Paediatric Cardiology: A

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Paediatric CardiologyA review of Congenital
Heart Disease and Clinical Problems

• Dr. Suzie Lee
• Pediatric Cardiologist
• Assistant Professor, University of Ottawa

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Objectives

• To provide an outline of congenital heart disease
• List criteria for Kawasaki syndrome
• Describe the common innocent murmurs of childhood
• Review of pediatric ECGs

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An Outline of Congenital Heart Disease

• Pink (Acyanotic)
• Blue (Cyanotic)
• Critical outflow tract obstruction

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Acyanotic Congenital Heart Disease

• Normal Pulmonary Blood Flow
• ? Pulmonary Blood Flow

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Acyanotic Congenital Heart Disease

• Normal Pulmonary Blood Flow
• Valve Lesions
• Not fundamentally different from adults

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Acyanotic Congenital Heart Disease

• ? Pulmonary Blood Flow

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Shunt Lesions

• Atrial Level Shunt

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ASD

• Physiology
• Left to Right shunt because of greater compliance
  of right ventricle
• Loads right ventricle and right atrium
• Increased pulmonary blood flow at normal pressure

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ASD

• History
• Usually asymptomatic in childhood
• Occasionally frequent respiratory tract
  infections
• Presentation with murmur in childhood

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ASD

• Physical Examination
• Right ventricular lift
• Atrial level shunts result in right-sided volume
  overload
• Wide fixed S2
• Blowing SEM in pulmonic area
• Murmur due to increased flow across the pulmonary

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ASD
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ASD
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ASD

• Natural History
• Generally do well through childhood
• Major complication atrial fibrillation
• Can develop pulmonary hypertension / RV failure
  but not before third or fourth decade of life

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ASD

• Management
• Device closure around three years of age or when
  found
• Surgery for very large defects or outside fossa
  ovalis (eg. sinus venosus defect)

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ASD
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Shunt Lesions

• Ventricular Level Shunt

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VSD

• Physiology
• Left to Right shunt from high pressure left
  ventricle to low pressure right ventricle
• Loads left atrium and left ventricle (right
  ventricle may see pressure load)

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VSD

• History
• Small defects
• Presentation with murmur in newborn period
• Large defects
• Failure to thrive (6 wks to 3 months)
• Tachypnea, poor feeding, diaphoresis

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VSD

• Physical Examination
• Active left ventricle
• Small defect
• Pansystolic murmur, normal split S2
• Large defect
• SEM, narrow split S2, diastolic murmur at apex
  from high flow across mitral valve

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VSD
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VSD
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VSD

• Natural History
• Small defect
• Often close
• No real significance beyond endocarditis risk
• Large defect
• Failure to thrive
• Progression to pulmonary hypertension as early as
  1 year

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VSD

• Management
• Small defect
• Conservative management
• Large defect
• Semi-elective closure if growth failure or
  evidence of increased pulmonary hypertension
• Occasionally elective closure if persistent
  cardiomegaly beyond 3 years of age

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Shunt Lesions

• Great Artery Level Shunt

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PDA

• Physiology
• Left to Right shunt from high pressure aorta to
  low pressure pulmonary artery
• Loads left atrium and left ventricle (right
  ventricle may see pressure load)

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PDA

• History
• Premature duct
• Failure to wean from ventilator /- murmur
• Older infant
• Usually murmur from early infancy
• Occasionally signs of heart failure

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PDA

• Physical Examination
• Active left ventricle
• Hyperdynamic pulses
• Premature duct
• SEM with diastolic spill
• Older infant
• Continuous murmur

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PDA

• Management
• Premature Duct
• Trial of indomethacin
• Surgical ligation
• Older infant
• Leave until 1 year of age unless symptomatic
• Coil / device closure
• Rarely surgical ligation

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Coarctation

• Obstruction of the aortic arch
• Classically juxtaductal, although may occur
  anywhere along the aorta
• May develop over time
• Femoral pulses should be checked routinely
  throughout childhood

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Coarctation of the Aorta

• History
• Presentation varies with severity
• Severe coarctation
• Failure (shock) in early infancy
• Mild coarctation
• Murmur (in back)
• Hypertension

