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Congenital Heart Defects
Craig T. Carter, D.O.
Assistant Professor – EM/Peds
University of Kentucky
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A nurse approaches you -
“Doctor, Doctor” - a kid just checked in
with a history of Hypoplastic Left Heart
syndrome – What is that again??”
You reply …..
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“ I need to go to the bathroom, as
soon as I get back, I will tell you all
about it....”
So you sit and think…..
And think some more….
Back to Basics-Fetal Circulation
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Four shunts of blood flow : placenta,
ductus venosus, foramen ovale and
ductus arteriosus
Back to Basics-Fetal Circulation
Changes after birth:
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Shift of blood flow for gas exchange,
from the placenta to the lungs.
Closure of ductus venosus :no placenta
increase in pulmonary blood flow
functional closure of foramen ovale
(increased LA pressure)
Closure of ductus arteriosus (^O2)
Newborn Cardiac Exam
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Vital signs: RR, HR, BP
respiratory effort
color
palpate point of maximal impulse (PMI)
of heart
palpate pulses of all extremities
auscultate
Case 1: 2 day old infant
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2 day old baby girl is being examined
for discharge physical.
Vitals: HR:120 RR:40 BP: r arm 75/40
Gen : low hairline, webbed neck,
edemetous dorsum of feet
Case 1: cardiac exam
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Normal PMI, no thrill
No hepatosplenomegally
unable to palpate pulses in lower
extremities
BP’s all four extremities: both legs
systolic BP lower 40/30
Case 1: Auscultation
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S2 splits normally
ejection click
systolic ejection murmur III/VI at the
URSB and LLSB
systolic murmur radiates to the back
early diastolic decrescendo murmur at
3LICS
Case 1:
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What do you think is the congenital
defect?
Case 1:
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What further evaluation do you need?
Coarctation of Aorta (COA)
Coarctation of Aorta (COA)
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Incidence: 8% of all
congenital heart
defects
Turner’s syndrome:
30% have COA
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Preductal: 40%
associated with
other cardiac
defects,symptomatic
early in life
Postductal: less
likely to have
symptoms early
COA
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EKG: LVH (but may
be normal in 20%)
X-rays: heart size
may be normal or
slightly enlarged
Rib notching in older
children
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Echo: can see
narrowing
Bicuspid aortic valve
Doppler:disturbed
flow
COA complications
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COA can cause CHF, HTN (intracranial
bleeding,hypertensive encephalopathy)
Bicuspid aortic valve: stenosis or regurg
LV failure
infective endocarditis
Preductal COA in Newborn
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80% of infants with preductal COA
develop CHF by 3 months of age!
Symptoms of CHF: poor feeding, poor
weight gain, dyspnea in first 2-6 weeks
of life.
Case 2: Two week old with
murmur
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Two week old infant, who you saw as a
newborn with normal exam, now is
noted to have a heart murmur.
PMI LSB, not hyperdynamic
pulses equal all extremities
No HSM
Case 2: Cardiac exam
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Grade III/VI holosystolic murmur at
LLSB
Case 2: What would you like to
do next?
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EKG: LVH,LAH
Blood pressures all four extremities
Xray: look for cardiomegally and
increase in pulmonary vascularity
oxygenation
Echo: VSD
Ventricular Septal Defect (VSD)
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The most common form of congenital
heart disease: 20-25%
may be located in different anatomical
locations
may be associated with many other
cardiac defects ( in many cases
essential for survival)
may be small or large (can cause CHF)
Case 3: One day old infant
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One day old infant with tachypnea and
cyanosis
Gen: cyanotic
RR:65, HR 140, BP 40/20
pulse ox on RA: less than 80
Case 3: Respiratory and Cardiac
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Lungs clear, no retractions, RR rapid
Cardiac: PMI at LSB
pulses palpable all extremities
S2 single and loud. No heart murmur.
What would you like to do?
