SEGMENTAL ANALYSIS OF CHD

1. SEGMENTAL ANALYSIS OF
CONGENITAL HEART DISEASE
Murtaza Kamal M.D., D.N.B.
Fellow Pediatric Cardiology
murtaza.vmmc@gmail.com (Dt: 12-14/12/2017) 1

2. MAUDE ELIZABETH SEYMOUR ABBOTT
1936
McGill University, Canada
1st
systematic study
Based on study of 1000 heart specimen
2

3. Richard Van praagh
• 1964
• Pediatric Cardiologist
• Boston Children
Hospital, Massachusetts
• Segmental approach to
CHD
3

4. History Cont…
• Kirklin(1973): Modified the segmental approach
using concordance/discordance to describe
connections between 3 main cardiac segments
• Shinebourne/Anderson/Tynan(1976): Popularized
the sequential segmental approach to CHD
4

5. 5

6. 6

7. THORACO-ABDOMINAL SITUS
Situs: Topology/ spatial position of structureSitus: Topology/ spatial position of structure
1st
segment evaluated is visceral situs
Atrial and visceral situs considered together
because they usually are concordant ( atrial and
visceral situs are same)
7

8. Pulmonary Situs (Sidedness)
• Determined by positions of morphologic rt & lt lungs
• Pulmonary morphology, in turn, defined by
relationship of PAs to their adjacent bronchi, and not by
the number of lobes
• PA of a morphologic right lung travels anterior to its
upper and intermediate bronchi, whereas that of a
morphologic left lung travels over its main bronchus
and posterior to the upper lobe bronchus
8

9. Pulmonary Situs (Sidedness)
Cont…
• Rt bronchus branches, then RPA crosses it
• LPA crosses lt bronchus, then it branches
• Clinically, pulmonary situs may be inferred by
comparing relative lengths of 2 main bronchi, as
measured on a CXR that shows an air bronchogram
• Distance from carina to origin of upper lobe
bronchus is 1.5- 2.5 X greater for morphologic left
lung than for right lung, and this ratio holds true
regardless of the sidedness of the aortic arch
9

10. 10

11. The morphologically left bronchus is long, and it branches only after it has been crossed by its
accompanying pulmonary artery, making the bronchus hyparterial. In contrast, the
morphologically right bronchus is short, and is crossed by its pulmonary artery only after it
has branched, giving an eparterial pattern of branching
LR
11

12. 2D ECHO: STRUCTURE??
12

13. 2D ECHO: STRUCTURE??
13

14. 14

15. Cardiac malposition
• Often an integral part of complex associated
anomalies of visceral atrial situs
• Prevalence 1 in 10, 000 live births
Cardiac malpositions:
Dextrocardia
Mesocardia
Levocardia (isolated)
Ectopia cordis
15

16. Cardiac Malposition
16

17. 17

18. DEXTROCARDIA
• Detro position
• Dextro version
• Mirror image dextrocardia
18

19. Dextroposition: Heart pushed/ pulled into right
chest
Push:
Lt sided tension pneumothoax
Lt congenital lobar emphysema
Lt diaphragmatic hernia
Pull:
Hypoplasia/ agenesis of rt. lung
DEXTROCARDIA
19

20. DEXTROCARDIA Cont…
Dextroversion: Failure of pivoting of cardiac apex to
left, frequently associated with A-V discordance
Mirror-image dextrocardia with atrial situs inversus
There may be major and complex pathology
associated with this type of dextrocardia
20

21. 21

22. MESOCARDIA
Location of heart with cardiac base apex axis
directed to midline of thorax or with ventricular
apices equally directed to both right and left
sides
This is often ignored as being infrequent and
atypical
22

23. Isolated levocardia
Occurring in conjunction with situs inverses
and situs ambiguous
 An abnormal atrial and visceral situs, the
heart is in its normal location in the left hemi
thorax with the apex pointing to left
23

24. Ectopia Cordis
• Partially/ completely
exteriorized
• Pentalogy of Cantrell:
– 1. Deficiency of ant. Diaphragm
– 2. Midline supra umbilical
abdominal wall defect
– 3. Defect in diaphragmatic
pericardium
– 4. CHD
– 5. Defect of lower sternum
24

