Transcript Paediatric Cardiology - Dr. Herchel Rosenberg

Paediatric Cardiology:
An Outline of Congenital Heart
Disease
Dr. H.C. Rosenberg
[email protected]
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
Pink
(Acyanotic)
Blue
(Cyanotic)
Resistance=
?
Acyanotic Congenital Heart
Disease
Normal
Pulmonary Blood Flow
↑ Pulmonary Blood Flow
Acyanotic Congenital Heart
Disease
Normal
Valve
Not
Pulmonary Blood Flow
Lesions
fundamentally different from
adults
Acyanotic Congenital Heart
Disease
↑
Pulmonary Blood Flow
Shunt Lesions
Atrial Level Shunt
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
 Low resistance
ASD
History
 Usually
asymptomatic in childhood
 Occasionally
 Presentation
frequent respiratory tract infections
with murmur as pre-schooler or older
ASD
Physical Examination
 Right
ventricular “lift”
 Wide fixed S2
 Blowing SEM in pulmonic area
ASD
ASD
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
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)
ASD
Shunt Lesions
Ventricular Level Shunt
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)
VSD
History
 Small
defects
 Presentation
 Large
with murmur in newborn period
defects
 Failure
to thrive (6 wks to 3 months)
 Tachypnea, poor feeding, diaphoresis
VSD
Physical Examination
 Active
left ventricle
 Small defect
 Pansystolic
 Large
murmur, normal split S2
defect
 SEM,
narrow split S2, diastolic murmur at apex from
high flow across mitral valve
VSD
BVH
VSD
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
VSD
Management
 Small
defect
 Large defect
 Semi-elective
closure if growth failure or evidence of
increased pulmonary hypertension
 Occasionally elective closure if persistent
cardiomegally beyond 3 years of age
Shunt Lesions
Great Artery Level Shunt
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)
PDA
History
 Premature
 Failure
 Older
duct
to wean from ventilator +/- murmur
infant
 Usually
murmur from early infancy
 Occasionally signs of heart failure
PDA
Physical Examination
 Active
left ventricle
 Hyperdynamic pulses
 Premature duct
 SEM
 Older
with diastolic spill
infant
 Continuous
murmur
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
Truncus Arterisosus
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
Causes of Cyanosis
Respiratory
Cardiac
 Hematologic
 Polycythemia
 Hemoglobins
with decreased affinity
 Neurologic
 Decreased
Respiratory drive
Cyanosis
Respiratory
Cardiac
Hyperoxic
Place
test
infant in 100% 02
 Lung
disease should respond to 02
 Failure of saturation to rise to > 85% suggest
cardiac disease
Cyanotic Congenital
Heart Disease
↓Pulmonary
Blood Flow
↑Pulmonary Blood Flow
Cyanotic Congenital Heart
Disease
 Decreased
Pulmonary Blood Flow
Cyanotic Congenital Heart
Disease - ↓ Pulmonary Flow
= RVOT
Obstruction
+ Shunt
Tetralogy of Fallot
VSD
Over-riding aorta
Pulmonary stenosis
RVH

Tetralogy of Fallot
History
 Presentation
 Severe
depends on severity of PS
stenosis
 Cyanosis shortly after birth (as duct closes)
 Mild stenosis
 May present as heart murmur (from shortly after
birth)
Tetralogy of Fallot
Physical Examination
 Variable
cyanosis (remember the 50g/l rule)
 Right ventricular “tap”
 Decreased P2 +/- ejection click
 “Tearing” SEM
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 Fallot
Hypercyanotic Spells (“Tet” Spells)
 Episodes
of profound cyanosis
 Most frequently after waking up or exercise
Tetralogy of Fallot
Hypercyanotic Spells (“Tet” Spells)
Fall in P02
Increased R to L
shunt
Hyperventilation
Increased Return of
deeply desaturated
venous blood
Tetralogy of Fallot
Hypercyanotic Spells (“Tet” Spells
 Treatment
 Tuck
knees to chest (pinches off femoral veins)
 In hospital
O2
 Bicarbonate
 Phenylephrine
 Morphine
 IV beta blocker

