Congestive heart failure

in children

Prepared by:
Ezra F. (MD)
Pediatrics Resident
Mekelle University
02/19/2023 1
Outline
• Introduction
• Definition
• Causes of pediatric HF
• Pathophysiology
• Diagnosis
• Staging and Severity
• Management

02/19/2023 2
Introduction
• In the United States, Heart failure is estimated to affect
12,000 to 35,000 children below the age of 19 years in the
United States each year.
• In our setup, study in black lion hospital shows acute heart
failure accounts for 2.9% of emergency department
admissions, with RHD being leading cause and precipitating
factors like pneumonia, infective endocarditis, rheumatic
recurrence and anemia.

02/19/2023 3
Definition
•  Heart failure (HF) results from structural or functional
cardiac disorders that impair the ability of the ventricle(s) to
fill with and/or eject blood in which it is unable to meet the
metabolic needs of the body.

02/19/2023 4
Causes of pediatric HF
• Causes of pediatric HF based on pathophysiologic
categories:-
1. Ventricular dysfunction
2. Volume overload with preserved ventricular
contractility
3. Pressure overload with preserved ventricular
contractility

02/19/2023 5
1. Ventricular dysfunction
A. Structurally normal heart
Cardiomyopathy – DCM (50 - 60 %)
– HCM (25 - 40 %)
Myocarditis – Viruses
Myocardial ischemia/infarction – Uncommon in children
Arrhythmias - Complete heart block
-Supraventricular and ventricular arrhythmias
Drug/toxins – Anthracyclines (e.g Doxorubicin)

02/19/2023 6
1. Ventricular dysfunction
A. Structurally normal heart
Noncardiac causes
Sepsis
Chronic kidney disease
Respiratory disorders
HIV
SLE

02/19/2023 7
1. Ventricular dysfunction
B. Congenital heart disease
Ventricular dysfunction may develop in children born with
complex CHD who undergo surgical repair or palliation in
early childhood.

02/19/2023 8
2. Volume overload with preserved
ventricular contractility
• occurs due to a moderate or large communication (shunt)
between systemic and pulmonary circulations and may occur
with the following cardiac lesions:
PDA
VSD
ASD
Aortopulmonary window
Atrioventricular septal defect
Valvular insufficiency – AR, MR, PR
02/19/2023 9
3. Pressure overload with preserved
ventricular contractility
• Ventricular outflow obstruction leads to pressure overload

Aortic stenosis
Coarctation of the aorta
Pulmonary stenosis

02/19/2023 10
 Causes of heart failure based on age

02/19/2023 11
Pathophysiology
• Systemic oxygen transport= Cardiac output X systemic
oxygen content
• Cardiac output = Heart rate x Stroke volume.
The primary determinants of stroke volume
1.After load (pressure work),
2.Preload (volume work), and
3.Contractility (intrinsic myocardial function).

02/19/2023 12
02/19/2023 13
Compensatory mechanisms
1. The Frank-Starling mechanism,
2. Activation of neurohormonal systems,

02/19/2023 14
 1. The Frank-Starling mechanism

02/19/2023 15
2.Activation of neurohormonal systems,
I. Sympathetic Nervous System Activation
II. RAAS Activation

02/19/2023 16
I. Sympathetic Nervous System Activation

1. Vasoconstriction,
2. Positive chronotropism - increased heart rate,
3. Positive inotropism - increased contractile force of the
ventricle,
4. Positive lusitropism - enhanced ventricular relaxation

02/19/2023 17
I. Sympathetic Nervous System Activation

02/19/2023 18
II. RAAS Activation

• The RAAS is essential to the maintenance of fluid and

sodium balance and hemodynamic stability.
• Moreover, RAAS activation plays an important role in both
adaptive and maladaptive mechanisms in response to heart
failure

02/19/2023 19
02/19/2023 20
 Maladaptive response

02/19/2023 21
MALADAPTIVE RESPONSE

Angiotensin II
• fibrosis of the heart, kidneys, and other organs.
• lead to worsening neurohormonal activation by enhancing
the release of NE from sympathetic nerve endings,
• stimulating the zona glomerulosa of the adrenal cortex to
produce aldosterone.

02/19/2023 22
MALADAPTIVE RESPONSE

Aldosterone
• provoke hypertrophy and fibrosis within the vasculature and
the myocardium, contributing to reduced vascular
compliance and increased ventricular stiffness.
• provokes endothelial cell dysfunction,
• baroreceptor dysfunction, and
• inhibition of NE uptake,

02/19/2023 23
Cardiac Remodeling
• Cardiac Remodeling is defined as alteration in the structure
(dimensions, mass, shape) of the heart (called cardiac or
ventricular remodeling) in response to hemodynamic
load and/or cardiac injury in association with neurohormonal
activation.

