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Pediatric
Cardiology 101
Misty Carlson, M.D.
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DISCLAIMER:
This lecture is based on generalizations.
In reality, a congenital heart defect (CHD)
can act completely different from onepatient to the next (eg- classic ToF vspink ToF).
There are many more CHDs than whatIve listed and I hope you can use these
principles to help you out with those.
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Fetal Circulation For the fetus the placenta is the oxygenator
so the lungs do little work
RV & LV contribute equally to the systemic
circulation and pump against similarresistance
Shunts are necessary for survival
ductus venosus (bypasses liver) foramen ovale (RL atrial level shunt)
ductus arteriosus (RL arterial level shunt)
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Transitional Circulation
With first few breaths lungs expand andserve as the oxygenator (and the placenta
is removed from the circuit) Foramen ovale functionally closes
Ductus arteriosus usually closes within
first 1-2 days
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Neonatal Circulation
RV pumps to pulmonary circulation and LVpumps to systemic circulation
Pulmonary resistance (PVR) is high; soinitially RV pressure ~ LV pressure
By 6 weeks pulmonary resistance drops
and LV becomes dominant
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Normal Pediatric Circulation
LV pressure is 4-5 x RV pressure (this isfeasible since RV pumps against lower
resistance than LV) RV is more compliant chamber than LV
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No shunts
No pressure gradients
Normal AV valves Normal semilunar
valves
If this patient wasdesaturated whatwould you think?
75% 100%
20/ 90/
20/890/
60
100%
75%
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If you have a hole in the heart what
affects shunt flow?1. Pressure easy enough to understand
2.
Resistance impedance to blood flow
Remember, the LV has higher pressure and
a higher resistive circuit relative to the RV.
Now onto the nitty-gritty
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Congenital Heart Disease (CHD)
Occurs in 0.5-1% of all live births
Simple way to classify is:
LR shunts
Cyanotic CHD (RL shunts)
Obstructive lesions
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LR Shunts (Acyanotic CHD)
Defects
1. VSD
2. PDA
3. ASD
4. AVSD (or complete atrioventricular canal)
May not be apparent in neonate due tohigh PVR (ie- bidirectional shunt)
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LR Shunts General Points
PDA & VSD
Presents in infancy w/heart failure, murmur,and poor growth
Left heart enlargement(LHE)
Transmits flow andpressure
ASD Presents in childhood w/
murmur or exerciseintolerance (AVSD or 1oASD presents earlier)
Right heart enlargement
(RHE) Transmits flow only
AVSD can present as either depending on size of ASD & VSD component
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Increased PBF
Left Heart
Overload
Right Heart
Overload
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Pulm vasc
markings
equal in
upper and
lower zones
Cardiomegaly
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Eisenmengers Syndrome
A long standing LR shunt will eventuallycause irreversible pulmonary vascular
disease This occurs sooner in unrepaired VSDs
and PDAs (vs an ASD) because of the
high pressure Once the PVR gets very high the shunt
reverses (ie- now RL) and the patient
becomes cyanotic
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RL Shunts (CCHD)
PBF
Truncus arteriosus
Total anomalous pulm.
venous return (TAPVR) Transposition of the great
arteries (TGA)
PBF
Tetralogy of Fallot
Tricuspid atresia
Ebsteins anomaly
Blue blood bypasses the lungs Degree of cyanosis varies Classify based on pulmonary blood flow (PBF)
Please note: This is a generalization. In reality most of these defects can present with
low or high PBF (eg- ToF with little PS acts more like a VSD with high PBF)
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RL Shunts
PBF
Presents more often
with heart failure(except TGA)
Pulmonary congestionworsens as neonatal
PVR lowers Sats can be 93-94% if
there is high PBFEqual
pressures
here too
There is unimpeded
PBF; thus, extreme
pulmonary
overcirculation.
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RL Shunts
PBF
Presents more often
with cyanosis See oligemic lung
fields
Closure of PDA mayworsen cyanosis
Dynamic subvalvular
obstruction here
causes Tet spells
Why are
pressures
equal?
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70%
70%
99%
99%
90%
60%
60%
99%
99%
70%
Pulmonary
overcirculation Too little
PBF
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Different amounts of PBF
(Truncus vs ToF)
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Obstructive Lesions
Ductal Dependent
1. Critical PS/AS
2. Critical CoA/IAA3. HLHS
Presents in CV shock at2-3 days of age when
PDA closes +/- cyanosis
Needs PGE1
Non-Ductal Dependent
1. Mild-moderate AS
2. Mild-moderate CoA3. Mild-moderate PS
Presents in olderchild w/ murmur,
exercise intolerance,or HTN (in CoA)
Not cyanotic
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Without a PDA there is no
blood flow to the abdomen
and lower extremities.
(Blue blood is better than no blood.)
