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Segmental approach to normaland abnormal situs arrangement
- Echocardiography -
Jan MarekGreat Ormond Street Hospital
London
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How the heart should be imaged?“European Chaos”
•“Transatlantic”way
•“Adult” way
•“Dutch” way
LV
LV
LV
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SEQUENTIAL SEGMENTAL ANALYSIS
ANALYSE SEGMENTS
ATRIUMS
VENTRICLES
ARTERIAL TRUNKS
ATRIOVENTRICULARCONNECTIONS
VENTRICULO-ARTERIALCONNECTIONS
Courtesy RH Anderson
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• Atriums - Atrial situs(systemic and pulmonary venous connections)
• AV connections
• Ventricles
• VA connections
• Aorta, PA trunk(AO arch and PA branches)
• Abdominal situs !
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LATERALIZED BODILY ARRANGEMENT
Courtesy RH Anderson
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VISCERAL HETEROTAXY
Courtesy RH Anderson
Atrial Isomerism
Right Left
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• Abdominal situs• Atriums - Atrial situs(systemic and
pulmonary venous connections)
• AV connections
• Ventricles
• VA connections
• Aorta, PA trunk(AO arch and PA branches)
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A
R
Usual Arrangement (Solitus)
ST
SP
Liver
IVCAO
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Mirror Imagery (Inversus)A
R
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Right Isomerism (Asplenia)A
R
Liver Liver
AO
IVC
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Left Isomerism (Polysplenia)A
R
Liver Liver
AO
AZ
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Left Isomerism (Polysplenia)
Fetus32 WoG
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• Abdominal situs
• Atriums - Atrial situs (systemic and pulmonary venous connections)
• AV connections
• Ventricles
• VA connections
• Aorta, PA trunk(AO arch and PA branches)
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Usual Arrangement (Solitus)- Atrial appendages -
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Right Isomerism (Asplenia)- Atrial appendages -
Courtesy V.Povysilova
Fetus 20. WoG
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Left Isomerism (Polysplenia)- Atrial appendages -
Courtesy V. Povysilova
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Atrial appendages- Juxtaposition -
Courtesy V. Povysilova
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“Usual” arrangementSOLITUS
“Unusual arrangement”ISOMERISMUS
Courtesy Ian D Sullivan
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• Abdominal situs
• Atriums - Atrial situs(systemic and pulmonary venous connections)
• AV connections• Ventricles
• VA connections
• Aorta, PA trunk(AO arch and PA branches)
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•Concordant
•Discordant
•Univentricular AV connections
Absent AV connection
Common AV valve
Double inlet connections
Atrio-ventricular Connections
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Concordant Connections
RA LA
AO
PV
PV
RVLV
Offsetting
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DiscordantAV
connection
Concordant AV
connection
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Discordant ConnectionsCCTGADORV/VIPA/VSD/VI
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Univentricular AV Connections•Absent AV connection (right / left)
Tricuspid / mitral atresia•Common AV valve
With single ventricle•Double inlet ventricle
Connection of both AV valve to the sameventricle:
Anatomically leftAnatomically rightUndetermined
(rudimentary chamber always present)
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Univentricular AV connections
RALA
RALA
*
* *
*
RALA
RA RARA
Common AV valve Double inlet
Absentright AV
connection
Absentleft AV
connection
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Common AV valveDouble inlet
Absent right AVconnection
Absent left AVconnection
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• Abdominal situs
• Atriums - Atrial situs(systemic and pulmonary venous connections)
• AV connections
• Ventricles
• VA connections• Aorta, PA trunk(AO arch and PA
branches)
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•Concordant (=normal)Ventricular septal defect, tetralogy of Fallot…
• DiscordantTransposition
•Double outlet ventricleWith subaortic VSD
subpulmonary VSDdoubly committed VSDnon-committed VSD
•Common arterial trunk
Ventriculo-arterial connections
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(AV & VA discordance = Congenitally corrected TGA)
Courtesy RH Anderson
Discordant AV / VA connections
RA
LVRV
LA
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Discordant AV / VA connections
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Overriding aortic valve: 50% ruleVSD TOF DORV
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Double outlet right ventricle
RH Anderson 2005
AoPa
TOF vs Double outlet right ventricle
RV LV
TOF
Ao PaAo
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Double conus = Double outlet right ventricle?
Double conus does not determine DORV!
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How to diagnose
Correct definition of disease?
AV concordance&
VA discordance
•Simple vs. Complex transposition?•Transposition or Double outlet right ventricle?
•Transposition or Criss-cross heart or Supero-inferior ventricles
•Transposition or Malposition(corrected or not, right or left?)
AV discordance&
VA discordance
AV discordance&
VA concordance
TGA CCTGA CCMGA
Right/Left ventricle
withtwo outlets
DORVDOLV
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AV discordance & VA concordance(Congenitally corrected malposition)
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Segmental approach
•Important to understand situs arrangement
•Provides complete coverage of lesion
•Minimises potential errors
•Easy for reading and interpretation
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Sequential Segmental Approach
KISS
KeepItSimple,Stupid