25. BASIC CARDIAC POSITIONS
95% CHD 100% CHD
3-5%CHD<1%CHD
25

26. 26

27. SEGMENT BY SEGMENT
ANALYSIS
27

28. SEGMENT CONNECTION
Great veins
Atria
Ventricles
Great arteries
Venoatrial
Atrioventricular
Ventriculoarterial
28

29. 29

30. 30

31. VENOUS SEGMENT
Systemic veins: IVC, hepatic
veins, SVC, CS
Pulmonary veins
HEPATIC VEINS: SC view,
drains into RA
CS – RA
IVC – RA
31

32. Venous Segment Cont…
ABNORMALITY OF IVC: Subcostal view
Interrupted IVC (Polysplenia):
Large azygous vein entering SVC seen along the spine in
abdomen, can be either right or left sided
Even then there is another vein draining into RA called:
Suprahepatic IVC
Suprahepatic IVC always drains into RA
Suprahepatic IVC differentiated from IVC in being small and
can’t be traced below liver
32

33. SVC
• SVC: Single right sided
• Subcostal view, high parasternal or suprasternal view
• LSVC:
• Connected to CS posteriorly/ laterally
• Border of LA (dilated CS)
• Directly visualized in a long axis plane
• Parasternal short axis images: Circular vessel anterior to
LPA near bifurcation
• SVC less reliable than IVC for situs
33

34. CS & LSVC
34

35. Pulmonary veins
• Subcostal, suprasternal, apical
• Color Doppler: Distinguishes from atrial
appendage
• 4 in number
• Left 2 veins may join: 3 entries into LA
35

36. ATRIAL SITUS
The identification of the morphologic right
atrium is important for establishing atrial
situs
Anatomic hallmark is the limbus of the
fossa ovalis
36

37. Atrial Situs
• Describes location of atria
• Solitus: Morphological RA on rt
• Inverses: Morphological RA on lt
• Ambiguous: Undifferentiated atria
37

38. Right atrium Left atrium
SEPTUM:
Limbus of fossa ovalis Flap valve of fossa ovalis
APPENDAGES:
Broad based, triangular, anterior Long and narrow, finger like,
posterior
MYOCARDIAL FEATURES:
Crista terminalis, tinea sagittalis,
extension of pectinate muscles towards
AV valve
Pectinate muscle confined to
appendage
VEINS:
Receives IVC, suprahepatic IVC, SVC,
coronary sinus
Receives pulmonary veins
38

39. Rightatrium Left atrium
39

40. 40

41. Subcostal sagittal views demonstrating eustachian valve (arrow in A) and flap
valve (arrow in B).
41

42. ATRIAL APPENDAGE
42

43. 43

44. Viscero atrial situs and its abnormality
Isomerism of Lt atrial
appendage:
•B/l lt atrial appendage:
Smooth pectinate muscles
•Interrupted IVC: 85%
Isomerism of Rt atrial
appendage:
• B/l rt atrial appendage:
Coarse pectinate muscles
•Abscent coronary sinus:
100%
•Abnormal relation of aorta
and IVC
44

45. ATRIAL ABNORMALITIES
azygous
45

46. 46

47. VENTRICULAR SITUS AND
MORPHOLOGY
Right ventricle Left ventricle
Large apical trabeculations Small apical trabeculations
Coarse septal surface Smooth upper surface
Moderator band No moderator band
Receives tricuspid valve Receives mitral valve
Tricuspid-pulmonary discontinuity Mitral-aortic continuity
Crescentic in cross section Circular in cross section
Thin free wall (3-5 mm) Thick free wall (12-15 mm)
47

48. Apical four-chamber view in a patient with ventricular inversion
with L-TGA
48

49. VENTRICULAR LOOPING
After morphology Determine looping
Bulboventricular loop: Describes location of ventricles
d-Loop: Morphologic RV on right
l-Loop: Morphologic RV on left
49

50. VENTRICULAR LOOPING Cont…
 Performed by imagining one is standing on the right
ventricle side facing the right ventricular face of the
interventricular septum
 The palm of one hand is placed against the septum
 The looping is determined by establishing which of the two
hands allow the thumb to point into the atrioventricular
valve and the fingers to point into the outflow tract
 If rt. hand meets criteria: d-looped, if lt hand meets criteria:
l-looped
50

51. The palmar surface of the right hand can
be placed on the septal surface of the normal morphologically right ventricle
with the thumb in the inlet component and the fingers extending into the
ventricular outlet. (d-ventricular loop.)
51