Ductal-Dependent
Lesion
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Physical Exam
Inspection and palpationCardiac cyanosis must be central
Differential cyanosis = RL PDA shuntDifferential edema/congestion implies
obstruction of SVC or IVC
Increased precordial activity
Displaced PMI
RV heave = RV hypertension
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Physical exam
Lungs Respiratory rate and work of breathing
Oxygen saturations
Abdominal exam Liver size
Extremities
Perfusion Edema
Clubbing
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Physical Exam
Pulses (very important)Differential pulses (weak LE) = CoA
Bounding pulse = run-off lesions (LR PDAshunt, AI, BT shunt)
Weak pulse = cardiogenic shock or CoA
Pulsus paradoxus is an exaggerated SBP
drop with inspiration tamponade or badasthma
Pulsus alternansaltering pulse strength LV mechanical dysfunction
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Physical Exam
Heart sounds Ejection click = AS or PS
Mid-systolic click = MVP
Loud S2 = Pulmonary HTN Single S2 = one semilunar valve (truncus),
anterior aorta (TGA), pulmonary HTN
Fixed, split S2 = ASD, PS
Gallop (S3) may be due to cardiacdysfunction/ volume overload
Muffled heart sounds and/or a rub = pericardialeffusion tamponade
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Physical Exam
Types of Murmurs
Systolic Ejection Murmur (SEM) =
turbulence across a semilunar valveHolosystolic murmur = turbulence begins
with systole (VSD, MR)
Continuous murmur = pressure difference
in systole and diastole (PDA, BT shunt)
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Innocent murmurs
Peripheral pulmonic stenosis (PPS)Heard in newborns disappears by one year
of age (often earlier)Soft SEM at ULSB w/ radiation to axilla and
back (often heard best in axilla/back)
Need to differentiate b/w PPS and actual
pulmonic stenosis. PS often associated witha valvular click and heard best overprecordium
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Innocent murmurs
Stills murmur Classic innocent murmur Heard most commonly in young children (3-5 yrs of
age) but can be heard in all ages Vibratory low-frequency murmur often heard along
LSB and apex Positional increases in intensity when pt is in supine
position
Also louder in high output states (i.e. dehydration,fever)
Need to differentate from VSD
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Innocent murmurs
Pulmonary flow murmur
Often heard in older children and adolscents
Soft SEM at ULSB, little radiation; normal secondheart sound
Not positional
Need to differentiate b/w mild PS and especially an
ASD Hint: ASD would have a fixed split second heart sound
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Innocent murmurs
Venous hum
Often heard in toddlers, young children
Low pitched continuous murmur often heard best ininfraclavicular area, normal heart sounds
Positional diminishes or goes completely awaywhen pt in supine position or with compression of
jugular vein
Need to differentiate between a PDA
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Syndrome Associations
DownAV canal and VSD
TurnerCoA, AS
Trisomies 13 and 18 VSD, PDA
Fetal alcoholLR shunts, ToF
CHARGE conotruncal (ToF, truncus)
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Hereditary Diseases
Marfan (AD) aortic root aneurysm dissection, MVP,MR, AI
HCM (AD) outflow tract obstruction, arrhythmias
Noonan (AD) HCM, PS DMD/BMD (X-link) DCM (>12 y.o.)
Williams (AD) supravalvar AS
Tuberous sclerosis rhabdomyoma
Romano-Ward AD LQTS Jervell & Lange-Nielsen AR LQTS & deafness
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Kawasaki Disease (KD)
Now the #1 cause of acquired heartdisease
A systemic vasculitis (etiology-unknown) Tests CBC, CMP, CRP, ESR, EKG,
ECHO
Rx IVIG at 2g/kg and high-dose ASA PrognosisCoronary artery dilatation in
15-25% w/o IVIG and 4% w/ IVIG (if givenwithin 10 days of fever onset). Risk of
coronary thrombosis.
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Kawasaki Clinical criteria
Fever for at least 5 days AND 4 of thefollowing 5 criteria:
Eyes - conjunctival injection (ie- no exudate) Lips & mouth - erythema, cracked lips, strawberry
tongue
Hands & feet - edema and/or erythema
Skin - polymorphous exanthem (ie- any rash)
Unilateral, cervical lymphadenopathy
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Rheumatic Fever
A post-infectious connective tissue disease
Follows GAS pharyngitis by 3 weeks (vs. nephritogenicstrains of GAS)
Injury by GAS antibodies cross-reacting with tissue Dx JONES criteria (major and minor)
Tests Throat Cx, ASO titer, CRP, ESR, EKG, +/-ECHO
Rx PCN x10 days and high-dose ASA or steroids 2o Prophylaxis daily po PCN or monthly IM PCN
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Rheumatic Fever organs
affected1. Heart muscle & valves myocarditis &
endocarditis (pericarditis rare w/o the others)
2. Joints polyarthritis3. BrainSydenhams Chorea (milkmaids grip or
better yet, motor impersistance)
4. Skin erythema marginatum (serpiginous border)
due to vasculitis5. Subcutaneous nodules non-tender, mobile and on
extensor surfaces
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In case you havent had enough.
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A ductal-dependentlesion
One ventricle pumpsboth PBF & SBF
Difficult to balancePBF & SBF
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Norwood Procedure
What is the purposeof the BT shunt?
Is there a murmur? What is your guess
for the arterialsaturation?
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Bidirectional Glenn
What is the purposeof the Glenn?
Is there a murmur? What is your guess
for the arterialsaturation?
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Fontan circuit
What is the path ofblood?
Is there a murmur? What is your guess
for the arterialsaturation?
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