Master Class Tétralogie de Fallot Hands-on session• Tetralogy of Fallot – With pulmonary...
Transcript of Master Class Tétralogie de Fallot Hands-on session• Tetralogy of Fallot – With pulmonary...
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Master Class Tétralogie de Fallot
Hands-on session
Lucile HOUYEL
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• Uniquemechanism=anteriormalalignmentandanteriordeviationoftheoutletseptum
Outlet VSD Aortic dextroposition Subpulmonary stenosis
Right ventricular hypertrophy
Tetralogy of Fallot : « monology » of Fallot
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RV
RV
VSD
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Earlylooping ConvergenceWEDGING
Ao AP
Wedging=rotationoftheaorticvalveandtransfertoLV
Normalheart
VD
VG
O
CT
Cardiacneuralcrest
Secondheartfield
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Outletseptum=betweentheaorticandpulmonaryvalves
EndocardialcushionsoftheoutflowtractWedging(44-49days)
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PA
TVSB
MB
Fomation of the outlet septum
RVLV
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The outlet septum
VGVGRV RV
RVLV LV
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Abnormal development of the outlet septum :Outlet VSD
❑ «Y»oftheseptalband=Zoneoffusionbetweentheoutletseptumandthemuscularprimitiveseptum
❑ Absenceoffusion(outletseptalmalalignment) outletVSD
❑ Anomaliesofthecardiacneuralcrestandsecondheartfield
AP
PA
OS
SBT
VSD
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Conotruncal malformations (cardiac neural crest / second heart field defects)
• OutletVSD(malalignedorfibrousoutletseptum)
• TetralogyofFallot– Withpulmonarystenosis– Withpulmonaryatresia– Withabsentpulmonaryvalve
• DORVwithsubaorticordoublycommittedVSD
• Commonarterialtrunk• InterruptedaorticarchtypeB
PA
OS
SBT
VSD
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• Anomaliesofwedging(rotation)andpositionoftheoutletseptum
• AllsharethesameVSD:outletVSD
Null Incomplete NormalExcessiveWEDGING
CAT TOFwPAtFallotOutletVSDNormalheartIAAtypeB
Conotruncal malformations (cardiac neural crest / second heart field defects)
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Tetralogy of Fallot
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Tetralogy of Fallot The ventricular septal defect
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Tetralogy of Fallot : the VSD
• OutletVSD• Locatedbetweenthetwo
limbsoftheseptalband• Beneaththeoverriding
aorticvalvarorifice• Variations:
– Postero-inferiorrim– Absent(fibrous)outletseptum
• AdditionalmuscularVSD
Ao
PA
VSD
T
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Ao-tricuspidfibrouscontinuity
47/71:66% Mostefa-KaraJTCS2015
139/164:85% KhanEJCTS2019
PA
T
AoAo
PA
The VSD in TOF: the postero-inferior rim
T
Fibrous(«perimembranous») Muscular
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Tetralogy of Fallot : the VSD
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The VSD: the conduction tissue• Importanceofthepostero-
inferiorrimforthesurgeon• Conductiontissue
vulnerableiffibrous(«perimembranous»)
OutletVSDwithfibrousposteroinferiorrim(perimembranousextension)
AoAo
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Fibrouspostero-inferiorrim Muscularpostero-inferiorrim
PV
Outletseptum
The VSD in TOF: the postero-inferior rim
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Tétralogie de Fallot Septum conal long
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TetralogyofFallot:theVSD Juxta-arterialdoublycommittedVSD
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Tetralogy of Fallot or DORV?
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Tetralogy of Fallot or DORV « Fallot-type » ?
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Tetralogy of Fallot : overriding aorta
Ao
PA
LVRV
DORV, subaortic VSD, subpulmonary stenosis « Fallot-type »
PA
Ao
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Tetralogy of Fallot The pulmonary valve
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TOF : the pulmonary valve
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Tetralogy of Fallot with absent pulmonary valve
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Tetralogy of Fallot The coronary arteries
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TOF: the coronary arteries
HouyelL,BajolleF.etal.JAnat2013;222:349-357.
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Anterior interventricular coronary artery connected to the RCA and crossing the RVOT
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Tetralogy of Fallot: associations
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Tetralogy of Fallot and atrioventricular septal defect
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Tetralogy of Fallot Aorto-pulmonary window
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Conclusion The anatomic variants of tetralogy of Fallot
❑ Commonanatomicbasis=Anteriormalalignmentanddeviationoftheoutletseptum➢ OutletVSD
➢ Overridingaorta➢ RVOTobstruction
❑ Anatomicvariants➢ Postero-inferiorrimoftheVSD
❑ Fibrous«perimembranous»❑ Muscular
➢ Outletseptum ❑ Muscular❑ Fibrous
➢ Pulmonaryvalve❑ Absent❑ Atretic
Fetalheart15WG
SubpulmonarystenosisOutlet
septum
Ao
RV
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