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Transcript heart and circulation sdg
Paediatric Cardiology:
Congenital Heart
Disease and Clinical
Problems
Dr. Suzie Lee
Pediatric Cardiologist
Assistant Professor, University of Ottawa
Objectives
Review
of fetal circulation and the
transitional circulation
To provide an outline of congenital heart
disease
List criteria for Kawasaki syndrome
Describe the common innocent murmurs
of childhood
Review of pediatric ECGs
Fetal Circulation
Basic
units:
• fetal heart
• placenta
• connections between placenta and fetal
heart
• umbilical vein and arteries
Fetal Circulation - The
Placenta
Maternal
to placenta via endometrial arteries
(oxygenated)
from placenta via endometrial veins
Fetal
blood:
blood
from placenta via umbilical vein (oxygenated)
to placenta via 2 umbilical arteries
(deoxygenated)
The fetal circulation
Fetal Circulation
Right Ventricle:
• lungs - 10-15%
• placenta - 50%
• lower body 35-40%
Left Ventricle:
• brain
• upper extr,
head and neck
The post-natal circulation
Closure of
ductus
arteriosus
Closure of
foramen
ovale
Loss of
umbilical
flows
Postnatal changes in PVR
Immediate
- abrupt decrease in PVR to
levels less than systemic
24 hours - PVR ~ 50% systemic
6 weeks - PVR < 30% systemic (adult
levels)
An Outline of Congenital Heart
Disease
Pink
(Acyanotic)
Blue
(Cyanotic)
Critical
outflow tract
obstruction
Acyanotic Congenital Heart
Disease
Normal
Pulmonary Blood Flow
↑ Pulmonary Blood Flow
Acyanotic Congenital Heart
Disease
Normal
Pulmonary Blood Flow
Valve Lesions
• Not fundamentally different from
adults
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
Physical Examination
Absent/weak femoral pulses
Arm leg gradient +/- hypertension
Left ventricular tap/heave
Bruit over back
Coarctation
Management
Newborn with CHF
Infant
Semi-elective repair in uncontrolled hypertension
Older child
Emergency surgical repair
Balloon arterioplasty +/- stenting
Surgery on occasion
Failure to repair prior to adolescence may lead to
life long hypertension
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
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
ASD
ASD
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 with murmur in newborn period
Large defects
Failure to thrive (6 wks to 3 months)
• Tachypnea, poor feeding, diaphoresis
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
VSD
VSD
VSD
Management
Small defect
Conservative management
Large defect
Semi-elective closure if growth failure or evidence of
increased pulmonary pressures
Occasionally elective closure if persistent
cardiomegaly beyond 3 years of age
VSD
VSD
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
Physical Examination
Active left ventricle
Hyperdynamic pulses
Premature duct
SEM with diastolic spill
Older infant
Continuous murmur
PDA
Management
Premature Duct
Trial of indomethacin
Surgical ligation
Older infant
Leave until at least 1 year of age unless symptomatic
Coil / device closure
Rarely surgical ligation
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
Cyanosis
Cardiac
Hyperoxic test – response to 100% O2
• Lung disease should respond to O2
• PO2 should rise to greater than 150 mmHg
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 Disease
↑Pulmonary
Blood Flow
d-Transposition
Normal Heart
Body
RA
RV
PA
AO
LV
LA
Lungs
Circulation is in “series”
d-Transposition
Circulation
is in “parallel”
Body
RA
RV
Ao
Lungs
LA
LV
PA
d-Transposition
Circulation is in
“parallel”
Need for mixing
TGA
Must
bring oygenated blood into the systemic
circulation
Great artery level shunt - PDA
Atrial level shunt – PFO
Prostaglandin
Re-opens and maintains patency of the ductus
arteriosus
Balloon
E1 (PGE)
atrial septostomy (BAS)
Increase intracardiac shunting across the atrial septum
d-Transposition
Body
RA
PFO BAS
RV
Lungs
LV
LA
Ao
PDA PGE
PA
Transposition
History
Presentation
Cyanosis shortly after birth
• Particularly with restrictive ASD and/or
closure of the ductus arrteriosus
Minimal or no murmur
Physical Examination
Significant cyanosis
Right ventricular “tap”
Loud single S2
Little or no murmur
TGA
Management
Prostaglandins to maintain PDA
Balloon atrial septostomy to improve mixing
Arterial switch repair in first week
Balloon Atrial Septostomy
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
Cardiac or supracardiac
• Rarely obstructed
• Can present like big ASD with cyanosis
Infradiaphragmatic
• Almost always obstructed
• Cyanosis and respiratory distress shortly after birth
Not
a PGE dependent lesion
TAPVD
TAPVD
Management
If severe cyanosis in newborn – ie obstructed
Emergency surgical repair
Unobstructed
Semi-elective surgical repair when discovered with
medical treatment of CHF
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
Disease
Decreased
Pulmonary Blood Flow
Cyanotic Congenital Heart
Disease - ↓ Pulmonary Flow
= RVOT
Obstruction +
Shunt
Cyanotic Congenital Heart
Disease
Tetralogy of Fallot
1.
