UMANAND PRASAD SCHOOL OF

MEDICINE AND HEALTH SCIENCES

PEDIATRIC

MANAGEMENT OF HEART FAILURE

2023
 INTRODUCTION
• A clinical and pathological syndrome that results from
ventricular dysfunction, volume, or pressure overload, alone or
in combination.

• It leads to characteristic signs and symptoms, such as poor

growth, feeding difficulties, respiratory distress, exercise
intolerance, fatigue, and is associated with circulatory, neuro-
hormonal, and molecular abnormalities. Heart failure has
numerous etiologies that are a consequence of cardiac and non
cardiac disorders, either congenital or acquired.
 PATHOPHYSIOLOGY
 Volume overload
 Pressure overload
 Poor cardiac muscle contractility
 High output state
• The heart can be viewed as a pump with an output
proportional to its filling volume and inversely proportional to
the resistance against which it pumps. As ventricular end-
diastolic volume increases, a healthy heart increases cardiac
output until a maximum is reached and cardiac output can no
longer be augmented (the Frank-Starling principle).

• The increased stroke volume obtained in this manner is a

result of stretching of myocardial fibers, but it also results in
increased wall tension, which elevates myocardial oxygen
consumption. Heart working under various types of stress
function along different Frank-Starling curves.

• If a cardiac chamber is already dilated because of a lesion

causing increased preload (e.g. a left-to-right shunt or valvular
insufficiency), there is little room for further dilation as a
means of augmenting cardiac output.
• Cardiac output can be calculated as the product of heart rate
and stroke volume. The primary determinants of stroke volume
are the afterload (pressure work), preload (volume work), and
contractility (intrinsic myocardial function).

• Abnormalities in heart rate can also compromise cardiac output;

for example, tachy-arrhythmias shorten the diastolic time
interval for ventricular filling.

• In some cases of heart failure, cardiac output is normal or

increased, yet because of decreased systemic oxygen content
(e.g., secondary to anemia) or increased oxygen demands (e.g.,
secondary to hyperventilation, hyperthyroidism, or hyper-
metabolism), an inadequate amount of oxygen is delivered to
meet the body's needs. This condition, high-output failure ,
results in the development of signs and symptoms of heart
failure when there is no basic abnormality in myocardial
function and cardiac output is greater than normal.
• Heart failure results when the demand for cardiac output
exceeds the ability of the heart to respond.

• There are multiple systemic compensatory mechanisms used

by the body to adapt to chronic heart failure. Some are
mediated at the molecular/cellular level, such as up-regulation
or down-regulation of various metabolic pathway components
leading to changes in efficiency of oxygen and other substrate
utilizations.

• One of the principal mechanisms for increasing cardiac output

is an increase in sympathetic tone secondary to increased
secretion of circulating epinephrine by the adrenals and
increased release of norepinephrine at the neuromuscular
junction.
• The initial beneficial effects of sympathetic stimulation
include an increase in heart rate and myocardial contractility,
mediated by these hormones’ action on cardiac β-adrenergic
receptors, increasing cardiac output. These hormones also
cause vasoconstriction, mediated by their action on
peripheral arterial α-adrenergic receptors.

• Peripheral vasoconstriction can result in decreased renal,

hepatic, and gastrointestinal tract function. Chronic exposure
to circulating catecholamine leads to a decrease in the
number of cardiac β-adrenergic receptors (down-regulation)
and also causes direct myocardial cell damage.
 ETIOLOGY
• Fetal: • Premature Neonate:
• Severe anemia • Fluid overload.
• Supraventricular • PDA.
• VSD.
tachycardia
• Cor pulmonale.
• Ventricular tachycardia
• Hypertension.
• Complete heart block. • Myocarditis.
• Severe Ebstein • Genetic/ metabolic
anomaly or other cardiomyopathy.
severe right-sided
lesions.
• Myocarditis
• Full-Term Neonate: • Infant-Toddler:
• Asphyxial • Left-to-right cardiac
cardiomyopathy. shunts.
• Arteriovenous • Hemangioma.
malformation. • Anomalous left
• Left-sided obstructive coronary artery.
• Genetic/ metabolic
lesions.
cardiomyopathy.
• Large mixing cardiac • Acute hypertension.
defects. • Supraventricular
• Myocarditis. tachycardia.
• Genetic/ metabolic • Kawasaki disease.
cardiomyopathy. • Myocarditis
• Child-Adolescent:
• Congenital heart disease.
• Rheumatic fever.
• Acute hypertension.
• Myocarditis.
• Thyrotoxicosis.
• Hemochromatosis-hemosiderosis.
• Cancer therapy.
• Sickle cell anemia.
• Endocarditis.
• Cor pulmonale.
• Genetic/ metabolic cardiomyopathy.
 CLINICAL PRESENTATION
Infants: Young Children: Older Children:
• Tachypnea • Gastrointesti • Exercise
nal intolerance
• Diaphoresis
symptoms • Anorexia
during feeds • Poor weight • Abdominal
• Irritability gain pain
• Decreased • Easy • Wheezing
volume of feeds fatigability • Dyspnea
• Poor weight • Recurrent or • Edema
gain. chronic • Palpitations
cough with • Chest pain
wheezing. • Syncope
 PHYSICAL EXAMINATION
• Tachycardia – Tachycardia is a response to decreased cardiac output in patients with depressed
myocardial contractility.
• Poor perfusion – Poor perfusion as a result of diminished cardiac output is manifested by cool
and mottled extremities, decreased capillary refill, decreased peripheral pulses, and lowered
systemic blood pressure.
• Gallop rhythm – A third heart sound (S3) gallop may be present in children with diminished
cardiac output or volume overload.
• Pulmonary findings – Pulmonary congestion is manifested primarily with changes in respiratory
status:
• Tachypnea is the most common finding of pulmonary congestion. Normal respiratory rate varies
with age.
Other signs of respiratory distress seen in patients with HF include retractions, use of accessory
respiratory muscles, and grunting with nasal flaring (in infants).
• Systemic congestion – Systemic congestion may be manifested by the following findings:
• Hepatomegaly (the most common finding)
• Peripheral edema
• Ascites and splenomegaly (may be present in severe right HF)
• Jugular venous distension (not generally observed in infants and young children)
• Other findings – Other findings may suggest an underlying
etiology for HF, as demonstrated by the following examples:
• High blood pressure limited to upper extremities and/or weak
pulses in lower extremities are suggestive of aortic coarctation
• The presence of a systolic murmur may be seen in patients with
outflow obstruction in hypertrophic cardiomyopathy or aortic
stenosis, congenital heart defects with left-to-right shunting (eg,
ventricular septal defects), or mitral regurgitation
• Precordial examination may reveal a "thrill" in patients with
shunt lesions, whereas those with a longstanding
cardiomyopathy may have a "heave" with a laterally displaced
point of maximal impulse.

