropic effects
 Stresses produce demands for cardiac output and MVO2
LABORATORY STUDIES
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PULSE OXIMETRY
ECG
ABG
CXR
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Size of the heart is difficult to determine radiologically,
particularly if there is a superimposed thymic shadow.
Enlarged cardiac shadow unassociated with signs of CHFsuspect that shadow noncardiac
Absence of cardiomegaly in a good inspiratory film (with
diaphragm near the 10th rib posteriorly) practically excludes
CHF except due to a cause like obstructed total anomalous
pulmonary venous connection (TAPVC)
CT Ratio method, > 60%
Massive cardiomegaly
RA dilation
Pulm plethora
LV Dialatation
ECHOCARDIOGRAPHY
Not useful for the evaluation of HF, which is a clinical
diagnosis
Essential for identifying
 Causes of HF such as structural heart disease
 Ventricular dysfunction (both systolic and diastolic)
 Chamber dimensions
 Effusions (both pericardial and pleural)
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Assessment of right and single ventricular function is more
complicated because of altered geometry
RV tissue Doppler imaging correlates with measurements of
RVEDP obtained during cardiac catheterization
Doppler myocardial performance index has been used to
assess function in children with SVs and abnormal RVs
Single (left) ventricle physiology-remodeling to a spherical
shape associated with deterioration
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CMR- Geometric assessment of RV and SV function
3D echo -additional detail of intracardiac anatomy
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Worse EF and FS at presentation -poor outcome in children
with DCM
LV remodeling to a more spherical shape -predict a poorer
prognosis in children with DCM
Myocarditis- children can present with severely depressed
ventricular function but recover normal function within a few
weeks to months
Lack of improvement in EF % over time –correlate worse
outcome.
HF BIOMARKERS
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Released primarily in response to atrial stretching
Sensitive marker of cardiac filling pressure and diastolic
dysfunction
BNP levels can distinguish between cardiac and pulmonary
causes of respiratory distress in neonates and children
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In acute decompensated heart failure due to cardiomyopathy
a BNP level 300 pg/Ml strongly correlate with poor outcome
than symptoms or echocardiographic findings
BNP levels can be different in children with DCM and
congenital heart disease despite similar NYHA class, EF, and
MVO2
PRINCIPLES OF MANAGING
HEART FAILURE
Recognition and treatment of underlying systemic disease
 Timely Surgical Repair of Structural Anomalies
Afterload Reduction
 ACE inhibitors
 ARB
 Milrinone Type 4 phosphodiesterase inhibitors
 Nitrates
 Recombinant BNP
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Preload Reduction
 Diuretics
 BNP
Sympathetic Inhibition
 Beta blockers
 Recombinant BNP
 Digoxin
Cardiac Remodeling Prevention
 Mineralocorticoid inhibitors
Inotropy
 Digoxin
MEDICAL THERAPY
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Medical management aims to maximize cardiac output and
tissue perfusion while minimizing stresses that increase
MVO2
Goals are accomplished by reducing afterload stress and
preload
Treatments that “rest” the heart such as vasodilators are
preferred to inotropic agents that increase MVO2
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Few drugs have evidence based efficacy compared to adults
Pediatric dosing is necessary
Scaling adult doses for pediatric use solely based on weight
can result in either inadequate or excessive drug levels
GENERAL MEASURES
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Bed rest and limit activities
Nurse propped up or in sitting position
Control fever
Expressed breast milk for small infants
Fluid restriction in volume overloaded
Optimal sedation
Correction of anemia ,acidosis, hypoglycemia and
hypocalcaemia if present
Oxygen –caution in LT-RT shunt as pulmonary vasodilation my
increase shunt
CPAP or mechanical ventilation as necessary
CONGENITAL HEART DISEASE:
VOLUME OVERLOAD
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General therapeutic approach is to minimize symptoms and
optimize growth until a definitive procedure can be
performed.
Mainstays of medical therapy are digitalis and diuretics.
DIGITALIS
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Digitalis considered as essential component
Evidence for efficacy is less in volume-overload lesions with
normal function where the mild inotropic effect of digitalis is
unnecessary
Sympatholytic properties may modulate pathological
neurohormonal activation
LOOP DIURETICS
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Furosemide improved clinical symptoms on a background of
digitalis administration
Decrease pulmonary congestion and thus decrease the work
of breathing
It is one of the least toxic diuretics in pediatrics
Associated with sensorineural hearing loss after long-term
administration in neonatal respiratory distress
Deafness related to speed of infusion
Torasemide is also safe and effective in this group

