• •

ear 01 ure 1n

Ninfa Joson - Villanueva, MD, MHPEd, FPPS, FPCC, FPSE

Heart Failure in Children
• Definition
• Pathophysiology
• Diagnosis
• History and Physical Examination
• Loborotory / Ancillary Procedures

• Management
• Prognosis
• Cardiogenic Chock
I

• Failure of the heart to supply blood

to either systemic or pulmonary
circulation at an appropriate rate
of flow
OR
to receive venous return at an
appropriate filling pressure, resulting
in adverse effects on the heart, the
circulation and the patient.
Heart Failure in Children:
DEFINITION

• A progressive clinical and pathophysiological

syndrome caused by cardiovascular and
noncardiovascular abnormalities that result in
characteristic signs and symptoms including
edema, respiratory distress, growth failure, and
exercise intolerance, and accompanied by
circulatory, neurohormonal, and molecular
derangements

Hsu and Pearson 2009

 Cardiac Output= Stroke Volume X Heart Rate

Determinants of Stroke Volume:

Preload
Afterload
Myocardial Contractility
I
Systemic Oxygen Transport= Cardiac Output X Systemic 02 content
Frank-Starling Law
No congestion Congestion

normal
QI
E
-:,
0
systolic fa, Iure
>QJ
..lie + positive inotroptc stimulation
0
....
'-
V,

systolic failu e

End-Diastolic Ventricular Pressure

~ (Preload)
 PATHOPHYSIO LOGY OF HEART FAILURE

! Cardiac Output
/
t baroreceptor stimulation I ! renal perfusion

Activation of Activation of Renin-Angiotensin-

L Sympathetic Nervous System Aldosterone System --
Heart Rate Vasoconstriction ( ·- afterload)
7 cardiac contractility Na+ & Water retention ( j preload)
Vasoconstriciion ( j afterload)

~.....f-+I Myocardial Myocardial

toxicity apoptosis / fibrosis

I Myocardiald ysfunction I
 do
f Gontrac . ty I Na• reMirptlon
~ Va-on,.rktlo" I K♦ and H♦ •11tretlon
t llenil'I, Vat.OfUKS,ln and
Aldasterone AockzSensjn II
I Alclo11e,0M
EcdoSbtfln I Vas.opr.nf"
VffOCOrutrktio" I t:ndoth4fln
Hy"rtrophy
Apoptosb
lr,otropy
r ~.,.,_

r Prgc1YSS;i99 of BNP & ANP Atrial notriuretic peptides

N:atril.l,.taa Brain (B-type) natriuretic pep1ides
Va~odllmtlocl
0.CtelMJ: C.rdiK fibfoslJ, SffS
k!fvatilon, Aldast,nona and R-mln
 PATHOPHYSIOLOGY OF HEART FAILURE

I Low cardiac output

Natriuretic Peptide System

~Ang1oten_s_1n_11_ --1t
I
Epinephr ne
t P (A P, 8 P)
1' P Recept r Density
AT1 Recepto~
t
t
orep,nephrine
Rf!n1n - 1 --
Vasodilation
I
I ~ Blood Pressure
Vasoconstr,ct on .J, Sympa hetic tone
I"" Blood Pres$ure Ant ,-hypertrophlc/ -ftbrot
""'Sympathetic tone D ures,s and atnuresis _J
A dosterone
F,bros1s
""' Ventrlcu ar Hypertrophy
Apoptos1s t clearance of NP by NEPRIL YSIN
. -
- ~-:::::::::~.J Chronic heart failure-
,.,·&a .. '
Heart Failure in Children
Epidemiology

• 20% are due to CHDs (8/1 ooo livebirths)

• Dramatic change bf outcomes due to early
surgery
• Cardiomyopathies (90% are dilated CMP)
• Rheumatic Fever and Rheumatic Heart Disease -
in developing countries
Causes of Heart Failure in Infants &
Children

Overcirculation Failure
Pump Failure
CAUSES OF OVER-CIRCULATION
HEART FAILURE
• Conditions associated with j pulmonary blood flow
• Lefl - lo - right shunts like VSD, PDA. P window and AVSD

• Admixture lesions
• TAPVR. Truncus orteriosus, Single Ventricle, e tc.

