Conotruncal anamolies

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The conotruncus comprises collectively two myocardial subsegments, the conus and the truncus.
Conus is the myocardial segment between ventricle and semi lunar valves which gives rise to sub arterial coni.
Truncus is the fibrous segment between semi lunar valves and aortic sac which gives rise to great arteries.

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Conotruncal anamolies

  1. 1. Conotruncus Embryology & Anomalies Msn Pavan Kumar Nizam’s Institute of Medical Sciences ,Panjagutta,Hyderabad,India. msnpavan@gmail.com
  2. 2. Conotruncus Embryology & Anomalies The conotruncus comprises collectively two myocardial subsegments, the conus and the truncus. Conus is the myocardial segment between ventricle and semi lunar valves which gives rise to sub arterial coni. Truncus is the fibrous segment between semi lunar valves and aortic sac which gives rise to great arteries.
  3. 3. Conotruncus Embryology & Anomalies  Embryology 1. Septation of conus and truncus. 2. Rotation and absorption. 3. Development of semi lunar valves. 4. Important structures involved a) Neural crest cells. b) Secondary heart field.  Congenital heart diseases d/t conotruncus  Genetic defects in Conotruncal
  4. 4. Conotruncus Embryology & AnomaliesEmbryology - Septation of conus and truncus.  4 truncus and 2 conal cushions develop.  Dextro- sinistro cushions of both conus and truncus fuse to form Conotruncal septum.  Intercalated cushions play an role in formation of semi lunar valves
  5. 5. Conotruncus Embryology & AnomaliesEmbryology - Septation of conus and truncus.  Because the cushions are dextro-superior and sinistro inferior in truncus and dextro-dorsal and sinistro- ventral in conus union forms a spiral septum than true lineal relation. For convenience it is represented as linear structure
  6. 6. Conotruncus Embryology & AnomaliesEmbryology - Rotation and absorption.  Aorta will be in connection with RV and PA with LV.  There are two rotations one at conoventricular junction and other at Conotruncal junction.  Both rotations are counterclockwise around 110º
  7. 7. Conotruncus Embryology & AnomaliesEmbryology - Rotation and absorption.  Conoventricular rotation brings aorta in continuation with LV and PA with RV.  Conotruncal rotation brings the normal position of aorta in relation to PA ( left and posterior to PA)
  8. 8. Conotruncus Embryology & AnomaliesEmbryology - Rotation and absorption.  Second most important thing is absorption of conus.  Out of the two coni which ever remains persistent and grows pushes the artery more anterior and superior direction bringing it in direct connection with RV.  In normal heart sub aortic conus is absorbed completely.
  9. 9. Conotruncus Embryology & AnomaliesEmbryology – Semilunar valves. Septation at valvular level occurs in intercalated cushions leading to formation partial aortic and pulmonary valve. The remaining part is formed by Conotruncal cushions.
  10. 10. Conotruncus Embryology & AnomaliesEmbryology – Important Structures For Development Of Conotruncus.  Second heart field cells and neural crest cell play important role in development of conotruncus
  11. 11. Conotruncus Embryology & Anomalies  Conotruncal defects 1. Truncus 2. TOF (with absent PV& PA) 3. DORV 4. DOLV 5. D-TGA 6. Conoventricular septal defects 7. Interrupted aortic arch type B
  12. 12. Conotruncus Embryology & AnomaliesTruncus arteriosus. Failure of aortopulmonary septum to Septation give rise to persistent truncus arteriosus. Truncus Conus
  13. 13. Conotruncus Embryology & AnomaliesTOF: Infundibular septum which arises from conal septum moves anteriorly and superiorly causing components of TOF
  14. 14. Conotruncus Embryology & AnomaliesDORV Conoventricular rotation is based on which coni persists. Artery with coni will be anterior and superior and connected to RV If both coni are present both arise from RV - DORV
  15. 15. Conotruncus Embryology & AnomaliesDORV In DORV, rotation of great arteries driven by conal development that changes not the position of VSD. Type More prominent Less prominent VSD commited coni coni to TOF(40%) Subpulmonic Sub aortic Aorta VSD (15%) Subpulmonic Sub aortic Aorta TGA(20%) Subaortic Sub pulmonic PA Variants ---- Both Both (DC<10%) Variants Both ---- Non committed (NC<10%)
  16. 16. Conotruncus Embryology & AnomaliesDOLV In DOLV both coni are absent hence both arteries are posterior and arising from LV
  17. 17. Conotruncus Embryology & AnomaliesD - TGA
  18. 18. Conotruncus Embryology & AnomaliesCono ventricular septal defects.  Absence of conal(Infundibular) septum leads to formation of malaligned , subinfundibular , subpulmonic VSD .
  19. 19. Conotruncus Embryology & Anomalies
  20. 20. Conotruncus Embryology & Anomalies
  21. 21. Conotruncus Embryology & Anomalies
  22. 22. Thank You Sharing This.

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