2.
3.
4.
Pulmonary stenosis
Overriding aorta
RVH
VSD
Generally not a PGE
dependent lesion
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
Physical Examination
Variable cyanosis
Right ventricular “tap”
Decreased P2 +/- ejection click
“Tearing”/harsh 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
Episodes of profound cyanosis
Most frequently after waking up or exercise
Stress leading to
catecholamine release, O2
consumption and fall in P02
Increased R to L shunt
with fall in SVR and
reduced RV preload
Tachycardia and
Hyperventilation
reduced mixed venous O2 content
Tetralogy of Fallot
Hypercyanotic Spells (“Tet” Spells
Treatment
Tuck knees to chest
• Reduces systemic venous return by compressing
femoral veins
• Increases systemic vascular resistance
Oral betablockers
In hospital
•
•
•
•
O2
Morphine
IV beta blocker
Phenylephrine
Tetralogy of Fallot
Tetralogy of Fallot
Decreased
Pulmonary Blood Flow
Pulmonary atresia/VSD
Tetralogy
of Fallot
with atretic pulmonary
valve
Variable pulmonary
artery anatomy
Generally
a PGE
dependent lesion
Critical pulmonary stenosis
Severe
pulmonary
stenosis with inadequate
pulmonary flow
Pulmonary atresia/intact
ventricular septum
Cyanotic due to R-L
shunting at atrial level
PGE
dependent lesion
Tricuspid atresia
1.
2.
3.
4.
5.
tricuspid atresia
severely hypoplastic RV
VSD
ASD – large
pulmonary stenosis
Variable
Generally
a PGE
dependent lesion
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)
Case 1 (continued)
BTS
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
Critical Left-Sided
Obstruction
Neonatal
presentation
Coarctation
Critical aortic stenosis
Hypoplastic left heart syndrome
Cardiogenic
shock
PGE dependent lesion
Left-sided Obstruction
Coarctation
of
the aorta
Critical narrowing
of the
“juxtaductal” aorta
Blood cannot get
past the
obstruction
SHOCK
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
Surgical
correction following initiation of
PGE and stabilization
Left-Sided Obstruction
Critical
Aortic
Stenosis
CRITICAL
Inadequate forward
flow to maintain
cardiac output
SHOCK
Critical Aortic Stenosis
Management
Prostaglandins to provide source of systemic blood
flow
Balloon valvuloplasty
Rarely surgery
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
Hypoplastic left heart
Management
Prostaglandins
Norwood procedure
Heart Transplant
HLHS
Initially
cyanotic
With closure of the PDA
SHOCK
Tachycardia, tachypnea, low blood pressure, weak
pulses, poor perfusion, cyanotic/grey colour
PGE
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 Findings
Sterile 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
Aneurysm in ~18% of untreated patients
~4-8 % if treated with high dose gammaglobulin
and ASA
Mortality
~0.1%
Innocent Murmurs
Characteristics
Always Grade III or less
Always systolic (rarely continuous)
Blowing or musical quality
Not best heard in back
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 with occlusion of neck veins
Physiologic peripheral pulmonary artery stenosis
• Blowing SEM best heard in PA radiating out to both axillae
Aortic Bruit
• Short systolic murmur heart supraclavicularly secondary to flow
from the Ao to the head and neck vessels
Pediatric ECGs
Brief
review of pediatric ECGs
Physiologic
reasons for differences
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
Pediatric ECGs
ECG differences compared to adults
Gestational Age
Birth
1 month
6 months
Adult
ratio LV/RV mass
0.8:1
1.5:1
2.0:1
2.5:1
Pediatric ECGs
P wave
Amplitude
<2.5 mm
all age
Pediatric ECGs
QRS
Morphology
Axis
progressive leftward axis with increasing age
Morphology
age dependent
< 80 msec < 3 years
< 90 msecs < 18 years
Voltage
age dependent
small variability seen with sex.
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.
Heart Rate
0-1 month 120/min
10 years 100/min
>16 years 70/min
QRS Axis
0-1 month 180-70 (120)
1 year
35-30 (60)
>16 year 110-(-)15 (60)
Pediatric ECGs
English
http://pediatriccardiology.uchicago.edu/MP/ECG/E
CG2.htm
Français:
http://www.cardioped.org/abrege/notion.htm
Questions?
Coarctation of the Aorta
History
Presentation varies with severity
Severe coarctation
• Failure (shock) in early infancy
Milder coarctation
• Murmur (in back)
• Upper limb hypertension
• Weak pulses