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Segmental approach in congenital heart disease [autosaved].pptx 2.pptx final

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Segmental approach in congenital heart disease [autosaved].pptx 2.pptx final

  1. 1. Segmental approach in congenital heart disease SHABNAM.MOHAMMADZADEH.MD CARDIOLOGIST FELLOW OF ADULT CONGENITAL HEART DISEASE
  2. 2. Segmental approach Step1 • Cardiac position Step2 • Great veins Step 3 • Visceral and atrial situs Step 4 • Venoatrial connection Step 5 • Ventricular looping Step 6 • Atrioventricular connections Step 7 • Relationship between great arteries Step 8 • Ventriculoarterial connection Step 9 • Associated anomalies SHABNAM.MOHAMMADZADEH, MD
  3. 3. Cardiac Orientation -Relationship or axis of the base to the apex of the heart . (Levocardia,Dextrocardia, Mesocardia) - If the cardiac apex fails to shift, it may result in situs solitus with dextrocardia which is termed dextroversion or situs inversus with levocardia called levoversion. SHABNAM.MOHAMMADZADEH, MD
  4. 4. Nomenclature for Thoracic and Visceral Situs Situs or sidedness: position or arrangement of structures or organs that are not bilaterally symmetric. Solitus, :Normal Inversus : Mirror image of normal Ambiguous: (not clearly solitus or inversus). Thoracic and abdominal organs cannot be lateralized , have neither the normal nor mirror image arrangement. SHABNAM.MOHAMMADZADEH, MD
  5. 5. Situs Definitions -Thoracic Situs: The right main bronchus takes a more vertical course and branches at an earlier point than the more horizontally oriented left bronchus. -The morphologically left bronchus is long, and it branches only after it has been crossed by its accompanying pulmonary artery, making the bronchus hyparterial. -The morphologically right bronchus is short, and is crossed by its pulmonary artery only after it has branched, giving an eparterial pattern of branching SHABNAM.MOHAMMADZADEH, MD
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  10. 10. SHABNAM.MOHAMMADZADEH, MD -Abdominal Situs:
  11. 11. POINTS Situs inversus with dextrocardia (complete situs inversus, mirror image dextrocardia, usually occurs without coexisting congenital heart disease. Isolated atrial inversion is rare  Situs solitus with dextrocardia is only occasionally associated with a structurally normal heart left-to-right shunts at atrial level or ventricular level usually coexist. Situs inversus with levocardia is consistently associated with coexisting congenital heart disease SHABNAM.MOHAMMADZADEH, MD
  12. 12. Situs Inversus with Dextrocardia -The incidence rate in the general population is estimated at 1/8000 to 1/25,000. - The heart and the thoracic and abdominal viscera are mirror images of normal . -The bronchi are inverted - The heart is right-sided -the right hemidiaphragm is lower than the left hemidiaphragm -The descending aorta is on the right - the ascending aorta, aortic knuckle, and pulmonary trunk are in their mirror image positions; -L-Loop ventricle. SHABNAM.MOHAMMADZADEH, MD
  13. 13. Situs Solitus with Dextrocardia The lungs and abdominal viscera are situs solitus, -The heart is right thoracic (dextrocardia) -The ascending aorta and aortic knuckle occupy their normal positions and the descending aorta runs its normal course along the left vertebral border -The major cardiac shadow lies to the right of midline (dextrocardia), the base to apex axis points to the right, and the right hemidiaphragm is lower than the left hemidiaphragm SHABNAM.MOHAMMADZADEH, MD
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  15. 15. Situs Inversus with Levocardia The left hemidiaphragm is lower than the right hemi-diaphragm because the apex is on the left Inversion of the bronchi ,coincides with inversion of the atria and lungs. The stomach is on the right, and the liver is on the left SHABNAM.MOHAMMADZADEH, MD
  16. 16. RA LA -RAA: triangular, broad-based -Crista terminalis -The thicker superior limbus of the atrial septum around the fossa ovalis -CS entrance -LAA: smaller, thinner, and finger-like -The flap of the valve at the oval fossa -CS course SHABNAM.MOHAMMADZADEH, MD
  17. 17. - The superior vena cava (SVC) is not an acceptable criterion to identify the morphologic right atrium -Identifying pulmonary venous connections is not a stern rule for identifying the morphologic left atrium. - Atrial situs follows abdominal situs in about 70% to 80% of the cases -It is often called situs inversus, even though the atrial chambers are not upside down. SHABNAM.MOHAMMADZADEH, MD POINTS
