Double outlet right ventricle.

1. DORV
Dr. PRAMOD KUMAR

2.  DORV is a heterogeneous group of congenital heart defects in which both the aorta and
main pulmonary artery arise entirely or predominately (>50%) from the morphologic
right ventricle .
 DORV accounts for 1–5 % of all congenital heart diseases with incidence
of 1 per 10,000 births.
 No known racial or gender predilection.
 Most cases are sporadic and
 No identifiable genetic cause.

3. DORV is the spectrum of conotruncal defects.
Conus: is a circular tube of muscle beneath the valves of each great artery in equal
distribution, creating fibrous discontinuity with the atrioventricular valves.
Normally, the muscle under the aortic valve resorbs whereas the subpulmonary conus
continues to grow.
This therefore produces a right and posterior position of the aortic valve with mitro-
aortic fibrous continuity and Left & anterior position of the pulmonary valve with a
fibrous discontinuity with the tricuspid valve .
In conotruncal defects, there is a spectrum between heart anomaly, in which minimal
conus exists beneath the aorta, as seen in tetralogy of Fallot, and no conus exists under
the pulmonary valve, as with transposition of the great arteries .

4. DORV falls in the middle of the spectrum, that have variable amounts of conus under each
semilunar valve.
One end of spectrum is TOF type DORV:
“TOF type”: Near-normal length of conus beneath the pulmonary valve and minimal conus
beneath the aortic valve. Consequently, there is No aorto-mitral continuity, and the
pulmonary valve is left, anterior and superior.
Other end of spectrum is TGA type DORV:
“Transposition type”: Large amount of conus under the aortic valve with relatively little
under the P-Valve. Aorta is pushed anteriorly and superiorly, resulting in rightward
positioning of the aorta relative to the pulmonary artery. No pulmonary-mitral continuity.
Between the TOF type and TGA type:
There are many variants with bilateral conus that have neither aorto-mitral , nor pulmonary-
mitral continuity and have variable distribution of conal septum.

6. Solitus (Normal)
Inversus
Side by side
D-Malposition
(D-MAG)
Side by side
L-Malposition
(L-MAG)
L-Transposition
(L-TGA/CC-TGA)
D-Transposition
(D-TGA)
Spatial Relationship Of Great Arteries

7. Van Praagh developed the most widely accepted classification of DORV, which included
the following:
• DORV with a subaortic VSD
• DORV with a subpulmonic VSD
• DORV with a doubly committed (both subaortic and subpulmonic) VSD
• DORV with a remote VSD (noncommitted)

8. Type of DORV % of
DORV
case
Subaortic VSD-type
DORV
(tetralogy of Fallot-type)
>50 %
Subpulmonary VSD-type
(k/a Taussig–Bing
anomaly)
30%
Doubly committed VSD-
type
10%
Noncommitted or remote
VSD-type DORV
10%

9. Four types of great artery relationships at the level of the semilunar valves have been
described in DORV:
• Aorta right and posterior to the pulmonary artery (normal)
• Aorta right and lateral to the pulmonary artery (side by side)
• Aorta right and anterior to the pulmonary artery (dextro-malposition)
• Aorta left and anterior to the pulmonary artery (levo-malposition)

10. Sridaromont et al
evaluated
angiographically 72
patients with proven
DORV and identified 16
possible combinations.
•1st MC : Subaortic VSD (68%)  subaortic VSD with side-by-side great vessels (46%)  subaortic VSD
with dextromalposition of the great vessels (right anterior aorta) (16%).
• 2nd MC: Subpulmonary VSDs usually with dextro-malposition of the great vessels (10%)
subpulmonary VSD with side-by-side great vessels (8%) .
1st MC
1st MC 2nd MC
MC

11. Sridaromont et al
evaluated
angiographically 72
patients with proven
DORV and identified 16
possible combinations.
• Doubly committed VSDs (3%)VSD is usually large and in the superior position, closely
related to both semilunar valves.
• Remote or noncommitted VSD (7%) with muscular VSD.

12. Pulmonary Stenosis (PS) is the most common lesion associated with DORV.
Occurs in ~50% of patients and may be valvular or subvalvar.
Secundum-ASD are seen in 25% of all types, whereas primum-ASD are seen in the 8% of
DORV patients .
Coronary arterial anomalies occur in about 10% of patients—most commonly
anomalous origin of the LAD from the RCA. It is of surgical importance, because they
may alter considerations for surgical repair due to their effect on feasibility of conduit
placement or coronary arterial transfer .
Associated aortic arch coarctation, hypoplasia, or interruption—also found in about 10%
of patients.
Rarely, the VSD will be absent, and it is accompanied by a small ASD and hypoplasia of
the mitral valve and hypoplastic left ventricle .

