1. Double Outlet Right Ventricle
Dr. Kshitij Mavade

2. Introduction Newfield et al –
Both great arteries and arterial trunks arise exclusively from RV, neither semilunar valve is in fibrous continuity with either AV valve, and usually, a VSD is present and represents the only outlet from the left ventricle.
Pulmonary valve or subpulmonary stenosis may be present or absent.

3. Introduction
Lev et al. One complete arterial trunk and at least half of the other arterial trunk emerge from RV, and there may or may not be mitral-aortic or mitral-pulmonary continuity
Van Praagh et al – Relied on subaortic and subpulmonary conus to produce mitral discontinuity as diagnostic marker.
Kirklin- Malformation in which the whole of one great artery and more than half of the other originates from the right ventricle

4. Anderson and his colleagues(Tynan et al)
DORV- abnormal ventriculoarterial connection characterised by more than half of each great artery originating above the morphologic right ventricle

5. HISTORY
1703
The earliest report in French
1793
John Abernathy, an assistant surgeon at St Bartholomew’s Hospital in London, described “partial transposition”
1898
Karl von Vierordt called double outlet right ventricle partial transposition to signify that the aorta was transposed but the pulmonary trunk was normally aligned
1957
Witham introduced double outlet right ventricle as a diagnostic term for a partial transposition complex

6. HISTORY
Kirklin, first repair of DORV with subaortic VSD, at the mayo clinic
Neufeld et al – physiological classification based on presence or absence of PS & position of VSD
1968- Patrick & Mcgoon- surgical classification based on relationship of GA & VSD

7. Incidence <1% of CHD 0.06 case per 1000 live birth
No sexual or racial predilection
No associated Genetic defect identified
Mostly sporadic, 22q11 deletion associated with some cases DORV

8. EMBRYOLOGY
Goor & Edwards: - DORV appears to represent a primitive embryologic condition because of failure to achieve conotruncal rotation and left shift of the conus.

10. CONUS: A circular tube of muscle, upon which semilunar valve sits up.
The more conal muscle present beneath a semilunar valve, the more that valve is pushed superiorly and anteriorly.

11. Van praagh theory of conal underdevelopment
“ the distal or semilunar part of infundibulum or conus arteriosus performs an arterial switch during cardiogenesis”
TOF: underdevelopment of pulmonary conus: AO is more anterior and to the right and superior, Conoventricular VSD with anterior malalignment of conal septum: RVOTO.
Extension of Van Praagh:

12. DORV represent a part of the whole spectrum of conotruncal anomaly
Spectrum of conotruncal anomalies
VSD TOF DORV TGA
(double coni) P P A A A P A P

14. PATHOLOGIC CLASSIFICATION
Based on
Location of VSD to great arteries
Great artery relationship

15. 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.
Conoventriccular: between the two limbs of TSM.
Sub aortic: TOF end: no sub aortic conus: superior margin of VSD is the Ao valve itself.
Sub pulmonary: TGA end: Progressive Subaortic conus; Ao Valve moves cephalad; and PV more intimately associated: Subpulmonary.
Non committed: VSD not in conal septum or Jn of conal and interventricular septum(subaortic) : remote from aorta: difficult to direct LV flow to aorta:: inlet type
Sub aortic VSD: VIF: posterior margin of VSD. Bundle away
Subaortic Perimembranous VSD near TV : Posterio division of TSM is deficient: Bundle lies in poesterior inferior margin of VSD.

16. RELATIONSHIP OF GREAT ARTERIES AT SEMILUNAR VALVE LEVEL
Right posterior aorta
Right lateral aorta
(side by side relationship)
Right anterior aorta
Left anterior aorta.

