1. feb04
PEDIATRIC CARDIOLOGY RA RV LV LA
ICV
SCV PA AO
Normal Heart

2. GENERAL PRINCIPLES Pediatric Cardiology and Adult Cardiology
1. Congenital Heart Disease (CHD, PJB)
Occurs since organogenesis
Prevalence tends to increase
2. Acquired Heart Disease (AHD, PJD)
Prevalence tends to decrease

3. INCIDENCE CHD : 6-8/1000 live births 8 types of CHD (85%) :
VSD, ASD, PDA, PS, AS, TF, TGA
CHD in siblings : 3-4 x
Mortality : - 30% in the first months
- 10% before the age of 1 yr
AHD :
Neonatus : virus (Echo, Influenzea)
yrs : RF

4. ETIOLOGY CHD : 90% genetic – environmental factors
- genetic : Mendel - chromosomal abnormalities (Down’s)
- 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)

5. Non cardiac malformation :
% CHD (e.g. Cleft lips)
Kartagener Syndrome (bronchiectasis,
sinusitis paranasal, dextrocardia)
AHD : - infection (RF, diphtheriae)
- neonatus (Coxsackie B virus)

6. CONGENITAL HEART DISEASE (CHD)
Early signs of CHD
Cyanosis
Inadequate intake, dyspnea, irritable heart failure
Heart murmur
Unpalpable femoral and brachial pulse :
left heart outflow obstruction
Circulation collaps
Arrhythmia

7. FETAL CIRCULATION

8. FETAL CIRCULATION Parallel systemic and pulmonary circulations
Signs :
Parallel systemic and pulmonary circulations
Pressure : RV = LV
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
Placenta : gas exchange (respiration), nutrition,
excretion

9. CIRCULATION AFTER BIRTH
After birth : Expansion of lung
- Placenta circulation ended
Systemic and pulmonary circulation  serial type
Pressure : LV > RV
Opening of pulm.vascular bed  pulmo. vascular
resistance drops
Uptake of O2 increased  supply to the whole body
No cross circulation in RA
Foramen ovale, d. Botalli & d. Venosus  closed

10. 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 :
measurment of arterial O2 content (N=95%).

11. 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 -)

12. Central C :
a. Pulmonary C :
Lung disorders (tissue, blood vessels, hamodinamic)  diffusion, ventilation, perfusion
 Cause : - intra & extra thoracal
- not a CHD
b. Cerebral C:
brain disorders  center of respiration
e.g. : injury, infection / meningitis

13. c. Intracardial C:
Hyperperfusion of pulm. circulation + R-L shunt
e.g. PS + VSD
Mixed of pulmonary and systemic circulation
e.g. TF
Separated of pulmonary and systemic circulation
e.g. TGA (parallel circulation)
Pulmonary obstruction
disturbance of O2 uptake, L-R shunt

14. Hyperoxic (100% O2) test / Crying :
pulmonary C  less/no C
intracardial C  C still persist
Peripheral C
 Decreased cardiac output
e.g. - MS. PVO, PS, AS
- hemodynamic disturbances

15. ATRIAL SEPTAL DEFECT (ASD)RA LA RV LV

16. ATRIAL SEPTAL DEFECT (ASD)
Any opening (defect) in the atrial septum  shunt
3 types :
1. Ostium Secundum (50-70%)  fossa ovalis
2. Ostium primum  lower part of atrial septum
3. Sinus venosus defect (entry of SVC/IVC into RA)
4. Atrioventriculer Canal (AV canal = AVSD)

17. Hemodynamic : depends on the size, compliance of V
and resistance of Pulm. and Syst. circulation
- Neonatus : R-L shunt, mild cyanosis
- Infants/Children : L-R shunt, no cyanosis
- Bidirectional shunt
Signs/Symptoms :
Usually asymptomatic, mmr is found by chance
Fatigue, dyspnea, recurrent respiratory infection.

