Heart Failure in Children

Cardiac physiology
TISSUE PERFUSION

BLOOD PRESSURE

CARDIAC OUTPUT x VASCULAR RESISTANCE

STROKE VOLUME x HEART RATE

1. PRELOAD: DIASTOLIC FILLING

2. AFTERLOAD : SYSTOLIC EMPTYING
3. MYOCARDIAL CONTRACTILITY (IANOTROPHY)
 Definitions
1. Stroke volume: The amount of blood ejected in one
cycle; around 70ml
2. Ejection fraction: amount of blood ejected per cycle
(60-80%)
3. Cardiac output (CO): the product of the stroke volume
(SV) and the heart (5L / Mt)
4. Preload: end diastolic volume and pressure
5. Afterload:
1. Left ventricular afterload: systemic vascular
resistance
2. Right ventricular afterload: pulmonary vascular
resistance
• Contractility: intrinsic ability of the cardiac muscle
fibres to contract and is independent of the degree
of preload and afterload and is often referred to as
the degree of inotropy
• Systolic dysfunction: reduced ventricular contraction
• Diastolic dysfunction: inadequate ventricular
relaxation
• Heart failure: It is syndrome in which cardiac output
is insufficient to meet the metabolic demands of the
body.
 Types of Heart Failure: LVF
1. Left-sided heart failure: Reduction in the left
ventricular output
2. Systolic failure (systolic dysfunction): LV loses its
ability to contract normally. Eg. MI
3. Diastolic failure (diastolic dysfunction): LV loses its
ability to relax normally Eg. Cardiomyopathy
4. Signs and symptoms: Pulmonary congestion -
pulmonary edema, dyspnoea, orthopnea, paroxysmal
nocturnal dyspnoea, cough, brongospasm, cardiac
asthma, fatigue, oliguria, cyanosis
 Right heart failure
1. Reduction in right ventricular output
2. Causes: Mitral stenosis, cor- pulmonale, TI, TS or PS
infective endocarditis
3. Signs and symptoms:
1. Systemic congestion - enlarged liver, ascites, jugular
venous distention, dependent pitting edema; pleural
effusion,
2. Fatigue, oliguria, nocturia, cyanosis
 Biventricular heart failure
1. BHF:
1. Causes: most common-LV failure leads to RV failure
2. MI and cardiomyopathy
3. Symptoms: Dyspnoea, dependent edema, jugular
venous distension, hepatomegaly, pulmonary vascular
congestion
2. Acute heart failure: Myocarditis; MI
3. Chronic heart failure: progressive valvular heart disease.
variety of compensatory changes may take place
 Compensatory Mechanisms During Heart Failure
Pathophysiology
1. Cardiac
1. Frank-Starling mechanism: stretching of the ventricular
wall increases cardiac muscle contraction
2. Ventricular hypertrophy
2. Autonomic Nerves
1. Increased adrenergic activity
2. Reduced vagal activity to heart
3. Hormones
1. Renin-angiotensin-aldosterone system
2. Vasopressin (antidiuretic hormone)
3. Circulating catecholamines
4. Natriuretic peptides
 Frank–Starling Mechanism
1. Incomplete chamber emptying
2. Blood that accumulates in the ventricle
during diastole
3. Increased stretch on the myofibers
4. A greater stroke volume on subsequent
contraction
5. Marked elevation of the end-diastolic volume
and pressure results in pulmonary congestion
and edema
 Ventricular Hypertrophy and Remodelling
1. Alterations in the size, shape, structure, and
function of the ventricle is known as remodeling
2. Synthesis of new sarcomeres make myocytes to
elongate and thicken
3. Initially acts to increase ventricular volume and
CO
4. Finally it leads to an increase in fibrosis and
myocardial apoptosis
 Sympathetic nervous system (SNS)

1. Stimulation of the sympathetic nervous

system (SNS) and release of catecholamines
(norepinephrine and epinephrine).
2. Increase HR and myocardial contractility and
vasoconstriction which increase cardiac
output
 Renin–angiotensin–aldosterone system (RAAS)

