Double outlet right ventricle (DORV) is a congenital heart defect where both the aorta and pulmonary artery arise primarily or entirely from the right ventricle. There are several surgical techniques used to repair DORV depending on factors like the location of the ventricular septal defect and relationship between the tricuspid and pulmonary valves. The most common repairs include an intraventricular baffle technique or arterial switch operation with VSD closure for Taussig-Bing anatomy. Early survival rates for DORV repair are over 95% at 15 years post-op, though outcomes depend on the specific DORV subtype and presence of other cardiac anomalies.

1. DOUBLE OUTLET RIGHT VENTRICLE

2. JOHN ABERNETHY

3. INTRODUCTION
 1793 - John Abernethy 1st described the
condition.
 1957 - Witham coined the term as DORV.
 1957 - 1st repair: Kirklin

4. DEFINITION
 When one great artery arises nearly or
wholely from RV and the other more than
50%.
 TOF with DORV is same as DORV with
pulmonary stenosis
 Taussig Bing Heart: variability in origin of
pulmonary artery

5. DORV

6. EMBRYOLOGY AND ANATOMY
 Conus: The muscle between semilunar and
AV valve.
 Lev’s theory of Conotruncal malseptation
 Van Praagh’s theory of Conal
underdevelopment

7. VAN PRAAGH THEORY OF CONAL
UNDERDEVELOPMENT
 “ the distal or semilunar part of infundibulum
or conus arteriosus performs an arterial
switch during cardiogenesis”

8. EXTENSION OF VAN PRAAGHS THEORY

9. VENTRICULAR SEPTAL DEFECT
 The only outflow tract of the left ventricle.
 Mostly conoventricular.
 Accordingly however DORV can be classified
with respect to VSD location.
 Subaortic
 Subpulmonary
 Doubly committed
 Non committed.

10. VSD IN DORV

11. CORONARY ARTERY ANATOMY
 Depends upon the great arteries
 At the transposition end of the spectrum:
LMCA and LAD pass anterior to PA
 TOF: LMCA and LAD pass posterior to PA.

12. HELLEN TAUSSIG AND RICHARD BING

13. THE TAUSSIG BING HEART
 Applied in physiological and anatomical
sense.
 Physiological
 Anatomical:
 Helen Taussig and Richard Bing (1949)
 Van praagh (1968) summarized anatomic
definition

14. THE TAUSSIG BING HEART

15. ASSOCIATED ANOMALIES
 Coarctation of aorta
 Arch hypoplasia
 Interrupted aortic arch

16. CLINICOPATHOLOGY AND HEMODYNAMICS
 Highly variable
 Large VSD with severe fixed pulmonary
stenosis: cyanotic child
 VSD with no pulmonary stenosis: features of
heart hailure with progression towards
pulmonary vascular disease.

17. STREAMING
 Determined by relationship of semilunar
valves to VSD and position and presence of
infundibular septum.
 Simple DORV: High Qp; heart failure; without
cyanosis
 Subpulmonary VSD: Taussig Bing heart:
SpaO2> SaO2. Infants present like TGA

18. CLINICAL FEATURES AND EVALUATION
 Type of symptoms and age of appearance
depends upon degree of pulmonary stenosis
 Echo:
 Size of VSD,
 Relation to semilunar valves
 Subvalvular conus
 AV valve abnormalities
 Position of great arteries
 Coronary artery anatomy

19. EVALUATION
 Cardiac catheterization: not in infants;
indicated in older children for
 PVR
 Ventricular end diastolic pressure
 MAPCA
 Cineangiography: Assessing complex
interrationships in DORV with superior-
inferior ventricles and criss cross hearts

20. MANAGEMENT: MEDICAL AND CATHETER BASED
 Decongestive medications: in cases of high
Qp heart failure; followed by surgery
 BAS: at the transposition end of DORV:
 Allows improved mixing
 Atrial septal defect allows decompression of left
side of heart: better surgical field

21. INDICATIONS AND TIMING OF SURGERY
 Diagnosis is sufficient for surgery
 Timing of surgery depends upon the subset
of the patients in question
 Simple DORV: electively by 3-6 months of age
 Sooner if symptoms of heart failure or failure to thrive
appear
 DORV with pulmonary stenosis: same as for
TOF
 Taussig Bing: during 1st month of age
 Operations including extracardiac conduit but
excluding ASO: 3-6 years of age.

