Materi - Gagal Jantung Neonatus

Heart Failure in Neonates
QUICK REVIEW
Etiology of Heart Failure in
Neonates
Etiology of HF in Neonates
• Arrhythmia: SVT,
Severe bradycardia
(TAVB) • Ductal-dependent systemic
circulation CHD (severe
• Anemia At birth AS/aortic coarctation, >2nd week of • Valvular regurgitation
• Severe TR (Ebstein’s HLHS, TAPVC) life • Infective endocarditis
anomaly), MR (AV • Premature with PDA
canal defect) • Fetal • Myocarditis
• Adrenal insufficiency, • L to R shunt CHD
• Myocarditis cardiomyopathies Neonatal thyrotoxicosis (VSD: 6-8 weeks, • Anemia
• Extracardial PDA, aortopulmonary • Cardiomyopathies
windows)
conditions
1st week after • LCAPA
Fetus (asphyxia, sepsis, Beyond infancy
hypoglycaemia, birth
hypocalcaemia)
• Volume of L to R increase as PVR falls
• Closure of duct è • Medical management important in
• Recognized from fetal VSDè may close on follow up
echocardiography
severe reduction of
• LCAPAè curable and often missed
• Severe CHFè hydrops end-organ perfusion
fetalis with ascites,
pleural and pericardial
effusions and anasarca
• Masarone D, Valente F, Rubino M, Vastarella R, Gravino R, Rea A, Russo MG, Pacileo G, Limongelli G. Pediatric Heart Failure:
A Practical Guide to Diagnosis and Management. Pediatr Neonatol. 2017 Aug;58(4):303-312.
• Sharma CM, Nair MNG, Jatana SK, Shahi BN. Congestive heart failure in infants and children. MJAFI. 2003;59:228-33.
Inherited Cardiomyopathy
u Five General Phenotype Classifications:
Left ventricular
Dilated Hypertrophic
noncompaction
cardiomyopathy cardiomyopathy
cardiomyopathy
(DCM) (HCM)
(LVNC)
Arrhythmogenic
Restrictive
ventricular
cardiomyopathy
cardiomyopathy
(rare in neonates)
(rare in neonates)
• Ryan TD, Kindel SJ, O’Connor MJ. Clinical diagnosis and management of pediatric heart failure: Heart failure in the neonate. Philadelphia: Elsevier;2018. p.383-
Inflammatory and Infectious
Cardiomyopathies
Rheumatic
Myocarditis Heart
Disease
Kawasaki
Endocarditis
Disease
Pathophysiology of Heart Failure
Index event (congenital or acquired)
Norepinephrine Angiotensin II
Cathecolamine Aldosterone
• Masarone D, Valente F, Rubino M, Vastarella R, Gravino R, Rea A, Russo MG, Pacileo G, Limongelli G. Pediatric Heart Failure: A Practical Guide to Diagnosis and
Management. Pediatr Neonatol. 2017 Aug;58(4):303-312.
Diagnosis of Heart Failure in
Neonates
Diagnosis
History Taking Physical Examination

u Mother’s prenatal history (pregnancy and u General Examination
labour + exposure to toxic or illness) u Weight compared to birth weight + normal
u Birth and perinatal history + any need for value
resuscitation u Vital sign: Blood pressure + saturation in right
upper arm and lower extremity (CoA,
u Family history (at least 3 generation)è hypertension)
screen recessive genetic condition
u Cardiac examination: abnormal murmur,
u Family history of sudden, unexplained, gallop, thrill, intensity of heart sound
unusual death
u Signs of HF
u History of stillbirth
u Other risk factors
u Symptoms of HF
Further assessment and work-up:

Chest-X ray, ECG, Lab, Echocardiography, etc.
• Das BB. Current State of Pediatric Heart Failure. Children (Basel). 2018;5(7):88.
• Ryan TD, Kindel SJ, O’Connor MJ. Clinical diagnosis and management of pediatric heart failure: Heart failure in the neonate. Philadelphia: Elsevier;2018. p.383-97.
Symptoms and Signs
u In children, the clinical signs of HF may not be obvious on physical examination (may
mimic other childhood diseases, e.g. bronchitis and GI tract disease)
u Resting tachycardia and tachypnea are commonly present in all ages
u Tachycardia >170x/min (severe), >220x/min (SVT)
u Tachypnea >60/min (moderate-severe)
u Blood pressure is usually normal except in patients with cardiogenic shock or impending
shock
u Signs of fluid overload, such as hepatomegaly (>3 cm below costal margin) and a gallop
rhythm, are common in children
u Other findings of congestion (edema of the lower extremities, abdominal ascites, rales,
and jugular venous distention) are identified less frequently
Price JF. Pediatrics in Review. 2019 Feb;40(2):60-70.