1. Kidneys secrete renin that acts on angiotensinogen in

the liver to make angiotensin I.
2. Circulating angiotensin i is converted by angiotensin-
converting enzyme (ace) in the lungs to angiotensin ii
3. Angiotensin ii produce vasoconstriction and promotes
the release of aldosterone. It promotes sodium
reabsorption, stimulate vasopressin release
4. Vasopressin causes vasoconstriction as well as
increased water retention
 OTHER NEUROHORMONES
1. Atrial natriuretic peptide (ANP) from atria released following
atrial or ventricular stretch
2. Brain natriuretic peptide (BNP) from ventricles released in
response to excessive stretching of heart muscle
3. c-type natriuretic peptide (CNP) secreted by the vascular
endothelium
4. Functions:
1. Counteract the actions of the renin-angiotensin system
2. Cause vasodilation, salt and water excretion; and inhibit
secretion of renin, aldosterone, and vasopressin.
3. Elevated bnp is used to follow the progression of disease.
 inflammatory mediators
1. Cytokine production also increases in HF and
include tumor necrosis factor α, interleukin
1α, interleukin 6, and interferon α.
2. These molecules are negative inotropes and
decrease contractility, and elevated levels are
associated with worse clinical outcomes.
 Protective mechnisms
1. Stimulation of insulin-like growth factor and
growth hormone
2. Secretion of atrial and brain natriuretic
peptides (ANP and BNP) by cardiac tissue
3. These factors increase vasodilatation and
diuresis
4. Prevent inflammation, cardiac fibrosis, and
hypertrophy
 Etiology of CCF
1. Volume overload: CHDs like VSD, PDA, ECD ,rheumatic
valvular HD, Anemia
2. Pressure overload: aortic stenosis; Coarctation of
aorta; Systemic hypertension
3. Systemic hypertension: Nephritis
4. Pulmonary hypertension: PPH, PPHN, Sec.PH
5. Tachyarrhythmia: Supraventricular tachycardia
6. Heart block
7. Myocarditis: viral, Kawasaki; Rheumatic
8. Cardiomyopathy: primary , secondary
9. Pulmonary disease
10. Collagen vascular disease
11. Endocardial fibroelastosis
12. Neuromuscular disease: Friedreich’s ataxia
13. Drug: anthracyclines
14. Post cardiac surgery
 Where CCF is uncommon:

1. Children with tetralogy of Fallot (TOF)

2. Atrial septal defect (ASD)
3. Large left-to-right shunt lesions, such as VSD
and PDA, do not cause CHF before 6 to 8 weeks
of age because the pulmonary vascular
resistance does not allow left-to-right shunt
until this age.
 Clinical Manifestations-Infants

1. Feeding difficulties due to dyspnoea: suck - rest cycle

2. Cold sweat on the face
3. Increased fatigability
4. Babies fail to thrive.
5. Tachypnea > 60 in neonate; > 40 in infants ; dyspnoea
with grunting, nasal flaring and intercostal retractions
6. Tachycardia > 160/mt; a gallop rhythm (s3, s4) and
neck veins may be difficult to recognise
7. Hepatomegaly
8. Peripheral edema is uncommon in infants and is
associated with only very severe heart failure.
9. Cool extremities, weakly palpable pulses
10. Mottling of the extremities
11. Slow capillary refill
 Presentation in Childhood
1. Closely resemble those in the adult
2. Exertional dyspnea; orthopnea
3. Chronic hacking cough, due to pulm.Congestion
4. Fatigue and weakness
5. Coolness, pallor, and cyanosis of the digits, with poor
capillary refill
6. Flank pain or tenderness owing to stretching of the
liver capsule
7. Peripheral edema, ascites, pericardial effusion, and,
occasionally, hydrothorax
 Signs
1. Cardiomegaly; hyperactive cardiac impulse
2. Third heart sound occurring in mid-diastole
3. Pulsus alternans, characterized by a regular rhythm
with alternating strong and weak pulsations, can be
felt occasionally; more easily appreciated while
measuring blood pressure.
4. Pulsus paradoxus (a fall in blood pressure on
inspiration and a rise on expiration), secondary to
marked swings in intrapulmonary pressure that affect
ventricular filling
 New York Heart Association (NYHA) Heart Failure
Symptom Classification System

1. Level I: No symptom limitation with ordinary

physical activity
2. II: Ordinary physical activity somewhat limited by
dyspnea (e.g., long-distance walking, climbing two
flights of stairs)
3. III: Exercise limited by dyspnea with moderate
workload (e.g., short-distance walking, climbing one
flight of stairs)
4. IV : Dyspnea at rest or with very little exertion
 American Heart Association Heart Failure Stages

