chf
TRANSCRIPT
HEART FAILURE IN
NEONATE AND INFANT
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.
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
Renin-aldosterone angiotensin system
Sympathetic nervous system
Cytokine-induced inflammation
“signaling” cascades that trigger cachexia.
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?
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.
Variety of age dependent clinical presentations
In neonates, the earliest clinical manifestations
may be subtle
CLINICAL MANIFESTATIONS IN
INFANTS WITH HF
CLINICAL MANIFESTATIONS IN
INFANTS WITH HF
Feeding difficulties
Rapid respirations
Tachycardia
Cardiac
enlargement
Gallop rhythm (S3)
Hepatomegaly
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.
Tachycardia
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
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
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
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
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.
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
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
The time of onset of CHF holds the key to the
etiological diagnosis in this age group
Parallel circulation becomes series at birth
Cardiac anomalies present at that point are
Critical AS
HLHS
Mitral atresia
Functional closure PDA 1 to 2weeks
PDA dependent lesions ,depend on patent duct for either
pulmonary blood flow- Fallots with pulmonary atresia
systemic blood flow-IAA/COA
mixing of systemic and pulmonary blood-TGA
Present at 1 to 2weeks
Anatomic closure of PDA by 2to4 weeks
Coarctation of aorta
Pulmonary vascular resistance falls 4to
6weeks
Congestive heart failure due to L-R shunt
Large VSD
PDA
ALCAPA
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
FETAL BLOOD FLOW
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
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
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
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).
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
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
CHF beyond second week of
life 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
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)
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)
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
Often combined volume and pressure overload
Both systemic and pulmonary circulations can be affected
Cyanosis in CCHD-risk of subendocardialischemia 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
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
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
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
Volume overload of the RV
Can arise through significant PR or TR
Compensatory dilation to decompensated
dilation occur slowly
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
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
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.
These factors can occur individually or in various combinations
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
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
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
Cardiomyopathy
Myocarditis
Cardiac ischemia
Acquired valve disorders
Hypertension
Kawasaki syndrome
Arrhythmia
(bradycardia or tachycardia)
NONCARDIAC
Anemia
Sepsis
Hypoglycemia
Diabetic ketoacidosis
Hypothyroidism
Other endocrinopathies
Arteriovenous
Renal failurefistula
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
Restrictive cardiomyopathy
Idiopathic
Infiltrative or storage diseases
hemochromatosis
Pompe disease
Hypertrophic cardiomyopathy
Idiopathic hypertrophic subaortic stenosis,
rarely associated with pediatric HF.
ARRHYTHMIAS
Arrhythmias cause HF when the heart rate is
too fast or too slow to meet tissue metabolic
demands
TACHYCARDIA
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
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
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
PRELOADSeptic 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
PULSE OXIMETRY
ECG
ABG
CXR
Size of the heart is difficult to determine radiologically, particularly if there is a superimposed thymic shadow.
Enlarged cardiac shadow unassociated with signs of CHF- suspect 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)
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
CMR- Geometric assessment of RV and SV
function
3D echo -additional detail of intracardiac
anatomy
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
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
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
Preload Reduction
Diuretics
BNP
Sympathetic Inhibition
Beta blockers
Recombinant BNP
Digoxin
Cardiac Remodeling Prevention
Mineralocorticoid inhibitors
Inotropy
Digoxin
MEDICAL THERAPY
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
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
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
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
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
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
26. Faris R FM, Purcell H, Poole‐Wilson PS, Coats AJS. Diuretics for heart failure. Cochrane Database of Systematic Reviews. 2006.
27. Ward OC, Lam LK. Bumetanide in heart failure in infancy. Arch Dis Child. 1977 Nov;52(11):877‐82.
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.
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.
30. Lowrie L. Diuretic therapy of heart failure in infants and children. Prog Pediatr Cardiol. 2000 Nov 4;12(1):45‐55.
31. Arnold WC. Efficacy of metolazone and furosemide in children with furosemide‐resistant edema. Pediatrics. 1984 Nov;74(5):872‐5.
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
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
Propranolol to the combination of digoxin and
diuretics shown to improve HF symptoms and
improve growth
SPIRONOLACTONE
Literature supporting the role in paediatric HF
is limited
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.
62. Buck ML. Clinical experience with spironolactone in pediatrics. Ann Pharmacother. 2005 May;39(5):823‐8.
NESIRITIDE
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
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
Transcatheter device closure of muscular VSD
Weight atleast 5.2 kg.
CONGENITAL HEART DISEASE
PRESSURE OVERLOAD
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
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.
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
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
Fontan procedure
Systemic and pulmonary circulations are separated and SV is pumping to the systemic circulation
A large cross-sectional study of 546 Fontansurvivors 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
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.
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
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
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
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
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
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
Pacemaker and implantable defibrillator
therapy
Biventricular pacing
Ventricular assist devices
Heart transplantation
THANK U