• Parallel circulation as in TGA

• Conditions causing l cardiac output
• Anemia, systemic AV fistula .beriberi. etc.

• Regurgitant valvular lesions

• Mitro! and aortic regurgitation - congenital, rheumatic
• Infective Endocarditis
CAUSES OF PUMP FAILURE
• Congenital causes
• Obstructive lesions: LV outflow tract obstructions (aortic stenosis,
Coarctation of aort ,) and RV outflow tract obstruction in
Pulmonary Stenos[s
• Anomalous Origin of left coronary artery from pulmonary artery
• Postoperative CHD with ventricular dysfunction
• Inflammatory- Viral myocarditis, HIV -related
• Dilated Cardiomyopathies - ldipathic, familial, neuromuscular
disease and metabolic
• Rhythm disturbance- tachycardiomyopathy and complete
heart block
• Others: LV noncompaction,anthracycline toxicity
DIAGNOSIS OF HEART FAILURE IN
CHILDREN
• History and Physical Examination
~
• Investigations
• Chest X-ray
• ECG
• Echocardiography
• Laboratory Tests/ Biomarkers
• Special Investigations
DIAGNOSIS OF HEART FAILURE IN CHILDREN
HISTORY
FETUS FIRST WEEK OF LIFE
SVT/ Severe bradycardia due Severe Aortic Stenosis
to Complete Heart Block Coarctation of Aorta
Severe TR due to Ebsteins' s Hypoplastic Left Heart Syndrome
Anomaly
MR from AV canal defect, Obstructed TAPVR
Sytemic AV fistula
Myocarditis TGA w ith intact ventricular septum
NEWBORN
Fetal cardiomyopathies PDA in prematures
Extracardiac conditions: sepsis,
hypoglycemia, hypocalcemia
DIAGNOSIS OF HEART FAILURE IN CH ILDREN
HISTORY
2 nd week of life Older Children
Large VSD, PDA, AP window Acute Rheumatic Fever with
~ . carditis
Truncus a rter1osus
Unostructed TA PVR Decompensated chronic
RHO
4 -6 weeks of life
Myocarditis
VSD, PDA, AP window Cardiomyopathies
Infants Rhythm disturbances
Dilated Cardiomyopathies- Palliated C HO
idiopathic, inborn errors of metabolism
& moiformation syndromes
DIAGNOSIS OF HEART FAILURE IN CH ILDREN
PHYSICAL EXAMINATION
PATHOPHYSIOLOGIC
MECHANISMS PHYSICAL FINDINGS

Compensatory responses tachycardia, gallop rhythm

to impaired cardiac weak & thready pulses
function cardiomegaly

Signs of increased increased perspiration

sympathetic response cold wet skin
DIAGNOSIS O F HEA RT FAILURE IN CHI LDREN
PHYSICAL EXAMINATION
PATHOPHYSIOLOGIC
MECHANISMS PHYSICAL FINDINGS
Hepat omegaly
Signs of systemic
venous congestion: Puffy eyelids/ sacral edema in infants
RIGHT-SIDED FAILURE
Dist ended neck veins/
Ankle edema in older child
tachypnea/ dyspnea on exertion:
Signs of pulmonary • poor feeding in small infant s
venous congestion: • orthopnea in older child
LEFT-SIDED HEART
FAILURE nasal flaring, retractions
wheezing+ rales
pulmonary edema
DIAGNOSIS OF HEART FAILURE IN CHILDREN
CLIN ICAL FEATURES

NEWBORNS OLDER CHILDREN/ ADOLESCENTS

Nonspecific Fatigue / effort intolerance
INFANTS Orthopnea
achycardia Abdomi.n a l pain
Tachypnea Oliguria / Dependent edema
Feeding difficulties Ascites
irritability during feeding Unequal upper & lower limb
pulses
refusal to feed
Peripheral bruits
Diaphoresis
Raised or asymmetric BP
Hepatomegaly
 STAGES OF HEART FAILURE
International Society for Heart and Lung Transplantation (ISHLT)

