conotruncal anamolies
DESCRIPTION
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.TRANSCRIPT
Msn Pavan KumarNizam’s Institute of Medical
Sciences ,Panjagutta,Hyderabad,India.
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
Embryology1.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
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.
Embryology - Septation of conus and truncus.
Conotruncus Embryology & Anomalies
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
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 - Rotation and absorption.
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 – Semilunar valves.
Second heart field cells and neural crest cell play important role in development of conotruncus
Conotruncus Embryology & Anomalies
Embryology – Important Structures For Development Of Conotruncus.
Conotruncal defects1. Truncus 2. TOF (with absent PV& PA)3. DORV4. DOLV5. D-TGA6. Conoventricular septal
defects7. Interrupted aortic arch
type B
Conotruncus Embryology & Anomalies
Failure of aortopulmonary septum to Septation give rise to persistent truncus arteriosus.
TruncusConus
Conotruncus Embryology & Anomalies
Truncus arteriosus.
Infundibular septum which arises from conal septum moves anteriorly and superiorly causing components of TOF
Conotruncus Embryology & Anomalies
TOF:
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
Type More prominent coni
Less prominent coni
VSD commited to
TOF(40%) Subpulmonic Sub aortic Aorta
VSD (15%) Subpulmonic Sub aortic Aorta
TGA(20%) Subaortic Sub pulmonic PA
Variants(DC<10%)
---- Both Both
Variants(NC<10%)
Both ---- Non committed
Conotruncus Embryology & Anomalies
DORV
In DORV, rotation of great arteries driven by conal development that changes not the position of VSD.
In DOLV both coni are absent hence both arteries are posterior and arising from LV
Conotruncus Embryology & Anomalies
DOLV
Conotruncus Embryology & Anomalies
D - TGA
Absence of conal(Infundibular) septum leads to formation of malaligned , subinfundibular , subpulmonic VSD .
Conotruncus Embryology & Anomalies
Cono ventricular septal defects.
Conotruncus Embryology & Anomalies
Conotruncus Embryology & Anomalies
Conotruncus Embryology & Anomalies
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