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ABG: before oxygenation
Give 100% O2 and then repeat ABG
EKG, 4 extremity BP
CXR
Results of ABG
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Before O2:
Pa O2 40
PH: 7.15,PaCO2 30 and
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After 100% O2: PH: 7.12, PaCO2 25
and PaO2 50
Transposition of Great Arteries
Transposition of Great Arteries
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5% of all congenital heart defects
Aorta arises anteriorly from RV, PA
arises posteriorly from LV
defects (VSD,ASD,PDA) that permit
mixing of the two circulations are
necessary for survival
TGA:
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ABG:hypoxemia is
unresponsive to O2
EKG: RVH
X-rays: egg-on-astring silhouette
cardiomegally with
increased
pulmonary
vascularity
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Echo: PA from LV
associated
anomalies:VSD,ASD
,PDA
Immediate Treatment
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Prostaglandin E1 to reopen PDA
Oxygen
cardiology/surgery referral
DDX of Cyanotic Heart Dz
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Transposition of the Great Arteries
Tetralogy of Fallot
Total Anomalous Pulmonary Venous
Return
Tricuspid Atresia
Pulmonary Atresia
Truncus Arteriosus
Other congenital cyanotic defects
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Ebstein’s anomaly
single ventricle
Double-outlet right ventricle (depends
on associated defects…if cyanotic or
not)
Tetralogy of Fallot
Tetralogy of Fallot (TOF)
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Large VSD
RV outflow obstruction
right ventricular hypertrophy
overriding of the aorta
Tetralogy of Fallot
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10% of all congenital heart defects
The MOST COMMON CYANOTIC
cardiac defect beyond infancy
TOF: physical exam
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Varying degrees of cyanosis and
clubbing
systolic thrill LSB
S2 single with ejection click
loud III-V/VI SEM LSB
continuous murmur of PDA
TOF
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EKG: RAD, RVH
Xray: “boot-shaped”
heart (hypoplastic
MPA)
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Echo: image of four
defects associated
Tetralogy of Fallot
TOF complications
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Hypoxic spells
growth retardation with severe cyanosis
brain abscess and CVA
infective endocarditis
polycythemia
Hypoxic Spell ( “TET Spell”
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Paroxysm of hyperpnea (rapid and
deep)
irritability and prolonged cry
increasing cyanosis
decreased intensity of heart murmur
(may lead to limpness, convulsion,CVA
or death)
Treatment of “TET Spell”
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Knee-chest (squat )position
morphine sulfate
treat acidosis
oxygenation
Total Anomalous Pulmonary
Venous Return
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One percent of all congenital heart
defects
Defect: no direct communication
between the pulmonary veins and the
left atrium (they can drain:
supracardiac,cardiac,infracardiac or
mixed)
Total Anomalous Pulmonary Venous
Return
TAPVR findings
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S2 widely split and
fixed
S3 or S4 gallop
SEM: III-IV/VI
middiastolic rumble
at LLSB
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Xrays:
cardiomegally
“Snowman”
Echo can define
anatomy
EKG:RAD
Tricuspid Atresia
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1-2% of all congenital heart disease in
infancy
tricuspid valve is absent and RV is
hypoplastic
associated defects of VSD,ASD or PDA
are necessary for survival
Tricuspid Atresia
Tricuspid Atresia: findings
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Exam: cyanosis
S2 single, often syst murmur of VSD,
and occ of PDA present
early CHF
Tricuspid Atresia
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EKG: superior QRS
between O and -90
LVH
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Pulmonary
vascularity is
decreased
Echo: defines
minimal RV, and
large LV
Pulmonary Atresia
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Less than 1% of congenital heart
diseases
valve is atretic, RV cavity is hypoplastic
need other defects: ASD,PDA for
survival
Pulmonary Atresia
Pulmonary Atresia: findings
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PE: S2 is single
murmur of PDA
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EKG: normal
axis,LVH
Xray: decreased
pulmonary
vascularity
Echo:atretic
pulmonary valve
and hypoplastic RV
Pulmonary Atresia
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Prostaglandin E1
cardiac surgery
Truncus Arteriosus
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Less than 1% of all congenital heart Dz
Only a single arterial trunk leaves the
heart and gives rise to the pulmonary,
systemic and coronary circulations
large VSD is always present
Truncus Arteriosus
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PE: cyanosis
wide pulse pressure
and bounding
pulses
harsh VSD murmur
LSB
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EKG:normal
axis,LAH
Xrays:
cardiomegally and
increased pulm
vascularity
50% R aortic arch
Echo:single great
artery,VSD
Hypoplastic Left Heart
Hypoplastic Left Heart
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Hypoplastic left heart syndrome refers to
underdevelopment of the left side of the
heart.
This syndrome may include:
– Small aorta: This is the major blood vessel
from the left ventricle to the body.
Hypoplastic Left Heart
May Include:
– Aortic valve atresia (absence): This valve normally
opens and closes to let blood flow from the left
ventricle to the aorta. When atresia is present, there
is no connection between the left ventricle and aorta,
and no forward blood flow.
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– Mitral valve stenosis or atresia: This valve normally
opens and closes to let blood flow between the left
atrium and left ventricle. Stenosis causes little blood
flow; atresia causes no blood flow. Either atresia or
stenosis may be present.