Auscultatory findings, including wheezing and rales, are more

commonly seen in older children as compared with infants.
INVESTIGATIONS:
 Other Investigations:
• BNP and NT-proBNP
Appear to be effective markers of structural and functional heart disease in children
and are useful in the integrated evaluation of children with HF. In patients with left-to-
right shunting defects (eg, atrial or ventricular septal defects, patent ductus arteriosus),
BNP levels correlate with the degree of shunting and in children with ventricular
dysfunction, BNP levels correlate negatively with ejection fraction

• Troponin 
Cardiac troponin I and troponin T are sensitive biomarkers for myocyte injury. Troponin
levels are elevated in myocarditis and myocardial ischemia. Among children presenting
with LV dysfunction an elevated troponin level may suggest acute myocarditis rather
than dilated cardiomyopathy

• Echocardiography
Echocardiography is the primary imaging modality to assess ventricular size and
function in children with signs and symptoms of HF. It also establishes whether the
child has a structurally normal heart or underlying structural congenital heart disease
(CHD).
Chest X-ray
 Cardiomegaly may be seen in a number of cardiac diseases,
including:

 Left-to-right shunting defects – In this setting, cardiomegaly

reflects the presence of a moderate to large shunt with volume
overload and subsequent trial and ventricular dilatation
 Dilated cardiomyopathy – Cardiomegaly reflects the dilatation
of left ventricle (LV)
 Myocarditis – Ventricular dilation may not be severe in this
setting, or ventricular size may be normal
 Arrhythmogenic right ventricular (RV) cardiomyopathy – RV
dilation may sometimes be seen
 Restrictive cardiomyopathy – Bi-atrial enlargement may be seen
 Pericardial effusion
 
 ECG

1. ST segment and T wave abnormalities are common in all forms of

cardiomyopathy and myocarditis
2. Increased QRS voltage that meets criteria for ventricular hypertrophy may be
seen in hypertrophic or dilated cardiomyopathy
3. Decreased QRS voltage may suggest myocardial edema or pericardial
effusion and may be present in children with myocarditis
4. Bi-atrial enlargement may be present in restrictive cardiomyopathy.
5. A deep q wave in inferior and lateral leads (I, aVL, and V5-V6) with ST
segment and T wave changes is suggestive of a myocardial infarct and is a
classic finding in infants with anomalous left coronary arising from the
pulmonary artery (ALCAPA)
6. Varying degrees of heart block may sometimes be observed in patients with
rheumatic or Lyme carditis or in patients with neonatal lupus.
7. Atrial, junctional, or ventricular tachycardia or frequent atrial or ventricular
ectopy may suggest arrhythmia as an underlying cause of ventricular
dysfunction or may represent a complication.
• Sinus tachycardia is the most common ECG finding and is
nonspecific. It represents a physiologic compensation for
reduced stroke volume.

In some cases, the ECG may point toward an underlying cause.

