Download Congenital heart defects Anatomic consideration

Congenital heart defects
Anatomic consideration
Dr. Yasser Salem
Defects from abnormal cardiac
development
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Defects of cardiac positioning
Defects of atrial septation
Defects of ventricular septation
Defects of A.V. canal and A.V. valves
Defects of cono-truncal development and
semi-lunar valves
• Defects in aortic arch
• Defects in venous system (systemic or
pulmonary)
Variants of Situs (sidedness)
• Solitus
• Inversus
• Ambiguous
– Left isomerism
– Right isomerism
Situs solitus
Left
Right
Situs inversus
Right
Left
Situs ambiguous
Left isomerism
Left
Left
Situs ambiguous
Right isomerism
Right
Right
Situs
Situs ????
RA
LA
Cardiac position
Defects of cardiac positioning
• Levocardia (normal orientation)
Defects of cardiac positioning
• Mesocardia
– Centrally located heart or
– Inferior base apex axis
Defects of cardiac positioning
• Dextrocardia
Defects of cardiac positioning
• Dextrocardia
– Isolated dextrocardia (with situs solitus)
accompanied by severe cardiac anomalies
e.g. A.V. or V.A. discordance single ventricle
or heterotaxy syndrome
– Dextrocardia with situs inversus less likely to
be accompanied with other anomalies
Defects of cardiac positioning
• Levocardia (normal orientation)
Atrio-ventriculo-arterial sequence
Atrio-ventriculo-arterial sequence
Atrio-ventriculo-arterial sequence
• Concordance
Normal sequence AV & VA
• Discordance
Reversed sequence AV & VA
Corrected TGA = AV discordance + VA discordance
Atrio-ventriculo-arterial sequence
Corrected TGA = AV discordance + VA discordance
Concordant flow with spatial
discordance
Discordant flow with spatial
concordance
Defects from abnormal cardiac
development
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Defects of cardiac positioning
Defects of atrial septation
Defects of ventricular septation
Defects of A.V. canal and A.V. valves
Defects of cono-truncal development and
semi-lunar valves
• Defects in aortic arch
• Defects in venous system (systemic or
pulmonary)
Defects of atrial septation
Embryologic cardiac tube
Defects of atrial septation
Defects of atrial septation
Defects of atrial septation
Defects of atrial septation
• P.F.O.
• Ostium secundum
defect
• Ostium primum defect
• Sinus venosus A.S.D.
• Single atrium
• Septal aneurysm
Defects in ventricular septation
1. Outlet
conal defect, conal septal
hypoplasia, sub-pulmonary
defect, infundibular defect,
supracristal defect, doubly
committed juxta-arterial defect
2. Membranous
peri-membranous defect, paramembranous defect,
conoventricular defect
3. Inlet
peri-inlet defect, AV canal type
defect, AV septal defect,
endocardial cushion defect.
4. Muscular
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2
Gerbode defect
Defects of A.V. canal & A.V. valves
• A.V. canal defect
• Tricuspid and mitral valve defects
• Ebstien anomaly
Defects of A.V. canal & A.V. valves
• A.V. canal defect
– Atrial communication
– Ventricular communication
– Abnormal A.V. valve development
Defects of A.V. canal & A.V. valves
• A.V. canal defect
– Complete
• Rastelli A
• Rastelli B
• Rastelli C
Defects of A.V. canal & A.V. valves
• A.V. canal defect
– Complete
– Transitional
– Partial
Defects of A.V. canal & A.V. valves
• A.V. canal defect
– Complete
• Balanced ventricles
• Unbalanced ventricles
Defects from abnormal cardiac
development
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Defects of cardiac positioning
Defects of atrial septation
Defects of ventricular septation
Defects of A.V. canal and A.V. valves
Defects of cono-truncal development and
semi-lunar valves
• Defects in aortic arch
• Defects in venous system (systemic or
pulmonary)
Defects of conotruncal
development & semilunar valves
Defects of conotruncal
development & semilunar valves
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Tetralogy of fallot
DORV
Transposition of great arteries T.G.A.
Truncus arteriosus
Pulmonary stenosis and atresia
Aortic stenosis and subaortic membrane
Tetralogy of fallot
Tetralogy of fallot
Tetralogy of fallot
Tetralogy of fallot
McGoon ratio
• Definition
– Diameter of left plus right pulmonary arteries
at the pre-branching point divided by diameter
of descending aorta at the level of diaphragm
• Normal 2.1
• More than 1.2 adequate for V.S.D. closure
• Less than 0.8 inadequate
Tetralogy of fallot
• Nakata index
• TNPAI total neo-pulmonary artery index
DORV
F4
DORV
TGA
DORV
F4
DORV
TGA
DORV
• Types in relation to great arteries
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Sub-aortic
Sub-pulmonic
Doubly committed
Non committed
Truncus Arteriosus
Truncus Arteriosus
Transposition of Great Arteries
D-type
•PFO
•VA discordance
•Connecting vessel (PDA)
Transposition of Great Arteries
L-type
•AV discordance
•VA discordance
Transposition of Great Arteries
L-type
Corrected TGA = AV discordance + VA discordance
Defects in aortic arch
Defects in aortic arch
• Right side aortic arch
Defects in aortic arch
• Coarctation of Aorta
Defects of pulmonary venous
drainage
• Total anomalous pulmonary venous drainage
• Partial Anomalous pulmonary venous drainage
Defects of systemic venous
drainage
• Persistent left SVC
• Interrupted IVC