52. The mirror-imaged normal heart. In this setting,
it is the palmar surface of the left hand that can be placed on the septal
surface of the morphologically right ventricle with the thumb in the inlet
and the fingers in the outlet. (l-ventricular loop)
52

53. VENTRICULAR LOOPING Cont…
 In general, convexity of aorta points to the position of the
right ventricle and thus helps indicate bulboventricular loop
 Definitive indicator of bulboventricular loop, however, is
relative positioning of ventricular inlets/ AV valves
 Thus, in a d-loop the tricuspid valve is to the right of the
mitral valve, and in an l-loop the tricuspid valve is to the left
of the mitral valve
 In a normal d-loop the apex pivots to the left hemithorax;
in a "normal" l-loop (i.e., one in the setting of situs inversus)
the apex pivots to the right hemithorax
53

54. SINGLE VENTRICLE
• Single ventricle: Determined by morphology
of ventricle
• Single vent of RV morphology: Hypo plastic LV
remnant, posterior to main ventricular
chamber
• LV morphology: Rudimentary RV, anterior 54

55. 55

56. 56

57. AV Connections and AV Valve
Morphology
First see: Bi/uni ventricular connection
If double see: AV concordance/discordance
If single ventricle see: Double inlet, single inlet,
common inlet
57

58. 50% rule
 Anderson et al: Rule of 50% for determining
whether a cardiac chamber is a ventricle
 For the assessment of AV connections, an atrium is
considered to join the ventricle into which >50% of
the valve orifice empties
 This rule states that a chamber is a ventricle if it
receives 50% more of an inlet
 The inlet consists of the fibrous ring of the AV valve
and need not always include a patent AV valve with
well formed valve leaflets 58

59. 50% RULE Cont…
 Eg: In HLHS with aortic and mitral atresia, the
fibrous ring of the MV contains an imperforate
membrane and is situated over the small LV
 Thus, this small left sided chamber is a ventricle
because it receives 100% of an inlet
 A chamber need not have an outlet to be a ventricle
 The rule of 50% has also been used to define VA
connections
 Thus, if 50% or more of a great artery arises above a
chamber, the great artery is defined as being
59

60. 50% RULE IN AV, VA
• A great vessel should be related to a
ventricle by 50% of its dimension to be
considered committed to it
• Av valve is committed to that ventricle to
which >50% of that valve is connected
60

61. SINGLE VENTRICLE
61

62. ATRIOVENTRICULAR VALVE
MORPHOLOGY
Tricuspid valve Mitral valve
Septal chordal attachments No septal chordal
attachments
Low septal annular
attachment
High septal annular
attachment
Triangular orifice
(midleaflet)
Elliptical orifice (midleaflet)
Three leaflets Two leaflets
Several papillary muscles Two large papillary muscles
Empties into right ventricle Empties into left ventricle
62

63. ATRIOVENTRICULAR CONNECTIONS
63

64. OVERRIDING AV VALVE
Abnormality of AV valve
alignment or connection in
which one valve annulus
opens into another chamber
through a VSD
64

65. STRADDLING
A feature of the tensor apparatus (chordae tendineae
and papillary muscles) of an AV valve and indicates
anomalous insertion into the contralateral ventricle,
either along its septum or its free wall
65

66. 66

67. 67

68. VENTRICULOARTERIAL CONNECTION
If Ao from LV and PA from RV: Concordant
If Ao from RV and PA from LV: Discordant
Origins of Ao and PA are evident on PLAX view, sweeping the
transducer inferiorly from the basal short-axis view, A5CV and
SC coronal and sagittal views
3rd
type of VA connection: Double outlet, almost always from
RV
Final type of VA connection: Single outlet (truncus
arteriosus)
68

69. VA Connection
Aorta:
•Coronary artery
•Carotids
•Absence of proximal
division
PA:
•Bifurcation
69

70. Parasternal short-axis images demonstrating different relationships between
the great vessels.
An echocardiogram from an infant with tetralogy of Fallot and pulmonary
atresia demonstrates the identifying features of a pulmonary artery (MPA) with
its bifurcation into right and left pulmonary arteries (RPA, LPA). The great
vessels are normally related
70

71. 71

72. 72

73. CONAL MORPHOLOGY
Conus/ infundibulum: Cavitary space formed by muscular
segment of heart that connects ventricles with great arteries
and separates the AV and semilunar valves
Abnormalities in conal development consist of variations in
the presence, length and diameters of subpulmonary and
subaortic conus
These variations can lead to (or be associated with) complex
malformations, such as TOF, IAA,TGA and DORV
73