Examples include:
 ventricular hypertrophy may be seen in hypertrophic or dilated
cardiomyopathy
 may suggest myocardial edema or pericardial effusion and may
be present in children with myocarditis
 present in restrictive cardiomyopathy.
 suggestive of a myocardial infarct and is a classic finding in
infants with anomalous left coronary arising from the
pulmonary artery (ALCAPA)
 MANAGEMENT
A. ACUTE B. MANAGEMENT GOALS:
1. Ensure airway patency. 1. Improve oxygen delivery
2. Intubate and provide 2. Reduce preload
oxygen as deemed 3. Reduce afterload
necessary. 4. Enhance cardiac
3. Ensure adequate contractility
perfusion (refer to Shock 5. Improve nutrition
Protocol if in shock).
4. Consider sepsis and
initiate management
early
5. Correct calcium
 C. MEDICAL MANAGEMENT
Medical management is dependent on the cause and degree of failure. Some patients may only require mono-therapy, while
others may need multiple drugs to achieve management goals.
 D. NUTRITION
• Carbohydrates 6 g/kg/day Lipids 2.5 g/kg/day Protein
1.2 to 1.5 g/kg/day
• Low salt if child is edematous
• Fluid restriction if edematous or hyponatremic.
• Consider the least stressful route of feeding – tube
feeding to reduce stress on the infant/child in heart
failure. Small and frequent meals are better
tolerated.
 E. SURGICAL MANAGEMENT
• Drainage of pericardial effusion
• Rashkind Balloon Septostomy
• Once cardiac failure is controlled, discuss with local
cardiac team for urgent overseas surgical referral or
await overseas visiting teams.
MANAGEMENT AND PREVENTION OF
HF COMPLICATIONS
Thromboembolism:
Children with HF due to systemic ventricular dysfunction are at risk for the formation
of intra-cardiac thrombi, which may result in pulmonary embolus, cerebral embolic
strokes, and, in some cases, death. Aspirin can also be considered in children with
restrictive cardiomyopathy if there is marked atrial dilation. Children who develop
intra-cardiac thrombi, or other clinically significant thromboembolic events, are
managed with anticoagulation therapy (initially with unfractionated heparin/ LMWH,
and subsequently with either LMWH or Warfarin).

Arrhythmias:
 Cardioversion or defibrillation if necessary (eg, if the patient is acutely unstable)
 Antiarrhythmic therapy
 Ablation therapy, particularly in the setting of chronic atrial tachyarrhythmias
 LONG-TERM HEALTH MAINTENANCE
• Immunizations – Children with HF should receive all routine childhood
vaccinations, including pneumococcal and yearly influenza vaccine. In addition,
respiratory syncytial virus (RSV) immunoprophylaxis should be provided to eligible
infants

• Monitoring of growth parameters – It is important to monitor growth and

development in children with HF, as it is in all children. Failure to thrive may be the
main clinical sign of HF in young infants and children.

• Monitoring for cardiac symptoms – Between visits with the cardiac specialist, the
primary care provider should monitor for symptoms related to HF. If the patient
develops new or worsening HF symptoms (eg, poor weight gain, tachypnea,
dyspnea, syncope), the patient should be promptly referred to the specialist for
cardiac evaluation.

• Treatment of respiratory illnesses – Respiratory illnesses can be associated with

considerable morbidity and mortality in children with HF. It is important to
promptly recognize acute respiratory illnesses and to provide appropriate
treatment
• Exercise and sports participation – Promoting healthy and safe physical activity in
patients with HF is an important part of management. The challenge is to balance
encouraging routine daily physical activity and limiting inactivity while minimizing
any potential risks from exercise. Recommendations should be tailored for each
individual based on his or her specific diagnosis and a comprehensive assessment
of the child's exercise capacity

• Antibiotic prophylaxis – Antibiotic prophylaxis for the prevention of bacterial

endocarditis should be provided to cyanotic patients and those with indwelling
central lines. 

• Planning of non-cardiac surgery – Children with HF are at increased risk for

adverse events when undergoing surgery and other procedures under anesthesia.
Careful perioperative planning (including consultation with cardiac anesthesia,
coordination with the cardiologist, and appropriate postprocedural monitoring) are
important for pediatric patients with HF undergoing surgery or other procedures
requiring anesthesia/sedation.

• Airplane travel – Airplane travel should be avoided in children with HF who are in
an unstable or decompensated condition. Supplemental oxygen may be warranted
in select patients during airplane travel.
 DIFFERENTIAL DIAGNOSIS
• Respiratory distress – Non-cardiac causes of respiratory distress in
neonates include transient tachypnea of the newborn, respiratory distress
syndrome, meconium aspiration, congenital diaphragmatic hernia,
pneumothorax, pneumonia, and pulmonary hypoplasia. In older infants and
children, common causes include pneumonia, bronchiolitis, and asthma.

• Poor weight gain – Other causes of poor weight gain include

gastrointestinal disorders (eg, protein-milk allergy, cystic fibrosis, celiac
disease), chronic infections, hyperthyroidism, and metabolic disorders.

• Edema – Peripheral edema may be caused by renal failure, venous

thrombosis, or adverse drug effects.

• Shock – Shock may be due to overwhelming sepsis or hypovolemia. 

 REFERENCES
• Pediatric Intensive Care Unit Guidelines; pages 75-79
• Nelsons textbook
• WHO blue book
• UpToDate