17. POSITION OF VSD Sub-aortic type Sub-pulmonary type
(Taussig Bing complex)
Doubly committed
Remote type

18. GREAT ARTERY RELATIONSHIPS AT THE LEVEL OF SEMILUNAR VALVES
Right posterior aorta The aortic valve and trunk originate from the right ventricle at a location posterior and to the right of the pulmonary valve and its arterial trunk
Right lateral aorta (side-by-side relationship) The aorta is to the right of the pulmonary artery, and the semilunar valves lie approximately in the same transverse and coronal plane. This is the classically described great artery relationship in DORV

19. Right anterior aorta (D-malposition)The aorta is to the right and anterior to the pulmonary artery. This grouping also may include some cases with the aorta directly anterior
Left anterior aorta (L-malposition)The aorta is to the left and anterior to the pulmonary artery. Thihs is least common great artery position.

21. 4 x 4 TYPES OF DORV, AND MORE…
16 possible variations of DORV based on the great artery relationships and the location of the VSD
In addition, an intact ventricular septum allows four other possible types of DORV, depending on the great artery relationships

22. However, these include only cases with situs solitus of the atrial and viscera, AV concordance, and two well-developed ventricles and AV valves
Multiple other variations and combinations are possible if one also includes situs inversus and situs ambiguus as well as AV discordance

24. DORV with Subaortic VSD
Aorta
VSD

25. DORV with Subpulmonic VSD
Aorta
DORV with Subpulmonic VSD PA
VSD

26. PHYSIOLOGIC CLASSIFICATION
Depends on:
Presence or absence of associated pulmonary stenosis.
Relationship of VSD to great arteries.
DORV may simulate that of large VSD, TOF, TGA.

27. ASSOCIATED ANOMALIES Pulmonary Stenosis - most common – 40-70%,
- frequently co-exists with subaortic VSD (80%);
- bicuspid pulmonary valve;
- rarely seen in subpulmonary VSD type.

28. ASSOCIATED ANOMALIES Subaortic stenosis – 3%;
Frequently co-exist with sub-pulmonary VSD type (50%).
Coarctation – 12%;
frequent in subpulmonary VSD type 50%.
Mitral valve anomalies – 10%; -
frequent in remote VSD
ASD – 10%
TAPVC – 2%
AV canal – 5%, Common in remote VSD.

29. CORONARY ANOMALIES- Normal Similar to TOF- LAD from RCA
Similar to TGA- RCA from the right posterior aortic cusp & LCA from the left posterior cusp
Anomalous origin of the left Cx from RCA,
single coronary ostium, origin of right Cx from LCA.

30. DORV – MAJOR CLINICAL PATERNS
Group 1 - Sub aortic VSD with PS (resembles TOF) Group 2-Sub pulmonary VSD, with or without PS (resembles TGA). Group 3-Sub aortic VSD, no PS, (resembles VSD) Group4- Sub aortic VSD with PVOD (Resembles Eisenmenger– complex

31. SUB AORTIC VSD WITH PS 50 % of DORV with subaortic VSD have PS
RV aorta
LV aorta
Aortic saturation decreased
PBF decreased
Resemble TOF

32. SUBPULMONARY VSD LV pulmonary trunk RV aorta
Pulmonary saturation > Aortic
If PVR decreased good aortic saturation;
LV volume overload
If PVR increases Blood from LV and RV Aorta Aortic saturation decreases.

33. SUBAORTIC VSD WITH DECREASE PVR WITH NO PS
- Resembles VSD
- PBF increased
-Aortic saturation is normal.

34. SUBAORTIC VSD – INCREASE PVR
-Increased blood to aorta
-Decreased flow to pulmonary trunk
-Aortic saturation falls

35. CLINICAL FEATURES

36. GROUP 1: SUBAORTIC VENTRICULAR SEPTAL DEFECT AND PULMONARY STENOSIS
Features similar to TOF
PS is severe- early cyanosis,
failure to thrive,
exertional dyspnea,
squatting,
polycythemia
Cyanosis
clubbing may be evident.

37. GROUP 1: SUBAORTIC VENTRICULAR SEPTAL DEFECT AND PULMONARY STENOSIS..
The precordium show evidence of a right ventricular impulse at the left sternal border, and a prominent systolic thrill upper left sternal border.
Grade 4 to 5/6 systolic ejection murmur, which radiates into the lung fields
The first heart sound is normal, and the second heart sound is usually single.

