Congenital heart disease

1. DORV
2019

2. Double outlet right ventricle (DORV

4. Great vessels
Side by side Aorta usually to right to PA
Both usually arise at same level
Each from a conus and separated by outlet
septum ( conus septum)
No fibrous continuity

6. DORV • Upper panel shows
sub types of
Double outlet right
ventricle. Lower
panel shows
different
anomalies
encountered
depending on
degree of
overriding of the
pulmonary valve in
the trabacular
region. (DORV-
Double outlet right
ventricle, TGA-
Transposition of
great arteries, Ao-
Aorta, VIF-
Ventriculoinfundib
ular fold, PA-
Pulmonary artery,
OS- Outlet septum,
VSD- Ventricular
septal defect, TSM-
Trabacula septo-
marginalis)

7. • The variations in the
double-outlet RV complex.
The outlet septal location
defines the location of the
VSD. In those with a
subaortic VSD, (70% ½ of
them with and ½ without
PS) the outlet septal
attaches to the anterior
(Ant.) limb of the septal
band VSD is to right to
conus septum
• . subpulmonic VSD (10-
30%), the outlet septal
attaches to the posterior
(Post.) limb of the septal
band. VSD is to left to conus
septum
• In the doubly committed
VSD, there is absence of the
outlet septal.
• A remote VSD ( AV canal
Type is often so large that
the patient functionally has
a single ventricle.

10. • Obstruction of systemic
flow occurs with small
VSD or stenosis at
Subaortic conus
• Mitral valve obstruction
occur in 20%`

11. Clinically
• Subaortic resemble VSD
with systolic murmur acc P2
hyperactive pericordum But
with Mild Cyanosis
• Sub Aortic + PS ---Fallot
How to differniat
• Sub pulmonary Resmble
TGV with cyanosis HF but
PA appear in chest x ray
• Increase the intensity of
VSD murmur during follow
up may signify reduction in
VSD size which may close
spontaneously with
catastrophic event
• Obstruction of Systemic
flow may be present (
restrictive VSD , Sub aortic
obstruction ( stenosis)

12. ECG
RV, RA
Left axis in patients with DORV with
subaortic VSD with no PS
Left Ventricular Hypertrophy in
those with Restrictive VSD and
those with Big subaortic VSD with
no PS

13. Dextrocardia and DORV

14. • Echo
• site and size of VSD
• Relation of GV
• Coni
• 2 vessels arise from RV
with overriding > 50%

15. Sub
costal
ao to
left of
PA
sub
pulmon
ary VSD
Short
axis

19. Pulmonary
stenosis

20. Ao anterior
and to right
with sub
aortic VSD
Ao anterior and to right with
sub pul VSD

21. Ao anterior with
sub pul VSD

23. O2 saturation in Ao>
PA in sub aortic VSD
O2 sat in PA >AO in
sub pulmonary

24. DORV
Sub Aortic VSD
GV side by side arise each from a conus
Ao to R
Both Ao and Pul Valves arise at same level

25. DORV
Sub Pulmonary VSD
GV side by side arise each
from a conus
Ao to R
Dil PA

26. DORV
Doubly committed VSD
GV side by side arise each from a conus
Ao to R
Both Ao and Pul Arteries show simultaneous
opacification

27. DORV Remote VSD GV side by side
LV inj > > LV out Flow Deficiency ,only exit is VSD

29. • DORV with
subpulmonary VSD ,Ao
ant & Left
• Bilateral conus
• Two valves at same
level

30. TTT
• PG
• PA Banding
• Shunt
• Balloon atrial sep in case with sub pul VSD to
decompresss LA

31. • Repair of double outlet
right ventricle with a
subaortic VSD is
accomplished by creating
an intraventricular tunnel
that channels left
ventricular blood through
the VSD to the aorta. This
is facilitated by the use of
a patch that corresponds
to the circumference of
the aorta.
• double outlet right
ventricle with
subpulmonary
ventriculoseptal defect
• repair of the VSD to direct
the left ventricular blood
to the pulmonary artery,
followed by an arterial or
atrial switch procedure.
• LV to aorta may be done (
difficult)

32. • Repair of double outlet right
ventricle with doubly
committed ventriculoseptal
defect
• . The VSD, which is typically
large, usually does not create
difficulty in channeling left
ventricular blood to the aorta
with an intraventricular tunnel.
Concurrent pulmonary stenosis
or obstruction of the right
ventricular outflow tract due to
the tunnel may necessitate the
creation of a right ventricle
outflow patch or even a right
ventricle–to–pulmonary artery
conduit.
• Repair Of fallot type
VSD – aorta tunnel and relief PS
by patch graft or conduit
Remote VSD
AO to LV ( obstruction between
Mitral and tricuspid valve OR
PA to LV then switch
Fontan

33. Repair of double outlet right ventricle with noncommitted
ventriculoseptal defect
• Often involves univentricular repair.
• Crucial to biventricular repair is the distance
between Tric and mitrall annuli because the
aortic tunnel is constructed in this area.
• subaortic band between the subaortic conus
and conal septum is resected. The distance
between the tricuspid annulus and the ostium
infundibulum is measured. This last
measurement should allow for a patch or tunnel
that is at least the diameter of the aorta
• When the VSD is distant, tunnel repair is
associated with clinically significant subaortic
stenosis
• When the VSD is situated in the inlet septum, a
tunnel to connect the VSD to the ostium
infundibuli, followed by an arterial switch
procedure, the creation of a tunnel to the
pulmonary artery does not depend on the
pulmonary-tricuspid distance.
• Contraindications to performing a biventricular
repair include significant left ventricular
hypoplasia, major overriding, or straddling of
the atrioventricular valve