STAGES I Examples
Patients with increased risk of Exposure to cardiotoxic agents
developing HF, but with
History of Rheumatic Fever
NORMAL cardiac function &
chamber size Hereditary diseases causing CMP ( e.g.
I neuromuscular disorders

Patients with abnormal cardiac Mitral or aortic insufficiency with LV

enlargement
morphology or function, with
I
I NO symptoms of HF, past or L-to-R shunt lesions with LV
present enlargement
I
LV dysfunction
 STAGES OF HEART FAILURE
International Society for Heart and Lung Transplantation {ISHLT)

Stages Examples
-
Patients with structural or f Symptomatic CHDs with PBF
C RF/ RH Din heart f ailure
functional heart disease, &
past or current SYMPTOMS of Symptomatic CMP
heart failur~e

Heart failure requiring special Severe heart failure in congenital or

D acquired heart diseases NOT controlled
interventions by maximal m edical therapy
 HEART FAILURE: FUNCTIONAL CLASSIFICATION

Class New York Heart Association Modified Ross ( < 6 y/o)

Without limitation of physical activity
Ordinary physical activity does not cause Asymptomatic
, undue fatigue, palpitation, dyspnea, or angina!
•
pain ~

I -
With slight limitation of physical Mild tachypnea or
activity. diaphoresis with feeding in
1
Comfortable at rest. infants.
Ordinary physical activity resu lts in fatigue,
palpitation, dyspnea, or angina! pain. Dyspnea on exertion in
older children.

,..,. &ii
 Heart Failure: Functional Classification
-
I Class New York Heart Association Modified Ross ( <6 y/o)
-
With marked limitation of physical activity. Marked tachypnea or diaphoresis
111 Comfortable at rest. with feeding in infants.
Prolonged feeding times with growth
Le\s than ordinary physical activity causes fatigue, failure.
palpitation, dyspnea, or anginal pain . Marked dyspnea on exertion in older
children.
Cannot carry on any physical activity with out
IV discomfort. Symptoms such as tachypnea,
retractions, grunting, or diaphoresis
Symptoms of cardiac insufficiency or of the at rest.
angina! syndrome may be present even at
rest.

If any physical activity is undertaken, discomfort is

increased.
,.,♦ Eil
 INVESTIGATIONS IN HEART FAILURE
Chest X-ray
I
Cardiomegaly
CT ratio > 60% in neonates
> 55% in older children
/ Highly predictive of ventricular
dilatation on echocardiography
Pulmonary edema, septal lines
(Kerley B lines)
Pleural effusion
L-to- R shunts: cardiomegaly,
enlarged main & branch pulmonary
arteries & pulmonary plethora

CT ra tio = 0.57
 Investigations in Heart Failure
ECG
Sinus Tachycardia
Common Findings:
tachycardia
LV hypertrophy
r ST- T changes
l-+-
1
Ml patterns- inferolateral Q waves
Pros&iro oYcrlood Volume overload in anomalous LCA from PA
1,1 R ...-.~ ,.;r, '3111,,;,d ST . .J J « I
,n-iedT wa,o :.t-n• PL~
conduction blocks
Tac hycardiomyo pa thy
Complete Heart Block

V6 V6
INVESTIGATIONS IN HEART FAILURE
ECHOCARDIOGRAPHY

• Indicated in all cases of pediatric

HF to exclude possible structural
diseases
• Baseline echo needed fo future
•
comparison
• Screening of cancer patients
undergoing anthracycline
chemotherapy, patients with
storage disorders, neuromuscular
diseases, etc.
• For surveillance of disease
progression & response to therapy
4 - chamber view o f the heart
INVESTIGATIONS IN HEART FAILURE
LABORATORY TESTS