26. Faris R FM, Purcell H, Poole‐Wilson PS, Coats AJS. Diuretics for heart failure. Cochrane
Database of Systematic Reviews. 2006.
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27. Ward OC, Lam LK. Bumetanide in heart failure in infancy. Arch Dis Child. 1977
Nov;52(11):877‐82.
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28. Muller K, Gamba G, Jaquet F, Hess B. Torasemide vs. furosemide in primary care patients
with chronic heart failure NYHA II to IV‐‐efficacy and quality of life. Eur J Heart Fail. 2003
Dec;5(6):793‐801.
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29. Senzaki H, Kamiyama MP, Masutani S, Ishido H, Taketazu M, Kobayashi T, et al. Efficacy and
safety of Torasemide in children with heart failure. Arch Dis Child. 2008 Mar 12.
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30. Lowrie L. Diuretic therapy of heart failure in infants and children. Prog Pediatr Cardiol. 2000
Nov 4;12(1):45‐55.
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31. Arnold WC. Efficacy of metolazone and furosemide in children with furosemide‐resistant
edema. Pediatrics. 1984 Nov;74(5):872‐5.
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32. Rosenberg J, Gustafsson F, Galatius S, Hildebrandt PR. Combination therapy with
metolazone and loop diuretics in outpatients with refractory heart failure: an observational study
and review of the literature. Cardiovasc Drugs Ther. 2005 Aug;19(4):301‐6.
ACE INHIBITION
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Improved growth was seen in some children with CHF
Captopril and enalapril
Concerning incidence of renal failure particularly in
premature and very young infants.
No efficacy data on ARBs in children with heart failure
B BLOCKER
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Propranolol to the combination of digoxin and diuretics
shown to improve HF symptoms and improve growth
SPIRONOLACTONE
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Literature supporting the role in paediatric HF is limited
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61. Hobbins SM, Fowler RS, Rowe RD, Korey AG. Spironolactone therapy in infants
with congestive heart failure secondary to congenital heart disease. Arch Dis Child.
1981 Dec;56(12):934‐8.
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62. Buck ML. Clinical experience with spironolactone in pediatrics. Ann
Pharmacother. 2005 May;39(5):823‐8.
NESIRITIDE
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Recombinant form of BNP
Promotes both diuresis and vasodilation
Drug reduces both preload and afterload
Directly inhibits the sympathetic nervous system,
mineralocorticoid expression, and cardiac fibroblast
activation and promotes myocyte survival.
Studies in the pediatric age group are lacking
INTRACARDIAC REPAIR
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Early transcatheter or surgical intervention, often before age
6 months is possible
Minimizes time of significant symptoms or medication
Minimizes the risk of pulmonary vascular disease.
Contemporary data indicate that early repair of a VSD, even in
the first month of life and at weights 4 kg, does not confer
increased risk compared with older, larger infants.
TRANSCATHETER DEVICE CLOSURE
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Transcatheter device closure of muscular VSD
Weight atleast 5.2 kg.
CONGENITAL HEART DISEASE
PRESSURE OVERLOAD
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Ventricular response to pressure overload is determined by
the severity and duration of the load
Critical AS can cause acute LV failure in early infancy
“Critical "implies a requirement for maintaining PDA with
prostaglandin infusion
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Optimizing hemodynamics until urgent intervention
Balloon valvuloplasty, first described in neonates in 1986
replaced surgical valvotomy, as the first-line intervention in
uncomplicated AS, including critical AS.
Ventricular function improves and usually normalizes after
catheter based or surgical intervention.
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Higher AV gradient -associated with lower FS, decreased
exercise capacity, increased risk of SCD and serious
arrhythmias
Severe AS (Doppler MG 50 mm Hg(40)) - intervention to
prevent or ameliorate symptoms
Mild AS (Doppler MG 25 mm Hg) could be followed up
These criteria continue to guide contemporary management
along with other criteria such as symptoms, exercise capacity,
ventricular hypertrophy, wall stress, and evidence of
arrhythmia.
COMPLEX CONDITIONS
RV failure in children
 There is no systematic clinical evidence for anticongestive
therapy
 Furosemide- relieve the clinical symptoms
 RV dysfunction - betablocker therapy did not improve
ventricular function
 Suggest a different pathophysiological process in RV failure
and thus a requirement for novel treatment strategies
RV functioning as systemic ventricle


