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Paediatric Cardiology: Congenital Heart Disease and Clinical Problems. Dr. Suzie Lee Pediatric Cardiologist Assistant Professor, University of Ottawa. Objectives. To provide an outline of congenital heart disease List criteria for Kawasaki syndrome

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paediatric cardiology congenital heart disease and clinical problems

Paediatric Cardiology:Congenital Heart Disease and Clinical Problems

Dr. Suzie Lee

Pediatric Cardiologist

Assistant Professor, University of Ottawa

objectives
Objectives
  • To provide an outline of congenital heart disease
  • List criteria for Kawasaki syndrome
  • Describe the common innocent murmurs of childhood
an outline of congenital heart disease
An Outline of Congenital Heart Disease
  • Pink (Acyanotic)
  • Blue (Cyanotic)
  • Critical outflow tract obstruction
acyanotic congenital heart disease
Acyanotic Congenital Heart Disease
  • Normal Pulmonary Blood Flow
  • ↑Pulmonary Blood Flow
acyanotic congenital heart disease1
Acyanotic Congenital Heart Disease
  • Normal Pulmonary Blood Flow
    • Valve Lesions
      • Not fundamentally different from adults
acyanotic congenital heart disease2
Acyanotic Congenital Heart Disease
  • ↑Pulmonary Blood Flow
shunt lesions
Shunt Lesions

Atrial Level Shunt

slide8
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
slide9
ASD

History

  • Usually asymptomatic in childhood
    • Occasionally frequent respiratory tract infections
  • Presentation with murmur in childhood
slide10
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
slide13
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
slide14
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)
shunt lesions1
Shunt Lesions

Ventricular Level Shunt

slide19
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)
slide20
VSD

History

  • Small defects
    • Presentation with murmur in newborn period
  • Large defects
    • Failure to thrive (6 wks to 3 months)
      • Tachypnea, poor feeding, diaphoresis
slide21
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
slide24
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
slide25
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
shunt lesions2
Shunt Lesions

Great Artery Level Shunt

slide27
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)
slide28
PDA

History

  • Premature duct
    • Failure to wean from ventilator +/- murmur
  • Older infant
    • Usually murmur from early infancy
    • Occasionally signs of heart failure
slide29
PDA

Physical Examination

  • Active left ventricle
  • Hyperdynamic pulses
  • Premature duct
    • SEM with diastolic spill
  • Older infant
    • Continuous murmur
slide30
PDA

Management

  • Premature Duct
    • Trial of indomethacin
    • Surgical ligation
  • Older infant
    • Leave till 1 year of age unless symptomatic
    • Coil / device closure
    • Rarely surgical ligation
coarctation
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
coarctation of the aorta
Coarctation of the Aorta

History

  • Presentation varies with severity
    • Severe coarct
      • Failure (shock) in early infancy
    • Mild coarct
      • Murmur (in back)
      • Hypertension
coarctation1
Coarctation

Physical Examination

  • Absent femoral pulses
  • Arm leg gradient +/- hypertension
  • Left ventricular “tap”
  • Bruit over back
coarctation2
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
cyanotic congenital heart disease
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
causes of cyanosis
Causes of Cyanosis
  • Respiratory
  • Cardiac
  • Hematologic
    • Polycythemia
    • Hemoglobins with decreased affinity
  • Neurologic
    • Decreased Respiratory drive
cyanosis
Cyanosis
  • Respiratory
  • Cardiac
    • Hyperoxic test – response to 100% O2
      • Lung disease should respond to 02
      • PO2 should rise to greater than 150 mmHg
cyanotic congenital heart disease1
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
cyanotic congenital heart disease2
Cyanotic Congenital Heart Disease

↑Pulmonary Blood Flow

normal heart
Normal Heart

Body

RA

RV

PA

AO

LV

LA

Lungs

Circulation is in “series”

d transposition1
d-Transposition

Circulation is in “parallel”

Body RA RV Ao

Lungs LA LV PA

d transposition2
d-Transposition
  • Circulation is in “parallel”
  • Need for mixing
slide46
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

d transposition3
d-Transposition

Body RA RV Ao

PFO BAS PDA PGE

Lungs LA LV PA

transposition
Transposition

History

  • Presentation
    • Profound cyanosis shortly after birth (as duct closes)
    • Minimal or no murmur
slide49
TGA

Physical Examination

  • Profound cyanosis
  • Right ventricular “tap”
  • Loud single S2
  • Little or no murmur
slide50
TGA

Management

  • Prostaglandins to maintain mixing
  • Balloon atrial septostomy
  • Arterial switch repair in first week
total anomalous pulmonary venous return
Total Anomalous Pulmonary Venous Return

Pulmonary veins communicate with systemic vein

Pulmonary veins fail to connect to left atrium

total anomalous pulmonary venous return supracardiac
Total Anomalous Pulmonary Venous Return - Supracardiac

