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PEDIATRIC CARDIOLOGY
SCV
AO
PA
LA

RA

LV
RV

ICV

Normal Heart
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GENERAL PRINCIPLES
Pediatric Cardiology :
1. Congenital Heart Disease (CHD, PJB)
Occurs since organogenesis
2. Acquired Heart Disease (AHD, PJD)

Disturbance of normal heart

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INCIDENCE
CHD :
6-8/1000 live births
8 types of CHD (85%) :
VSD, ASD, PDA, PS, AS, TF, TGA

AHD :
Neonatus : virus (Echo, Influenzae)
5 - 15 yrs : RF
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ETIOLOGY
CHD : 90% genetic environmental factors
Environment :
1st trimester pregnancy organogenesis of the
heart : radiation, smoking, drugs (thalidomide),

maternal infection (rubella), mother age (young /

old), high geografic location (less O2 ),
metabolic disorders (DM)
AHD : - infection (RF, diphtheriae)
- neonatus (Coxsackie B virus)
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FETAL CIRCULATION

FETAL CIRCULATION
Signs :

Parallel systemic and pulmonary circulations

Foramen ovale, ductus Botalli, ductus
venosus : still open
RA : enlargement, cross circulation
Head, heart and upper extremities are
supplied by high O2 content
Minimal pulmonary circulation
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CIRCULATION AFTER BIRTH

After birth :
Expansion of lung
Placenta circulation ended

Systemic and pulmonary circulation

serial type
No cross circulation in RA

Foramen ovale, d. Botalli & d. Venosus

closed
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Cyanosis
Reduced Hb > 5 gr% (N=2,25 gr%)
2 types :
a. Central C :
arterial unsaturation
generalized and severe

b. Peripheral C :
without arterial unsaturation
localized and milder

Distinction between a and b :

measurement of arterial O2 content (N=95%).
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Influence of Hb levels on C:
1. Hb. 20 gr %, 70% saturation

Reduced Hb = 30 % x 20 gr % = 6 gr % (C +)
2. Hb 6 gr %, 70% saturation

Reduced HB = 30 % x 6 gr% = 1.8 gr % (C -)

a. Central C :

Pulmonary C :
Lung disorders (diffusion, ventilation,
perfusion)
Cerebral C:
Brain disorders center of respiration
Cardial C:
R L shunt
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Hyperoxic (100% O2) test / Crying :

pulmonary C less/no C
cardial C C still persist

b. Peripheral C

Decreased cardiac output

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CONGENITAL HEART DISEASE (CHD)

Early signs of CHD
Cyanosis
Inadequate intake
Heart murmur
Unpalpable femoral and brachial pulse
Circulation collaps
Arrhythmia
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ATRIAL SEPTAL DEFECT (ASD)

LA
RA
LV

ASD
RV

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ATRIAL SEPTAL DEFECT (ASD)

Any opening (defect) in the atrial septum shunt
Ostium Secundum (50-70%)
Hemodynamic : depends on the size, compliance
of V and resistance of Pulm. and Syst. circulation

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Signs/Symptoms :
Usually asymptomatic, mmr is found by chance
Fatigue, dyspnea, recurrent respiratory

infection.
Ausc. : ( mmr may be absent in infants)

widely split and fixed S2

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X ray : increased PBF

ECG : RAD, RVH

Echo : position and size of the defect
Catheterization : O2 in RA > CV

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Management
To favour the spontaneous closure of ASD
(87%)
Transcatheter closure (Amplatzer Septal
Occluder)
Surgical closure :
Indication : P / S ratio 1.5 : 1

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VENTRICULAR SEPTAL DEFECT (VSD)

LA
LV

RA
RV
VSD

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VENTRICULAR SEPTAL DEFECT (VSD)

Defect in the ventricular septum

Prevalence : CHD no.1 (25%)

Hemodynamic :

Depends on the size and pressure between

RV and LV
Pressure LV > RV L-R shunt
R-L, L-R, R-L (Eisenmenger S)
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SIMPLE VSD
20 % of CHD, 25 % of VSD
Small 1-5 mm, Moderate 5-10 mm
Asymptomatic : Rogers disease
Normal G-D, mmr heard at Week 1

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MODERATE VSD
Fatigue, intol.activity, dyspnea, recurrent
resp.tr infection

Pansystolic (holosystolic) 3-4/6, pm LSB

3-5

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X-ray : - increased PBF

ECG : Small VSD normal

Moderate VSD LVH (+LAE)

Catheterization : O2 in RV > RA
ECHO : nr, size, location

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Management :
Nonsurgical closure :
Amplatzer septal occluder
Surgical : infant with large VSD + CHF
Prognosis :
Perimembranous : surgical intervention
Muscular defect : spontaneous closure

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PATENT DUCTUS ARTERIOSUS (PDA)

PDA
AO
AP
LA

RA

LV
RV

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PATENT DUCTUS ARTERIOSUS (PDA)