Symptoms and Signs
u Signs of poor perfusion may be present, including delayed capillary refill and
cool distal extremities
u A blowing holo systolic murmur at the apex may be appreciated in patients
with a dilated left ventricular chamber and an incompetent mitral valve
u Growth failure in heart failure is likely multifactorial and may be related to
suboptimal energy intake secondary to exercise intolerance, malabsorption,
and/or end-organ dysfunction due to impaired cardiac output.

Age-Related Specific Signs and Symptoms of HF
Infant and young children:

- Difficulty in feeding (prolonged
feeding time intake to frank
intolerance)
- <3 oz/feed or >40 min/feed
- Vomiting
- Poor weight gain
- Tachypnea
- Sinus tachycardia
- Cyanosis
- Pallor
- Fatigue
- Diaphoresis
Das BB. Current State of Pediatric Heart Failure. Children (Basel). 2018;5(7):88.
Masarone D, Valente F, Rubino M, Vastarella R, Gravino R, Rea A, Russo MG, Pacileo G, Limongelli G. Pediatric Heart Failure: A
Practical Guide to Diagnosis and Management. Pediatr Neonatol. 2017 Aug;58(4):303-312.
Clinical Features of Decompensated Pediatric HF

Classification of Heart Failure
Hoover JD, et al. Pediatr Crit Care Med 2018

Masarone D, Valente F, Rubino M, Vastarella R, Gravino R, Rea A, Russo MG, Pacileo G, Limongelli G. Pediatric Heart Failure: A Practical Guide to Diagnosis and Management. Pediatr Neonatol. 2017
Aug;58(4):303-312.
Heart Failure Scores for Infants With CHF
Sharma CM, Nair MNG, Jatana SK, Shahi BN. Congestive heart failure in infants and children.
MJAFI. 2003;59:228-33.
Chest Radiography
u Chest radiography is indicated in all children with suspected HF
u Heart Size (cardiomegaly reflects the

nature of any anatomic or functional
lesion)
è CTR >60% in newborn or >55% in
older infant
u Semiquantification of pulmonary blood
flow or vascular congestionè pulmonary
edema (marked by fluffy infiltrates with
perihilar haziness, septal lines or Kerley B
lines) + pleural effusions
u Children with HF frequently have normal lung markings on chest
radiographs
• Price JF. Pediatrics in Review. 2019 Feb;40(2):60-70.

• Masarone D, Valente F, Rubino M, Vastarella R, Gravino R, Rea A, Russo MG, Pacileo G, Limongelli G. Pediatric Heart Failure: A Practical Guide to Diagnosis and Management. Pediatr Neonatol. 2017
Aug;58(4):303-312.
• Hsu DT, Pearson GD. Heart failure in children: part I: history, etiology, and pathophysiology. Circ Heart Fail. 2009 Jan;2(1):63-70.
Electrocardiogram (ECG)
u Sinus tachycardia is common in acute HF

