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Pediatric Cardiology 101

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Kawasaki Disease (KD) Now the #1 cause of acquired heart disease ... Kawasaki Clinical criteria. Fever for at least 5 days AND 4 of the following 5 criteria: ... – PowerPoint PPT presentation

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Title: Pediatric Cardiology 101


1
Pediatric Cardiology 101
  • Misty Carlson, M.D.

2
DISCLAIMER
  • This lecture is based on generalizations.
  • In reality, a congenital heart defect (CHD) can
    act completely different from one patient to the
    next (eg- classic ToF vs pink ToF).
  • There are many more CHDs than what Ive listed
    and I hope you can use these principles to help
    you out with those.

3
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 similar resistance
  • Shunts are necessary for survival
  • ductus venosus (bypasses liver)
  • foramen ovale (R?L atrial level shunt)
  • ductus arteriosus (R?L arterial level shunt)

4
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5
Transitional Circulation
  • With first few breaths lungs expand and serve as
    the oxygenator (and the placenta is removed from
    the circuit)
  • Foramen ovale functionally closes
  • Ductus arteriosus usually closes within first 1-2
    days

6
Neonatal Circulation
  • RV pumps to pulmonary circulation and LV pumps to
    systemic circulation
  • Pulmonary resistance (PVR) is high so initially
    RV pressure LV pressure
  • By 6 weeks pulmonary resistance drops and LV
    becomes dominant

7
Normal Pediatric Circulation
  • LV pressure is 4-5 x RV pressure (this is
    feasible since RV pumps against lower resistance
    than LV)
  • RV is more compliant chamber than LV

8
100
  • No shunts
  • No pressure gradients
  • Normal AV valves
  • Normal semilunar valves
  • If this patient was desaturated what would you
    think?

90/ 60
20/8
100
75
75
20/
90/
9
If you have a hole in the heart what affects
shunt flow?
  • Pressure easy enough to understand
  • 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

10
Congenital Heart Disease (CHD)
  • Occurs in 0.5-1 of all live births
  • Simple way to classify is
  • L?R shunts
  • Cyanotic CHD (R?L shunts)
  • Obstructive lesions

11
L?R Shunts (Acyanotic CHD)
  • Defects
  • VSD
  • PDA
  • ASD
  • AVSD (or complete atrioventricular canal)
  • May not be apparent in neonate due to high PVR
    (ie- bidirectional shunt)

12
L?R Shunts General Points
  • ASD
  • Presents in childhood w/ murmur or exercise
    intolerance (AVSD or 1o ASD presents earlier)
  • Right heart enlargement (RHE)
  • Transmits flow only
  • PDA VSD
  • Presents in infancy w/ heart failure, murmur,
    and poor growth
  • Left heart enlargement (LHE)
  • Transmits flow and pressure

AVSD can present as either depending on size of
ASD VSD component
13
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14
Increased PBF
Left Heart Overload
Right Heart Overload
15
Pulm vasc markings equal in upper and lower zones
Cardiomegaly
16
Eisenmengers Syndrome
  • A long standing L?R shunt will eventually cause
    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 R?L) and the patient becomes cyanotic

17
R?L Shunts (CCHD)
  • Blue blood bypasses the lungs
  • Degree of cyanosis varies
  • Classify based on pulmonary blood flow (PBF)
  • ? PBF
  • Truncus arteriosus
  • Total anomalous pulm. venous return (TAPVR)
  • Transposition of the great arteries (TGA)
  • ? PBF
  • Tetralogy of Fallot
  • Tricuspid atresia
  • Ebsteins anomaly

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)
18
R?L Shunts
  • ? PBF
  • Presents more often with heart failure (except
    TGA)
  • Pulmonary congestion worsens as neonatal PVR
    lowers
  • Sats can be 93-94 if there is high PBF

There is unimpeded PBF thus, extreme pulmonary
overcirculation.
Equal pressures here too
19
R?L Shunts
  • ? PBF
  • Presents more often with cyanosis
  • See oligemic lung fields
  • Closure of PDA may worsen cyanosis

Dynamic subvalvular obstruction here causes Tet
spells
Why are pressures equal?
20
Pulmonary overcirculation
Too little PBF
90
70
99
99
70
60
99
99
70
60
21
Different amounts of PBF(Truncus vs ToF)
22
Obstructive Lesions
  • Ductal Dependent
  • Critical PS/AS
  • Critical CoA/IAA
  • HLHS
  • Presents in CV shock at 2-3 days of age when PDA
    closes
  • /- cyanosis
  • Needs PGE1
  • Non-Ductal Dependent
  • Mild-moderate AS
  • Mild-moderate CoA
  • Mild-moderate PS
  • Presents in older child w/ murmur, exercise
    intolerance, or HTN (in CoA)
  • Not cyanotic

