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Congenital heart defects Anatomic consideration Dr. 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 ambiguous Left isomerism Left
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Situs ambiguous Right isomerism 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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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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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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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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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 F4F4 TGA
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F4F4 DORVTGA DORV
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Types in relation to great arteries –Sub-aortic –Sub-pulmonic –Doubly committed –Non committed
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Truncus Arteriosus
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T ransposition of G reat A rteries D-type PFO VA discordance Connecting vessel (PDA)
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Transposition of Great Arteries L-type AV discordance VA discordance
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Corrected TGA = AV discordance + VA discordance Transposition of Great Arteries L-type
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Defects in aortic arch
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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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