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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
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Congenital heart defectsAnatomic 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 ambiguousLeft isomerism Left Left
Situs ambiguousRight isomerism Right Right
Situs ???? RA LA
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
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 • P.F.O. • Ostium secundum defect • Ostium primum defect • Sinus venosus A.S.D. • Single atrium • Septal aneurysm
Defects in ventricular septation • Outlet conal defect, conal septal hypoplasia, sub-pulmonary defect, infundibular defect, supracristal defect, doubly committed juxta-arterial defect • Membranous peri-membranous defect, para-membranous defect, conoventricular defect • Inlet peri-inlet defect, AV canal type defect, AV septal defect, endocardial cushion defect. • Muscular 1 2
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