18. Phys.Exam :
Relatively slender body (gracile habitus)
Normal BP and pulse
Cyanosis  PH
Insp./palp. : increased activity at lower LSB
Ausc. : ( mmr may be absent in infants)
widely split and fixed S2,
2-3/6 systolic ej.mmr at LSB2  interscapular

19. X ray : enlargement of RA and RV
prominent PA segment and increased PBF
ECG : RAD, RVH/ RBBB with rsR’ pattern in V1
Echo : 2D is diagnostic
- position and size of the defect
- indirect signs of L-R shunt (RAE, RVE, PA>>)
Catheterization : O2 in RA > CV

20. Management CHD be treated to favour the spontaneous closure of ASD
Transcatheter closure (Amplatzer Septal Occluder)
Surgical closure :
Indication : P / S ratio ≥ 1.5 : 1
Contra Ind : high pulm.vasc.resistance
Timing : after 3-4 yrs except for infant with CHF
Mortality : < 1%

21. Prognosis: location and diameter :
foramen ovale  good
AV Canal  poor
Possibility of spontaneous closure 30%
conservative until 10 yrs
Complication : CHF,CVA, HP, PVO, Infection (SBE, Pni)

22. VENTRICULAR SEPTAL DEFECT (VSD)RA LV LA RV

23. VENTRICULAR SEPTAL DEFECT (VSD)
Defect in the ventricular septum
Prevalence : CHD nr.1 (25%)
Pathology :
diameter : 0,5 - 3 cm
permembranous & muscular portion
no septum  single ventricle
VSD + (ASD, PDA, Co.A, PS)

24. Embryology : disturbance of inferior V septum development
Hemodynamic :
Depends on the size and pressure between RV and LV
Pressure LV > RV  L-R shunt
L-R, R-L (Eisenmenger S)  VSD, ASD
Defect <<  small shunt
Defect >>  large shunt
Septum muscle contraction  diameter
Other lesions

25. SIMPLE VSD
20 % of CHD, 25 % of VSD
Small 1-5 mm, Moderate 5-10 mm, RVH (-)
Asymptomatic : Roger’s disease
Normal G-D, mmr heard at Week 1
MODERATE VSD
fatigue, intol.activity, dyspnea, recurrent resp.tr infection
Solid food : - better growth, defect <<  normal child.

26. Thorax
mild vousure cardiaque
Thrill at lower LSB
Normal S1 & S2
Pansystolic (holosystolic) 3-4/6, pm LSB 3-5

27. X-ray : - normal heart / slightly enlarged
- AP : normal / slightly enlarged
- PBF increased
ECG : Small VSD  normal
Moderate VSD  LVH (+LAE)
Large  CVH (±LAE)
Catheterization : O2 in RV > RA
ECHO : 2D & Doppler: nr, size, location

28. Management :
Treatment of CHF
No surgery on infant wthout CHF/PH
Nonsurgical closure : umbrella device
Surgical : infant with large VSD + CHF not
responded to drugs, P/S ratio > 2 : 1
Complication : SBE, HP
Prognosis : - Spontaneous closure depends
on the position of VSD.
Perimembranous : surgical intervention
Muscular defect : spontaneous closure

29. PATENT DUCTUS ARTERIOSUS (PDA)LV AO AP LA RV RA

30. PATENT DUCTUS ARTERIOSUS (PDA)
Incidence : 12 % CHD (nr. 2), F > M
Anatomy/physiology : diameter mm - 1 cm
Intrauterine: AP  d.Botalli  Aorta
Extrauterine: d. Botalli 10–15 hrs still open thin wall, circular smooth muscle .
During the last week of pregnancy :
D. Botalli is closing
Decreased blood flow through the ductus
Increased pulmonary blood flow

31. D. Botalli :
1. Prostaglandin tipe E1  vasodilator, open
2. Increased fetal blood O2 level  close
3. Respiratory disturbances  open
4. Pulm. Vascularization/ PA pressure  open/close
RV - LV pressure  direction of shunt
L-R shunt (syst-diast)  continuous mmr (+ 90% PDA)
R-L, L-R, R-L shunt  Eisenmenger S
Pressure P > S  R-L shunt, diastol mmr (-)
Continuous mmr (-)  Eisenmenger S, neonatus
10 % PDA + PH (atypical PDA)