13. > 50 % of DORV cases .
Great arteries spatial relationship : Maintained, Aortic origin posterior and to the right
of the pulmonary origin .
Pulmonary stenosis is present in up to 50 % of patients. In these patients, the
physiology resembles that of tetralogy of Fallot, where the aorta completely overrides
the right ventricle.
In the absence of pulmonary stenosis, the physiology resembles that of a large isolated
VSD . This anatomy may result in congestive heart failure.
Subaortic VSD is associated with L-transposition of the great arteries and an
anomalous course of the right coronary artery, which crosses the pulmonary outflow
tract.

14. c) Aorta and the PA are arising from the RV.
Most of the ventricular outflow is directed toward the
aorta, which is larger than the pulmonary artery
a). Great arteries
spatial relationship :
Maintained.
Aorta posterior and
right to the pulmonary
artery
b) Large VSD (arrow).

15.  30 % of patients with DORV .
 The left ventricular outflow is directed toward the pulmonary artery, resulting in
pulmonary artery saturations greater than aortic saturations.
The great artery relationship is transposed . The aortic and pulmonary origins have
either a parallel arrangement (positioned side by side) or the aorta is to the right and slightly
anterior to the pulmonary artery (D-Malposition) .
In the absence of pulmonary stenosis, the physiology is similar to that of transposition of
the great arteries (TGA).
 If there is associated pulmonary stenosis, the physiology is similar to that of tetralogy of
Fallot (TOF).
It is associated with subaortic stenosis, aortic arch obstruction (aortic coarctation and
interrupted aortic arch), straddling and cleft mitral valves.

16. a) Aorta and PA in a parallel arrangement (positioned side by side).
b) Aorta and PA are arising from the RV.
Most of the ventricular outflow is directed toward the pulmonary artery

17. a) Aorta anterior and right to the PA (D-transposition).
b/c) Aorta and PA are arising from the dilated RV.
(c) Very large VSD.

18.  10 % of cases of DORV.
The left ventricular outflow is equally directed to the aorta and pulmonary artery.
The great arteries are normally related .
Since the left ventricular outflow is equally shared by the aorta and pulmonary
artery, the pathophysiology resembles that of a VSD.
4. Non-committed or remote VSD-type DORV
10 % of DORV cases.
 Most commonly the great arteries are normally related .
The anatomy and physiology is similar to that of an isolated VSD .

19.  Patients present by age range 1 day–4 years.
Presentation is variable and is dependent on the type of DORV and associated cardiac
anomalies.
DORV with subaortic VSD +PS (TOF type):
C/F indistinguishible from classic Tetrology of Fallot.
Cyanosis develops and progresses during the early month of life..
Hypoxic spell may occur.

20. DORV with subaortic VSD without PS:
In absence of PS Increased flow to pulmonary circulation PAH.
C/F resembles that of large isolated VSD.
DORV with subpulmonary VSD ( Taussing Bing malformation):
C/F similar as TGA with VSD.
Cyanosis in newborn.
Early development of Heart failure.
Increasing breathlessness, poor feeding and slow weight gain are prominent feature.
Associated coarctation of aorta is frequent and leads to early (1st week of life) onset of HF.

21. Most patients with DORV are diagnosed in the first month of life and undergo
Palliative repair (pulmonary artery banding or Blalock–Taussig shunt) or Surgical repair.
The surgical interventions depend on the location of the VSD, the size of the left ventricle,
type of ventriculo-arterial connection, and the type of pulmonary blood flow (restricted on
unrestricted) .
Definitive repairs vary and include :
Arterial switch operation to connect the left ventricle to the neo-aorta in combination with
VSD closure. The subpulmonic or “Taussig–Bing” anomaly with physiology similar to
transposition may be treated with an arterial switch operation.
Bidirectional Glenn shunt (superior vena cava to pulmonary artery) .
Intraventricular repair: for the subaortic and doubly committed VSDs .
Fontan procedure: For directing the systemic flow of venous blood to the lungs without
passing through a ventricle.

22. Fig: Original Fontan procedure,
SVC was connected to the RPA and the
RA to MPA.
Fig: Modified Fontan procedure.
RA was connected to MPA.

24. SCA was connected to PA

25. Potential complications after surgical repair include :
 Residual or recurrent VSD,
 Residual or recurrent outflow tract obstructions,
 Atrioventricular valve regurgitation
stenosis/narrowing of the Glenn shunt or Fontan procedure.

26. THANK YOU