1. Congenital Heart Disease
Cardiac Malpositions
Approach to Diagnosis

4. Congenital Heart Disease
Malpositions : Diagnosis
Identify situs
Identify chambers and vessels
Arrange chambers and vessels
Identify additional anomalies
Define route of blood flow
Diagnosis =

6. Congenital Heart Disease
Situs
Solitus usual normal
Inversus mirror image normal
Ambiguous Indeterminate
Aplenia, Polysplenia,
Palpation, Percussion, X-ray

7. Congenital Heart Disease
Malpositions : Asplenia
Absent spleen
Malrotation of bowels
Symmetrical liver
Complex CHD
(Bilateral – right sidedness)

9. Congenital Heart Disease
Malposition : Polysplenia
Multiple spleens
Malrotation of bowels
Symmetrical liver
Complex CHD
(Bilateral left sidedness)

12. Atria RA LA
Ventricles RV LV
Great arteries PA AO

16. Congenital Heart Disease
Sequential Chamber Localisation
Define situs
Visceroatrial situs rule
Tracheal bifurcation
ECG
ECHO
Angiogram

17. Congenital Heart Disease
Atrial Situs
Morphologically right atrium
Receives IVC
Broad appendage
Crista terminalis
Fossa ovalis

18. Congenital Heart Disease
Atrial situs
Morphologically left atrium
Finger like appendage with narrow neck
Valve of fossa ovalis

19. Congenital Heart Disease
Atrial situs
Visceroatrial situs rule
ECG – unreliable
Thoracic roentgenogram – bronchial morphology
Echo – IVC to RA
Femoral vein – contrast inj.
Angiogram – shape of appendage

20. Congenital Heart Disease
Atrial situs : Viscero atrial situs rule
Stomach, spleen and LA
Liver & RA Same side
Not applicable with
Situs ambiguus
Asplenia - bilateral RA
Polysplenia - bilateral LA

21. Congenital Heart Disease
Situs rule : fallacies
Thoracoabd. discordance %
(No splenic abn.)
Solitus or Inversus predicted %
as ambiguus
Midline liver Do no predict %
Absent spleen S. ambiguus

22. Congenital Heart Disease
Atrial situs : Situs rule
87 cases : Levo, meso, dextrocardia
Position of liver (central = ambiguus) 37%
Liver & Howell Jolly bodies 45%
Rt or Lt sided liver (no polysplenia) 59%

23. Congenital Heart Disease
Atrial situs : situs rule
3 lobed lung RA
2 lobes lung LA
Accuracy 72%

24. Congenital Heart Disease
Atrial situs : Tracheal bifurcation
RA follows Rt bronchus
LA follows Lt bronchus
Asymmetrical division
Symmetrical – asplenia, polysplenia

26. Congenital Heart Disease
Atrial situs : bronchial morphology
LA follows left bronchus – Hyparterial, horizontal, longer
RA follows right bronchus – Eparterial, vertical, shorter

28. Bronchial length (cm) 6
Left
Right 5 4 3 2 1 2 4 6 8 10 12 14 16
Age (Yrs.)

29. Congenital Heart Disease
Malpositions : Atrial situs
Bronchial anatomy : Length
Left bronchus :  2 cms
Right bronchus :  2 cms
(overlap below age 2 yrs.)

30. Congenital Heart Disease
Atrial situs : bronchial anatomy
Lt / Rt bronchus
2.0 or  Normal or Inversus
1.5 to 2.0 Tomography
1.5 or less Atrial isomerism

31. Congenital Heart Disease
Atrial situs : ECG
P  in 1, II, aVF Normal
 in aVR
P  in I, II aVF Inversus
 in aVR

32. Congenital Heart Disease
Atrial situs
‘P’ wave specificity poor
i Sinus node – left side
ii Ectopic atrial rhythm
iii Bilateral sinus node
(asplenia, polysplenia)

33. * S V C LA RA
IVC
LIVER

34. LA RA 34

35. RA CS LV RV 35

36. LAA LA
RAA LV RA 36

38. Liver

39. Liver

41. Liver

44. Congenital Heart Disease
Ventricular relationship
d loop RV Right side
LV Left side
l loop RV Left side
LV Right side
Indeterminate : Sup. Inferior vent.