02/19/2023 24
Cardiac Remodeling
• It develops in response to a series of complex events that
occur at the cellular and molecular levels.
• These changes include
1. Alterations in Myocyte Biology
2. Myocardial Changes
3. Alterations in Left Ventricular Chamber Geometry

02/19/2023 25
02/19/2023 26
Diagnosis
• The diagnosis of HF in children is based on a combination of
clinical, radiographic, echocardiographic, and laboratory
findings.

02/19/2023 27
History
• Symptoms of HF vary with the age of the patient
Newborns and Infants
Feeding history- Frequency, volume, length of time to finish
a feeding.
Tachypnea that worsens during feeding, poor weight gain,
and cold sweat on the forehead.
 Cyanosis

02/19/2023 28
History

Newborns and Infants

• The child's history regarding pregnancy, labor and delivery,
and neonatal course are important.

02/19/2023 29
History
Older children
May complain of shortness of breath, especially with
activities, easy fatigability, puffy eyelids, or swollen feet.
sleep history –orthopnea, PND
The past medical history should include documentation of
significant illnesses, previous hospitalization, previous
operations, immunization status, and symptoms of poor
growth as an infant.

02/19/2023 30
Physical Examination

• Physical findings of CHF may be classified as follows,

depending on their pathophysiologic mechanisms.
• The following are found as compensatory responses to:
1. Impaired cardiac function
2. Pulmonary venous congestion
3. Systemic venous congestion

02/19/2023 31
Physical Examination
Impaired cardiac function:
Tachycardia, gallop rhythm, and weak and thready pulses
are common.
Cardiomegaly is almost always present
There are signs of increased sympathetic discharges (e.g.,
growth failure, sweat, cold and wet skin).

02/19/2023 32
Physical Examination
Pulmonary venous congestion (from left-sided failure) results
in the following manifestations:
Tachypnea is common and is an early manifestation of CHF
in infants.
Dyspnea on exertion (equivalent to poor feeding in small
infants) is common in children.
Orthopnea may be seen in older children.
Wheezing and pulmonary crackles are occasionally audible
02/19/2023 33
Physical Examination
Systemic venous congestion (related to right-sided failure)
results in the following:
Hepatomegaly
Puffy eyelids are common in infants.
Distended neck veins and ankle edema, which are common
in adults, are not seen in infants.

02/19/2023 34
Laboratory tests
• CBC
• Serum chemistry (electrolytes, LFT, RFT)
• B-type natriuretic peptide
• Troponin
• Investigations based on clinical evidence( TFT, Blood
culture, ESR…)

02/19/2023 35
 Chest X-ray

02/19/2023 36
Investigations

Electrocardiography
• ECGs help determine the type of heart defect causing heart
failure but are not helpful in deciding whether CHF is
present.
• It is important in diagnosing Ventricular hypertrophy and
arrhythmias

02/19/2023 37
Investigations

Echocardiography
• Echo studies may confirm enlargement of ventricular
chambers and impaired LV systolic function (decreased
fractional shortening or ejection fraction) as well as impaired
diastolic function by the use of Doppler techniques.
• A more important role of echo may be due to its ability to
determine the cause of CHF.
• Echo is also helpful in serial evaluation of the efficacy of
therapy.
02/19/2023 38
Staging and Severity
• Categorization of the stage and severity of the patient's HF is
important for monitoring the disease progression and guiding
management decisions .

02/19/2023 39
Staging 
• The staging system of pediatric HF (stages A to D) is used to
describe the development and progression of disease
following exposure to a risk factor for HF .

02/19/2023 40
02/19/2023 41
Severity 
• The two main classification systems used for describing
severity of pediatric HF are the New York Heart Association
(NYHA) and Modified Ross classifications. The NYHA
classification relies on patient reporting of symptom severity
and thus has limitations in children.

02/19/2023 42
Severity 
NYHA class — The New York Heart Association (NYHA)
Classes (I to IV) are most commonly used to quantify the
degree of functional limitation imposed by HF in adults and
may be used in adolescents
Ross classification — The Ross HF Classification is an
adaptation of the NYHA system that is used to describe HF
severity in infants and children based on a history of feeding
intolerance, growth problems, exercise intolerance, and
physical findings
02/19/2023 43
02/19/2023 44
02/19/2023 45
Management
• The management of pediatric HF is dependent on its etiology
and severity.
• Management begins with a thorough assessment of the
underlying cause of HF. 
• The approach to the management of cardiac failure in
children developed from combination of clinical experience,
small scale studies in pediatric populations and the
application of adult trial data to the pediatric population.

