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feb04feb04

PEDIATRIC CARDIOLOGYPEDIATRIC CARDIOLOGY

Normal HeartNormal Heart

RA

RV

LV

LA

ICV

SCV

PA

AO

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GENERAL PRINCIPLESGENERAL PRINCIPLES

Pediatric Cardiology :Pediatric Cardiology :

1. Congenital Heart Disease (CHD, PJB)1. Congenital Heart Disease (CHD, PJB)

Occurs since organogenesisOccurs since organogenesis

2. Acquired Heart Disease (AHD, PJD)2. Acquired Heart Disease (AHD, PJD)

Disturbance of normal heart Disturbance of normal heart

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INCIDENCEINCIDENCE

CHDCHD : : 6-8/1000 live births6-8/1000 live births8 types of CHD (85%) : 8 types of CHD (85%) :

VSD, ASD, PDA, PS, AS, TF, TGAVSD, ASD, PDA, PS, AS, TF, TGA

AHD :AHD :Neonatus : virus (Echo, Influenzae)Neonatus : virus (Echo, Influenzae)5 - 15 yrs : RF5 - 15 yrs : RF

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ETIOLOGYETIOLOGYCHD :CHD : 90% genetic – environmental factors90% genetic – environmental factors

Environment : Environment :

11stst trimester pregnancy trimester pregnancy organogenesis of the organogenesis of the heart : heart : radiation, smoking, drugs (thalidomide), radiation, smoking, drugs (thalidomide), maternal infection (rubella), mother age (young / maternal infection (rubella), mother age (young / old), high geografic location (less O2 ), metabolic old), high geografic location (less O2 ), metabolic disorders (DM)disorders (DM)

AHDAHD : : -- infection (RF, diphtheriae)infection (RF, diphtheriae)

- neonatus (Coxsackie B virus)- neonatus (Coxsackie B virus)

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FETAL CIRCULATIONFETAL CIRCULATION

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FETAL CIRCULATIONFETAL CIRCULATION

Signs :Signs :Parallel systemic and pulmonary circulationsParallel systemic and pulmonary circulations

Foramen ovale, ductus Botalli, ductusForamen ovale, ductus Botalli, ductus

venosus : still openvenosus : still open

RA : enlargement, cross circulationRA : enlargement, cross circulation

Head, heart and upper extremities are Head, heart and upper extremities are supplied by high O2 content supplied by high O2 content

Minimal pulmonary circulation Minimal pulmonary circulation

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CIRCULATION AFTER BIRTH CIRCULATION AFTER BIRTH

After birth : After birth : Expansion of lung Expansion of lung Placenta circulation endedPlacenta circulation ended

Systemic and pulmonary circulation Systemic and pulmonary circulation serial typeserial type

No cross circulation in RANo cross circulation in RA

Foramen ovale, d. Botalli & d. Venosus Foramen ovale, d. Botalli & d. Venosus closedclosed

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CyanosisCyanosis

Reduced Hb > 5 gr% (N=2,25 gr%)Reduced Hb > 5 gr% (N=2,25 gr%)

2 types :2 types :a.a. Central C :Central C :

arterial unsaturationarterial unsaturation generalized and severegeneralized and severe

b.b. Peripheral C :Peripheral C : without arterial unsaturationwithout arterial unsaturation localized and milderlocalized and milder

Distinction between a and b : Distinction between a and b :

measurement of arterial O2 content (N=95%).measurement of arterial O2 content (N=95%).

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Influence of Hb levels on C: Influence of Hb levels on C:

1.1. Hb. 20 gr %, 70% saturationHb. 20 gr %, 70% saturation

Reduced Hb = 30 % x 20 gr % = 6 gr % Reduced Hb = 30 % x 20 gr % = 6 gr %

(C +)(C +)

2.2. Hb 6 gr %, 70% saturationHb 6 gr %, 70% saturation

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

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a.a. Central CCentral C : :

Pulmonary C :Pulmonary C :

Lung disorders (diffusion, ventilation, Lung disorders (diffusion, ventilation,

perfusion)perfusion)

Cerebral C:Cerebral C:

