1 congenital heart disease cardiac malpositions approach to diagnosis
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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 usual normal
Inversus mirror image normal
Ambiguous 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
RV LV
Atria
Ventricles
PA AOGreat 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 7.2%(No splenic abn.)
Solitus or Inversus predicted 3.6%as ambiguus
Midline liver Do no predict 30%Absent spleen S. ambiguus
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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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LeftRight
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4 6 8 10 12 14 1620
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Bronchial length (cm)
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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Atrial situs
‘P’ wave specificity poor
i Sinus node – left side
ii Ectopic atrial rhythm
iii Bilateral sinus node
(asplenia, polysplenia)
Congenital Heart Disease
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RAIVC
LACV
S
LIVER
*
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LA
RA
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RA
RVLV
CS
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LAA
LA
RALV
RAA
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Liver
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Liver
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Liver
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Ventricular relationship
d loop RV Right side
LV Left side
l loop RV Left side
LV Right side
Indeterminate : Sup. Inferior vent.
Congenital Heart Disease
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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
Congenital Heart Disease
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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
Congenital Heart Disease
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LV
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LV
RV
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LV
RA LA
RV
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LV
RV
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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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V
RA
LA
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LA
RV
RA
LV
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RA
RVLV
LA
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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
Transposition
LV PA• l loop RV Ao CCTGA
LV PA
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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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A
P
R L
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P
A
R L
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A
P
R L
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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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PAAo
PAAo
Normally related Great Vessels Great Vessels in Situs Inversus
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a
b
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a
b
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Ao
PA
LARA
Ao
PA
D Malposition L Malposition
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RARV
PAAo
TR
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LV
RV
TR
Ao
RPATR
LA
RA
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A
P
RL
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A
P
R L
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A
P
R L
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Congenital Heart Disease
Establish :
Ventriculo–arterial relationship
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LVRA
AoRPAMPA
Ao
RARV
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RV
Ao
LVLVRA
PA
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PARV
RA
LV
PA
LVRA
RV
Ao
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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
LV
PA
LA
RV
Ao
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RA
RV
Ao
LA
LV
PA
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LARA
RV
Ao
LV
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 bowelsBilateral left bronchusBilateral SVC, Inferior vena cava - azygos or
hemiazygos continuationPAPVC, TAPVCBilateral LA, ASD, ECD, Single AVSD, Single V.(DORV, TGA with PS rare)
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Congenital Heart Disease
Malposition : S. AmbiguousAbd. VisceraBronchial anatomyPeripheral smear – Howell Jolly bodiesECGSplenic scintigraphyUS Abdomen Multiple spleen
Absent spleenIntrahepatic 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 Asplenia (21)PS or Pulm. Atresia 18Heterotaxy 17ASD / Single A. 19VSD / Single V. 20Bilateral SVC 13Howell Jolly bodies 13Bilateral 3 lobed lung 12ECD 14
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