22. SURGICAL MANAGEMENT
 1957 – Kirklin and 1958 – Barratt-Boyes: 1st
successful repair of DORV

23. ANATOMICAL DETERMINANTS OF METHOD OF
REPAIR
1. Separation of Pulmonary and tricuspid valve
2. Prominance of Conal septum
3. Presence of subpulmonic stenosis

24. SEPARATION OF PULMONARY AND TRICUSPID
VALVES

25. PROMINENCE OF CONAL SEPTUM
 Length of conal septum largely determined
by the development of subaortic conus
 A prominent conal septum will necissitate a
longer baffle pathway along its inferior
margin
 Long conal septum associated with shorter
distance between TV and PV: prone to
stenosis.

26. PRESENCE OF SUBPULMONARY STENOSIS
 At the tetralogy end of DORV spectrum
 Requires relief of stenosis.
 LV baffle pathway will further cause RVOT
narrowing: Infundibular outflow patch
 Significant stenosis precludes the technique
of ASO for Transposition end of DORV
spectrum

27. SURGICAL TECHNIQUES
 SA-VSD with adequate TV-PV distance:
intraventricular baffle technique
 PA-VSD in Tassig Bing without PS: ASO with
VSD closure
 Doubly committed VSD: Baffle
 Noncommitted VSD: baffle with myocardial
flap reconstruction of straddling TV

28. INTRAVENTRICULAR BAFFLE FOR
UNCOMPLICATED DORV
 Standard midline sternotomy/cardioplegic
arrest
 RA approach:
 technically difficult, No significant studies to show
that safer than ventricular approach
 Opened cephalocaudal near AV groove

29. INTRAVENTRICULAR BAFFLE
 RV approach:
 Transverse ventriculotomy in low RVOT
 Vertical infundibular ventriculotomy: if the
distance between LAD and RCA small
 Assessment of anatomy:
 VSD
 Distance between TV and PV

30. ASSESSMENT OF ANATOMY

31. INTRAVENTRICULAR REPAIR
 Enlargement of VSD: anteriorly,
 Risk of damaging LAD
 Risk of damaging MV
 VSD patch: Polyester tube graft diameter
>20% of Ascending aorta, Length: Anterior
edge of VSD to sub Ao conus
 Neonates and infants: standard pledgetted
interrupted suture technique
 Older: continuous

32. VSD PATCH IN INTRAVENTRICULAR BAFFLE
Flat patch : Knitted Daron Velour
Tube: PTFE

33. COMPLETED BAFFLE

34. REPAIR OF TAUSSIG BING ANOMALY
 Closure of VSD followed by ASO
 Approach
 Perimembranous VSD: RA
 Rvoutlet VSD and PA not overriding : Proximal
aorta (after harvesting coronary buttons)
 PA overridin: PA

35. TAUSSIG BING REPAIR BY INTRAVENTRICULAR
APPROACH
 Intraventricular tunnel repair: Kawashima
Method
 If insufficient space between TV and PV:
Patrick-McGoon method
 Lecompte Inraventricular repair

36. RASTELLI PROCEDURE

37. REV

38. NIKAIDOH PROCEDURE

39. DAMUS-STANSEL-KAYE PROCEDURE

41. KAWASHIMA METHOD
RV opened through vertical or transverse
incision

42. KAWASHIMA METHOD
VSD enlarged by incision anteriorly and
excision of some portion of infundibular
septum

43. KAWASHIMA METHOD
Intraventricular tunnel posterior to PA

44. PATRICK-MCGOON METHOD
Transverse ventriculotomy and VSD
enlargement of inlet septum

45. PATRICK-MCGOON METHOD
PTFE tube graft with diameter >20% of
ascending aorta;

46. PATRICK-MCGOON METHOD
Continuous sutuiring of the tube graft

47. PATRICK-MCGOON METHOD
Completion of spiralled tube graft

48. RESULTS
 Early survival: current hospital mortality <2%
for simple baffle procedure.
 Taussig Bing heart ~ 3.7%
 Time related survival: >95% @ 15 yrs
 Age at repair: Older age is a risk factor:
ecplained by absence or presencec of
pulmonary vascular disease

49. RESULTS
 Type of repair:
 Favourable subtypes: Simple DORV, DORV with
doubly committed VSD
 Unfavourable: DORV with subpulmonic VSD
 Major cardiac anomalies
 Pulmonary stenosis: incremental risk factor
 CAVCD, Hypoplasia of cardiac chamber, mitral
valve anomalies

50. COMPLICATIONS OF REPAIR
 Major reoperation are mainly for
 Leakage and obstruction of baffle: <1% catheter
based evidence of incidence
 LVOTO: manly due to potentially obstructing
muscle in the LVOTO

51. THANK YOU