Brain disorders Brain disorders center of respiration center of respiration

Cardial C: Cardial C:

R – L shuntR – L shunt

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Hyperoxic (100% O2) test / Crying :Hyperoxic (100% O2) test / Crying :

pulmonary C pulmonary C less/no C less/no C

cardial C cardial C C still persist C still persist

b.b. Peripheral CPeripheral C

Decreased cardiac outputDecreased cardiac output

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CONGENITAL HEART DISEASE (CHD)CONGENITAL HEART DISEASE (CHD)

Early signs of CHDEarly signs of CHD

CyanosisCyanosis

Inadequate intakeInadequate intake

Heart murmurHeart murmur

Unpalpable femoral and brachial pulse Unpalpable femoral and brachial pulse

Circulation collapsCirculation collaps

ArrhythmiaArrhythmia

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ASD

RA

LA

RVLV

ATRIAL SEPTAL DEFECT (ASD)ATRIAL SEPTAL DEFECT (ASD)

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Any opening (defect) in the atrial septum Any opening (defect) in the atrial septum shunt shunt

Ostium Secundum (50-70%)Ostium Secundum (50-70%)

Hemodynamic : depends on the size, compliance Hemodynamic : depends on the size, compliance

of V and resistance of Pulm. and Syst. circulationof V and resistance of Pulm. and Syst. circulation

ATRIAL SEPTAL DEFECT (ASD)ATRIAL SEPTAL DEFECT (ASD)

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Signs/Symptoms :Signs/Symptoms :

Usually asymptomatic, mmr is found by chanceUsually asymptomatic, mmr is found by chance

Fatigue, dyspnea, recurrent respiratory infection.Fatigue, dyspnea, recurrent respiratory infection.

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

widely split and fixed S2widely split and fixed S2

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X ray : increased PBF X ray : increased PBF

ECG : RAD, RVHECG : RAD, RVH

Echo : position and size of the defectEcho : position and size of the defect

Catheterization : OCatheterization : O22 in RA > CV in RA > CV

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ManagementManagement

To favour the spontaneous closure of ASD To favour the spontaneous closure of ASD (87%)(87%)

Transcatheter closure (Transcatheter closure (Amplatzer Septal Amplatzer Septal OccluderOccluder))

Surgical closure :Surgical closure :

Indication : P / S ratio Indication : P / S ratio ≥ 1.5 : 1≥ 1.5 : 1

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VENTRICULAR SEPTAL DEFECT VENTRICULAR SEPTAL DEFECT (VSD)(VSD)

VSD

RA LV

LA

RV

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VENTRICULAR SEPTAL DEFECT VENTRICULAR SEPTAL DEFECT (VSD)(VSD)

Defect in the ventricular septumDefect in the ventricular septum

Prevalence : CHD no.1 (25%)Prevalence : CHD no.1 (25%)

Hemodynamic :Hemodynamic :

– Depends on the size and pressure between Depends on the size and pressure between RV and LVRV and LV

– Pressure LV > RV Pressure LV > RV L-R shunt L-R shunt

– R-L, L-R, R-L (R-L, L-R, R-L (Eisenmenger SEisenmenger S) )

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SIMPLE VSDSIMPLE VSD

20 % of CHD, 25 % of VSD20 % of CHD, 25 % of VSD

Small 1-5 mm, Moderate 5-10 mmSmall 1-5 mm, Moderate 5-10 mm

Asymptomatic : Asymptomatic : Roger’s diseaseRoger’s disease

Normal G-D, mmr heard at Week 1Normal G-D, mmr heard at Week 1

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MODERATE VSDMODERATE VSD

Fatigue, intol.activity, dyspnea, recurrent Fatigue, intol.activity, dyspnea, recurrent

resp.tr infectionresp.tr infection

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

3-53-5

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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 Management : :

Nonsurgical closureNonsurgical closure : :

Amplatzer septal occluderAmplatzer septal occluder

SurgicalSurgical : infant with large VSD + CHF : infant with large VSD + CHF

PrognosisPrognosis : :

