This document discusses clinical congenital heart disease (CCHD) and provides classifications and descriptions of various conditions. It classifies CCHD into cyanotic types with increased or decreased pulmonary blood flow, acyanotic stenotic types, and unclassifiable types. For each type, it describes symptoms, physical exam findings including auscultation findings, and chest x-ray appearances. It also discusses unusual causes of cyanosis, Fallot physiology, complications of CCHD with decreased pulmonary blood flow, and keys to clinical diagnosis including focusing on pulse characteristics and the second heart sound.

1. Clinical congenital heart
disease
Prof M S Ranjit MD DCH
Senior consultant paed. Cardiologist
Chennai.

2. Some clinical aspects
“paediatric and adolescent accent”
 Classification (modified for simplicity)
 cyanotic - with ↑ pulm blood flow
- with ↓ pulm blood flow
- unclassifyiable – ebsteins/ TGA IVS
 acyanotic – largely shunt lesions
 stenotic - outflow & arterial obstructions

3. Cyanosis
caused by > 5gm/dl reduced Hb
 Clinical detection depends on
- % arterial blood that is desaturated
- Hb Concentration !!
 If art O2 satn is 60%,
cyanosis is detectable if Hb > 12.5gm/dl !
but not if Hb < 10 gm/dl !
ie 4gm/dl insufficient for detection of cyanosis !

4. Detection of cyanosis
 Astute physician/ paed cardiologist
detects when reduced Hb 3 gm/dl
Others detect at 5gm/dl
 Better to overdiagnose than underdiagnose !
 Clinical diagnosis of cyanosis is inaccurate
M Tynan in Andersons Paediatric cardiology 2007

6. Cyanosis -some aspects
 Some CCHD with Rt to Lt shunt and ↑ P B flow
UO TAPVR/ Truncus/ TGA-VSD/ Single ventr Physiol etc
- may have low saturations
- but undetectable cyanosis clinically
i.e. 88-92% !!
 Polycythemic patients appear cyanosed
 Methhaemoglobinaemia !!

7. Hyperoxic test
cyanosed or not
 Pulse oximeter - not always reliable
“a random number generator”
 Rt radial ABG in air and after 5-10 min O2
paO2 > 250mmHg -excludes CCHD
paO2 > 160 -CCHD unlikely
( UO TAPVR False negative !)
paO2 < 100 -CCHD likely (usually lower)
(severe Lung disease (high paCo2), PPHN/PFC)

8.  “Radial ABG more useful than ECG or CXR
in detection of cyanotic heart disease”
Warburton 1981

9. C C H D
in 3 major circumstances
 Pulmonary obstruction with avenue for right to
left shunting
 Discordant AV connection i.e transpositions
 Common mixing situations i.e common atrium
single ventricle etc

10. Unusual causes of cyanosis
without murmurs !
surviving to adolesc./ adult life
 Left SVC to LA
 IVC to LA
 Rt. SVC to LA
 Pulm. AV Fistulae (Ostler Rendu Weber syndr)

11. LV

12. Cyanosis – which category?
 Symptomatology
 Clinical examination
 Chest X ray

13. Fallot physiology
 Systemic venous return unable to reach lungs
 Shunted right to left away from pulm circulation
 ASD/VSD essential for this to occur;
 Or a common chamber !
PLUS

14. Fallot physiology
 Obstruction at
- RA outlet - i.e Tric atresia
- infund/valvar Pulm stenosis
- rarely branch PA stenosis/ DCRV
- High PVR – Eisenmenger !
obstructed pulm arterioles !!

15. CCHD with ↓ pulm blood flow
pulmonary oligaemia on CXR
 Symptomatology
 Inspection findings
 Auscultatory findings
 Chest Skiagram

16. CCHD with ↓ PBF - symptoms
 Exertional dyspnoea
 Cyanosis, spells, seizures
 CNS complications
 No recurrent RTI/ no diaphoresis
 No breathlessness at rest
except in extremes / anaemia

17. CCHD - ↓ PBF
- inspection /palpatory findings
 Cyanosis & clubbing
 polycythemia
 Quiet precordium to inspection & palpation
 No Harrisons sulcus or precordial bulge
 Apex well within limits if visible
 No palpable sounds or thrills

18. CCHD with ↓ PB Flow
auscultatory findings
 Normal first heart sound
 Single second heart sound
 Pulm component inaudible
 Stenotic pulmonary murmur
slightly after S1
stops short of S2
 Other murmurs – ductal/ MAPCA/ AR