Blood glucose
Serum electrolytes
Screening for sepsis and hypoxia in newborns with HF
ASO & CRP in suspected RF or reactivation of chronic RHD
Metabolic & genetic testing
INVESTIGATIONS IN HEART FAILURE
LABORATORY TESTS

Test Rationale
Complete Assess anemia which may aggravate HF
Blood Count
(CBC) · WBC may result from stress or signal underlying
infection

Electrolytes ,. Na+ refl.e cts expansion of extracellular fluid volume

-
! K+ and Cl can be result of pro longed diuretics use

~K+ can be result of impaired renal perfusion & marked

reduction of GFR or
from intracellular K+ release due to impaired tissue
perfusion
INVESTIGATIONS IN HEART FAILURE
LABORATORY TESTS

Test Rationale
Renal Function Tests -~ BUN and BUN/creatinine ratio seen in
decompensated HF

Liver Function Tests Congestive hepatomegaly associated with impaired

hepatic function: j AST, ALT, LOH and other liver
enzymes

Hyperbilirubinemia (j B1 and B2) related to hepatic

venous congestion - common in severe right heart
failure

Prolonged PTT

Hypoalbuminemia- from impairment in hepatic synthesis

INVESTIGATIONS IN HEART FAILURE
LABORATORY TESTS
Test
Natriuretic peptides:
Brain natriuretic peptide (BNP)
amino terminal (NT- proBNP)
Rationale
Useful for differentiation of HF from pulmonary causes of
respiratory distress
Reliable test for recognizing ventricular dysfunction in
children with a variety of CHO

Guide to therapy or to monitor HF status

CPK-MB Useful if clinical scenario is suggestive of an ischemic

Troponin I & T process of myocarditis
Lactate j lactate in decompensated HF as a result of
decreased tissue perfusion &/or decreased metabolism
due to secondary liver dysfunction

Useful serologic marker for response to therapy

 INVESTIGATIONS IN HEART FAILURE
LABORATORY TESTS
Test Rationale
Thyroid function Both severe hyper- or hypothyroidism can cause
tests ~ heart failure

/ Arterial blood gas Mild hypoxemia in patients with with mild to

(ABG) moderate HF

Severe hypoxemia to hypoxia in severe HF

Hypocapnia in early stages of pulmonary edema

because of V /Q mismatch, progressing to
hypercapnia & respiratory acidosis
(related to ! vital capacity & poor ventilation)
INVESTIGATIONS IN HEART FAILURE
SPECIAL INVESTIGATIONS
Test Rationale
Polymerase chain In viral myocarditis
reaction (PCR)
Endomyocardial Invasive procedure with significant risk
biopsy (EMB) To confirm clinical diagnosis of myocarditis/ choose
appropriate therapy eg. Giant cell myocarditis

Cardiac MRI Less invasive than EMB

To study complex CHDs or for tissue c haracterization
(diagnosis, risk stratification) with specific forms of CMPs
Cardiac Accurate evaluation of pressure gradients in patients
Catheterization with complex valve diseases
Evaluation of hemodynamic parameters {PVR, SVR, CO,
Cl) in Fontan patients or during pre-transplant screening
TREATMENT
Principles of Management
Treatment of the cause
• Medical or surgical
Correction of precipitating even,t
• lntercurrent infecl ions
• Anemia
• Electrolyte imba lances
• Arrhy thmia
• Rheumatic reactivation
• IE
• Drug interactions. toxicity, noncompiance

Treatment of systemic or pulmonary co.ngestion

 AIMS OF TREATMENT OF HEART FAILURE

1. Eliminate the causes ~

/
2. Control the symptoms and disease
•
progression
TREATMENT
1. Eliminate the causes

■ Corrective treatment in CHDs if available-

Surgical/ interventional procedures

• Correction of precipitating events:

intercurrent infection anemia
electrolyte imbalances arrhythmia
rheumatic reactivation IE
drug interactions, toxicity, noncompliance
HEART FAILURE IN CHILDREN:
SURGICAL INTERVENTIONS