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  20. 20. Segmental approach Step1 • Cardiac position Step2 • Great veins Step 3 • Venoatrial Step 4 • Visceral and atrial situs Step 5 • Ventricular looping Step 6 • Atrioventricular connections Step 7 • Relationship between great arteries Step 8 • Ventriculoarterial connection Step 9 • Associated anomalies SHABNAM.MOHAMMADZADEH, MD
  21. 21. Definitions for Cardiac Ventricles SHABNAM.MOHAMMADZADEH, MD -Subpulmonic conus -Coarse trabeculation -Moderator band -Septal attachment of TV chorda -More apical TV RV -Fibrous outlet -Thin trabeculation -No moderator band - No septal attachment of MV -Higher MV LV always
  22. 22. The feature of solitary and indeterminate ventricle Coarse trabeculation than the morphological right ventricle There is no other chamber in the ventricular mass  Rudimentary right ventricle usually situated anterosuperiorly either leftward or rightward position and rudimentary left ventricle is positioned inferiorly The heart where one AV valve confined to the ventricle and the other AV valve overrides more than 50% will be described as double inlet ventricle SHABNAM.MOHAMMADZADEH, MD
  23. 23. Ventricular topology - spatial relationship of one ventricle to the other - Right hand topology is the normal pattern. Determination of ventricular topology requires first identification of the morphologic right ventricle. If the palmar surface of right hand can be placed on the septal surface, thumb in the inlet and the fingers toward the outlet and the wrist is at the apex, then this is the right –hand pattern. If only the left hand palm can be placed on the septal surface of the right ventricle in the same manner, then this will be described as left hand topology. Ventricular topology allows analysis of the atrioventricular junction in hearts with isomeric arrangement of atrial appendages. , SHABNAM.MOHAMMADZADEH, MD
  24. 24. LEFT HAND TOPOLOGY SHABNAM.MOHAMMADZADEH, MD
  25. 25. RIGHT HAND TOPOLOGY SHABNAM.MOHAMMADZADEH, MD
  26. 26. Nomenclature for Atrioventricular connection  Concordant (normal), Discordant, : RA to the morphologic LV ,LA into the morphologic RV Ambiguous, : Half atrioventricular junction, concordant,half, discordantly Double-inlet, Absent connection. SHABNAM.MOHAMMADZADEH, MD
  27. 27. A-V connection in functionally univentricular hearts 1. Double inlet : functional univentricle connected to two separate atria with two separate atrioventicular valves. 2. Single inlet : two separate atria with only one of the atria connected to the functional univentricle via one atrioventricular valve. The other atria connection is atretic. 3. Common inlet : both atria are connected to a functional univentricle via one atrioventicular valve. SHABNAM.MOHAMMADZADEH, MD
  28. 28.  Straddling is a feature of the chordae tendineae of an atrio- ventricular valve and describes chordae that cross a ventricular septal defect and have their attachments in the opposite ventricle.  Overriding is a feature of the valve annulus which describes an annulus that crosses a ventricular septal defect and thus lies “over” more than one ventricle. SHABNAM.MOHAMMADZADEH, MD
  29. 29. Definitions for Great Arteries  The great vessels are typically described by the terms solitus, inversus, dextro, and levo. Solitus : Normal anatomically relationship between the great vessels. Inversus :mirror image anatomic relationship.  Dextro : great vessels on the right side of the body levo : the great vessels are on the left.  The great arteries :identified by their branches . The location of the conus can help to identify the pulmonary artery  A common arterial trunk : a vessel connected to the ventricle (or ventricles) via a common ventriculoarterial valve.  A solitary arterial trunk : vessel arising from ventricle or ventricles that does not give rise to intrapericardial pulmonary arteries. In this anomaly, the blood supply to the lung usually comes from collateral vessels originating from either the ascending or descending thoracic aorta. SHABNAM.MOHAMMADZADEH, MD
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  31. 31. SHABNAM.MOHAMMADZADEH, MD
  32. 32. Nomenclature for Ventriculoarterial SHABNAM.MOHAMMADZADEH, MD  Concordant,  Discordant,  Double-outlet right ventricle,  Double-outlet left ventricle.  Ventriculoarterial overriding :more than half of the area of the outlet overrides the ventricular septum.
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