Perimembranous Perimembranous : surgical intervention: surgical intervention

Muscular defectMuscular defect : spontaneous closure : spontaneous closure

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PATENT DUCTUS ARTERIOSUS (PDA)PATENT DUCTUS ARTERIOSUS (PDA)

PDA

LV

AO

AP

LA

RVRA

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PATENT DUCTUS ARTERIOSUS (PDA)PATENT DUCTUS ARTERIOSUS (PDA)

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

Anatomy/physiology : Anatomy/physiology : Intrauterine: AP Intrauterine: AP d. Botalli d. Botalli Aorta Aorta Extrauterine: d. Botalli 10–15 hrs still Extrauterine: d. Botalli 10–15 hrs still

openopen

L-R shunt (syst-diast) L-R shunt (syst-diast) continuous continuous mmr mmr

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TYPICAL PDA (SIMPLE PDA)TYPICAL PDA (SIMPLE PDA)Clin. Manifestations :Clin. Manifestations :

asymptomatic, recurrent resp. tr.infectionasymptomatic, recurrent resp. tr.infectioncontinuous mmr at LSB2continuous mmr at LSB2

Echo : direction of shunt & Ø PDAEcho : direction of shunt & Ø PDA

Prognosis : rarely closed spontaneously (1 yr),Prognosis : rarely closed spontaneously (1 yr),

except in premature babies)except in premature babies)

Management : Management :

Surgical closure (ligation)Surgical closure (ligation)

Nonsurgical closureNonsurgical closure : Amplatzer Ductal Occluder : Amplatzer Ductal Occluder

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PULMONARY STENOSIS (PS)PULMONARY STENOSIS (PS)

PS

RVLV

LA

RA

PA

AO

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PULMONARY STENOSIS (PS)PULMONARY STENOSIS (PS)

Difference of syst.pressure between RV and PA Difference of syst.pressure between RV and PA > 100 mmHg> 100 mmHg

Hemodynamic :Hemodynamic : RV activity increased RV activity increased RVH RVH PS + VSD PS + VSD R-L shunt (cardial cyanosis) R-L shunt (cardial cyanosis)

rarely CHFrarely CHF

Pulmonary ejection click (valve opening)Pulmonary ejection click (valve opening)

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Clin.Manif. Clin.Manif.

Eject. Syst mmr LSB2Eject. Syst mmr LSB2

X-ray : PBF <<, cardiomegaly X-ray : PBF <<, cardiomegaly

ECG : RAD , RVH ECG : RAD , RVH Echo : thick pulmonary valve, dilated PAEcho : thick pulmonary valve, dilated PA

CineangioCineangio : : a jet contrasta jet contrast Management :- Management :- Balloon valvuloplastyBalloon valvuloplastySurgery if balloon failtSurgery if balloon failt

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COARCTATION OF THE AORTA (CoA)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)COARCTATION OF THE AORTA (CoA)

CoA

LA

LV

RVRA

AO

PA

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Postductal CoAPostductal CoA

Clin.ManifestationsClin.Manifestations

Pain of calves, headaches, nose Pain of calves, headaches, nose bleeds, epistaxisbleeds, epistaxis

BP Hypertenssion (pathognomonic)BP Hypertenssion (pathognomonic)

Brachial – Femoral lagBrachial – Femoral lag

Reduced / abcent lower extremity Reduced / abcent lower extremity pulsespulses

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X-ray :X-ray :Rib notching (collateral vessels)Rib notching (collateral vessels)E sign on barium meals E sign on barium meals

ECHO / Doppler : ECHO / Doppler : Gradient and pattern of diastolic flowGradient and pattern of diastolic flow

Catheterization : Catheterization : Confirmation of diagnosisConfirmation of diagnosis

Management :Management :Surgery, balloon angioplastySurgery, balloon angioplasty

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TETRALOGI OF FALLOT (TF)TETRALOGI OF FALLOT (TF)

VSD

PS

OvA

RVH

AO

AP

LV

LA

RA

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TETRALOGI OF FALLOT (TF)TETRALOGI OF FALLOT (TF)

4 defects : 4 defects :