38. GROUP 2: SUBPULMONARY VENTRICULAR SEPTAL DEFECT
Features resembling those in TGA with VSD.
These patients present with cyanosis and heart failure in early infancy.
When PS is also present, the cyanosis and polycythemia may be more severe

39. GROUP 2: SUBPULMONARY VENTRICULAR SEPTAL DEFECT..
A precordial bulge and right ventricular impulse are present at the left sternal border.
A grade 2 to 3/6 high-pitched systolic murmur may be present at the upper left sternal border. .

40. GROUP 2: SUBPULMONARY VENTRICULAR SEPTAL DEFECT..
When PS is present,
a systolic thrill may be present, and the murmur is loud (grade 3 to 4/6).
The second heart sound is loud and single because of the proximity of the aorta to the chest wall .
With increased pulmonary flow, an apical diastolic rumble may be present.

41. GROUP 3: SUBAORTIC VENTRICULAR SEPTAL DEFECT WITHOUT PULMONARY STENOSIS
Patients present features typical of those with a large VSD and pulmonary hypertension.
Usually, little cyanosis is evident, but failure to thrive and heart failure are dominant features.
With increased pulmonary flow, respiratory tract infections are frequent.

42. GROUP 3: SUBAORTIC VENTRICULAR SEPTAL DEFECT WITHOUT PULMONARY STENOSIS..
A systolic thrill may be present at the upper left sternal border, and a grade 3 to 4/6 holosystolic murmur may be evident at the left sternal border.
An apical diastolic rumble and a third heart sound are audible at the cardiac apex.

43. GROUP 4: SUBAORTIC VENTRICULAR SEPTAL DEFECT WITH PULMONARY VASCULAR OBSTRUCTIVE DISEASE
Pulmonary flow is reduced, and heart failure and frequent respiratory infections are less evident.
Cyanosis and clubbing may be present.

44. GROUP 4: SUBAORTIC VENTRICULAR SEPTAL DEFECT WITH PULMONARY VASCULAR OBSTRUCTIVE DISEASE
On examination,
The systolic murmur may be diminished or absent
The second sound is very loud and single.
A decrescendo diastolic murmur of pulmonary valve insufficiency may be present.

45. ELECTROCARDIOGRAPHIC FEATURES
Right ventricular hypertrophy and right-axis deviation are the most common features
Combined ventricular hypertrophy - markedly increased pulmonary flow (observed in patients with subpulmonary VSD.
First-degree AV conduction delay is a common feature; however, it is not uniformly observed.

46. ELECTROCARDIOGRAPHIC FEATURES
Right atrial enlargement -patients with PS
left atrial enlargement may be observed in instances of increased pulmonary flow with intact atrial septum.
Patients with complete AV septal defect associated with DORV also typically have left axis deviation, combined ventricular hypertrophy, atrial enlargement, and first-degree AV conduction delay .

48. ECG showing deep S wave in V5-6 indicating right ventricular hypertrophy. Biventricular hypertrophy is manifested by large RS complexes in leads V3-6, in a case of DORV, subaortic VSD.

49. ECG showing peaked right atrial P waves in leads2 and V1
ECG showing peaked right atrial P waves in leads2 and V1. q waves appear in lead 1 and aVL despite right axis deviation. Right ventricular hypertrophy manifested by tall R waves in leads V1 and aVR. The qR pattern in leads V5-6 indicates that left ventricle is well developed, in a case of DORV with subaortic VSD and severe PS.

50. ECG showing peaked right atrial P waves in leads 2,3, aVF
ECG showing peaked right atrial P waves in leads 2,3, aVF. The QRS axis is rightward. Right ventricular hypertrophy is reflected by tall R waves in leads V1-2 and in leads aVR and by prominent S waves in left precordial leads, in a case of DORV and subpulmonary VSD.

51. ECG Distinguishing points from TOF
Counter clockwise initial force with q waves in leads I & aVL, even when the axis is vertical or rightward.
Deep & prolonged terminal force with broad, slurred S waves in leads I, aVL & V5-V6 and broad R wave in lead aVR.