32. TYPICAL PDA (SIMPLE PDA)
Clin. Manifestations :
asymptomatic, undernourished, recurrent resp. tr.infection
Large shunt : dyspnea, intolerance activ., pulsus celer
Thorax LVA  , thrill LSB2
S1 N, S2 split, continuous mmr at LSB2
split paradoxus (heard at expiration) :
- maximal shunt  maximal LV filling long syst early closure of pulm.valve
X-ray : small shunt  N heart
Large shunt  enlargment LA, LV , PA , Ao. PBF 
ECG : N or LVH
large shunt CVH, LAE

33. Cath. : O2 level increased at PA Echo : direction of shunt & Ø PDA
Prognosis : N life, rarely closed spontaneously (1 yr),
except in premature babies)
Complication : SBE, CHF, PVO
Management :
Surgical closure (ligation)
Nonsurgical closure : Amplatzer Ductal Occluder
Indomethacin, not for term infants

34. PULMONARY STENOSIS (PS)RV LV LA RA AP AO

35. PULMONARY STENOSIS (PS)
10% of CHD
Difference of syst.pressure between RV and PA > 100 mmHg
Hemodynamic :
Pressure of PA > LA  RV activity increased  RVH
Severe PS  RHV  infundibular stenosis (IS)  CO <<  peripheral cyanosis
PS + VSD  R-L shunt (intracardial cyanosis) rarely CHF

36. Clin.Manif. depends on the degree of stenosis:
Mild : normal G & D
Mild-moderate : asymptomatic
Severe : right CHD, cyanosis, clubbing fingers
Thorax : RVA , thrill
Pulmonary ejection click (valve opening)
S 1 N, S 2 N/>/<, S 4 (atrial contraction)
Eject. Syst mmr LSB2
X-ray : enlargement PA, RA & RV >>, PBF <<, cardiomegaly
ECG : mild PS : N
moderate PS : RAD
severe PS : RVH (+RAE)

37. Catheterization : - moderate PS : low PA pressure
- severe PS : RA pressure increased
- difference of pressure between PA & IS
Echo : thick pulmonary valve cups, PA dilated
pressure gradient across the stenotic valve
Cineangio : a jet contrast
Prognosis : mod/severe PS tends to progress with age
severe PS : CHF & cyanosis, sudden death
Management : - Balloon valvuloplasty
- Prostaglandin E1 (reopen the ductus in newborn)
- Surgery if balloon failt

38. COARCTATION OF THE AORTA (CoA)
KoA LA LV RV RA AO AP

39. 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)

40. Postductal CoA Clin.Manifestations Asymptomatic
Mmr and hypertension are found incidentally
Pain of calves, headaches, nose bleeds, epistaxis
Growth of lower body be compromised
BP discrepacies between upper and lower extremities (pathognomonic)
Adolescent : easily fatique and intolerance activity
Early / later life : CHF

41. Brachial – femoral lag
Reduced / abscent lower extremity pulses
Lower legs : cold, shorter and pain in calves
Deformity of chest, suprasternal pulsation
LVA increased, collateral vessels pulsation
Loudly S2, paradoxus split
Systolic mmr: left infraclavicular / under scapula
continuos mmr : collateral / severe CoA

42. X-ray :
Cardiomegaly, prominent LA and LV
Rib notching (collateral vessels)
E sign on barium meals
ECHO / Doppler: gradient and pattern of diastolic flow
ECG : neonate : RVH
late onset : LVH, ischemia
Catheterization : confirmation of diagnosis
Prognosis : early presentation : CHF
late presentation : hypertension
Management :
Prostaglandin E1 to open the ductus
Surgery, balloon angioplasty

43. PREDUCTAL CoA Babies : CHF and cyanosis Systolic BP arms > legs
X-ray : - no E sign or rib notching
- cardiomegaly
Died usually at the first days / weeks