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Coarctation

• Physical Examination
• Absent femoral pulses
• Arm leg gradient /- hypertension
• Left ventricular tap
• Bruit over back

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Coarctation

• Management
• Newborn with CHF
• Emergency surgical repair
• Infant
• Semi-elective repair in uncontrolled hypertension
• Older child
• Balloon arterioplasty /- stenting
• Surgery on occasion
• Failure to repair prior to adolescence recipe for
  life long hypertension

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Cyanotic Congenital Heart Disease

• Blue blood (deoxygenated hemoglobin) enters the
  arterial circulation
• Systemic oxygen saturation is reduced
• Cyanosis may or may not be clinically evident
• 5g deoxygenated HgB

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Causes of Cyanosis

• Respiratory
• Cardiac
• Hematologic
• Polycythemia
• Hemoglobins with decreased affinity
• Neurologic
• Decreased Respiratory drive

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Cyanosis

• Respiratory
• Cardiac
• Hyperoxic test response to 100 O2
• Lung disease should respond to 02
• PO2 should rise to greater than 150 mmHg

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Cyanotic Congenital Heart Disease

• Increased pulmonary blood flow
• Truncus arteriosus
• Transposition of the great arteries
• Total anomolous pulmonary venous return
• Decreased pulmonary blood flow
• Tetralogy of Fallot/pulmonary atresia
• Tricuspid atresia
• Critical pulmonary stenosis

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Cyanotic Congenital Heart Disease

• ?Pulmonary Blood Flow

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Cyanotic Congenital Heart Disease

• Increased Pulmonary Blood Flow
• TGA
• TAPVD
• Truncus arteriosus

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TGA
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Normal Heart
Body
RA
RV
PA
LA
LV
AO
Lungs
Circulation is in series
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TGA

• Circulation is in parallel
• Body RA RV Ao
• Lungs LA LV PA

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TGA

• Circulation is in parallel
• Need for mixing

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TGA

• Must bring oygenated blood into the systemic
  circulation
• Great artery level shunt - PDA
• Atrial level shunt PFO
• Prostaglandin E1 (PGE)
• Re-opens and maintains patency of the ductus
  arteriosus
• Balloon atrial septostomy (BAS)
• Increase intracardiac shunting across the atrial
  septum

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TGA

• Body RA RV Ao
• PFO BAS PDA PGE
• Lungs LA LV PA

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TGA

• History
• Presentation
• Profound cyanosis shortly after birth
• Particularly with restrictive ASD and/or closure
  of the ductus arrteriosus
• Minimal or no murmur

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TGA

• Physical Examination
• Profound cyanosis
• Right ventricular tap
• Loud single S2
• Little or no murmur

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TGA

• Management
• Prostaglandins to maintain mixing
• Balloon atrial septostomy
• Arterial switch repair in first week

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Balloon Atrial Septostomy
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Total Anomalous Pulmonary Venous Return
Pulmonary veins communicate with systemic vein
Pulmonary veins fail to connect to left atrium
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Total Anomalous Pulmonary Venous Return -
Supracardiac
Pulmonary veins communicate with systemic vein
Pulmonary veins fail to connect to left atrium
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Total Anomalous Pulmonary Venous Return -
Infracardiac
Pulmonary veins fail to connect to left atrium
Pulmonary veins communicate with systemic vein
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TAPVD

• History
• Presentation depends on presence or absence of
  obstruction to venous return
• Infradiaphragmatic
• Almost always obstructed
• Cyanosis and respiratory distress shortly after
  birth
• Cardiac or supracardiac
• Rarely obstructed
• Can present like big ASD with cyanosis
• Not a PGE dependent lesion

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TAPVD

• Physical Examination
• Variable cyanosis (again depends on obstruction)
• Right ventricular tap
• Wide split S2
• Blowing systolic ejection murmur

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TAPVD
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TAPVD

• Management
• If severe cyanosis in newborn
• Emergency surgical repair
• Unobstructed
• Semi-elective surgical repair when discovered

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Truncus arteriosus

• 1. common, single outflow tract with pulmonary
  arteries originating from the ascending aorta
• 2. abnormal truncal valve
• 3. large VSD
• 4. not a PGE dependent lesion