52. CHEST X RAY In patients with PS Features may resemble TOF
Mild degree of cardiomegaly
Pulmonary vascularity is diminished
MPA segment is absent, which results in a concave upper left border of the heart

54. DORV subaortic VSD pulmonary atresia

55. Generalized cardiomegaly Prominent main pulmonary artery segment
In cases of subaortic VSD without PS -
Generalized cardiomegaly
Prominent main pulmonary artery segment
Increased pulmonary vascularity

56. DORV with subaortic VSD

57. Taussig Bing anomaly

58. When PVR is high -
Prominent MPA
peripheral pruning

59. ECHO 4 OBSERVATIONS NOTED FOR DIAGNOSIS
Origin of one great vessel from the RV and overlie of at least 50% or more of the other great artery over the RV
Mitral- semilunar discontinuity with conus
Absence of LVOT other than VSD
Spatial relationship of great arteries determined by bifurcation of pulmonary artery and branching of aorta.

60. DORV: Aortic-mitral Discontinuity
Aortic - mitral discontinuity in DORV is best shown in the parasternal long-axial view. LA LV RV Ao

62. DORV with subaortic VSD

65. DORV with sub pulmonic VSD

66. DORV with doubly committed VSD

67. DORV with remote VSD

68. DORV with remote VSD

69. Echo Evaluation of DORV: Role of Echocardiography in Planning Surgical Strategy:
The crucial question:
IS TWO VENTRICLE REPAIR FEASIBLE?
Two ventricle repair is preferred over the single ventricle (Fontan) option whenever feasible:
Better long term survival
Less arrhythmias
Better functional capacity

70. Role of Echocardiography in Planning Surgical Strategy: Criteria for Two Ventricle Repair
Two good-sized ventricles
No straddling of either of the AV valves
The VSD should be suitably located for intra-ventricular re-routing
No significant AV valve tissue in the way between VSD and the aorta

71. Echo Evaluation of DORV: Suitability for two-ventricle repair
Good separation between Pulm. and Tricusp. Valves:
Separation between Pulm. and Tricusp. Valves < Ao annulus
Minimum separation between Pulm. and Tricusp. Valves

72. Repair of DORV with remote VSD requires creation of a complex intraventricular baffle using multiple patches (the Barbero-Marciel technique)

73. Echo Evaluation of DORV: Two Ventricle Repair Candidates- Additional Issues
Branch PAs
Origin of LAD in the TOF type of DORV
The origin of coronary arteries in the Taussig-Bing anomaly prior to an arterial switch operation
Additional muscular VSDs which may require to be closed at the time of surgery
Other associated anomalies

74. Echo Evaluation of DORV Prior to Single Ventricle Repair
Echo complements cardiac catheterization
Ventricular function
Presence and severity of AV valve regurgitation
Size of the branch pulmonary arteries
Peripheral pulmonic stenosis if any
Severity of pulmonic stenosis (indirect estimate of PA pressures)

75. CARDIAC CATHETERISATION
OBJECTIVES: to evaluate
Routability of VSD to aorta
Branch PA anatomy
Pulmonary vascular resistance
Coronary artery and aortic arch anatomy.

78. Natural History
1 -Infant without PS may develop severe CHF -Later PVOD -Spontaneous closure of VSD –fatal,rare 2 –When PS present ,complications of CCHD (Polycythemia,CVA)

79. Natural history
3 -Taussig Bing –severe PVOD develop early as in TGA 4 -Associated anomalies –COA - LV hypoplasia - poor prognosis

80. Management Includes Medical care and surgical management
Optimize medical treatment before surgical intervention
Surgical – palliative or definitive
Thorough evaluation important before any plan is made

81. MEDICAL MANAGEMENT
Inadequate pulmonary blood flow – maintain ductal patency.
Prostaglandin E1(ie, alprostadil) is the standard of care until repair can take place.