02/19/2023 46
COMPONENTS OF THERAPY
1. General supportive care
2. Identification and correction of precipitating factors
3. Pharmacologic therapy to relieve symptoms, slow the
progression of ventricular dysfunction,
4. Additional therapeutic interventions for patients with
advanced heart failure refractory to pharmacologic therapy
5. Therapeutic interventions to reduce the risk of and/or treat
associated complications
02/19/2023 47
General Measures
• A “cardiac chair” or “infant
seat” - used to keep infants in a
semi-upright position
• Bed rest
• Restrictions on activities
within the context of the specific
diagnosis & the patient's ability.
• Daily weight measurement is
essential in hospitalized patients.
02/19/2023 48
Diet
• Fail to thrive - combination of increased metabolic demands
and decreased caloric intake.
• Increasing caloric density of feeding may be required, and it
may be accomplished with fortification of feeding.
Eg- Formula concentration to 24Kcal/Oz (0.8 kcal/ml ) can be
achieved by
1 cup powdered formula + 3 cups water

02/19/2023 49
Diet
• The required caloric intake may be as high as 150 to 160
kcal/kg/day for infants in CHF.

• NG tube feedings - Severely ill infants and children may lack

sufficient strength for effective sucking because of extreme
fatigue, rapid respirations, and generalized weakness.

02/19/2023 50
Diet
• The use of low-sodium formulas in the routine management
of infants with heart failure is not recommended because
these preparations are often poorly tolerated and may
exacerbate diuretic-induced hyponatremia.
• Human breast milk is the ideal low-sodium nutritional
source.
• Most older children can be managed with “no added salt”
diets. (<0.5 g/day)

02/19/2023 51
Pharmacologic therapy
To achieve improvement in symptoms
Diuretics
Digoxin
 ACE inhibitors
To achieve improvement in survival
 ACE inhibitors
 ARBs
 B blockers
 Spironolactone
02/19/2023 52
Diuretics
• Decrease preload by promoting natriuresis and provide relief of
volume overload.
• Have two main effects
• increased water loss 
• increased sodium loss 
• Loop diuretics increase the fractional excretion of sodium by 20–
25%, thiazide diuretics increase it by only 5–10% and tend to
lose their effectiveness in patients with moderate or severe renal
insufficiency.
02/19/2023 53
02/19/2023 54
 Diuretics
• Diuretics are used to treat children with stage C or D heart failure
• Diuretic therapy may augment neurohormonal activation due to
volume depletion, with potentially deleterious effects on the
progression of heart failure.
• For this reason, it is preferable to avoid the use of diuretics in the
subset of patients with asymptomatic LV dysfunction and to use
the minimal dose necessary to maintain euvolemia in patients with
symptoms related to volume retention.

02/19/2023 55
Loop diuretics

Act by reversibly inhibiting the Na+-K+-2Cl− symporter

(cotransporter) on the apical membrane of epithelial cells in
the thick ascending loop of Henle
• Includes
Furosemide,
Bumetanide,
Torsemide,

02/19/2023 56
Loop diuretics

• Furosemide dose 1-4 mg/kg/24 hr given between 1 and 4

times a day.
• Furosemide acts as a venodilator and reduces right atrial and
pulmonary capillary wedge pressure within minutes when
given intravenously.
• The bioavailability of furosemide ranges from 40% to 70% of
the oral dose. By contrast,the oral bioavailability of
bumetanide and torsemide exceeds 80%.

02/19/2023 57
Loop diuretics

• Careful monitoring of electrolytes is necessary with long-

term furosemide therapy because of the potential for
significant loss of potassium.
• Potassium chloride supplementation is usually required
unless the potassium-sparing diuretic spironolactone is given
concomitantly.

02/19/2023 58
Loop diuretics

Side Effects
• Hyponatremia
• Hypokalemia
• Contraction alkalosis
• Ototoxicity
• Allergic reaction

02/19/2023 59
Thiazide diuretics

• Block the Na+-Cl− transporter in the distal convoluted

tubule.
• Commonly used thiazide diuretics are
 Chlorothiazide ,
 Hydrochlorothiazide ,
 Metolazone
• Side effects – Hyponatremia, hypomagnesemia, hypokalemia
and hypercalcemia