VSD Patch Closure

PDA Ligation
HEART FAILURE IN CHILDREN:
INTERVENTIONAL PROCEDURES

Step 1 Step 2

Step 3 Step 4

PDA Occluder Device ASD Device Closure

 TREATMENT
2. Control of symptoms and d isease progression
General measures
• Nutritional support/ supplementation:
caloric intake of 150 kcal/kg/d in infants
25 - 30 kcal/kg/day in children & adolescents
• Oxygen support
• Salt and water restriction in older children

Medical Therapy - Goals:

• Decrease of pulmonary wedge pressure
• Increase of cardiac output & the improvement of end-organ
perfusion
• Delay of d isease progression
 PHA RMACOLOGIC THERAPY
DIURETICS
• dec rease preload by pro moting natriuresis, & provide relief of volume overload
symptoms such as pulmo nary and p'eripheral ed ema
• use d to treat child ren with stage C or D HF

• Thlazide diuretics used as 2nd -line agents & of ten in

combination with loop d iuretics (clorothiazide,
hydrochorothiazied & metazolone)

• K- sparing diuretics: Spironolac tone & eplerenone

• Loop diuretics: Furosemide

Bumetanide & torsemide- more potent drugs, reserved for
more severe or furosemide-resistant fluid overload
Side effec ts: electrolyte abnonnalities (hyponatremia,
hypochloremia & hypokalemia) , metabolic alkalosis, &
renal insufficiency.
Long-term therapy: nephrocalcinosis & ototoxicity (usually
with high IV doses), Increased risk of bone fractures
PHARMACOLOGICAL THERA PY

MINERALOCORTICOID RECEPTOR
ANTAQONISTS MRA~ - - - - - - - - - - - '
• For children with chronic systolic HF
Actions:
• decrease Na+ reabsorption and K+ excretion in collecting ducts of
kidneys ~
• K+- sp aring diuretic effect; suitable for use in conjunction with loop
diuretics & thiazides
• inhibition of myocardial fibrosis (LV remodeling)

Examples: Spironolactone
Eplerenone
Side effects include hyperkalemia (with both drugs) and gynecomastia
(with spironolactone) .
PHARMACOLOGICAL THERAPY
DIGOXIN

• not recommended for child ren with asymptomatic ventricular dysfunction;

however
• commonly used in the t{eatment of infants and children with stage C HF,
particularly those with persistent symptoms despite treatment with other
agents
• Actions:
• positive inotropic effect (mediated by No+/K + ATPose inhibition & increase in
intracellular Ca+)
• negative ch,ronotropic effect that slows atrial conduction
• vagotonic properties that counter symptoms and signs mediated by the
activation of the sym pathetic nervous system in HF
PHARMACOLOGICAL THERAPY
ANGIOTENSIN-CONVERTING ENZYME (ACE)
INHIBITORS
• 1st - line component therapy for c hildren with stage Band C
HF; ~ BP & renal function should be closely monitored, especially in
neonates
• Decrease afterload by antagonizing the RAAS, improve
cardiac output and mediate reversal of LV remodeling (on
chronic use)
• Captopril - preferred in neonates
• Enalapril - 1st choice for those older than 2 years of age

ANGIOTENSIN II RECEPTOR BLOCKERS

• For those intolerant of ACEls {cough, angioedema}
• Examples: candesartan, losartan, valsartan
• New drug: Sacubitril-Valsartan (an ARB plus neprilysin inhibitor}
PHARMACOLOGICAL THERAPY I
ANGIOTENSIN-CONVERTING ENZYME (ACE)
INHIBITORS
• 1st - line component therapy for c hildren with stage Band C
HF; BP & renal function should be closely monitored, especially in
neonates
• Decrease afterload by antagonizing the RAAS, improve
cardiac output and mediate reversal of LV remodeling (on
chronic use)
• Captopril - preferred in neonates
• Enalapril - 1st choice for those older than 2 years of age