82. MEDICAL MANAGEMENT
Clinical picture of congestive heart failure –
diuresis,
inotropic support

83. When double outlet right ventricle repair is planned – review :
PS or PAH
Single or Two ventricle repair
Relationship of great vessels
Location of the ventriculoseptal defect (VSD) and its size
Associated lesions

84. Accurately determine surgically relevant features :
Separation of the pulmonary valve from the tricuspid valve relative to the diameter of the aortic valve annulus
Location of the VSD, including degree of involvement of the conal septum
Chordal attachments to the conal septum, VSD ridge, and presence of straddling chordae

85. Degree of subpulmonary stenosis and whether it is fixed or dynamic
Degree of pulmonary valvar stenosis
Coronary anatomy
Relative size of the great vessels and their relationship
The aortic arch and the presence of coarctation

86. Surgical Care - Palliative
As with medical treatment, this approach helps improve the patient's clinical condition, allowing the baby to gain weight to achieve optimal conditions for definitive surgical repair
With pulmonary stenosis, a systemic-to-pulmonary shunt, such as a modified Blalock-Taussig shunt, may be required if SpO2< 70%

88. Surgical Care - Palliative
Variations with increased PBF and CHF–
may first require palliation with
pulmonary artery banding.
NOT RECOMMENDED for
infants with Sub Aortic VSD or Doubly Committed VSD.
Primary repair is a better choice

89. Palliative Procedures
Taussig-Bing type –
Balloon /blade atrial septostomy,
- for better mixing
- for decompression the LA, which causes PV congestion.

90. SURGICAL TREATMENT
Analysis of the results of surgery requires consideration of
1. Morphologic subset of this defect
2. Associated congenital heart defect
In this analysis the commitment of the VSD to great arteries appears to determine the outcome of repair.

91. SURGERY GOALS OF REPAIR Establish connection from LV to aorta
To connect RV to PA
To close the VSD

92. Repair of double outlet right ventricle with subaortic ventriculoseptal defect
Repair of double outlet right ventricle with a subaortic VSD - intraventricular tunnel.
If the VSD is suspected to
be smaller than the aorta
before or during surgery,
the VSD is enlarged.
Mortality rate is <5% (subaortic VSD)

93. Similar to those with TOF
Repair of double outlet right ventricle with subaortic ventriculoseptal defect and pulmonary stenosis
Similar to those with TOF
Intraventricular tunnel repair of the VSD - similar to that used for patients who have double outlet right ventricle and subaortic VSD without pulmonary stenosis.

94. During intracardiac repair PS may require: - Pulmonary valvotomy
- Infundibular resection
- Patch enlargement of RVOT
If, coronary artery crosses the right ventricular outflow tract - conduit may be added as an additional outflow path from the right ventricle.
Carried out at 3-4 years of age
Carried out between 6 months and 2 yrs of age.

95. Taussig-Bing Anomaly: - 3 possible approaches.
Anatomic repair of double outlet right ventricle with subpulmonary ventriculoseptal defect
Taussig-Bing Anomaly: - 3 possible approaches.
Early surgery is recommended because of rapid development of Pul vascular obstructive disease.
1. An Intraventricular tunnel between the VSD and PA with the arterial switch operation. Preferred surgical method
Mortality 10-15%.

96. Anatomic repair of double outlet right ventricle with subpulmonary ventriculoseptal defect

97. The second method –
Consists of construction of a long intraventricular tunnel to establish continuity between the left ventricle and the aorta and between the right ventricle and pulmonary artery.

99. IMPORTANCE OF TRICUSPID TO PULMONARY VALVE DISTANCE
Dist > diameter of AV – tunnel can be created post to PV
Dist is extremely short – tunnel created to keep PV on LV side
Dist < diameter of AV – if tunnel is created post to PV, subaortic stenosis will develop. Tunnel created ant to PV

100. The third method - involves closure of the VSD with baffling of the left ventricular outflow to the pulmonary artery with a subsequent atrial baffle (eg, Senning procedure, Mustard procedure).
This method is associated with high operative and late mortality rates.

101. Because coarctation of the aorta is commonly observed in this situation, patients may have undergone coarctation repair with a pulmonary artery band
The subsequent procedure is a single stage complete repair with VSD enlargement if restrictive, repair of the VSD to direct the left ventricular blood to the pulmonary artery, followed by an arterial switch procedure.
Aortic arch obstruction - repaired at the same time under hypothermic circulatory arrest

102. Repair of double outlet right ventricle with doubly committed ventriculoseptal defect
Surgical correction - similar to that described for double outlet right ventricle with subaortic VSD.
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.