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Cyanotic Congenital Heart Disease

• Decreased Pulmonary Blood Flow

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Cyanotic Congenital Heart Disease

• Decreased Pulmonary Blood Flow
• Tetralogy of Fallot/Pulmonary Atresia
• Tricuspid Atresia
• Critical pulmonary valve stenosis

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Cyanotic Congenital Heart Disease - ? Pulmonary
Flow

• RVOT Obstruction Shunt

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Cyanotic Congenital Heart Disease

• Tetralogy of Fallot
• 1. Pulmonary stenosis
• 2. Overriding aorta
• 3. RVH
• 4. VSD
• Generally not a PGE dependent lesion

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Tetralogy of Fallot

• History
• Presentation depends on severity of PS
• Severe stenosis
• Cyanosis shortly after birth (as duct closes)
• Mild stenosis
• May present as heart murmur (from shortly after
  birth)

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Tetralogy of Fallot

• Physical Examination
• Variable cyanosis (remember the 50g/l rule)
• Right ventricular tap
• Decreased P2 /- ejection click
• Tearing/harsh SEM

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Tetralogy of Fallot

• Management
• Outside the newborn period, surgical repair if
  symptomatic
• Elective repair at 6 months
• Role for beta blockers to palliate hypercyanotic
  spells

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Tetralogy of Fallot

• Hypercyanotic Spells (Tet Spells)
• Episodes of profound cyanosis
• Most frequently after waking up or exercise

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Tetralogy of Fallot

• Hypercyanotic Spells (Tet Spells)

Stress leading to fall in P02
Tachycardia and Hyperventilation
Increased R to L shunt
Increased Return of deeply desaturated venous
blood
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Tetralogy of Fallot

• Hypercyanotic Spells (Tet Spells
• Treatment
• Tuck knees to chest
• Reduces venous return by compressing femoral
  veins
• Increases systemic vascular resistance
• In hospital
• O2
• Phenylephrine
• Morphine
• IV beta blocker

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Tetralogy of Fallot
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Tetralogy of Fallot

• Decreased Pulmonary Blood Flow

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Pulmonary atresia/VSD

• Tetralogy of Fallot with atretic pulmonary valve
• Variable pulmonary artery anatomy
• Generally a PGE dependent lesion

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Critical pulmonary stenosis

• Severe pulmonary stenosis with inadequate
  pulmonary flow
• Pulmonary atresia/intact ventricular septum
• PGE dependent lesion

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Tricuspid atresia

• 1. tricuspid atresia
• 2. severely hypoplastic RV
• 3. VSD
• 4. ASD large
• 5. pulmonary stenosis
• Variable
• Generally a PGE dependent lesion

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Cyanotic Heart Disease

• Decreased blood flow due to RVOT obstruction may
  require augmentation of pulmonary blood flow via
  creation of a surgical systemic to pulmonary
  shunt
• Blalock-Taussig Shunt (BTS)

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Management of cyanotic HD

• BTS

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Duct Dependent Congenital Heart Disease

• Which of the following are examples of duct
  dependent CHD?
• Pulmonary atresia
• Patent ductus arteriosus
• Transposition of the great arteries

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Critical Left-Sided Obstruction

• Neonatal presentation
• Coarctation
• Critical aortic stenosis
• Hypoplastic left heart syndrome
• Cardiogenic shock
• PGE dependent lesion

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Left-sided Obstruction

• Coarctation of the aorta
• Critical narrowing of the juxtaductal aorta
• Blood cannot get past the obstruction

SHOCK
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Coarctation

• Characterized by weak or absent pulses
  particularly in the lower limbs
• Initiation of PGE lifesaving
• splitting of saturations seen in critical
  narrowings with patency of ductus arteriosus ie
  normal saturation in right arm and lower
  saturation in the lower limbs due to right to
  left shunting across the PDA

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Coarctation - treatment

• Surgical correction following initiation of PGE
  and stabilization

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Left-Sided Obstruction

• Critical Aortic Stenosis
• CRITICAL
• Inadequate forward
• flow to maintain
• cardiac output

SHOCK
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Critical Aortic Stenosis