If symptomatic ventricular dysfunction occurs
ISHLT Guidelines recommend diuretics, digitalis, and ACE
inhibition, based solely on expert consensus
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Fontan procedure
Systemic and pulmonary circulations are separated and SV is
pumping to the systemic circulation
A large cross-sectional study of 546 Fontan survivors aged 6 to 18
years found normal ejection fraction in 73% of subjects but
abnormal diastolic function in 72%.
Diastolic function was significantly worse in the group with RV
compared with LV or mixed ventricular morphology.
Overt heart failure after the Fontan operation is relatively
infrequent in the pediatric population but increases in the adult
Identifying and treating underlying causes of HF such as conduction
or rhythm abnormalities or residual structural lesions is initial
strategy.

Single ventricle

No compelling data to guide medical treatment
ISHLT guidelines recommend diuretics, digitalis, and ACE
inhibition but not beta blockade, based on expert consensus.

CARDIOMYOPATHIES

Primary or acquired DCM

ISHLT Guidelines reflect only data from studies in adults in
recommending both digitalis and diuretics only for
symptomatic LV dysfunction in children
Torasemide, a newer loop diuretic with potassium-sparing
properties, significantly improved New York University
Pediatric Heart Failure Index, decreased BNP levels, and
improved fractional shortening


Senzaki etal Efficacy and safety of Torasemide in children with heart failure. Arch
Dis Child. 2008 Mar 12
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ISHLT Guidelines recommend ACE inhibition for moderate or
severe degrees of LV dysfunction regardless of symptoms
ARB therapy if ACE inhibitor is indicated but not tolerated

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Although the carvedilol trial did not demonstrate efficacy
based on the primary end point improvement in FS and
clinical outcome seen in DCM patients who received
carvedilol has led to the empirical use of carvedilol in this
group of patients.
long-term responses to BB therapy have not been studied in
children
Close monitoring of potential adverse effects is essential
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Systemic exposure to carvedilol amongst paediatric heart
failure patients and has indicated that higher doses relative to
body weight are required to provide exposure comparable to
adults
Paediatric carvedilol doses
1mg/kg/day for adolescents
2mg/kg/day for children aged 2 to 11 years
3mg/kg/day for infants (aged 28 days to 23 months)
Carvedilol used in many of the studies have been lower than
these recommendations

Treatment of primary diastolic heart failure in children with
hypertrophic or restrictive cardiomyopathy are limited to the
judicious use of diuretics to decrease the degree of pulmonary
congestion.
Inotropes in acute cardiac failure

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Routine use of in children cannot be recommended
Used in treatment of exacerbating conditions and as a
bridging therapy pending transplantation
Dopamine as it possesses both the cardiac and renal effects is
more useful




Practice guidelines for pediatric heart failure, developed by
the International Society for Heart and Lung Transplantation
(ISHLT)
None of the 49 recommendations is level A evidence
7 are level B evidence
Remainder are level C (expert consensus).
NUTRITION AND EXERCISE IN
PEDIATRIC HEART FAILURE


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Important as medical therapy, particularly in infants
Increase the caloric density of feeds as soon as a diagnosis
Sodium restriction is not recommended in infants and young
children.
Sodium restriction can result in impaired body and brain
growth

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There is evidence that regular physical activity can result in
sustained improvements in physical functioning even in
children with complex congenital heart disease.
Significant, sustained improvements in exercise function,
behavior, self-esteem and emotional state.
SURGICAL AND DEVICE
THERAPY
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Pacemaker and implantable defibrillator therapy
Biventricular pacing
Ventricular assist devices
Heart transplantation
THANK U