45. Congenital Heart Disease
Ventricular identification – ECG
QRS morphology RV : LV
rS, Rs : 1
rsR, rSR, rRs 8 : 1
qRS, qR : 3
QRs, QR, Qr 1 : 5

48. Congenital Heart Disease
Ventricular identification : Echo
Short axis – mid LV level
4 chamber – apex – coarse trabeculation
& moderator band - RV
Papillary muscle orientation of LV
MV / TV – attachment (4 chamber)
Short axis – fish mouth – MV

49. LV

50. RV LV

51. LA RA RV LV

52. RV LV

56. Congenital Heart Disease
Chamber localization : Angiogram
IVC RA
RA : Broad appendage
LA : Finger like appendage
RV : Coarse trabeculation
LV : Fine trabeculation
(two views : AP/Lat, RAO / LAO)

57. Congenital Heart Disease
Establish
Atrio-ventricular relationship

58. LA RA V 58

59. LA RA LV RV

60. RA LA RV LV

61. Congenital Heart Disease
Great vessel relationship
Normal
Transposition
Malposition
Single artery

62. Congenital Heart Disease
Great Vessels : Normal
Atrioventricular concordance
Both ventricles present
PA from RV – ant & to left
Ao from LV – post & to right

63. Congenital Heart Disease
Great arteries : Transposition
PA from LV, Ao from RV
Vent. septum present
d loop : RV Ao Transposition
LV PA
l loop RV Ao CCTGA

64. Congenital Heart Disease
Great arteries : Malposition
All GV relations in SV
D.O. RV
D.O. LV
d loop - abn. relation
l loop - abn. relation

65. Congenital Heart Disease
Great arteries : Single vessel
Persistent truncus arteriosus
Pulm. atresia
Aortic atresia

66. P L R A

67. P R L A

68. P R L A

69. Congenital Heart Disease
Great arteries : Echo
Single
Two GV :
i) One circle the other wrap
around – normal.
ii) Two circles – transposition &
malposition

71. Normally related Great Vessels
Great Vessels in Situs Inversus PA Ao PA Ao

72. a b

73. a b

74. D Malposition
L Malposition Ao PA LA RA Ao PA

75. PA Ao TR RV RA

76. RV
RPA LV TR TR LA Ao RA

77. A L R P

78. A R L P

79. A L R P

80. Congenital Heart Disease
Establish :
Ventriculo–arterial relationship

81. MPA
RPA Ao Ao LV RA RV RA 81

82. PA Ao RA LV LV RV 82

83. RV Ao PA PA RV RA LV RA LV

84. Congenital Heart Disease
Malpositions : Diagnosis
Identify situs
Identify chambers and vessels
Arrange chambers and vessels
Identify additional anomalies
Define route of blood flow
Diagnosis =

85. RA LA RV LV PA Ao

86. LA RA RV LV Ao PA

87. RA LA LV RV Ao PA

88. Thank you

89. Congenital Heart Disease
Asplenia Syndrome
Symmetrical liver
Stomach : L, R, Midline
Bilateral Rt. Bronchus
Bilateral SVC, single atrium
IVC, Ao same side in Abd.
Common AVC, Single Vent
TGA & Pulm. Atresia, TAPVC

90. Congenital Heart Disease
Polysplenia syndrome :
Symmetrical liver (less common)
Malrotation of bowels
Bilateral left bronchus
Bilateral SVC, Inferior vena cava - azygos or
hemiazygos continuation
PAPVC, TAPVC
Bilateral LA, ASD, ECD, Single A
VSD, Single V.
(DORV, TGA with PS rare)

91. Congenital Heart Disease
Malposition : S. Ambiguous
Abd. Viscera
Bronchial anatomy
Peripheral smear – Howell Jolly bodies
ECG
Splenic scintigraphy
US Abdomen Multiple spleen
Absent spleen
Intrahepatic IVC

92. Congenital Heart Disease
Malposition : Asplenia
Blood picture
Polycythemia
Howell Jolly bodies
Siderocystosis
Target cells
Heinz bodies

93. Congenital Heart Disease
Malposition Asplenia (21)
PS or Pulm. Atresia 18
Heterotaxy 17
ASD / Single A. 19
VSD / Single V. 20
Bilateral SVC 13
Howell Jolly bodies 13
Bilateral 3 lobed lung 12
ECD