23
Ductal-DependentLesion
Without a PDA there is no blood flow to the
abdomen and lower extremities. (Blue blood is
better than no blood.)
24
Physical Exam
  • Inspection and palpation
  • Cardiac cyanosis must be central
  • Differential cyanosis R?L PDA shunt
  • Differential edema/congestion implies obstruction
    of SVC or IVC
  • Increased precordial activity
  • Displaced PMI
  • RV heave RV hypertension

25
Physical exam
  • Lungs
  • Respiratory rate and work of breathing
  • Oxygen saturations
  • Abdominal exam
  • Liver size
  • Extremities
  • Perfusion
  • Edema
  • Clubbing

26
Physical Exam
  • Pulses (very important)
  • Differential pulses (weak LE) CoA
  • Bounding pulse run-off lesions (L?R PDA shunt,
    AI, BT shunt)
  • Weak pulse cardiogenic shock or CoA
  • Pulsus paradoxus is an exaggerated SBP drop with
    inspiration ? tamponade or bad asthma
  • Pulsus alternans altering pulse strength ? LV
    mechanical dysfunction

27
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 cardiac dysfunction/
    volume overload
  • Muffled heart sounds and/or a rub pericardial
    effusion tamponade

28
Physical Exam
  • Types of Murmurs
  • Systolic Ejection Murmur (SEM) turbulence
    across a semilunar valve
  • Holosystolic murmur turbulence begins with
    systole (VSD, MR)
  • Continuous murmur pressure difference in
    systole and diastole (PDA, BT shunt)

29
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 with a valvular
    click and heard best over precordium

30
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

31
Innocent murmurs
  • Pulmonary flow murmur
  • Often heard in older children and adolscents
  • Soft SEM at ULSB, little radiation normal second
    heart sound
  • Not positional
  • Need to differentiate b/w mild PS and especially
    an ASD
  • Hint ASD would have a fixed split second heart
    sound

32
Innocent murmurs
  • Venous hum
  • Often heard in toddlers, young children
  • Low pitched continuous murmur often heard best in
    infraclavicular area, normal heart sounds
  • Positional diminishes or goes completely away
    when pt in supine position or with compression of
    jugular vein
  • Need to differentiate between a PDA

33
Syndrome Associations
  • Down AV canal and VSD
  • Turner CoA, AS
  • Trisomies 13 and 18 VSD, PDA
  • Fetal alcohol L?R shunts, ToF
  • CHARGE conotruncal (ToF, truncus)

34
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

35
Kawasaki Disease (KD)
  • Now the 1 cause of acquired heart disease
  • A systemic vasculitis (etiology-unknown)
  • Tests CBC, CMP, CRP, ESR, EKG, ECHO
  • Rx IVIG at 2g/kg and high-dose ASA
  • Prognosis Coronary artery dilatation in 15-25
    w/o IVIG and 4 w/ IVIG (if given within 10 days
    of fever onset). Risk of coronary thrombosis.

36
Kawasaki Clinical criteria
  • Fever for at least 5 days AND 4 of the following
    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

37
Rheumatic Fever
  • A post-infectious connective tissue disease
  • Follows GAS pharyngitis by 3 weeks (vs.
    nephritogenic strains 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

38
Rheumatic Fever organs affected
  • Heart muscle valves myocarditis
    endocarditis (pericarditis rare w/o the others)
  • Joints polyarthritis
  • Brain Sydenhams Chorea (milkmaids grip or
    better yet, motor impersistance)
  • Skin erythema marginatum (serpiginous border)
    due to vasculitis
  • Subcutaneous nodules non-tender, mobile and on
    extensor surfaces

39
In case you havent had enough.
40
  • A ductal-dependent lesion
  • One ventricle pumps both PBF SBF
  • Difficult to balance PBF SBF

41
Norwood Procedure
  • What is the purpose of the BT shunt?
  • Is there a murmur?
  • What is your guess for the arterial saturation?

42
Bidirectional Glenn
  • What is the purpose of the Glenn?
  • Is there a murmur?
  • What is your guess for the arterial saturation?

43
Fontan circuit
  • What is the path of blood?
  • Is there a murmur?
  • What is your guess for the arterial saturation?
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