u Non-specific abnormalities such as ventricular
hypertrophy by voltage criteria and Q waves, and ST-
segment or T-wave changes (myocardial inflammation
and/or ischemia)
u Electrical conduction disturbances, especially in
patients with advanced disease
u Rhythm disturbances are also common in patients with
HF and may include supraventricular tachycardia, atrial
fibrillation/flutter, atrioventricular block, and
ventricular tachycardia
Aug;58(4):303-312.
• Ryan TD, Kindel SJ, O’Connor MJ. Clinical diagnosis and management of pediatric heart failure: Heart failure in the neonate. Philadelphia: Elsevier;2018.
p.383-97.
Laboratory Test in HF
Masarone D, Valente F, Rubino M, Vastarella R, Gravino R, Rea A, Russo MG, Pacileo G, Limongelli G. Pediatric Heart Failure: A Practical Guide to Diagnosis and Management.
Pediatr Neonatol. 2017 Aug;58(4):303-312.
Laboratory Investigations
u The natriuretic peptides are useful in the acute settings for differentiation of HF from pulmonary
causes of respiratory distress
u ESR and CRP + blood PCR test (adenovirus, parvovirus B19, HHV-6, enterovirus, CMV, HIV, and additional
viral studies as is appropriate for the local epidemiology) should be done in cases of HF with suspected
myocarditis
u Blood glucose and serum electrolytes like calcium, phosphorous should be measured in all children with
HF as their abnormalities can cause reversible ventricular dysfunction
u Metabolic test (including urine organic acid, serum amino acid, serum carnitine) should be considered in
all neonates with newly discovered cardiomyopathy or HF
u Genetic testing may be considered as recent reports suggest genetic as the cause for more than 50% of
patients with DCM
u More helpful to confirm rather than exclude diagnosis (only 30-35% of familial cardiomyopathies have an
identifiable genetic etiology)
u Screening for hypoxia and sepsis should be done in newborn with HF
97.
• Jayaprasad N. Heart Views 2016
• Deshpande SR. Congenit Heart Dis 2011
• Ponikowski P, et al. Eur Heart J. 2016
Echocardiography
u Primary diagnostic modality: reliable imaging quality, noninvasive, nontoxic, widely available,
portable, low-cost test
u Neonates typically have excellent echocardiographic window
u Provides immediate data on cardiac morphology and structure, chamber volumes/diameters, wall
thickness, ventricular systolic/diastolic function (shortening fraction, ejection fraction), and pulmonary
pressure
u Evaluate for congenital and acquired condition in suspected cardiomyopathy in neonates (full anatomic
study)

Aug;58(4):303-312.
Cardiac MRI
u Assuming an increasingly important role in the assessment of
cardiomyopathies in all age groups
u Superior to echo in image quality + accurate volumetric calculation
of EF of both ventricles
u Provides information regarding tissue characteristics (myocardial
scarring via the presence of late gadolinium enhancement and T1
mapping)
u Weakness: required sedation in neonate
u Indicated to study complex CHDs or tissue characterization
à for diagnosis, risk-stratification, and ongoing management of patients with
specific forms of cardiomyopathies
Other Investigations
Cardiac Catheterization
u Have a role in evaluation of selected groups of neonates with new onset HF
u Confirmation of coronary anatomyè selective coronary injection or aortic root angiography
(suspicion of ALCAPA)
u Accurate evaluation of pressure gradients in patients with complex valve diseases
u Hemodynamic parameters (pulmonary and systemic vascular resistance, cardiac output, and cardiac
index) in Fontan patients
u Intracardiac electrophysiologic proceduresè for diagnosis confirmation or very rarely intervention
of refractory arrhytmias
Endomyocardial Biopsy
u Gold Standard for myocarditis
u The decision to biopsy in an infant has to be weighed against a higher risk of fascular or myocardial
injury
Management of Heart Failure in
Neonates
Target of Management
Treatment of Pediatric HF aims to:

u ELIMINATE the causes of HF
è Corrective treatment should be performed in CHDs
è Systemic disease (such as sepsis) or electrolytic imbalance (such as hypocalcemia) must be carefully
researched and treated
u CONTROL the symptoms and disease progression
è General measures and medical therapy
è Treat precipitating factors: infection, anemia, electrolyte imbalance, arrhythmias
Park MK (2020). Park’s pediatric cardiology for practitioners.
Management Strategies in Neonates HF
Assessmment of
Imaging to Symptomatic and
New onset heart clinical stability
+ provision of determine Definitive
failure etiology therapy
support
• “Wet or Dry”
(congestion) and Warm
or Cold” (cardiac output)
• Cautious monitoring +
careful consideration for
ICU
• Stabilization of
hemodynamic
Ryan TD, Kindel SJ, O’Connor MJ. Clinical diagnosis and management of pediatric heart failure: Heart failure in the neonate. Philadelphia:
Elsevier;2018. p.383-97.
Initial stabilization
u Inadequate CO due to systolic or diastolic dysfunction