VSD, PS, RVH, Overriding of the AortaVSD, PS, RVH, Overriding of the AortaFrequency : Frequency :

10-15% CHD, cyanotic CHD no.1 (75%)10-15% CHD, cyanotic CHD no.1 (75%)

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

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Clin.Manifestation : Clin.Manifestation :

Clubbing fingers, scoliosis, squatting Clubbing fingers, scoliosis, squatting

positionposition

Ejection systolic mmr LSB3-4Ejection systolic mmr LSB3-4

Lab : Hb, Ht, RBC levels inreasedLab : Hb, Ht, RBC levels inreased

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Echo : Echo : VSD, Overriding Ao, RVOT obstructionVSD, Overriding Ao, RVOT obstruction

X-Ray : X-Ray : couer en sabotcouer en sabot, RVH, PBF , RVH, PBF

Complication : Complication :

Cerebral Infarction (age < 2 yrs)Cerebral Infarction (age < 2 yrs)

Cerebral Absces (age > 2 yrs)Cerebral Absces (age > 2 yrs)

PolycytemiaPolycytemia

Treatment : Treatment :

Surgery : palliative / total correctionSurgery : palliative / total correction

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TRANSPOSITION OF THE GREAT ARTERIES TRANSPOSITION OF THE GREAT ARTERIES (TGA)(TGA)

RVLV

AO

AP

LA

RA

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TRANSPOSITION OF THE GREAT ARTERIESTRANSPOSITION OF THE GREAT ARTERIES (TGA) (TGA)

Ventriculoarterial discordance, Ao – RV and PA - Ventriculoarterial discordance, Ao – RV and PA - LVLVCyanotic CHD no.2, M > FCyanotic CHD no.2, M > FHemodynamic :Hemodynamic :

– parallel pulmonary and systemic circulation parallel pulmonary and systemic circulation (cyanosis(cyanosis))

– prolong life : mixing of oxy- and deoxygenated blood prolong life : mixing of oxy- and deoxygenated blood

(ASD, VSD, PDA)(ASD, VSD, PDA)

– deficient O2 supply to the heart, enlargement of the deficient O2 supply to the heart, enlargement of the

heart, heart, heart failureheart failure

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X-ray : X-ray : like an egg on its side like an egg on its side bootshaped heart (=TF)bootshaped heart (=TF)

Echo : Echo : double circledouble circle, parallel PA & Ao , parallel PA & Ao Management :Management :– Balloon atrial septostomyBalloon atrial septostomy– Surgery palliative or arterial switch Surgery palliative or arterial switch

procedureprocedure

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DEXTROCARDIADEXTROCARDIA

RVLV RA

RVLV

LA

VCI

VCS

AP

AO

RA

LA

AO

AP

Normal heartIsolated Mirror Image Dextrocardia

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The heart is located on the right side of the chest & the The heart is located on the right side of the chest & the

apex points to the right. Dextroposition is not a Dx.apex points to the right. Dextroposition is not a Dx.

Anatomy :Anatomy :

1. 1. Visceroatrial relationship :Visceroatrial relationship :

S (solitus), I (inversus)S (solitus), I (inversus) or or A (ambiguus)A (ambiguus)

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

X (uncertain or undeterminate)X (uncertain or undeterminate)

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

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

LA

VCI

AP

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Isolated mirror image dextrocardia (I,L,I)Isolated mirror image dextrocardia (I,L,I)

Kartagener syndrome: Kartagener syndrome:

Dextrocardia / situs inversusDextrocardia / situs inversus

BronkhiectasisBronkhiectasis

Paranasal sinusitis Paranasal sinusitis

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Clin. Manifestations :Clin. Manifestations :

loudest heart sound on the right chestloudest heart sound on the right chest

IMID 50-80% without CHDIMID 50-80% without CHD

X-ray IMID: liver – left, stomach bubble- rightX-ray IMID: liver – left, stomach bubble- right

Echo : dextrocardiaEcho : dextrocardia

Prognosis : depends on the lesionsPrognosis : depends on the lesions

Treatment : overcome the associatedTreatment : overcome the associated lesionslesions