1. Double Outlet Right Ventricle
Presenter
Dr. Md. Azizur Rahman
MD ( Phase-A) Resident
Paediatric Nephrology

2. 1. Definition
A congenital cardiac anomaly in which both great arteries ( the aorta and pulmonary artery ) rise wholly or in large part from the right ventricle.
Double outlet right ventricle also includes lesions in which one great vessel and at least 50% of the other great vessel emerge from the right ventricle.

4. Frequency
In the US: DORV accounts for 1-1.5% of all CHDs, with an incidence of 1 per 10,000 live births.
Internationally: Incidence is the same as in the United States

5. Embryology
Failure of proper development of the outflow tracts will cause both outflow tracts to emerge from the right ventricle.

6. Pathophysiology
Both great arteries arise from the right ventricle in association with a nonrestrictive VSD.
The pathophysiology depends on the position of the VSD & the presence/absence of pulmonary stenosis.
Left-to-right shunting across the VSD results in pulmonary over circulation, pulmonary hypertension, and congestive heart failure.
Pulmonary stenosis results in right-to-left shunting and cyanosis.

7. Hemodynamics of DORV

8. Morphology of DORV 1. VSD; . Subaortic 3. Great arteries
. Subpulmonary
. Doubly committed
. Noncommitted or remote
2. Infundibulum
. Absent
. Single
. Bilateral in general
3. Great arteries
. Normal or near-normal
. D-malposition
. L-malposition
4. Pulmonary stenosis
5. Associated anomalies
PS, CoA, PDA,,
A-V canal, multiple VSD
in 30 %

9. Locations of VSD in DORV
a ; Subaortic
b ; Subpulmonic
c ; Doubly committed
d ; Noncommitted

10. DORV with Subaortic VSD
Aorta
VSD

11. DORV with Subpulmonic VSD
Aorta
DORV with Subpulmonic VSD PA
VSD

12. Morphologic Syndromes
1. Simple DORV
2. Taussig-Bing Heart
3. DORV with doubly committed VSD
4. DORV with noncommitted VSD
5. DORV with L-malposition
6. DORV with complete ECD
7. DORV with superior-inferior ventricles

13. History It’s depends with PS or without PS
Without PS ( blood flow to pulmonal  )
- respiratory tract infection repeatedly
- congestive heart disease
With PS (blood flow to pulmonal  )
- hypoxic spell

14. Symptoms Symptoms of DORV may include:
Baby tires easily, especially when feeding
Bluish skin color (the lips may also be blue)
Failure to gain weight and grow
Pale coloring
Sweating
Swollen legs or abdomen
Trouble breathing

15. Physical Examination Without PS : sign of congestive heart disease
With PS : cyanosis
Auscultation :
- S2 is increase if there is PH
- S2 is single if position of PA behind of aorta( malposition )
- Ejection systolic murmur in ICS 2-3 left parasternal
if there is PS
- Holosystolic murmur in ICS 2-3 left parasternal
and mid diastolic rumble in apex if DORV+VSD
without PS

16. Lab Studies
Routine laboratory studies are not required for the initial diagnosis and management of children with DORV.
Obtain a hemoglobin and hematocrit assessment if children are thought to have polycythemia.
Monitor serum electrolytes after treating children with diuretics, glycosides, and afterload-reducing agents.

17. Electrocardiography
The RV will be hypertrophied with right axis deviation and possible RAE.

18. Imaging Studies DORV without PS :
Cardiomegaly, Pulmonary knob , plethora
DORV with PS
Size of Heart is normal , pulmonary segment is concave, pulmonary vascular is oligaemic
Patients with subaortic VSD and severe pulmonary stenosis demonstrate diminished pulmonary vascularity and concave left heart border (similar to appearance associated with tetralogy of Fallot).
Patients with subpulmonary VSD is similar to that in patients with transposition of the great arteries, revealing increased pulmonary vascularity and cardiomegaly.

19. Echocardiography
Echocardiography is the imaging technique most often used to diagnose DORV.
The principle diagnostic feature is appearance of both great arteries primarily committed to the right ventricle.
Parasternal long and short axis views reveal degree of commitment to the right ventricle.
Subcostal and apical 4-chamber views depict the separation between semilunar and atrioventricular valves (ventriculoinfundibular fold).
Use multiple views to determine the relationship between the ventricular septum and the outlet septum.

20. Echo 2 dimension :
- The location and size of VSD
- The position of aorta and pulmonary artery out from ventricle
PS +/-
Confluence and diameter of PA
The position of semilunar valve from atrioventricular valve.

