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