2. Introduction
Firstdescribed by Roger in 1879
Most common congenital heart disease
1.5 – 2.5/1000 live births
20% of CHD
Most common disorder in various chromosomal
disorders
Has Multifactorial etiology
3. Ventricular septum
Complex non – planar structure; 4 components
Inlet septum – lightly trabeculated; extends from
tricuspid annulus to attachments of tricuspid
valve
Trabecular septum – heavily trabeculated;
trabecular septum extends from inlet out to apex
and up to smooth-walled outlet
Outlet septum – non trabeculated; extends up to
pulmonary valve
3 diverge from small membranous septum
4.
5. Ventricular septation
Ventricles derived from 2 imporatant
components of primitive heart – inlet & outlet
Three septal components are necessary for
septation
Expansion of inlet & outlet components leads to
formation of partial septum between two –
primary interventricular septum
Two intrinsic septum in two segments called
inlet & outlet septum
mainly formed by inlet and primary septa
6. Ventricular septation
growth of two ventricles on either side causes
primary septum to be more prominent
inlet septum result from muscular
trabeculations in inlet region of ventricles
in same plane as that of atrial septum
third component comes from endocardial
cushion tissue – membranous portion of
ventricular septum
septation starts at about 37 days of gestation &
complete by 49th day of gestation
7. Muscular septum
During 5th week(day 30), muscular fold extends from
anterior wall of ventricles to floor
appear at middle of ventricle near apex and grows
towards AV valves with concave ridge
Most of initial growth achieved by growth of two
ventricles on each side of ventricular septum
In addition trabeculations from inlet region coalesce
grows into ventricular cavity at slightly different plane
than primary septum
inlet interventricular septum is at same plane as that of
atrial septum
8. Ventricular Outflow septation
from horse-shoe shaped condensed mesenchyme
embedded in endocardial cushion tissue
Just proximal to level of development of aorto-
pulmonary valves
Condensed mesenchyme will come in close
contact with outflow tract myocardium
Area just above bulboventricular fold appears to
reach out to condensed mesenchyme
Participate in septation of outflow tract by
providing an analogue to muscle tissue
9. Primary foramen
Communication between inlet & outlet
components
Exists because primary septum is incomplete
Divided into R & L by growing septation
L component forms LVO
Due to differential growth, LV apex formed by
inlet component
RV apex formed by outflow component
10. Interventricular Foramen
Bordered by concave upper ridge of muscular
interventricular septum and fused AV canal
endocardial tissue, closes at end of week 7
Achieved by growth of three structures: right and
left bulbar ridges and posterior endocardial
cushion tissue
Closes interventricular foramen and connect
ventricular septum to outflow septum
Connecting right ventricle to pulmonary trunk and
left ventricle to aortic trunk
11. Outflow Tract
Includes ventricular outflow tract and
aortopulmonary septum
Three embryological areas, conus, truncus and
pulmonary arterial segments
Each segment develop two opposing ridges of
endocardial tissue
Opposing pair of ridges and those from various
segments meet to form septum separating two
outflow tracts and aortopulmonary trunks
15. Perimembranous
most common defect
80% of surgical and autopsy series
usually extends into muscular, inlet, or outlet areas
synonyms: infracristal, membranous
16. Outlet
5%-7% of autopsy and surgical series (29% in Far
East)
situated just beneath the pulmonary valve
synonyms: supracristal, conal, infundibular,
subpulmonary,doubly committed subarterial
18. Muscular
5%-20%
Central: mid-muscular, may have multiple
apparent channels on RV side and coalesce to
single defect on LV side
Apical: multiple apparent channels on RV side
may be single defect on LV side as with central
defect
Marginal: along RV septal junction
"Swiss cheese" septum: large number of
muscular defects
19. a, outlet defect; b, papillary muscle of the conus; c, perimembranous defect
d, marginal muscular defects; e, central muscular defects; f, inlet defect; g, apical
muscular defects
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