21. Echocardiography with Doppler
Degree of PS
Is there regugitation of atrioventricular valve

23. Angiography To measuresment the increase of pressure in RV and PA
Opacification of both great arteries following right ventriculography
Similarity of aortic and pulmonary valve horizontal planes
Frequent anterior malposition of the aorta
Presence of a filling defect dividing the two outflow tracts
To measuresment the increase of pressure in RV and PA

24. DORV Angiography

25. Double Outlet Right Ventricle
1. Simple DORV : similar to simple large VSD
2. Taussig-Bing heart : similar to TGA+VSD,
but more unfavorable
3. DORV+PS or atresia :similar to TOF or atresia
Natural history in some patients is dominated by an associated cardiac anomalies such as AV canal defect.

26. Operative Techniques 1. Intraventricular tunnel repair
. Simple DORV
. Subaortic VSD & PS
. Doubly committed
. Noncommitted
. Taussig-Bing heart
2. Switch repair ( arterial, atrial )
3. Rastelli or Lecompte repair
. With intraventricular tunnel repair
4. Nikaidoh aortic translocation
5. Total cavopulmonary connection
. Noncommitted VSD with PS
6. Palliative operations

27. Surgical Strategy of DORV
Intraventricular Tunnel
DORV-Adequacy of distance between TV and PV for an intracardiac baffle vs. Rastelli

28. Intraventricular Tunnel Repair Taussig-Bing Heart

29. Rastelli Operation Taussig-Bing Heart

30. Taussig-Bing Heart with COA
Combined aortic arch repair and arterial switch
without coronary re implantation

31. Surgical Results of DORV
1. Survival
. Early deaths
. Time-related survival
2. Risk factors for premature death
1) Age at repair
2) Type of DORV
3) Associated cardiac anomalies
4) Surgical era
5) Type of operation
. Atrial switch operation
. Complex tunnel repair
. Transannular patch or conduit
3. Complications of intraventricular tunnel repair
. Leakage
. Obstruction

32. Operative Indications
Simple DORV with subaortic VSD
. Without PS : 6 months of life
. With PS : same as TOF
2. DORV with subpulmonary VSD
. Without PS : 1st month of life
. With PS : 2 to 4 years of age
3. DORV with doubly committed VSD
. Same as simple DORV
4. DORV with noncommitted VSD
. VSD enlargement & tunnel
. Extracardiac conduit repair
. LeCompte procedure
. Fontan-type repair

33. Treatment DORV with VSD subaotic or subaortic doubly commited and PS :
- Variant of TOF with overide of Aortic more
than 90 %, The treatment look at TOF algoritym

34. Treatment
DORV with VSD subaotic or subaortic doubly commited without PS
- Because pressure of PA as same as with systemic pressure , to prevent Pulmonary Vascular Disease
Paliative operation : PA banding.
When 1 years old and pulmonary vascular still reactive with O % have be done VSD closure and tunneling patch , if not reactive
 VSD closure with perforated patch or conservative

35. Treatment
Repair of double outlet right ventricle with subaortic ventricular septal defect.

36. DORV with VSD subpulmonic and PS :
- If with history severe spell hypoxic  BT shunt
- If PS non resectable  operation same as with
TGA,VSD and LVOTO(PS) : valve conduit
homograft from RV to PA ( Rastelli operation )
But if smallest LV : considerable with fontan
operation
- If PS resectable: ASO with PS resection
(Valvulotomy and subvalvar resection

37. DORV with VSD subpulmonic without PS
- Taussig Bing Anomaly
- If age < 6 month old : ASO and VSD closure
without catheterization
But if age > 6 month ( Suspect Pulm.Vasc Disease)
oxygen test .If reactive: ASO with VSD closure
with perforated patch
If not reactive : conservative.

38. Complex repair of double outlet right ventricle with subpulmonary ventricular septal defect.

39. DORV with VSD non commited with PS
- Univentricle Repair ( BCPS  Fontan )
- If age < 6 month : BT shunt
DORV with VSD non commited without PS
- PA Banding before 6 month old to prevent
Pulm.Vasc.Disease,
after that when 1 years old : catheterization
- BCPS , Fontan operation

40. Repair of double outlet right ventricle with noncommitted ventricular septal defect using a long ventricular septal defect patch.

41. Prognosis
Improvement in surgical techniques in recent years has resulted in good outcomes for most patients born with CHD.
Prognosis for infants born with DORV and TGA generally is good, although it is dependent on specific anatomy. For example, patients with DORV and TGA with a subaortic VSD and no other anatomic abnormalities (eg, left ventricular hypoplasia) are likely to do well after surgery.

42. Patients with restrictive VSD may not do as well because this is a particularly difficult problem. Enlargement of VSD is difficult and likely to result in complications, such as conduction abnormalities (AV block).

43. Thank you