02/19/2023 60
Aldosterone antagonists
• Rationale for use of aldosterone antagonists:
• Reduced sodium retention
• Reduced water retention
• Reduced myocardial apoptosis and fibrosis
• Reduced inhibition of nitric oxide release
• Slowed heart rate, improving balance of oxygen supply and
demand
• Anti‐arrhythmic effects
02/19/2023 61
Aldosterone antagonist
Spironolactone
• Inhibits aldosterone & enhances K+ retention
• Dose is 2mg/kg/24hrs in 2 divided doses
• Improved survival demonstrated in adult patients

02/19/2023 62
 Kaplan-Meier analysis of survival among 1663 patients
with advanced heart failure in the RALES trial shows that
spironolactone reduces mortality by 30 percent (35 versus
46 percent for placebo, p<0.001)
02/19/2023 63
Spironolactone

Side effects
• Hyperkalemia
• Endocrine side effects of spironolactone (gynecomastia,
breast pain, menstrual irregularities, impotence, and
decreased libido).

02/19/2023 64
Diuretic Resistance
• A patient with HF may be considered to be resistant to
diuretic drugs after being treated with maximal doses of a
loop diuretic.

02/19/2023 65
Mechanism of diuretic resistance
• Noncompliance with medication and salt free diet
• Simultaneous use of NSAIDs,
• Compensatory retention of sodium after the effective period
of the diuretic.
• Diminished renal natriuretic effect owing to compensatory
hypertrophy and hyperplasia of epithelial cells of the distal
convoluted tubule leading to increased reabsorption of
sodium

02/19/2023 66
Management of diuretic resistance
(A)Rule out non-compliance in medication and salt restriction
(B)Discontinue NSAIDs
(C)Combination diuretic therapy - adding a thiazide diuretic.
(D)Dose adjustment (loop diuretic)
(E)Intravenous bolus injection or continuos infusion
- more frequent administration of loop diuretics

02/19/2023 67
Angiotensin Converting Enzyme Inhibitors
• ACE inhibitors reduce the effects that result from the
activation of the renin‐angiotensin‐aldosterone (RAA)
system and SNS.

02/19/2023 68
Angiotensin Converting Enzyme Inhibitors
Mechanism of action
• Inhibit the formation of angiotensin II
• Reduce angiotensin II mediated sympathetic nervous system
activity
• Increase concentrations of the vasodilator bradykinin by
inhibiting its degradation
• Improvement in endothelial function

02/19/2023 69
Angiotensin Converting Enzyme Inhibitors

• Reduced Angiotensin II mediated aldosterone release

• Effects on arterial compliance, and prevention of adverse
cardiac and vascular remodeling
• Includes
 Captopril (0.3-6 mg/kg/24 hr given in 3 divided doses )
 Enalapril (0.05-0.5 mg/kg/24 hr given in 1 or 2 daily doses )

02/19/2023 70
Angiotensin Converting Enzyme Inhibitors
Side effects
• Cough
• Angioedema - Bradykinin
• Hypotension - Weakness, dizziness, or syncope may result
from an excessive reduction in blood pressure
• Hyperkalemia
• Maculopapular pruritic rash

02/19/2023 71
Angiotensin Receptor Blockers (ARBs)

• ARBs reduce some of the effects that result from the

activation of the RAAS.
• Inhibits the actions of angiotensin II

02/19/2023 72
Angiotensin Receptor Blockers (ARBs)
• In children with HF, there is a paucity of data on the use of
ARBs, which block the angiotensin receptor.
• Thus, ACE inhibitors are the preferred class of drugs for
inhibition of the RAAS.
• ARBs are usually reserved for patients unable to tolerate
ACE inhibitors due to cough or angioedema.

02/19/2023 73
Beta‐blockers
• Beta‐blockers counter the activation of the sympathetic
nervous system which is often seen in heart failure.
• Rationale for use of beta‐blockers:
 Slowed heart rate, improving balance of oxygen supply and
demand
 Reduced myocardial apoptosis and fibrosis
 Anti‐arrhythmic effects
 Synergism with ACE inhibitors

02/19/2023 74
Beta‐blockers
• Beta blockers should not be initiated in acute decompensated
heart failure.
• The initiation and increased dosing of these agents may lead
to worsening fluid retention because of the abrupt withdrawal
of adrenergic support to the heart and circulation.
• Therefore, it is important to optimize the dose of diuretic
before starting therapy with beta blockers.