• Management
• Prostaglandins to provide source of systemic
  blood flow
• Balloon valvuloplasty
• Rarely surgery

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Left Ventricular Outflow Tract Obstruction

• Hypoplastic Left Heart Syndrome (HLHS)
• 1. Mitral atresia
• 2. Aortic atresia
• 3. Hypoplastic left ventricle
• 4. Hypoplastic ascending aorta
• PDA is the only source of systemic blood flow
• PGE dependent lesion

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HLHS

• Initially cyanotic
• With closure of the PDA SHOCK
• Tachycardia, tachypnea, low blood pressure, weak
  pulses, poor perfusion, cyanotic/grey colour
• PGE

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Hypoplastic left heart

• Management
• Prostaglandins
• Norwood procedure
• Heart Transplant

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Overview

• Pink (acyanotic)
• ASD, VDS, PDA
• Blue (cyanotic)
• In order of most common TOF, TGA, Truncus
  arteriosus, Tricuspid atresia, TAPVD
• Critical outflow tract obstruction
• HLHS, coarctation

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Kawasaki Syndrome

• Small artery arteritis
• Coronary arteries most seriously effected
• Dilatation/aneurysms progressing to (normal)
  stenosis

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Kawasaki Syndrome

• 5 days of fever plus 4 of
• Rash
• Cervical lymphadenopathy (at least 1.5 cm in
  diameter)
• Bilateral conjuctival injection
• Oral mucosal changes
• Peripheral extremity changes
• Swelling
• Peeling (often late)

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Kawasaki Syndrome

• Associated Findings
• Sterile pyuria
• Hydrops of the gallbladder
• Irritability

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Kawasaki Syndrome

• Epidemiology
• Generally children lt 5 years
• Male gt Female
• Asian gt Black gt White

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Kawasaki Syndrome

• Management
• Gamma globulin 2g/kg
• 80 mg/kg ASA until afebrile then 5 mg/kg for 6
  weeks
• Aneurysm in 18 of untreated patients
• 4-8 if treated with high dose gammaglobulin
  and ASA
• Mortality 0.1

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Innocent Murmurs

• Characteristics
• Always Grade III or less
• Always systolic (occasionally continuous)
• Blowing or musical quality
• Not best heard in back

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Innocent Murmurs

• Types
• Stills
• Vibratory SEM best heard mid-left sternal border
• Pulmonary Flow murmur
• Blowing SEM best heard in PA
• Venous Hum
• peripheral pulmonary artery stenosis (PPS)
• Blowing SEM best heard in PA radiating out to
  both axillae
• Continuous murmur best heard in R infraclavicular
• Decreases lying flat or with occlusion of neck
  veins
• carotid Bruit
• Short systolic murmur heart supraclavicularly
  secondary to flow from the Ao to the head and
  neck vessels

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Pediatric ECGs

• Brief review of pediatric ECGs
• Physiologic reasons for differences

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Pediatric ECGs

• Electrical activation is the same as in adults
• Electrodes are placed in the same position
• Extra leads used in pediatric ECGs V3R, V4R, V7

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Pediatric ECGs

• ECG differences compared to adults
• Gestational Age ratio LV/RV mass
• Birth 0.81
• 1 month 1.51
• 6 months 2.01
• Adult 2.51

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Pediatric ECGs

• P wave
• Amplitude
• lt2.5 mm all age

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Pediatric ECGs

• QRS Morphology
• Axis
• progressive leftward axis with increasing age
• Morphology
• age dependent
• lt 80 msec lt 3 years
• lt 90 msecs lt 18 years
• Voltage
• age dependent
• small variability seen with sex.

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Pediatric ECGs

• T wave in V1
• Subject of confusion
• Upright at birth
• Normally inverts between 3-7 days of life
• Becomes upright again during adolecscence.

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• Heart Rate
• 0-1 m onth 120/min
• 10 years 100/min
• gt16 years 70/min
• QRS Axis
• 0-1 month 180-70 (120)
• 1 year 35-30 (60)
• gt16 year 110-(-)15 (60)

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Pediatric ECGs

• English
• http//medstat.med.utah.edu/kw/ecg/intro.html
• Français
• http//www.cardioped.org/abrege/notion.htm

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Questions?