• Stage A: Presence of heart failure risk factors but no

heart disease and no symptoms
• Stage B: Heart disease is present but there are no
symptoms (structural changes in heart before
symptoms occur)
• Stage C: Structural heart disease is present AND
symptoms have occurred
• Stage D: Presence of advanced heart disease with
continued heart failure symptoms requiring aggressive
medical therapy
 Ross classification of heart failure in infants
• Class I: No limitations or symptoms
• Class II: Mild tachypnea or diaphoresis during feeding
in infants. Dyspnea on exertion in older children. No
growth failure
• Class III: Marked tachypnea or diaphoresis during
feeds or exertion Prolonged feeding times Growth
failure
• Class IV: Symptoms at rest with tachypnea, retractions,
grunting or diaphoresis
 Lab
1. Proteinuria and high specific gravity of the
urine
2. Increase in the blood urea nitrogen and
creatinine levels, secondary to reduced renal
blood flow.
3. Hyponatremia, secondary to increased water
retention
4. Congestive hepatomegaly and cardiac
cirrhosis may lead to abnormalities in liver
enzymes
 CXR
1. cardiomegaly.
2. Excessive pulmonary blood flow; venous
congestion
3. Enlarged left atrium may cause collapse of the
left lower lobe.
4. Interstitial pulmonary edema in a butterfly
pattern around the hila.
5. Kerley's lines, sharp linear densities in the
interlobar septa.
1. The electrocardiogram is not useful in assessing heart
failure, but it may provide clues to the lesion causing
the heart failure.
2. ECG: points to heart defect
3. ECHO:
• Enlargement of ventricular chambers and impaired
LV systolic function & pericardial effusion
• It helps to determine the cause of CHF.
• Echo is also helpful in serial evaluation of the
efficacy of therapy.
 Biochemical Markers
1. Natriuretic peptides: Those with higher values on
admission do worse and those whose values decrease
after treatment fare better
2. serum troponin, creatinine phosphokinase level are
high in severe cases
3. CRP helps in identifying asymptomatic patients at risk
for CHF. It correlates with severity of CHF;
4. TNF and IL-6 predict development of heart failure in
asymptomatic patients
 Steps in management of CCF
1. Elimination of underlying cause:
1. Surgery for defects
2. Drugs for hypertension; hyperthyroidism
3. Pacemaker for arrhythmias
2. Treatment of contributing cause ( fever, anemia,
infection)
3. Control of CCF :
1. Semi upright position (infant chair)
2. O2
4. Restriction of exercise
 DIET
1. High calorie diet in frequent small feeding
2. Human breast milk is the ideal low sodium nutritional
source.
3. Formula or EBM can be enriched (24 cal/oz) by adding:
1. Medium-chain triglycerides (MCT)
2. Microlipid (safflower oil emulsion),
3. Polycose
4. Older children: Low salt diet(<.5 g/day) and fluid
restriction
 Diuretics
1. First mode of therapy ; reduces blood volume ,
pulmonary fluid overload and ventricular filling
pressure.
2. Furosemide: initial dose of 1-2 mg/kg iv/im; 1-4
mg/kg/24 hr/ 1 and 4 times a day + potassium chloride
3. Spironolactone: orally in 2 divided doses of 2
mg/kg/24 hr
4. Chlorothiazide: 10-40 mg/kg/24 hr in 2 divided doses.
Potassium supplementation is often required
 Afterload Reducers
1. ACEI:
1. Decrease peripheral vascular resistances;
2. Produce arterial dilatation by blocking the
production of angiotensin II,
3. Venodilation and consequent preload reduction
4. Interferes with aldosterone production and help
control salt and water retention.
2. ARBs: recently introduced in pediatric patients with
renal disease; data in children with heart failure are
limited.
 After load Reducers
1. Captopril : PO 0.1–2 mg/kg/day q8–12h

2. Enalapril : PO 0.08–0.5 mg/kg/dose q12–24h

3. Hydralazine: IV:0.1- 0.5 mg/kg/dose; PO:0.25–1.0

mg/kg/dose 8h

4. Nitroprusside : IV:0.5–8 μg/kg/min

5. Prazosin: PO:0.005–0.025 mg/kg/dose q6–8h

 Digitalis Glycosides
• Digoxin, was once the mainstay of heart failure
management in both children and adults.
• It is currently used less frequently, as a result of the
introduction of newer therapies and the recognition
of its potential toxicities.
• Many cardiologists will use digitalis as an adjunct to
ACEIs and diuretics in patients with symptomatic
heart failure, whereas others have moved away from
its use altogether.
 DIGOXIN

Digitalization Premature: 20 μg/kg PO;

(½ initially, followed IV dose is 75% of po dose
by ¼ q12h × 2)
Full-term neonate (up to 1 mo):
25 μg/kg