02/19/2023 75
 Beta‐blockers
Carvedilol
• In children with heart failure and related conditions, carvedilol has been the
most widely studied beta‐blocker.
• Carvedilol is a non selective beta blocker which also has an anti-oxidant
property.
• Due to its alpha blocking effect, carvedilol exerts a vasodilatory effect.
• Dose - 0.1 mg/kg/day in two divided doses, increase at 1-2 weekly interval
to 1 mg/kg/day with a maximum of 2 mg/kg/day.
• Side effects - dizziness, fatigue, hypotension, bradycardia, bronchospasm
02/19/2023 76
Contraindications
• Bronchial asthma
• Decompensated heart failure (class IV)
• Severe liver impairment
• Second or third degree AV block
• Cardiogenic shock
• Severe bradycardia
• Known hypersensitivity to the drug

02/19/2023 77
Digitalis Glycosides - Digoxin
Mechanism of action
1. Inhibits the Na+,K+-ATPase pump in cell membranes, including the
sarcolemmal Na+,K+-ATPase pump of cardiac myocytes leading to
an increase in intracellular calcium and hence increased cardiac
contractility
2. Sensitize Na+,K+-ATPase activity in vagal afferent nerves, leading
to an increase in vagal tone that counterbalances the increased
activation of the adrenergic system in advanced HF.
3. Also inhibits Na+,K+-ATPase activity in the kidney and may,
therefore, blunt renal tubular resorption of sodium
02/19/2023 79
How to Digitalize?
• Obtain a baseline ECG (rhythm and PR interval) , serum
electrolytes ( K+ and Mg++) & RFT ( BUN and Creatinine)
• Close monitoring of ECG and Serum electrolytes is necessary
before and after each doses.

02/19/2023 80
Digoxin
Expert recommendations on indications of digoxin in the
management of heart failure in children:
Primary myocardial disease with left and/or right ventricular
dysfunction (in symptomatic patients along with diuretics)
Symptomatic patients with severe valvular regurgitation
Atrial fibrillation
In acute phase of acute myocarditis at lower dose

02/19/2023 81
Digoxin Toxicity

Contraindication
• Hypertrophic obstructive cardiomyopathy
• High grade AV block

02/19/2023 82
Digoxin Toxicity
Caution
• Renal failure
• Hypokalemia
• Acute myocarditis
• Premature infants with impaired renal clearance
• Co administration with drugs inhibiting AV conduction

02/19/2023 83
Digoxin Toxicity
• Therapeutic trough blood levels are usually 2-4 ng/mL in
infants and 1-2 ng/mL in older children.
• Clinical manifestation - anorexia, nausea, vomiting,
diarrhea, restlessness, drowsiness, fatigue, and visual
disturbances in older children and Heart failure worsens.
• ECG changes- Prolongation of PR interval (sometimes
which may progress to second-degree AV block ) , Profound
sinus bradycardia or sinoatrial block

  
02/19/2023 84
Advanced Heart Failure
Stage D -Patients with end-stage HF requiring specialized
interventions
Pharmacologic – Ionotropic agents and Intravenous diuretics
Inotropes — Inotropic agents are used in the setting of low cardiac
output (eg, during acute exacerbations of HF to improve cardiac
output and to stabilize patients awaiting heart transplantation).
1. Catecholamines – Dopamine, Dobutamine, Isoproterenol,
Epinephrine
2. Phosphodiesterase Inhibitors - Milrinone
02/19/2023 85
Advanced Heart Failure
Nonpharmacologic interventions
• Noninvasive ventilation
• Mechanical circulatory support in patients with end-stage HF
• Heart transplantation

02/19/2023 86
02/19/2023 89
02/19/2023 90
References
1. Nelson, Textbook of pediatrics, 21st Edition
2. Uptodate, 2018
3. Harrison’s principles of internal medicine 19th Edition
4. Medical wikipedia
5. Christopher S. Lee, Nancy C. Tkacs. Concepts of neurohormonal
activation. 2008; 19(4) : 364–385
6. Braunwald’s Heart disease ,11th edition
7. Moss and Adams’ Heart disease in Infants, Children, and
adolescents including fetus and young Adult , 9 th edition
02/19/2023 91
References cont…
8. Anita Saxena, Drug Therapy of Cardiac Diseases in Children .2009 ;
46(1): 310 -338
9. Shreepal Jain, Balu Vaidyanathan. Digoxin in management of heart
failure in children. 2009 ; 2(2) :149 – 152
10. G. Michael Felker, Kerry L. Lee, David A. Bull. Diuretic Strategies in
Patients with Acute Decompensated Heart Failure. N Engl J Med
2011;364: 797-805.
11. Goodman & Gilman’ s, The Pharmacological Basis Of Therapeutics
11th Ed
12. Myung K. Park , Pediatric Cardiology , 5 th edition
02/19/2023 92
THANK YOU
02/19/2023 93
02/19/2023 94