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NORMAL HEART

Heart Diagrams by Dr. James L. Wilkinson MB. ChB.,FRCP, FRACP, FACC, FRCPCH. (Royal Childrens Hospital, Melbourne,
Australia)
(http://www.cc.umanitoba.ca/~soninr)

GENERAL PRINCIPLES
Pediatric Cardiology and Adult
Cardiology
Pediatric Cardiology :
1. Congenital Heart Disease (CHD)
Occurs since organogenesis
2. Acquired Heart Disease (AHD)
Disturbances occur after birth

INCIDENCE
CHD :
6-8/1000 live births
8 types of CHD (85%) :
VSD, ASD, PDA, PS, AS,
TF, TGA
AHD :
Neonatus : virus
5 - 15 yrs : RF

ETIOLOGY
CHD : 90% genetic environmental
factors
environment :
1st trimester pregnancy
organogenesis of the heart : radiation,
smoking, drugs (thalidomide), maternal
infection (rubella), mother age
high geografic location
(young/old),
AHD :
(less
O2), metabolic
disorders
(DM),
infection
(RF, diphtheriae)
Down syndrome (50% with CHD)

neonatus (Coxsackie B virus)

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

CIRCULATION AFTER BIRTH

Cyanosis
Reduced Hb > 5 gr% (N=2,25
gr%)
2 types :
A. Central C :
arterial unsaturation
B. Peripheral C :
without arterial unsaturation
Distinction between A and B :
measurement of arterial O2

Central C :
Pulmonary C :
Lung disorders diffusion,
ventilation, perfusion
Cerebral C:
brain disorders center of
respiration
Cardial C:
R-L shunt
Hyperoxic (100% O2) test / Crying :
pulmonary C less / no C
intracardial C C still persist
Peripheral C

Heart Diseases in
Children
Classification
CHD
Cyanotic Type
Non cyanotic type
AHD
Rheumatic Heart Disease
Rheumatic Fever
Myocarditis
Endocarditis

Congenital Heart Disease


Non Cyanotic type
VSD
ASD
PDA
Cyanotic type
TOF
Double Outlet Right Ventricel
Great Artery Transposition

Acyanotic Defect

PBF

Normal PBF
RVH

LVH or CVH

RVH

LVH

VSD
PDA
ECD

ASD
PAPVR
PVOD

AS or AR
COA
MR

PS
COA
MS

Cyanotic
PBF
LVH or CVH
Single Ventricel
TGA + VSD

PBF
RVH
TGA
HLHS

CVH
TGA + PS

LVH
AT
AP + Hypoplastic RV

RVH
TOF
PVOD

Congenital heart disease


(CHD)

Etiology
Unknown
Mothers disease (TORCH)
Rubella
Medicine : fenitoin, Alcohol,
lithium
Radiation
Genetics (dominan
autosomal)

Congenital heart disease


(CHD)

Early signs of CHD


Cyanosis
Inadequate intake
Heart murmur & sounds
Unpalpable femoral &
brachial pulse
Circulation collapse
Arrhythmia

Congenital heart disease


(CHD)

Diagnosis
Anamnesis and physical
examination
Simple investigation
Laboratory, ECG, X Ray
Echocardiography
Catheterization

ATRIAL SEPTAL DEFECT (ASD)

ATRIAL SEPTAL DEFECT (ASD)


Any opening (defect) in the atrial
septum shunt
Ostium Primum (15%) Ostium
Secundum (50%-70%) , Sinus
venosus defect
Hemodynamic : depends on the
size
compliance of Ventricles

ATRIAL SEPTAL DEFECT


(ASD)
CONTINUED
Signs/Symptoms :
Usually asymptomatic, murmur is
found by chance
Fatigue, dyspnea, recurrent
respiratory infection , FTT
Ausc: ( murmur may be absent in
infants)
widely split and fixed S2, HS 2nd

ATRIAL SEPTAL DEFECT


(ASD)
CONTINUED
X-ray:
increased PBF , RA
and Pulmonal Conus
protrude
ECG :
RAD, RVH
Echo :
position and size of
the defect

Management
Spontaneous closure of ASD, 40% (4
years) or become small

Transcatheter closure (Amplatzer


Septal Occluder)
Surgical closure :
Indication :
P / S ratio 1.5 : 1

ASD
VCS
VC I

RA

VP

LA

RV

LV

AP

AO

Ventricular septal defect (vSD)

Ventricular septal defect (vSD)


Defect in the ventricular septum
(perimembran, muscular,
subarterial)
Prevalence : CHD no. 1 (25%)
Hemodynamic :
Depends on the size and
pressure between RV and LV
Pressure LV > RV L-R shunt

Ventricular septal defect (vSD)


CONTINUED

SIMPLE VSD
20 % of CHD, 25 % of VSD
Small 1-5 mm, Moderate 5-10
mm, RVH (-)
Asymptomatic : Rogers disease
,
Ausc ( murmur holosistolik)

Ventricular septal defect (vSD)


CONTINUED

MODERATE VSD
fatigue, intolerance activity,
dyspnea, recurrent respiratory
tract infection, CHF
Pansystolic (holosystolic) 3-4/6,
punctum maximum LSB 3-5, P2
intensity >

Ventricular septal defect (vSD)


CONTINUED

X-ray :
Increased PBF, LAH, LVH

ECG :
Small VSD

: normal

Moderate VSD : LVH (+LAE)

Catheterization : O2 in RV > RA
ECHO : 2D & Doppler: number, size,
location

Ventricular septal defect (vSD)