u Goals of early therapy: appropariate hemodynamics,
maintenance of oxygen delivery, prevention of end organ
injury
u Stable infant with congestive symptomsè
u Diuretic may be adequate
u Loop diuretic (furosemid and bumetanide)
u Thiazdie (cholorhiazide)
u If inadequateè inotropic and vasoactive agent
Elsevier;2018. p.383-97.
Initial Stabilization
u Inotropic and vasoactive agent

u Milrinone (PDE inhibitor) up to 0.75 mcg/kg/minè increase inotropy, vasodilation, cardiac
relaxation (lusitropy)
u Dopamine
u Low dose <5 mcg/kg/minè vasodilation in brain, kidney, mesentery
u Moderate dose 5-10 mcg/kg/minè increase HR and inotropy + increased cardiac oxygen consumption
(beta receptor stimulation)
u High dose >10 mcg/kg/minè increase vascular tone and SVR (alpha adrenergic)
u Dobutamine
u Strong beta agonism, mild-moderate alpha antagonism
u Increase inotropy, vasodilation, modest effect on HR
u Epinephrine
u Activates alpha , beta 1, and beta 2 receptors in a dose-dependent manner
u Increase stroke volume and lower SVR via peripheral vasodilation (beta agonism predominates at lower
doses)
Elsevier;2018. p.383-97.
Vasodilator
u Helpful in patients with low CO + high SVR (driven by increased cathecolamines in response
to low organ perfusion pressure)
u Nitroprusside
u Rapid onset and effect
u Side effectt: cyanide toxicity from metabolic byproducts, should be monitored particularly in
small infants
u Nicardipine
u Dihydropyridine calcium-channel blocker (vasodilatory effects without intrinsic myocardial
inhibition)
u Caution: neonates have low calcium strores and stereotypic calcium sensitivity (concerns for
contractility effects)
u Nesiritide
u Recombinant form of BNP
u Benefits in urine output and decreased filling pressure, without significant hypotension
Elsevier;2018. p.383-97.
Chronic Medical Therapy
u Chronic therapy goals: reducing fluid overload (diuretic), reducing afterload (vasodilator),
downregulating neurohormonal response (blockade of angiotensin, renin, aldosterone, and
cathecolamine)
u Focuses on three main goals:
u Decrease of pulmonary wedge pressure
u Increase CO and the improvement of end-organ perfusion
u Delay of disease progression
u There is little evidence of HF therapy in childrenè Canadian Cardiovascular Society (CCS) and ISHLT
relied on ACC/AHA guidelines of evaluation and management of HF in adults.

Aug;58(4):303-312.
Diuretics
u Role: reduction of systemic, pulmonary, and venous

congestion (symptomatic relief)
u Administer oral form early in the day to prevent
increased urination during sleep hours
u Dilute if necessary in NaCl 0.9% (glucose solutions CAUTIONS!!!
are unsuitable) Check for contraindications
• Allergy to thiazides and
sulfonamides
• Electrolyte depletion
• Severe renal failure, anuria
Complications
• Electrolyte abnormalities
(hyponatremia, hypo- or
hyperkalemia, hypochloremia),
metabolic alkalosis
Aug;58(4):303-312.
• Hypotension
ACE Inhibitor
u Acts as anti-remodelling (prevent, attenuate, or

possibly reverse the pathophysiological myocardial
remodelling) + decrease afterload
u Recommended in all pediatric patients with HF
and left ventricular systolic dysfunction
MONITOR!!!
Beneftis: symptomatic relief, reduce
u
Blood pressure, renal function,
hospitalization, decrease mortality
serum potassium
u Preferred shorter half-life (captopril, enalapril)
over once daily (lisinopril) in neonatesè better
control of dosing

