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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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defects from abnormal cardiac development
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
Variants of Situs (sidedness)
  • Solitus
  • Inversus
  • Ambiguous
    • Left isomerism
    • Right isomerism
situs solitus
Situs solitus

Left

Right

defects of cardiac positioning
Defects of cardiac positioning
  • Levocardia (normal orientation)
defects of cardiac positioning1
Defects of cardiac positioning
  • Mesocardia
    • Centrally located heart or
    • Inferior base apex axis
defects of cardiac positioning3
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 positioning4
Defects of cardiac positioning
  • Levocardia (normal orientation)
atrio ventriculo arterial sequence2
Atrio-ventriculo-arterial sequence
  • Concordance

Normal sequence AV & VA

  • Discordance

Reversed sequence AV & VA

Corrected TGA = AV discordance + VA discordance

atrio ventriculo arterial sequence3
Atrio-ventriculo-arterial sequence

Corrected TGA = AV discordance + VA discordance

defects from abnormal cardiac development1
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 septation4
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
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
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 valves1
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 valves2
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 valves3
Defects of A.V. canal & A.V. valves
  • A.V. canal defect
    • Complete
    • Transitional
    • Partial
defects of a v canal a v valves4
Defects of A.V. canal & A.V. valves
  • A.V. canal defect
    • Complete
      • Balanced ventricles
      • Unbalanced ventricles
defects from abnormal cardiac development2
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 valves1
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 fallot3
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 fallot4
Tetralogy of fallot
  • Nakata index
  • TNPAI total neo-pulmonary artery index
slide51
DORV

DORV

TGA

F4

slide52
DORV

DORV

TGA

F4

slide53
DORV
  • Types in relation to great arteries
    • Sub-aortic
    • Sub-pulmonic
    • Doubly committed
    • Non committed
t ransposition of g reat a rteries d type
Transposition of Great ArteriesD-type
  • PFO
  • VA discordance
  • Connecting vessel (PDA)
transposition of great arteries l type
Transposition of Great ArteriesL-type
  • AV discordance
  • VA discordance
transposition of great arteries l type1
Transposition of Great ArteriesL-type

Corrected TGA = AV discordance + VA discordance

defects in aortic arch1
Defects in aortic arch
  • Right side aortic arch
defects in aortic arch2
Defects in aortic arch
  • Coarctation of Aorta
defects of pulmonary venous drainage
Defects of pulmonary venous drainage
  • Total anomalous pulmonary venous drainage
  • Partial Anomalous pulmonary venous drainage
defects of systemic venous drainage
Defects of systemic venous drainage
  • Persistent left SVC
  • Interrupted IVC
ad