CONTINUED

Management :
Nonsurgical closure : Amplatzer septal
occluder
Surgical : infant with large VSD + CHF

Prognosis :
Perimembranous : surgical intervention
Muscular defect : spontaneous become
small/ prolaps aorta , Infundibulum Stenosis,
PH, CHF, Endocarditis

VSD
VCS
VC I

RA

VP

LA

RV

LV

AP

AO

PATENT DUCTUS ARTERIOSUS (PDA)

PATENT DUCTUS ARTERIOSUS (PDA)

Incidence : 12 % CHD (no. 2), F >


M
Anatomy/physiology : diameter
mm - 1 cm
Intrauterine: AP d.Botalli
Aorta
Extrauterine: d. Botalli 1015 hrs
still open
L-R shunt (syst-diast)

TYPICAL PDA (SIMPLE PDA)


CONTINUED

Clinical
Manifestations :
Asymptomatic, recurrent
respiratory tract infection,
tachipneu
Continuous murmur at LSB2,
middiastolic murmur at apical

X-ray : PBF >>,

LVH, RVH

Echo : direction of

TYPICAL PDA (SIMPLE PDA)


CONTINUED

Management :
Surgical closure (ligation)
Nonsurgical closure : Amplatzer
Ductal Occluder

PDA
VCS
VC I

VP

RA

LA

RV

LV
AO

AP
DA

Pulmonary stenosis (PS)

Pulmonary stenosis (PS)


Incidens 5-7% of CHD , Abn
pulmonary Noonans syndrome
Asymptomatic
Difference of systolic pressure
between RV and PA > 100 mmHg
Hemodynamic :
RV activity increased RVH
Pulmonary ejection click (valve
opening)
Clinical Manifestation

Pulmonary stenosis (PS)


CONTINUED

X-ray : PBF <<, cardiomegaly


ECG : RAD, RVH
Echo :
thick pulmonary valve, dilated PA
Cineangio : a jet contrast
Management :
Balloon valvuloplasty
Surgery if balloon failt

COARCTATION OF THE AORTA (CoA)

CoA
CONTINUED

Narrowing of the aorta.


Frequency : 5 8% CHD, M > F
Location : distal of left subclavian
artery
2 types :
Preductal (CoA + Systemic LV/RV)
Postductal (CoA + Sytemic LV)

CoA
CONTINUED

Hemodynamic :
Adequate O2 to distal of CoA :
(Adaptation mechanism)
Increased systolic pressure at
proximal of CoA
Increased diastolic pressure at
distal of CoA (arterioles
vasoconstriction)
Collateral circulation (a
subclavian, intercostal, etc)

POSTDUCTAL CoA
Clinical Manifestations
Pain of calves, headaches,
epistaxis
Hypertension (pathognomonic)
Brachial Femoral lag
Reduced / abcent lower
extremity pulses

POSTDUCTAL CoA
CONTINUED

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

TETRALOGY OF FALLOT (TF)

TETRALOGY OF FALLOT (TF)


4 Defects :
VSD, PS, RVH, Overriding of the
Aorta
Frequency :
10-15% CHD, cyanotic CHD nr.1
(75%)
Hemodynamic :
PS + VSD R-L shunt
Cyanosis/ acyanotic pink TOF

TETRALOGY OF FALLOT (TF)


CONTINUED

Clinical Manifestation :
Clubbing fingers, scoliosis,
squatting position
Ejection systolic murmur LSB3-4,
single HS 2nd
Lab : Hb, Ht, RBC levels increased

TETRALOGY OF FALLOT (TF)


Echo :

CONTINUED

VSD, Overriding Ao, RVOT


obstruction

X-Ray :
couer en sabot, RVH, PBF ,
concav pulmonary segment

Complication :
Cerebral Infarction (age < 2 yrs)
Cerebral Absces (age > 2 yrs)

Treatment :
Surgery : palliative / total

VCS

TOF

AO
VCS
VC I

RA

VP
AKA
LA

RV

LV

AP

AP AKI

AO

VKI

VKA

AO
AKa

VKA

AP

AKI

VKI

TRANSPOSITION OF THE GREAT


ARTERIES (TGA)

TRANSPOSITION OF THE GREAT


ARTERIES (TGA)
Ventriculoarterial discordance, Ao RV
and PA - LV
Cyanotic CHD no.2, incidens 3-5%CHD, M
>F
Hemodynamic :
parallel pulmonary and systemic
circulation (cyanosis)
To prolong life : mixing of oxy- and
deoxygenated blood (ASD, VSD, PDA)
deficient O2 supply to the heart,
enlargement of the heart, heart failure

TRANSPOSITION OF THE GREAT


ARTERIES (TGA)

CONTINUED

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

DEXTROCARDIA

DEXTROCARDIA

CONTINUED

The heart is located on the right side


of the chest & the apex points to the
right.
Dextroposition is not a DIAGNOSIS.
Anatomy :

1. Visceroatrial relationship :
S (solitus), I (inversus) or A
(ambiguus)
2. Ventricular Loop : D (D-loop), L (Lloop) or
X (uncertain or undeterminate)
3. Great arteries (conotruncal) : S

DEXTROCARDIA
CONTINUED

Isolated mirror image dextrocardia


(IMID)
Kartagener syndrome:
Dextrocardia / situs inversus
Bronkhiectasis
Paranasal sinusitis

DEXTROCARDIA
CONTINUED

Clinical 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

ASD

VSD

PDA

SP

CoA

TF

TGA

DextrocardiaSolitus

DextrocardiaInversus

Dextrocardia

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