Download Congenital Heart Disease

Congenital Heart Disease
Cardiac Malpositions
Approach to Diagnosis
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Congenital Heart Disease
Malpositions : Diagnosis
1. Identify situs
2. Identify chambers and vessels
3. Arrange chambers and vessels
4. Identify additional anomalies
5. Define route of blood flow
Diagnosis = 1+ 2 + 3 + 4 + 5
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Congenital Heart Disease
Situs
Solitus
Inversus
Ambiguous
usual normal
mirror image normal
Indeterminate
Aplenia, Polysplenia,
Palpation, Percussion, X-ray
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Congenital Heart Disease
Malpositions : Asplenia
Absent spleen
Malrotation of bowels
Symmetrical liver
Complex CHD
(Bilateral – right sidedness)
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Congenital Heart Disease
Malposition : Polysplenia
Multiple spleens
Malrotation of bowels
Symmetrical liver
Complex CHD
(Bilateral left sidedness)
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RA
LA
Atria
Ventricles
RV
PA
LV
AO
Great arteries
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Congenital Heart Disease
Sequential Chamber Localisation
Define situs
1. Visceroatrial situs rule
2. Tracheal bifurcation
3. ECG
4. ECHO
5. Angiogram
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Congenital Heart Disease
Atrial Situs
Morphologically right atrium
Receives IVC
Broad appendage
Crista terminalis
Fossa ovalis
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Congenital Heart Disease
Atrial situs
Morphologically left atrium
Finger like appendage with narrow neck
Valve of fossa ovalis
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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
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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
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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
7.2%
3.6%
30%
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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%
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Congenital Heart Disease
Atrial situs : situs rule
3 lobed lung RA
2 lobes lung LA
Accuracy 72%
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Congenital Heart Disease
Atrial situs : Tracheal bifurcation
RA follows Rt bronchus
LA follows Lt bronchus
Asymmetrical division
Symmetrical – asplenia, polysplenia
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Congenital Heart Disease
Atrial situs : bronchial morphology
LA follows left bronchus – Hyparterial,
horizontal, longer
RA follows right bronchus – Eparterial,
vertical, shorter
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Bronchial length (cm)
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Left
Right
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2
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0
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Age (Yrs.)
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Congenital Heart Disease
Malpositions : Atrial situs
Bronchial anatomy : Length
Left bronchus :  2 cms
Right bronchus :  2 cms
(overlap below age 2 yrs.)
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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
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Congenital Heart Disease
Atrial situs : ECG
P
 in 1, II, aVF Normal
 in aVR
P
 in I, II aVF Inversus
 in aVR
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Congenital Heart Disease
Atrial situs
‘P’ wave specificity poor
i Sinus node – left side
ii Ectopic atrial rhythm
iii Bilateral sinus node
(asplenia, polysplenia)
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S
*
V
C
RA
LA
IVC
LIVER
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LA
RA
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RA
CS
LV
RV
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LAA
LA
RAA
LV
RA
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Liver
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Liver
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Liver
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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.
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Congenital Heart Disease
Ventricular identification – ECG
QRS morphology
RV : LV
rS, Rs
2:1
rsR, rSR, rRs
8:1
qRS, qR
1:3
QRs, QR, Qr
1:5
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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
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LV
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RV
LV
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LA
RA
LV
RV
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RV
LV
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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)
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Congenital Heart Disease
Establish
Atrio-ventricular relationship
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LA
RA
V
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LA
RA
LV
RV
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RA
LA
LV
RV
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Congenital Heart Disease
Great vessel relationship
Normal
Transposition
Malposition
Single artery
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Congenital Heart Disease
• Great Vessels : Normal
– Atrioventricular concordance
– Both ventricles present
– PA from RV – ant & to left
– Ao from LV – post & to right
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Congenital Heart Disease
Great arteries : Transposition
• PA from LV, Ao from RV
• Vent. septum present
• d loop : RV
Ao
LV
PA
• l loop RV
Ao
LV
PA
Transposition
CCTGA
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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
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Congenital Heart Disease
Great arteries : Single vessel
Persistent truncus arteriosus
Pulm. atresia
Aortic atresia
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P
L
R
A
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P
R
L
A
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P
R
L
A
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Congenital Heart Disease
Great arteries : Echo
Single
Two GV :
i) One circle the other wrap
around – normal.
ii) Two circles – transposition &
malposition
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Normally related Great Vessels
Great Vessels in Situs Inversus
PA
PA
Ao
Ao
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a
b
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a
b
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D Malposition
L Malposition
Ao
PA
RA
Ao
LA
PA
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Ao
PA
TR
RA
RV
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RV
LV
RPA
TR
LA
TR
Ao
RA
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A
L
R
P
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A
R
L
P
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A
L
R
P
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Congenital Heart Disease
Establish :
Ventriculo–arterial relationship
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MPA
RPA
Ao
Ao
RV
RA
LV
RA
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PA
Ao
RA
RV
LV
LV
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RV
PA
Ao
PA
RA
LV
RV
RA
LV
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Congenital Heart Disease
Malpositions : Diagnosis
1. Identify situs
2. Identify chambers and vessels
3. Arrange chambers and vessels
4. Identify additional anomalies
5. Define route of blood flow
Diagnosis = 1+ 2 + 3 + 4 + 5
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RA
LA
RV
LV
PA
Ao
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LA
RA
RV
LV
Ao
PA
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RA
LA
LV
RV
Ao
PA
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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
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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)
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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
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Congenital Heart Disease
Malposition : Asplenia
Blood picture
• Polycythemia
• Howell Jolly bodies
• Siderocystosis
• Target cells
• Heinz bodies
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Congenital Heart Disease
Malposition
PS or Pulm. Atresia
Heterotaxy
ASD / Single A.
VSD / Single V.
Bilateral SVC
Howell Jolly bodies
Bilateral 3 lobed lung
ECD
Asplenia (21)
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