ANGIOTENSIN II RECEPTOR BLOCKERS

 PHARMACOLOGICAL THERAP Y

BETA BLOCKERS
• used in children who are stable on other HF medications
diuretics, ACEi) , have systolic dysfunttion w ith stage C HF &
ave a systemic LV
• antagonizes the deleterious effects of chronic sympathetic
myocardial activation & can reverse LV remodeling &
impro,,v e systolic function
• _arvedilol
• Metoprolol
ide effects that may preclude dose increase: dizziness, fatigue , hypotension, bradycardia,
bronchospasm, & hypoglycemia.
 PHARMACOLOGICAL THERAPY

INOTROPES
Digoxin - main oral inotropic drug
IV I notropes
• used in the setting of low cardiac output
/ (eg, during acute exacerbations of HF to improve cardiac output
& to stabilize patients awaiting heart transplantation)
• effect is mediated through higher intracellular
cAMP levels, either by :
1. increased production (catecholamines)
2. decreased degradation (phosphodiesterase
Ill inhibition}.
PHARMACOLOGICAL THERAPY

Intravenous INOTROPES
Catecholamines - sympathomimetic stimulation; improves
myocardial contractility & may have an additional
beneficial effect on peripheral vascular beds
• Dopamine- the preferred drug during decompensated HF
{usually in combination with IV milrinone)
• Dobutamine- with additive effect of reducing afterload.
• Epinephrine - low dose used in setting of refractory
hypotension and/or poor end-organ perfusion .
Milrinone - a phosphodiesterase Ill inhibitor; increases
contractility and reduces afterload without a significant
increase in myocardial oxygen consumption
 AGENT PEDIATRIC DOSE COMMENT

PRELOAD REDUCTION

1 Furosemide 1 mg/kg/dose PO or IV May increase to qid

Hyd rochlorothiazide 2 mg/kg/d PO divided bid May increase to qid

Metolazone 0.2 mg/kg/dose PO Used with loop diuretic, may increase to bid

INOTROPIC

Prete rm infants: 0.005 mg/kg/d PO divided bid

Dlgoxin o r 75% of this dose IV; age 10 y: 0.005
mg/ kg/d PO qd or 75% of thi s dose IV

5-10 mcg/kg/min IV (usual dosage; maximal

Dopamine Gradually t itrate upward to desi red effect
dosage may be up to 28 mcg/kg/min)

Dobutamine 5-10 mcg/kg/min IV Gradually t itrate upward to desi red effect

Epinephrine 0.01-0.03 mcg/kg/mln IV Not to exceed 0.1-0.3 mcg/kg/min

I
I Typically used without loading dose, especially
M ilr inone 0.3-1 mcg/kg/min IV in unstable patients
Load: SO mcg/kg IV over 15 min
AFTERLOAD REDUCTION

Captopril 0.1-0.5 mg/kg/d PO divided q8h ...

Adults: 2.5-5 mg/day PO qd/bid initially;

0.1 mg/kg/d PO d ivided qd/bid, not to titrate slowly at 1- to 2-wk interva ls;
Enalapril
exceed 0.5 mg/kg/d target dose is 10~20 mg PO bid; not to
exceed 40 mg/day

Adults: Usual dosage is 10mg PO qd

Lisinopril Not established
(range, 2.5-10 mg)

Initial dose for hypertension is 0 .1

Losa rtan mg/kg/day PO; dosage for treatment of Adults: 25-100 mg/d PO qd or divided bid
CHF is not established in children

Nitroprusside 0.5-10 mcg/kg/min IV M ay need to monitor cyanide level

~
Nitroglycerin 0.1-0.5 mcg/kg/min IV Va sodilator

Initiate with 0.01 mcg/kg/min

Nesiritide 0.01-0.03 mcg/kg/m in IV
M ay cause dose-related hypotension

Alprostad iI* 0 .03-0.1 mcg/kg/min IV

 BETA-BLOCKADE

limited data suggest a therapeutic

dosage range of 0.2-0.4 mg/kg/dose PO
Adults: 12.5-25 mg PO bid
Carvedilol bid; initiate w ith lower dose and
Init iate w ith 3.125 mg PO bid
gradually increase dose q2-3wk to
therapeutic range

