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SEGMENTAL ANALYSIS OF CONGENITAL HEART DISEASE
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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