Infant or child: 30 μg/kg

Maintenance digoxin 5–10 μg/kg/day, divided q12h

 Digoxin
1. Half-life of 36 hr
2. Absorbed well by the gastrointestinal tract
3. Dosing must be adjusted according to the patient’s
renal function.
4. Should be discontinued if a new rhythm disturbance is
noted
5. Prolongation of the P-R interval; ST segment or t-wave
changes are common
6. Hypokalemia and hypercalcemia exacerbate digitalis
toxicity.
7. Bradycardia, vomiting, green vision
 α- and β-Adrenergic Agonists

1. β-adrenergic receptor agonist, but it has α-

adrenergic effects at higher doses.
2. selective renal vasodilation
3. 2-10 μg/kg/min→increased contractility
4. > 15 μg/kg/min →α-adrenergic effects and
vasoconstriction.
5. Dobutamine: 2-20 μg/kg/min
6. Epinephrine: mixed α- and β-adrenergic receptor
agonist reserved for patients with cardiogenic shock
and low arterial blood pressure
 Phosphodiesterase Inhibitors
1. Milrinone: useful in low cardiac output after
open heart surgery;
2. It has both positive inotropic effects and
peripheral vasodilatory effects
3. works by inhibition of phosphodiesterase,
which prevents the degradation of intracellular
cyclic adenosine monophosphate.
4. Intravenous infusion at 0.25-1 μg/kg/min
5. Hypotension secondary to vasodilation
 Beta - Blockers
1. Helps in counteracting adrenergic overstimulation
in CCF
2. Given only in compensated CCF with adequate fluid
balance and BP
3. Carvedilol ; 0.1 mg/kg/day divided bid
4. Propranolol used with some benefits in large Lt to
Rt shunts
5. Metoprolol PO, 0.2 mg/kg/day divided bid;
 OTHER APPROACHES TO HEART FAILURE
1. Functional capacity have been achieved in
selected adult patients with cardiomyopathy
using biventricular resynchronization pacing
2. Carnitine: a cofactor for transport of long
chain fatty acids is used for dilated
Cardiomyopathy
3. Surgery: cardiac transplant when other
measures fail.
 Diet
1. Infants with heart failure may fail to thrive. Increasing
the number of calories per ounce of infant formula (or
supplementing breast-feeding) may be beneficial.
2. Nasogastric feedings may be helpful in severely ill
infants
3. Human breast milk is the ideal low sodium nutritional
source.
4. Older children can be managed with “no added salt”
diets and abstinence from foods containing large
amounts of sodium.
6. High Calory Diet to improve nutritional status: Human milk
fortifier (Enfamil of Mead Johnson), 1 packet per 25 mL of breast
milk = 24 kcal/oz
7. Addition of any of the following to the milk formula to yield 26–
30 kcal/oz
Fat
1). Medium-chain triglycerides (MCT) oil
2). Microlipid (safflower oil emulsion),
Low-osmolality polymers
1.Polycose
2.Moducal
3.PediaSure
 For patients with pulmonary edema
1. Positive pressure ventilation
2. β-Adrenergic agonists such as dopamine,
epinephrine, and dobutamine are used in
combination with phosphodiesterase inhibitors
such as milrinone.
3. If the blood pressure is not low, afterload-
reducing agents (nitroprusside, angiotensin-
converting enzyme [ACE] inhibitors) may be
beneficial.
 Refractory CCF
1. The signs new weight gain and peripheral edema, gallop,
jugular venous distention
2. increased P2
3. Hemodynamic: left ventricular ejection fraction less than
20%, low cardiac output, arterial hypotension, sinusal
tachycardia, and kidney failure.
4. Biochemical: high values of norepinephrine and
natriuretic peptides, and hyponatremia.
5. Electrophysiological: presence of potentially serious
arrhythmias.
 Potentially Treatable Factors
1. Anemia, pulmonary embolism and infections
2. viral infections
3. Thyroid dysfunction
4. Atrial fibrillation
5. Obesity increases the risk of developing HF
6. Lack of salt restriction
7. Physical activity
 Pharmacological management
1. Nesiritide: acts as an arterial and venous vasodilator,
increases natriuresis and suppresses the activation of
the renin-angiotensin-aldosterone and adrenergic
systems.
2. Antidiuretic hormone arginine vasopressin (AVP)
antagonists are currently under study.
3. The phosphodiesterase inhibitors (amrinone,
milrinone): reduce pulmonary vascular resistance ;
also improve blood flow to the extremities