Incidence : 12 % CHD (nr. 2), F > M

Anatomy/physiology :
Intrauterine: AP d. Botalli Aorta

Extrauterine: d. Botalli 1015 hrs still

open
L-R shunt (syst-diast) continuous
mmr
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TYPICAL PDA (SIMPLE PDA)

Clin. Manifestations :
asymptomatic, recurrent resp. tr.infection
continuous mmr at LSB2

Echo : direction of shunt & PDA

Prognosis : rarely closed spontaneously (1 yr),
except in premature babies)
Management :
Surgical closure (ligation)
Nonsurgical closure : Amplatzer Ductal Occluder
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PULMONARY STENOSIS (PS)

AO
PA
LA

LV

RA
RV

PS
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PULMONARY STENOSIS (PS)

Difference of syst.pressure between RV and PA
> 100 mmHg
Hemodynamic :
RV activity increased RVH
PS + VSD R-L shunt (cardial cyanosis)
rarely CHF

Pulmonary ejection click (valve opening)

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Clin.Manif.
Eject. Syst mmr LSB2

X-ray : PBF <<, cardiomegaly

ECG : RAD , RVH

Echo : thick pulmonary valve, dilated PA

Cineangio : a jet contrast
Management :Balloon valvuloplasty
Surgery if balloon failt

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COARCTATION OF THE AORTA (CoA)

Narrowing of the aorta. Turner Syndrome
Frequency : 5 8% CHD, M > F

Location : distal of left subclavian artery

2 types : 1. Preductal (CoA + Systemic LV/RV)
2. Postductal (CoA + Sytemic LV)

Hemodynamic :
Adequate O2 to distal of CoA :(Adaptation mechanism)
1. Increased systolic pressure at proximal of CoA

2. Increased diastolic pressure at distal of CoA

(arterioles vasoconstriction)
3. Collateral circulation (subclavian a, intercostal, etc)
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COARCTATION OF THE AORTA (CoA)

CoA

AO
PA

LV

LA

RA

RV

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Postductal CoA
Clin.Manifestations

Pain of calves, headaches, nose

bleeds, epistaxis
BP Hypertenssion (pathognomonic)
Brachial Femoral lag
Reduced / abcent lower extremity
pulses
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X-ray :
Rib notching (collateral vessels)

E sign on barium meals

ECHO / Doppler :
Gradient and pattern of diastolic flow
Catheterization :
Confirmation of diagnosis

Management :
Surgery, balloon angioplasty
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TETRALOGI OF FALLOT (TF)

AO
AP

LA

LV

RA
PS

RVH
VSD

OvA
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TETRALOGI OF FALLOT (TF)

4 defects :
VSD, PS, RVH, Overriding of the Aorta
Frequency :
10-15% CHD, cyanotic CHD no.1 (75%)

Hemodynamic :
PS + VSD R-L shunt
Cyanosis
R-L shunt polycytemia & tromboemboly

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Clin.Manifestation :
Clubbing fingers, scoliosis, squatting

position
Ejection systolic mmr LSB3-4
Lab : Hb, Ht, RBC levels inreased

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Echo : VSD, Overriding Ao, RVOT obstruction

X-Ray : couer en sabot, RVH, PBF

Complication :
Cerebral Infarction (age < 2 yrs)

Cerebral Absces (age > 2 yrs)

Polycytemia

Treatment :
Surgery : palliative / total correction
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TRANSPOSITION OF THE GREAT ARTERIES (TGA)

AO
AP
RA
RV

LA
LV

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TRANSPOSITION OF THE GREAT ARTERIES (TGA)

Ventriculoarterial discordance, Ao RV and PA LV
Cyanotic CHD no.2, M > F
Hemodynamic :
parallel pulmonary and systemic circulation (cyanosis)
prolong life : mixing of oxy- and deoxygenated blood
(ASD, VSD, PDA)

deficient O2 supply to the heart, enlargement of the

heart, heart failure
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X-ray : like an egg on its side

bootshaped heart (=TF)
Echo : double circle, parallel PA & Ao
Management :
Balloon atrial septostomy
Surgery palliative or arterial switch
procedure

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DEXTROCARDIA

VCS
AO

AO

AP

AP

LA

LA

LV
RV

RA

RA

LV
RV

VCI
Isolated Mirror Image Dextrocardia

Normal heart

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The heart is located on the right side of the chest & the
apex points to the right. Dextroposition is not a Dx.
Anatomy :
1. Visceroatrial relationship :
S (solitus), I (inversus) or A (ambiguus)

2. Ventricular Loop : D (D-loop), L (L-loop) or

X (uncertain or undeterminate)
AP

3. Great arteries (conotruncal) : S (solitus), I (inversus),

LA

D (D-transposition) or L (L-transposition) RA
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VCI

Isolated mirror image dextrocardia (I,L,I)

Kartagener syndrome:
Dextrocardia / situs inversus

Bronkhiectasis
Paranasal sinusitis

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Clin. Manifestations :
loudest heart sound on the right chest
IMID 50-80% without CHD
X-ray IMID: liver left, stomach bubble- right
Echo : dextrocardia

Prognosis : depends on the lesions

Treatment : overcome the associated lesions

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