Metoprolol Not established Adults: 25-100 mg PO qd

SELECTIVE ALDOSTERONE ANTAGONISTS

It 1-3.3 mg/kg/day PO in single or divided Adults: 12.5-50 mg PO qd; reduce dose
Spiron olactone
doses to 25 mg qod if hyperkalemia occurs

Eplerenone Not established 25-50 mg PO qd

l(F) CURRENT INHIBITOR

Initial >6 mo and< 40 kg: 0.05 mg/kg PO Indicated for children aged 6 months or
o lder with stable symptoma1ic heart failure
BID
lvabradine caused by d ilated cordiomyopathy who ore
Maximum: 0.2 mg/kg BID (6 mo-1 y); 0.3 1n sinus rhythm with an elevated heari ro le.
mg/kg BID ( 1 y or older)
Blocks the hyperpolarizalion-activoted cyclic
nucleotide-gated (HCN) channel responsible
for the cardiac pacemaker lff} ' Funny'
current. which regulates heorl rote.
,·~
,. a::a
 DEVICE THERAPY:

I
Treatment of Rhythm Disturbances
Electrodes
lnser1ed into
A voln leading
to heett

l P, c.~,

lr,I.....,~
~llealON,-., 1>!,
t~•.
•tv ,,...
....;,, wo...-~,., ..,~

l ,...,..114' &•IMIIU

~--Utct-
I r1.tt.rt, Uftl ,uOt
=•l<N'd ""'itN..,.
Clectt vde,
In ttt,-,n ____.; Implant.a~•

EJectr<>des
defibrillator
Inserted
under skin
--
...nn.,,11w 1
.... "' ___
o.te ctt.

l>Y1»•"Qp4',e••""
~•C~•
in heart Rf9ht atrium and ven:nde

Pacemaker Implantable defibrillators Cardiac resynchronization

for odvo t1 ced 2 nd & 3rd for patients with history of therapy ( CRT)
degree Av Block cardiac arrest or symptomatic for pati~nts with EF < 35%, CLBBB
ventricular tac hycardia in pattern , prolonged QT
association with C HD
 DEVICE THERAPY
ECMO machine
r; = = oxygenates l-'"""""'
iiiiii;;;;;;;;;;'I

blood 't
Ca.'Tl.tas

81000 low In oxygen Bl~od ccn tanir,g '' '

flows lo ECIAO oxygen tli reLum!Kl \ ' \
\
' lV \
m:1<:h nc lo tile bOdy 'I •II"• 'II
I
I
I

Site of cannula
insertion ______.:__/ ______ ._ _ __J

Extracorporeal membrane Ventricular Assist Device (VAD)

oxygenation (ECMO) - For univentricular or biventricular circuit support
for those requiring a long-term mechanical support to
- for isolated HF that is believed to be
bridge to cardiac transplantation
reversible, as temporizing measure a bridge
to recovery of func tion

,.,·e "\
 CARDIAC TRANSPLANTATION
f

• Therapy of choice for end-stage HF in

children refractory to surgical and
Healthy donor
heart in pla-ce
medical therapy
, • Most common indication:
cardiomyopathies
• Others: Hypoplastic left heart
syndrome (HLHS)
Complex CHD, single Ventricle
Palliated heart disease
~
Management and Prevention of
HF Complica tions
Aspirin
Thro m bo er11_bo Iism Ant icoagulation therapy
• Pulmonary embolus
• initially with unfractionated heparin or LMWH, and
• Cerebral embolic stroke
subsequently w ith either LMWH warfarin
• deat h
Card ioversion or defibrillation for acute/ unstable HF
Arrhythmia Ant iarrhythmic therapy - not routine
• Sustained atrial &
Ablation therapy- for chronic atrial tachyarrhythmias
ventricular tachycardias
Implantable cardioverter defibrillator {ICD) - for those
Sudden death who have survived SCD & those who have high risk for SCD
due to ventricular arrhythmia
Outcome for Pediatric Patients with HF
• Mortality rate of 6 - 11 % among hospitalized patients
• lndepende'1t risk factors for mortality: comorbid renal failure and
respiratory failure
• Pediatric Carvedilol study: 16 1 childre n in c lass II & Ill HF, 8-mon study
Treatment: ACEi in all, diuretics + digoxin in 85%, b -blockers in 2/3,
spironolac tone in 1/3
Outcome: 56% improved , 18% remained stable, 26% worsened ( 11 deaths
& 18 tra nsplantation
• Registry of Dilated CMP 2000 - 2009 (549 c hildren - 1 year follow-up)
27% recovered - normal func tion and size
27% heart transplant
9% died
most commo n diagnosis: Dilated Cardiomyopathy
 SHOCK
HYPOVOLEMIC DISTRIBUTIVE OBSTRUCTIVE CARDIOGENIC