Pulmonary veins communicate with systemic vein

Pulmonary veins fail to connect to left atrium

total anomalous pulmonary venous return infracardiac
Total Anomalous Pulmonary Venous Return - Infracardiac

Pulmonary veins fail to connect to left atrium

Pulmonary veins communicate with systemic vein

tapvd
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
tapvd1
TAPVD

Physical Examination

  • Variable cyanosis (again depends on obstruction)
  • Right ventricular “tap”
  • Wide split S2
  • Blowing systolic ejection murmur
tapvd3
TAPVD

Management

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

cyanotic congenital heart disease3
Cyanotic Congenital Heart Disease
  • Decreased Pulmonary Blood Flow
cyanotic congenital heart disease4
Cyanotic Congenital Heart Disease

Tetralogy of Fallot

1. Pulmonary stenosis

2. Overriding aorta

3. RVH

4. VSD

Generally not a PGE dependent lesion

tetralogy of fallot
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)
tetralogy of fallot1
Tetralogy of Fallot

Physical Examination

  • Variable cyanosis (remember the 50g/l rule)
  • Right ventricular “tap”
  • Decreased P2 +/- ejection click
  • “Tearing”/harsh SEM
tetralogy of fallot2
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
tetralogy of fallot3
Tetralogy of Fallot

Hypercyanotic Spells (“Tet” Spells)

  • Episodes of profound cyanosis
  • Most frequently after waking up or exercise
tetralogy of fallot4
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

tetralogy of fallot5
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
tetralogy of fallot7
Tetralogy of Fallot
  • Decreased Pulmonary Blood Flow
pulmonary atresia vsd
Pulmonary atresia/VSD

Tetralogy of Fallot with atretic pulmonary valve

Variable pulmonary artery anatomy

Generally a PGE dependent lesion

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

1. tricuspid atresia

2. severely hypoplastic RV

3. VSD

4. ASD – large

5. pulmonary stenosis

Variable

Generally a PGE dependent lesion

cyanotic heart disease
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)

duct dependent congenital heart disease
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
critical left sided obstruction
Critical Left-Sided Obstruction
  • Neonatal presentation
    • Coarctation
    • Critical aortic stenosis
    • Hypoplastic left heart syndrome
  • Cardiogenic shock
    • PGE dependent lesion
left sided obstruction
Left-sided Obstruction

Coarctation of the aorta

Critical narrowing of the “juxtaductal” aorta

Blood cannot get past the obstruction

SHOCK

coarctation3
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
coarctation treatment
Coarctation - treatment

Surgical correction following initiation of PGE and stabilization

left sided obstruction1
Left-Sided Obstruction

Critical Aortic Stenosis

CRITICAL

Inadequate forward

flow to maintain

cardiac output

SHOCK

critical aortic stenosis
Critical Aortic Stenosis

Management

  • Prostaglandins to provide source of systemic blood flow
  • Balloon valvuloplasty
  • Rarely surgery
left ventricular outflow tract obstruction
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

slide88
HLHS
  • Initially cyanotic
  • With closure of the PDA SHOCK
    • Tachycardia, tachypnea, low blood pressure, weak pulses, poor perfusion, cyanotic/grey colour
  • PGE
hypoplastic left heart
Hypoplastic left heart

Management

  • Prostaglandins
  • Norwood procedure
  • Heart Transplant
kawasaki syndrome
Kawasaki Syndrome
  • Small artery arteritis
    • Coronary arteries most seriously effected
    • Dilatation/aneurysms progressing to (normal) stenosis
kawasaki syndrome1
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)
kawasaki syndrome2
Kawasaki Syndrome
  • Associated Findings
    • Sterile pyuria
    • Hydrops of the gallbladder
    • Irritability
kawasaki syndrome3
Kawasaki Syndrome
  • Epidemiology
    • Generally children < 5 years
    • Male > Female
    • Asian > Black > White
kawasaki syndrome4
Kawasaki Syndrome
  • Management
    • Gamma globulin 2g/kg
    • 80 mg/kg ASA until afebrile then 5 mg/kg for 6 weeks
innocent murmurs
Innocent Murmurs
  • Characteristics
    • Always Grade III or less
    • Always systolic (occasionally continuous)
    • Blowing or musical quality
    • Not best heard in back
innocent murmurs1
Innocent Murmurs
  • Types
    • Still’s
      • Vibratory SEM best heard mid-left sternal border
    • Pulmonary Flow murmur
      • Blowing SEM best heard in PA
    • Venous Hum
      • Continuous murmur best heard in R infraclavicular
      • Decreases lying flat or occlusion of neck veins
    • Physiologic peripheral pulmonary artery stenosis
      • Blowing SEM best heard in PA radiating out to both axillae
ad