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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