Tetralogy of Fallot
Tetralogy of Fallot
 Decreased
Pulmonary Blood Flow
Duct Dependent Congenital
Heart Disease

1.
2.
3.
Which of the following are
examples of duct dependent
CHD?
Pulmonary atresia
Patent ductus arteriosus
Transposition of the great
arteries
Cyanotic Congenital Heart
Disease With ↑Pulmonary
Blood Flow
Cyanotic Congenital Heart
Disease With ↑Pulmonary
Blood Flow
 Transposition
of the great arteries
 Total anomalous pulmonary venous
drainage
d-Transposition
Normal Heart
Body
RA
RV
PA
AO
LV
LA
Lungs
Circulation is in “series”
d-Transposition
 Circulation
is in
“parallel”
 Need for mixing
Transposition
History
 Presentation
 Profound
cyanosis shortly after birth (as duct
closes)
 Minimal or no murmur
Tetralogy of Fallot
Physical Examination
 Profound
cyanosis
 Right ventricular “tap”
 Loud single S2
 Little or no murmur
Tetralogy of Fallot
Management



Prostaglandins to maintain mixing
Balloon atrial septostomy
Arterial switch repair in first week
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
Pulmonary veins
communicate with
systemic vein
Pulmonary veins
fail to connect to
left atrium
Total Anomalous Pulmonary
Venous Return - Infracardiac
Pulmonary veins
fail to connect to
left atrium
Pulmonary veins
communicate with
systemic vein
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
TAPVD
Physical Examination
 Variable
cyanosis (again depends on obstruction)
 Right ventricular “tap”
 Wide split S2
 Blowing systolic ejection murmur
TAPVD
TAPVD
Management

If severe cyanosis in newborn


Emergency surgical repair
Unobstructed

Semi-elective surgical repair when discovered
Coarctation of the aorta
Coarctation of the Aorta
History
 Presentation
varies with severity
 Severe coarct
 Failure
 Mild
(shock) in early infancy
coarct
 Murmur
(in back)
 Hypertension
Coarctation
Physical Examination
 Absent
femoral pulses
 Arm leg gradient +/- hypertension
 Left ventricular “tap”
 Bruit over back
Coarctation
Management

Newborn with CHF


Infant


Emergency surgical repair
Semi-elective repair in uncontrolled hypertension
Older child


Balloon arterioplasty
Surgery on occasion
Failure
to repair prior to
adolescence recipe for
life long hypertension!
“Grey” Heart Disease
Critical
LVOT obstruction
Left Ventricular Outflow Tract
Obstruction
 Critical
Aortic
Stenosis
 “Critical”
shock
Critical Aortic Stenosis
Management



Prostaglandins to provide source of systemic blood
flow
Balloon valvuloplasty
Rarely surgery
Hypoplastic Left Heart
Syndrome
 “Duct
dependent “
congenital heart
disease
 Ductus arteriosus is
the only source of
systemic blood flow
Hypoplastic left heart
Management


Prostaglandins
Norwood procedure
Kawasaki Syndrome
 Small
artery arteritis
 Coronary
arteries most seriously effected
 Dilatation/aneurysms progressing to (normal)
stenosis
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 Syndrome
 Associated
 Sterile
Findings
pyuria
 Hydrops of the gallbladder
 Irritability!!!
Kawasaki Syndrome
 Epidemiology
 Generally
children < 5 years
 Male > Female
 Asian > Black > White
Kawasaki Syndrome
 Management
 Gamma
globulin 2g/kg
 80 mg/kg ASA until afebrile then 5 mg/kg for 6
weeks
Innocent Murmurs
 Characteristics
 Always
Grade III or less
 Always systolic (or continuous)
 Blowing or musical quality
 Not best heard in back
Innocent Murmurs

Types

Still’s


Pulmonary Flow murmur


Blowing SEM best heard in PA
Venous Hum



Vibratory SEM best heard mid-left sternal border
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
Questions?