Congenital heart defects
Anatomic considerationDr. 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
LeftLeft
Situs ambiguousRight isomerism
Right 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
Atrio-ventriculo-arterial sequence
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
Defects of atrial septation
Defects of atrial septation
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
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
Tetralogy of fallot
Tetralogy of fallot
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
F4DORV TGA
F4DORV TGA
DORV
DORV
• Types in relation to great arteries
– Sub-aortic– Sub-pulmonic– Doubly committed– Non committed
Truncus Arteriosus
Truncus Arteriosus
Transposition of Great ArteriesD-type
•PFO
•VA discordance
•Connecting vessel (PDA)
Transposition of Great ArteriesL-type
•AV discordance
•VA discordance
Corrected TGA = AV discordance + VA discordance
Transposition of Great ArteriesL-type
Defects in aortic arch
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