44. TETRALOGI OF FALLOT (TF)
VSD PS
OvA
RVH AO AP LV LA RA

45. TETRALOGI 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 diameter of VSD, PS, systemic vascular pressure
Limitation of RV pressure  CHF (-)
Small babies less cyanosis (mild PS)
R-L shunt  polycytemia & tromboemboly

46. Clin.Manifestation : G retard, fatique & dyspnea, clubbing fingers, retinal engorgement, scoliosis, squatting position, hemopthysis
Thorax : RVA >>, thrill lower LSB
S1 N, loud S2
Ejection systolic mmr LSB3-4
Lab : Hb, Ht, RBC levels inreased

47. Echo : VSD, overriding Ao, RVOT obstruction
X-ray : couer en sabot, RVH, PBF 
ECG : RAD, RAE, RVH
Complication :
cerebral Infarction (age < 2 yrs)
cerebral absces (age > 2 yrs)
Polycytemia

48. Coagulation disorders Terapi : Iron intake Prevention of dehydration
Relative iron deficiency anemia (Ht < 55%)
SBE
Right CHF (very rare)
Coagulation disorders
Terapi :
Iron intake
Prevention of dehydration
Squatting
Surgery : palliative / total correction

49. TRANSPOSITION OF THE GREAT ARTERIES (TGA)RV LV AO AP LA RA

50. TRANSPOSITION OF THE GREAT ARTERIES (TGA)
Ventriculoarterial discordance, Ao – RV and PA - LV
Cyanotic CHD nr.2, M > F
Embryology :
No spiral division but straight line division of arterial trunk
Anatomic subtypes :
1. TGA with intact ventricular septum
2. TGA with VSD
3. TGA with VSD & LVOT obstruction
4. TGA with VSD & PVO s

51. Hemodynamic :
parallel pulmonary and systemic circulation
prolong life : mixing of oxy and deoxygenated blood (ASD, VSD, PDA)
deficient O2 supply to the heart, enlargement of the heart, myocard failure (CHF)

52. Clinical manifestations
- TGA with intact ventricular septum :
cyanosis within hours after birth
- TGA with VSD :
mild cyanosis and signs of CHF, RVA >>, pansyst.mmr
- TGA with VSD & LVOT obstruction (as TF) :
extreme cyanosis, single S2, syst.ej.mmr
- TGA with VSD & PVO s. :
cyanosis, single S2, no mmr

53. X-ray : type 1 : like an egg on its side
type 2 : Cardiomegaly, PBF 
type 3 : bootshaped heart (=TF)
ECG : RAD
Catheterization : confirm the Echo findings
Echo : double circle, parallel PA & Ao
Prognosis : depends on the lesions
Management :
Prostaglandin E1 to maintain ductal patency
Balloon atrial septostomy
Surgery palliative or arterial switch procedure

54. DEXTROCARDIA VCS AO AO AP AP LA LA LV RA LV RA RV RV VCI Normal heart
Isolated Mirror Image Dextrocardia
Normal heart

55. 1. Visceroatrial relationship :
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)
3. Great arteries (conotruncal) : S (solitus), I (inversus),
D (D-transposition) or L (L-transposition) AP LA RA
VCI

56. Normal heart with situs solitus (S,D,S)
Isolated mirror image dextrocardia (I,L,I)
Sindrom Kartagener : dextrocardia, bronkhiectasis, paranasal sinusitis
Associated lesions : TGA, TF, PS

57. loudest heart sound on the right chest IMID 50-80% without CHD
Clin. Manifestations :
loudest heart sound on the right chest
IMID 50-80% without CHD
Cyanosis : PS or PVO
Acyanosis : ASD, VSD, single ventricle
X-ray IMID: liver – left, stomach bubble- right
Echo : dextrocardia
ECG :IMID :P axis +90 to +180, Q in V5R and V6R
Prognosis : depends on the lesions
Treatment : to overcome the associated lesions