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 (young/old), high
geografic location (less O2), metabolic disorders (DM),
Down syndrome (50% with CHD)
AHD :
infection (RF, diphtheriae)
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
Minimal pulmonary circulation
CIRCULATION AFTER
BIRTH
After birth :
Expansion of lung & O2 uptake
Systemic and pulmonary circulation serial
type
No cross circulation in RA

Foramen ovale, d. Botalli & d. venosus

closed
 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 content (N=95%)
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
Decreased cardiac output
 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

LVH or CVH RVH LVH RVH

VSD ASD AS or AR PS
PDA PAPVR COA COA
ECD PVOD MR MS

Cyanotic

PBF PBF

LVH or CVH RVH LVH RVH

CVH

Single Ventricel TGA AT TOF

TGA + PS
TGA + VSD HLHS AP + Hypoplastic RV PVOD
Congenital heart
disease (CHD)
Etiology
Unknown
Mothers disease (TORCH) Rubella
Medicine : fenitoin, Alcohol, lithium
Radiation
Genetics (dominan autosomal)
Syndrome (Down, Noonan,Turner)
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
resistance of Pulm. and Syst. circulation
 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 N , P2 intensity
N
ATRIAL SEPTAL DEFECT
(ASD) CONTINUED

X-ray:
increased PBF , RA and
Pulmonal Conus protrude
ECG :
RAD, RVH
Echo :
position and size of the defect
Catheterization :
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
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
R-L, L-R, R-L (Eisenmenger S)
 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)

ECG and X Ray : normal

 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
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) continuous
murmur (+ 90% PDA)
 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 shunt & PDA
Prognosis : rarely closed spontaneously (1 yr),
except in premature babies
 TYPICAL PDA (SIMPLE PDA)
CONTINUED

Management :
Surgical closure (ligation)
Nonsurgical closure : Amplatzer Ductal
Occluder
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
Eject. Syst murmur LSB2
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
R-L shunt polycytemia & tromboemboly
 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)
CONTINUED
Echo :
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 correction (BT
Shunt, Glenn, Waterstone)
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 (L-loop) or
X (uncertain or undeterminate)
3. Great arteries (conotruncal) : S (solitus), I (inversus),
D (D-transposition) or L (L-transposition)
 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
Whats
next?
ASD
VSD
PDA
SP
CoA
TF
TGA
 DextrocardiaSolitus

DextrocardiaInversus

Dextrocardia
 VCS
TOF
AO
AP AKI
VCS VP
VC I AKA

VKI
RA LA VKA

T M
AO
RV LV

AKa AP
AO AKI
AP

VKA VKI
PDA
VCS VP
VC I

RA LA

T M

RV LV

AO
AP

DA
VSD
VCS VP
VC I

RA LA

T M

RV LV

AO
AP
ASD
VCS VP
VC I

RA LA

T M

RV LV

AO
AP