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Conotruncus Embryology & Anomalies

                      Msn Pavan Kumar
             Nizam’s Institute of Medical Sciences
                ,Panjagutta,Hyderabad,India.
                   msnpavan@gmail.com
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.
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
Conotruncus Embryology & Anomalies
Embryology - 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
Conotruncus Embryology & Anomalies
Embryology - 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
Conotruncus Embryology & Anomalies
Embryology - 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
Conotruncus Embryology & Anomalies
Embryology - 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)
Conotruncus Embryology & Anomalies
Embryology - 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.
Conotruncus Embryology & Anomalies
Embryology – 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.
Conotruncus Embryology & Anomalies
Embryology – Important Structures For Development Of Conotruncus.
    Second heart field cells and neural crest cell play important role in
     development of conotruncus
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
Conotruncus Embryology & Anomalies
Truncus arteriosus.
   Failure of aortopulmonary septum to Septation give rise to
     persistent truncus arteriosus.




                                                         Truncus

                                                         Conus
Conotruncus Embryology & Anomalies
TOF:
  Infundibular septum which arises from conal septum moves
       anteriorly and superiorly causing components of TOF
Conotruncus Embryology & Anomalies
DORV
 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
Conotruncus Embryology & Anomalies
DORV

   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%)
Conotruncus Embryology & Anomalies
DOLV
  In DOLV both coni
       are absent hence
       both arteries are
       posterior and arising
       from LV
Conotruncus Embryology & Anomalies
D - TGA
Conotruncus Embryology & Anomalies
Cono ventricular septal defects.
       Absence of conal(Infundibular) septum leads to formation of
        malaligned , subinfundibular , subpulmonic VSD .
Conotruncus Embryology & Anomalies
Conotruncus Embryology & Anomalies
Conotruncus Embryology & Anomalies
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Conotruncal anamolies

  • 1. Conotruncus Embryology & Anomalies Msn Pavan Kumar Nizam’s Institute of Medical Sciences ,Panjagutta,Hyderabad,India. [email protected]
  • 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. 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. Conotruncus Embryology & Anomalies Embryology - 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. Conotruncus Embryology & Anomalies Embryology - 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. Conotruncus Embryology & Anomalies Embryology - 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. Conotruncus Embryology & Anomalies Embryology - 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. Conotruncus Embryology & Anomalies Embryology - 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. Conotruncus Embryology & Anomalies Embryology – 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. Conotruncus Embryology & Anomalies Embryology – Important Structures For Development Of Conotruncus.  Second heart field cells and neural crest cell play important role in development of conotruncus
  • 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. Conotruncus Embryology & Anomalies Truncus arteriosus. Failure of aortopulmonary septum to Septation give rise to persistent truncus arteriosus. Truncus Conus
  • 13. Conotruncus Embryology & Anomalies TOF: Infundibular septum which arises from conal septum moves anteriorly and superiorly causing components of TOF
  • 14. Conotruncus Embryology & Anomalies DORV 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. Conotruncus Embryology & Anomalies DORV 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. Conotruncus Embryology & Anomalies DOLV In DOLV both coni are absent hence both arteries are posterior and arising from LV
  • 17. Conotruncus Embryology & Anomalies D - TGA
  • 18. Conotruncus Embryology & Anomalies Cono ventricular septal defects.  Absence of conal(Infundibular) septum leads to formation of malaligned , subinfundibular , subpulmonic VSD .