Aug;58(4):303-312.
Beta-blocker
u Antagonize the deleterious effects of chronic

sympathetic myocardial activation, can reverse
left ventricular remodelling, improve systolic
function
u Start with a low dose, up-titrate every 2 weeks by
doubling the dose Cautions!!
u Adverse effect • Bradycardia, heart blocks,
u CNSè headache, fatigue, dizziness cardiogenic shock, stage IV
u GIè GI upset, nausea, vomiting, diarrhea failure
• Can be exacerbated by the
cardiac-suppressing effects of
these drugs
Aug;58(4):303-312.
Digoxin (Cardiac Glycoside)
u Increase the cardiac output (or contractile
state of the myocardium)
u Indication: symptomatic patients with left
and/or right ventricular systolic dysfunction
u Administration
u IVè may be administered undiluted (may also
dilute 1 mL of digoxin in 4 mL of sterile water
for injection, D5W, or 0.9% NaCl)
Cautions!!
u POè can be given with or without meal
• Digoxin has a narrow therapeutic
u Continues to be common in some pediatric
range
centers (use in dysrrhytmia), but it is no • Medication errors include
longer considered frontline for management miscalculation of pediatric doses
of HF
and insufficient monitoring

Aug;58(4):303-312.
Mechanical Circulatory Support (VAD and
ECMO)
• ECMO
• Need systemic anticoagulation
• Need large-bore cannulae with
central placement trough the
chest in infant
• Risk of both embolic and
hemorrhagic complications
• Berlin EXCOR pulsatile VAD
• Multiple pump size, capable of
supporting children as small as 3-4
kg
• Overall success rate for bridge to
transplant or explant and recovery
(75%)
• Superior survival after heart
transplant compared to ECMO
• Low survival rate for patient with
•
•
CHD and < 5 kg
Aug;58(4):303-312.
Adult Children
u Mainly related to ischemia (60-70% of u Mostly related to CHDs or cardiomyopathies
cases) u Lacking consensus for pediatric HF
u Well-established guidelines for the u More likely to undergo intubation, cardiac
management of adult HF bypass, cardioversion and other cardiac
u of intubation, cardiac bypass
A low utilization
procedures
and other cardiac procedures u Hospital stays were significantly longer
u The major components of their regimen
were medical
Masarone D, Valente F, Rubino M, Vastarella R, Gravino R, Rea A, Russo MG, Pacileo G, Limongelli G. Pediatric Heart Failure: A Practical Guide to Diagnosis and Management. Pediatr Neonatol. 2017 Aug;58(4):303-312.
Park MK (2020). Park’s pediatric cardiology for practitioners.
Conclusion
u Heart Failure in the neonate is a complex syndrome representing a constellation of symptoms
and signs with heterogenous etiology including CHD, inherited or acquired cardiomyopathy, and
systemic disease.
u Accurated diagnosis of the etiology, comprising of thorough evaluation including family history,
focused physical exam, radiography, ECG, noninvasive cardiac imaging, laboratory testing, and
in appropriate cases invasive testing, is the key for appropriate therapy.
u The management of heart failure in neonates include diuretic therapy, afterload reduction,
beta-blockade, inotropic and vasoactive medications, mechanical ventilatory support,
mechanical circulatory support, and ultimately heart transplantation in cases of intractable
disease.
u The definitive therapy of CHD-related HF in neonates is corrective surgery or intervention
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Heart Failure in Neonates

History Taking Physical Examination

Further assessment and work-up:

Price JF. Pediatrics in Review. 2019 Feb;40(2):60-70.

Price JF. Pediatrics in Review. 2019 Feb;40(2):60-70.

Infant and young children:

Price JF. Pediatrics in Review. 2019 Feb;40(2):60-70.

Hoover JD, et al. Pediatr Crit Care Med 2018

u Heart Size (cardiomegaly reflects the

• Price JF. Pediatrics in Review. 2019 Feb;40(2):60-70.

u Sinus tachycardia is common in acute HF

u Screening for hypoxia and sepsis should be done in newborn with HF

• Price JF. Pediatrics in Review. 2019 Feb;40(2):60-70.

Treatment of Pediatric HF aims to:

u Inadequate CO due to systolic or diastolic dysfunction

u Inotropic and vasoactive agent

• Price JF. Pediatrics in Review. 2019 Feb;40(2):60-70.

u Role: reduction of systemic, pulmonary, and venous

u Acts as anti-remodelling (prevent, attenuate, or

• Price JF. Pediatrics in Review. 2019 Feb;40(2):60-70.

u Antagonize the deleterious effects of chronic

• Price JF. Pediatrics in Review. 2019 Feb;40(2):60-70.

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