19. Ejection murmur in Fallot physiology
 Length & loudness inversely proportional to
severity of stenosis
In isolated PVS – the opposite !
 Absent murmur – acquired pulm atresia
- MAPCA murmur over back
- soft ductal murmur (tortuous)
 To & Fro – Aortic regurg / Abs PV syndrome

20. MAPCAS

21. CCHD with Pulm.blood flow
 Tetralogy of Fallot
 VSD - PS
 DORV – VSD – PS
 Tricusp. atresia - PS
 Single ventricle - PS
 TGA with VSD – PS
 Corr.transp.-VSD-PS
 ASD - PS

22. Chest skiagram in CCHD with ↓ PBF
 Small heart
 Pulmonary bay
 Pulmonary oligaemia
 Right aortic arch/ RA enlargement/ differential
vascularity/ narrow pedicle in various defects

23. Tetralogy of Fallot

24. Typical - Fallot CXR
Pulm bay
RV apex
Pulmonary
oligaemia

25. Tricuspid atresia
Normally related great arteries
Restrictive VSD
PFO / ASD
VSD / PDA
Fallot physiology

26. Fallot physiology
Tricuspid atresia

27. Transposition of the
Great arteries
With V S D and P S
TGA-VSD-PS
Fallot physiology

28. LV
RA
LA
PA
AO
Double inlet
Left ventricle
With PS CCHD with PB Flow
Single ventricle with PS
Fallot physiology

29. Atrial septal defect with pulmonic stenosis
ASD with PS
Fallot physiology
Fallot physiology
S2 variable
Pulm ESM

30. Corrected Transposition with VSD and PS
Atrio-ventricular &
ventriculo-arterial
Discordance
LV
RVRA
LA
AO
PA
Fallot Physiology
Fallot physiology
Single S2
Loud A2
pulmonic ESM

31. CNS complications of CCHD with ↓ PBF
 Paradoxic embolus
 Cerebral thrombosis
 Cerebral abcess
 Seizures
 Hypoxic damage
 Endocarditis & vegetations
 Postoperative strokes

32. CCHD with ↑ pulm blood flow
 Transpositions with VSD/Duct/ASD
 Common mixing situations
atrial level – TAPVR/Comm Atr
Mixing at ventric level – DORV/Single ventric
arterial level – comm art trunk
 Mild cyanosis, CCF, resp symptoms, ex dyspnoea

33. CCHD with ↑ Pulm blood flow
 Seldom survive to adolescence/ adulthood
 UO TAPVR/ comm atrium- the exceptions
 Most have Eisenmenger by then
and those features dominate

34. CCHD ↑ P B Flow
easy diagnosis – rare
 Clinical differentiation not always possible
(Tynan M, Andersons paed cardiology 2007)
 Brisk pulses, ej click, to& fro murmur – Truncus
 Sm. pulses, RV impulse, wide split S2,TV MDM – TAPVR
 AV regurg murmur, wide split, TV MDM – comm. atrium
 Sing S2, cont murmur over back – p atr / MAPCAS

35. CCHD with ↑ P B Flow - symptoms
 Respiratory symptoms predominate
 Growth retarded – weight & height
 Scrawny, sick, dyspnoeic patient
 Recurrent LRTI/Pneumonias
 Chronic lung disease- bronchiectasis etc
 Diaphoresis/ breathlessness at rest
 Exertional dyspnoea, limited activity.

36. CCHD with ↑ P B Flow
inspection findings
 Sickly underweight individual
 Cyanosis & clubbing -mild to moderate
 Severe PHT, Eisenmenger – modifies findings
 Harrisson’s sulcus, precordial bulge
Active precordium, RV, LV, PA pulsations
Obvious cardiomegaly

37. CCHD with ↑ P B Flow
palpatory findings
 Active precordium
 RV impulse – DORV, TAPVR, TGA VSD PS
 LV Impulse – Single ventricle, AVSD-AV regurg
 Palpable second sound / Thrills rare

38. Eminently operable Operable but PHT
Eisenmenger

39. CCHD with ↑ P B Flow
auscultatory findings
 Single second heart sound
 Loud pulm component, if heard
 Ejection click – pulmonary/ truncal

40. CCHD with ↑ P B Flow
auscultatory findings -2
 Pulm flow – ejection murmur
 MD murmur - if no severe PHT/ Eisenmenger
 PR/ TR murmurs may dominate
 To & fro murmurs in- Truncus/ abs PV syndr.
 MR murmur in complex AVSD /comm Atrium