• Inadequate blood Inappropriate • Obstructed b lood • Impaired cardiac

volume or oxygen- d istri bu tion of blood flow contractility
carrying capacity volume and flow
Etiology: Etiology: Etiology: Etiology:
Gastroenteritis Sepsis (Septic) Tension pneumothorax CHDs
Burns Cardiac tamponade Myocarditis
Hemorrhage Anaphylaxis Pulmonary embolism Cardiomyopathy
Inadequate fluid (Anaphylactic) Constriction of the Arrhythmia
intake ductus arteriosus with
Increased body fluid Spinal cord Injury ductal-dependent
losses (Neurogenic) congenital heart
Osmotic diuresis lesions (Infant)
Cardiogenic Shock
• may occur as a complication of
l) Severe cardiac dysfunction
~

2) Septicemia
3) Severe burns
4} Anaphylaxis
5) Cardiomyopathy
6} Myocarditis
7) Myocardial infarction or stunning
8} Acute CNS disorders
I
Recognition of Shock Flowchart

A Airway open and maintainable/ not maintainable

Increased
Normal to increased Labored •
B Normal •
•
•

• • Normal Crackles, grunting

(:!: crackles) •
•
•
•
Compensated Shock Hypotensive Shock •
•
•
•
Narrow Variable Narrow
•
Increased

C .. Weak
Sounding or
Weak
•
•
weak
•
•
Pale, cool Warm or cool Pale, cool •
• •
Delayed Variable Delayed
•
•
Decreased
•
•
'
D • •
Irritable early Lethargy late •
•
•
•
•
E Variable
I
> Oxygen > IV/10 access
> Pulse oximetry > BLS as indicated
> ECG > Point-of-care glucose testing

•
•
•
• 20 ml/kg NS/LR bolus, •
• Control external bleeding •
repeat as needed •
•
• 20 ml/kg NS/LR bolus, repeat 2 or 3x as needed •
• Consider colloid •
•
• Transfuse PRBCs as indicated •
•

•
'
'
•
•

•
• Antibiotic • IM epinephrine (or autoinjector) 20 ml/kg NS/LR bolus, •
•
'•
• 20 ml/kg NS/LR • Fluid boluses (20ml/kg NS/LR) repeat PRN
•
bolus, repeat PRN • Albuterol Vasopressor
•
• V asopressor • Antihistamines, corticosteroids •
•
•
•
• Epinephrine infusion •
•
I
> Oxygen > IV/ 10 a cc ess
> Pulse o>cimetry > BLS a s ind icated
> ECG monitor > Point-of-core glucose testing

•
•
•
•
onagement a lgorithm 5 to ~ 0 ml/kg NS/LR bolus repeat PRN •
•
Brodycord io Vosoac tive inrusion •
•
•
Tachycardia with poor perfusion Consider experl consultation •
•
•
•

•
•

•
•
Prostoglondin E1 Needle Pericardiocen tesis 20 ml/kg NS/LR bolus. •

repea t PRN • •
Expert consullation d ecompression 20 ml/kg NS/LR bolus •
Consider thrombolytics .
Tube thorocostomy •
onticoogulonts •
'
Expert consultation •
•
•
•
Thank you