Congenital heart defects Anatomic consideration
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Congenital heart defects
Anatomic considerationDr. Yasser Salem
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Defects from abnormal cardiac development
• 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)
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Variants of Situs (sidedness)
• Solitus
• Inversus
• Ambiguous– Left isomerism– Right isomerism
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Situs solitus
Left Right
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Situs inversus
Right Left
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Situs ambiguousLeft isomerism
LeftLeft
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Situs ambiguousRight isomerism
Right Right
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Situs
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Situs????
LA
RA
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Cardiac position
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Defects of cardiac positioning
• Levocardia (normal orientation)
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Defects of cardiac positioning
• Mesocardia– Centrally located heart or– Inferior base apex axis
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Defects of cardiac positioning
• Dextrocardia
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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
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Defects of cardiac positioning
• Levocardia (normal orientation)
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Atrio-ventriculo-arterial sequence
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Atrio-ventriculo-arterial sequence
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Atrio-ventriculo-arterial sequence
• Concordance
Normal sequence AV & VA
• Discordance
Reversed sequence AV & VA
Corrected TGA = AV discordance + VA discordance
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Atrio-ventriculo-arterial sequence
Corrected TGA = AV discordance + VA discordance
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Concordant flow with spatial discordance
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Discordant flow with spatial concordance
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Defects from abnormal cardiac development
• 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)
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Defects of atrial septation
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Embryologic cardiac tube
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Defects of atrial septation
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Defects of atrial septation
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Defects of atrial septation
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Defects of atrial septation
• P.F.O.• Ostium secundum
defect• Ostium primum defect• Sinus venosus A.S.D.• Single atrium• Septal aneurysm
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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, para-membranous defect, conoventricular defect
3. Inlet peri-inlet defect, AV canal type defect, AV septal defect, endocardial cushion defect.
4. Muscular
1
2
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Gerbode defect
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Defects of A.V. canal & A.V. valves
• A.V. canal defect
• Tricuspid and mitral valve defects
• Ebstien anomaly
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Defects of A.V. canal & A.V. valves
• A.V. canal defect– Atrial communication– Ventricular communication– Abnormal A.V. valve development
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Defects of A.V. canal & A.V. valves
• A.V. canal defect– Complete
• Rastelli A• Rastelli B• Rastelli C
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Defects of A.V. canal & A.V. valves
• A.V. canal defect– Complete – Transitional– Partial
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Defects of A.V. canal & A.V. valves
• A.V. canal defect– Complete
• Balanced ventricles• Unbalanced ventricles
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Defects from abnormal cardiac development
• 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)
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Defects of conotruncal development & semilunar valves
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Defects of conotruncal development & semilunar valves
• Tetralogy of fallot
• DORV
• Transposition of great arteries T.G.A.
• Truncus arteriosus
• Pulmonary stenosis and atresia
• Aortic stenosis and subaortic membrane
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Tetralogy of fallot
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Tetralogy of fallot
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Tetralogy of fallot
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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
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Tetralogy of fallot
• Nakata index
• TNPAI total neo-pulmonary artery index
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DORV
F4DORV TGA
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F4DORV TGA
DORV
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DORV
• Types in relation to great arteries
– Sub-aortic– Sub-pulmonic– Doubly committed– Non committed
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Truncus Arteriosus
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Truncus Arteriosus
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Transposition of Great ArteriesD-type
•PFO
•VA discordance
•Connecting vessel (PDA)
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Transposition of Great ArteriesL-type
•AV discordance
•VA discordance
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Corrected TGA = AV discordance + VA discordance
Transposition of Great ArteriesL-type
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Defects in aortic arch
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Defects in aortic arch
• Right side aortic arch
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Defects in aortic arch
• Coarctation of Aorta
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Defects of pulmonary venous drainage
• Total anomalous pulmonary venous drainage• Partial Anomalous pulmonary venous drainage
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Defects of systemic venous drainage
• Persistent left SVC• Interrupted IVC
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