74. Subpulmonary conus - Best
identified in subcostal views
In normal heart: Conus- Nearly
vertical tubular outflow portion of
RV
Separated from nearly horizontal
right ventricular inflow portion by
distinct muscle bands
These muscle bands form a near-
circular rim formed by parietal band
anteriorly, crista supraventricularis
posteriorly, and septal band
medially and prohibit PV to AV
valve continuity 74

75. Conus Cont…
 Subaortic conus is evident on
subcostal coronal and sagittal views
 Persistence of subaortic conus and
involution of subpulmonic conus is
the usual conal relationship in d- (or
l-) TGA
 Persistence of subaortic conus
prohibits continuity of the aortic
valve to either AV valve, and
involution of subpulmonary conus
allows continuity between PV and
both AV valves in TGA 75

76. BILATERAL CONUS
 B/l persistence of the subarterial
conus usually results in DORV
 Because the main goal of surgical
correction of DORV is to connect the
aorta with the morphologic left
ventricle through VSD, it is important
to determine the conal relationships
with each other and with the great
vessels 76

77. BILATERAL CONUS
When 2 coni are present, their relationship may be
classified as either anterior/posterior or side-by-side
With the anterior/posterior conal relationship, the
ventricular septal defect is usually subaortic; with the
side-by-side relationship, the defect is usually
subpulmonic
 The conal relationship can be determined by subcostal
coronal and sagittal imaging with anterior/posterior
and left/right sweeping, respectively 77

78. ABSENT CONUS
 A rare type of d-transposition
can exist in the context of
bilaterally deficient
subarterial conus
 This results in an unusual
heart in which d-TGA exists
with a doubly committed
VSD and a posterior aorta
 Associated with DOLV 78

79. 79

80. SEMILUNAR VALVES
80

81. Great Artery Relations
8 basic types of great artery relationships are possible
based on the aortic and pulmonary valve positions at the
level of the semilunar valves
• Right posterior aorta (normally related)
Right lateral aorta (side by side)
Right anterior aorta (d-malposed)
Directly anterior aorta
Left anterior aorta (l-malposed)
Left lateral aorta (left side by side)
Left posterior aorta (inverted normal)
Directly posterior aorta.
81

82. 82

83. 83

84. ARCH OF AORTA
 Unexpected position of aortic arch: Well recognized
associated anomaly of conditions such eg. TOF/ Truncus
 Distinction should be made b/w position of arch and side of
descending aorta, particularly in describing vascular rings
 Side of aortic arch depends on whether it passes to right or
left of the trachea
 Position of descending aorta is defined relative to vertebral
column
84

85. ARCH OF AORTA Cont…
 Aorta lies more posterior in center of
 Visualized in PLAX,PSAX, A5CV,SC , and suprasternal
notch views
 Arch best seen in subcostal oblique view and
suprasternal views
 Side of arch diagnosed by sweeping transducer in
suprasternal long-axis view and noting the
relationship of the arch to the trachea, the rings of
which resemble a stack of coins
85

86. This transducer position allows visualization of the ascending aorta
(Asc), aortic arch (Arch), origin of the brachiocephalic vessels
(arrows), descending thoracic aorta (Dsc), and right pulmonary
artery (*).
ARCH OF AORTA LONG-AXIS
86

87. The short-axis view of the aortic arch is obtained by rotating the
transducer clockwise, which also allows visualization of the right
pulmonary artery (RPA) in its long-axis format, located inferiorly to
the aortic arch (Arch). Inferior to RPA is the left atrial (LA) cavity
with connections of the four pulmonary veins (arrows
SHORT-AXIS VIEW
87

88. 88

89. FINAL DIAGNOSIS
• SS, LEVOCARDIA, NORMAL SYSTEMIC &
PULMONARY VENOUS COMMUNICATIONS, NRGAs,
AV & VA CONCORDANCE, INTACT IA & IV SEPTUM,
NO PDA, GOOD SIZED CONFLUENT BPAs, NORMAL
ARCH, NO COA, NORMAL CORONARIES, NORMAL
SIZED CHAMBERS, GOOD BV FUNCTION, NO
VEGETATION/ CLOT OR PE 89

90. THANKS FOR UR PATIENCE
90