41. Double outlet right ventricle with VSD & PAH
Normally related great arteries
DORV – VSD - PAHCCHD with
P B Flow
RV impulse
Single S2 - loud
Pulm ESM
Mitral MDM

42. Common atrium
CCHD with P B Flow
Often complex venous anatomy
VSD physiology
RV impulse
Wide split S2
Tricuspid MDM
Pulm. ESM

43. Transposition of the
Great arteries
With V S D

44. Truncus arteriosus
CCHD with P B Flow
Brisk pulses
Ejection click
Non specific ESM
EDM if truncal regurg.
Mitral MDM

45. Supra cardiac
Cardiac
Infra cardiac
Total anomalous pulmonary venous return
TAPVR
VSD physiology
ASD on
auscultation
Mild cyanosis

46. CCHD with ↑ P B Flow
radiographic findings
 Cardiomegaly (unless sev. PHT/Eisenmenger)
 Dilated PA
 Pulmonary plethora
 Atrial enlargement
 RV/LV/ Biventric. -Depends on anatomy/age

51. Keys to clinical diagnosis
 Work in order
 Pulses,pulses, pulses
 Colour ie. Cyanosis, pallor, polycythemia
 Inspect – for chest form, pulsations
 Palpate to determine – which ventricle ?
 Forget the murmur !!
 Listen first to S1, and then to S2
 Can you split the second sound ??
 Then concentrate on the components
 Finally the murmurs – systolic – ejection or pansyst.
 Is there a diastolic murmur

52. The second heart sound
the key to diagnosis of CHD
 Single
 Normal split
 Wide variable split
 Wide fixed split
 Reverse split
 Loud A2
 Loud P2

53. Unclassifiable CCHD
 TGA–IVS – do not survive
 Ebsteins – may have features of CCF & ↓PBF
cyanosis, cardiomegaly
multiple sounds, wide split, soft P2, Sail sound
TR murmur, MDM, scratchy sounds
 P Atresia IVS – seldom survive infancy

55. Acyanotic CHD
Stenotic CHD
 Few issues
 ASD, VSD , PVS, AVS too well known to
talk about

56. AAO ARCH
DAO
PA
Coarctation of aorta
COA

57. Localised coarct
membrane

58. Collateral
circulation
in
coarctation
1
2
3
Adapted from
Amplatz radiology in CHD

59. Cxr coarct adult – rib notching

61. Coarctation of aorta
 Asymptomatic adults – collaterals
 Hypertension !
 Femorals !!
 Bicuspid AV in 80% - ejection click !
 Collateral murmur over back
 AVS

64. DD of a continous murmur
 With or without cyanosis ?
 Continous or a To & Fro murmur ?

65. Continous murmurs without cyanosis
 PDA (Patent arterial duct)
 AP Window
 Venous Hum
 Coronary AV Fistula
 ALCAPA
 RSOV
 Periph Pulm. Stenosis
 Systemic AV Fistula
 Collaterals in COA
 Mammary Souffle
 Aortico-LV tunnel

68. AO
PA
LV
MR
AO
RA
Fi

69. LV
AO
LA
P
AR
RV
LA
LA

70. Continous murmur with cyanosis
 Duct in Tetralogy
 Pulm Atresia with Duct
 MAPCAS in Pulm atresia
 Supracard. TAPVR
 Pulm AV Fistulae
 Post BT shunt (Thomas-Blalock-Taussig shunt)
 Post - Pott’s, Waterston, Central shunts

71. Thomas-Blalock-Taussig
shuntWaterston shunt
Pott’s shunt
Central shunt

72. To & Fro Murmur
without cyanosis with cyanosis
 VSD AR
 MR AR
 AS AR
 PS PR
 Post op Tetralogy
 MR AR
 TR PR etc
 Tetralogy with AR
 Truncus with regurg
 Absent PV syndrome

73. LV

74. RV
PA
PR
LV
PV
AO
PA
PR

75. VSD
outcome
CCF > FTT > marasmus
pneumonias / death
PHT / PVOD / Eisenmenger
Infective endocarditis
Aortic prolapse & regurg.
Mitral regurgitation.
LV to RA shunts
RSOV
Infundibular pulm. stenosis
VSD gets smaller
spontaneous closure
Surgical closureArrhythmias
LV dysfunction
Subaortic
membrane

76. thanks
Thanks