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