103. .

104. Repair of double outlet right ventricle with noncommitted ventriculoseptal defect
Most difficult to correct.
High-risk procedure that often involves univentricular repair.
However, biventricular repair of double outlet right ventricle with noncommitted VSD has been described and done
The major feature of this anomaly is a persistent subaortic conus and a double infundibulum.

105. Repair of double outlet right ventricle with noncommitted ventriculoseptal defect
The subaortic conus is in excess to essentially normal right ventricular structures.
Represents malposition of the aorta, with a normally positioned pulmonary artery and with the great vessels usually side by side.
The VSD, usually perimembranous, often has inlet and/or trabecular extension and can be restrictive.

106. Copyright ©1999 The American Association for Thoracic Surgery

108. Contraindications to performing a biventricular repair
Significant left ventricular hypoplasia
Straddling of the atrioventricular valve

109. Timing for Univentricular repair
If the child is stable, mildly cyanosed-
Direct Fontan operation may be done at 3-4 years of age (Class I)
Stable, mildly cyanosed: Glenn at 1 year. Fontan at 3-4 year (Class IIa)

110. Timing for Univentricular repair
Significant cyanosis (SpO2< 70%) < 6 month-
Systemic to pulmonary artery shunt followed by Glenn at 9 months- 1 year, and Fontan at 3-4 years (ClassI).
Significant cyanosis (SpO2 <70%) > 6 months-
BD Glenn followed by Fontan at 3-4 years (ClassI).

111. POTENTIAL DANGER AREAS DURING SURGICAL CORRECTION

112. STATUS AFTER REPAIR Most patient surviving repair are NYHA class I
Early primary repair improve outcome

113. MAJOR CONCERNS AFTER REPAIR
PROGRESSIVE VENTRICULAR DYSFUNCTION.
Associated factors like Residual VSD
AV valve insufficiency
Prolonged circulatory arrest at intracardiac repair.

114. MAJOR CONCERNS AFTER REPAIR
LV Outflow Obstruction
due to proliferation of fibrous tissue at VSD,
distortion of intraventricular baffle, or
hypoplasia of the aortic annulus.
Late post op Arrhythmia and Sudden Cardiac Death
- Associated factors like repair at older age
-Ventricular Dysfunction
- Increased PA pressure.

115. Records were reviewed of all children with DORV
ANATOMIC FEATURES AND SURGICAL STRATEGIES IN DOUBLE-OUTLET RIGHT VENTRICLE
Records were reviewed of all children with DORV
undergoing surgery between 1978 and 1993
Biventricular repair can be achieved in most patients with DORV with low risk
In complex DORV, a Fontan procedure is associated with a lower surgical mortality
Circulation. 1997; 96:   doi: /​01.CIR

116. FOLLOW UP Long term regular follow up 6-12 month interval
Subaortic VSD without PS – excellent long term outcome

117. FOLLOW UP
-Ventricular arrythmia should be treated - may cause sudden death -20% require reoperation of intraventricular tunnel -Continue SBE prophylaxis

118. Consultations
Geneticist to discuss the possibility of subsequent children having this or other forms of CHD.
When CHD is detected, a detailed investigation for extracardiac malformation should be performed and vice versa.
Also, issues such as preterm birth and stillbirth should be taken into account in risk assessment and risk stratification in patients born with CHD.
Most prevalent extracardiac anomalies associated with conotruncal heart defects are anomalies of the GI and genitourinary systems.

119. Associations with DORV –
Omphalocele , gastroschisis, facial clefting.
Preterm infants have been shown to have more than twice as many cardiovascular malformations as do term infants
16% of all infants with cardiovascular malformations are preterm.
Prevalence of CHD is high among late stillbirths. In particular, a greater incidence of coarctation of the AO , double-inlet left ventricle, hypoplastic left heart, truncus arteriosus, DORV, and AV septal defect is noted among stillbirths.