Congenital heart defects Anatomic consideration Dr. Yasser Salem
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)
Variants of Situs (sidedness) Solitus Inversus Ambiguous –Left isomerism –Right isomerism
Situs solitus Left Right
Situs inversus Right Left
Situs ambiguous Left isomerism Left
Situs ambiguous Right isomerism Right
Situs
Situs ???? LA RA
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
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 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
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, para- membranous defect, conoventricular defect 3.Inlet peri-inlet defect, AV canal type defect, AV septal defect, endocardial cushion defect. 4.Muscular 1 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 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
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
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 F4F4 TGA
F4F4 DORVTGA DORV
Types in relation to great arteries –Sub-aortic –Sub-pulmonic –Doubly committed –Non committed
Truncus Arteriosus
T ransposition of G reat A rteries D-type PFO VA discordance Connecting vessel (PDA)
Transposition of Great Arteries L-type AV discordance VA discordance
Corrected TGA = AV discordance + VA discordance Transposition of Great Arteries L-type
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