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