Transcript DORV DR KSHITIJ

Dr. Kshitij Mavade
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.
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

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
HISTORY
1703 • The earliest report in French
• John Abernathy, an assistant surgeon at St Bartholomew’s
1793 Hospital in London, described “partial transposition”
• Karl von Vierordt called double outlet right ventricle partial
transposition to signify that the aorta was transposed but
1898 the pulmonary trunk was normally aligned
• Witham introduced double outlet right ventricle as a
diagnostic term for a partial transposition complex
1957

HISTORY
1957 - Kirklin, first repair of DORV with subaortic VSD, at the mayo
clinic

1961- 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
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

EMBRYOLOGY

Goor & Edwards: - DORV appears to
represent a primitive embryologic condition
because of failure to achieve conotruncal
rotation and left shift of the conus.
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.

Van praagh theory of conal
underdevelopment

“ the distal or semilunar part of infundibulum or
conus arteriosus performs an arterial switch during
cardiogenesis”
DORV represent a part of the whole
spectrum of conotruncal anomaly
Spectrum of conotruncal anomalies
P
P
A
VSD
A
TOF
A
A
P
P
DORV
TGA
(double coni)
PATHOLOGIC CLASSIFICATION
Based on
•Location of VSD to great arteries
•Great artery relationship
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.
RELATIONSHIP OF GREAT ARTERIES AT
SEMILUNAR VALVE LEVEL

Right posterior aorta
Right lateral aorta
(side by side relationship)


Right anterior aorta

Left anterior aorta.
POSITION OF VSD

Sub-aortic type
Sub-pulmonary type
(Taussig Bing complex)


Doubly committed

Remote type
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

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.
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
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
RELATIONSHIP OF GREAT ARTERIES AND VSD
Reationship
Location of VSD
Of great
Sub
Sub
Non –
remote
artereis
aortic pulmonary commited
Total
Normal
3%
Side by
side
Right
anterior
aorta
0
0
0
3%
46% 8%
3%
7%
64%
16% 10%
0
0
26%
Left
anterior
aorta
3%
0
0
7%
TOTAL
68% 22%
3%
7%
4%
DORV with
Subaortic VSD
Aorta
VSD
DORV with
Subpulmonic VSD
Aorta
PA
VSD
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.
ASSOCIATED ANOMALIES

Pulmonary Stenosis
- most common – 40-70%,
- frequently co-exists with subaortic VSD
(80%);
- bicuspid pulmonary valve;
- rarely seen in subpulmonary VSD type.
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.

CORONARY ANOMALIESNormal
 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.

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
SUB AORTIC VSD WITH PS
50 % of DORV with subaortic VSD have PS
RV
LV
aorta
aorta
Aortic saturation decreased
PBF decreased
Resemble TOF
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
Aortic saturation decreases.
Aorta
SUBAORTIC VSD WITH DECREASE
PVR WITH NO PS
- Resembles VSD
- PBF increased
-Aortic saturation is normal.
SUBAORTIC VSD – INCREASE PVR
-Increased blood to aorta
-Decreased flow to pulmonary trunk
-Aortic saturation falls
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.
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.
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
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.
.
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.
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.
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.
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.
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.
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.
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 firstdegree AV conduction delay .
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.
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.
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.
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

DORV
subaortic
VSD
pulmonar
y atresia

In cases of subaortic VSD without PS  Generalized cardiomegaly
 Prominent main pulmonary artery
segment
 Increased pulmonary vascularity

DORV with
subaortic
VSD

Taussig Bing
anomaly

When PVR is high  Prominent MPA
peripheral pruning
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.
DORV: Aortic-mitral Discontinuity
RV
Ao
LV
LA
Aortic - mitral
discontinuity in
DORV is best shown
in the parasternal
long-axial view.
DORV with subaortic VSD
DORV with sub pulmonic VSD
DORV with doubly committed VSD
DORV with
remote VSD
DORV with remote VSD
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
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
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
Repair of DORV
with remote VSD
requires creation of
a complex
intraventricular
baffle using multiple
patches (the
Barbero-Marciel
technique)
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
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)
CARDIAC CATHETERISATION
OBJECTIVES: to evaluate
• Routability of VSD to aorta
•
Branch PA anatomy

Pulmonary vascular resistance

Coronary artery and aortic arch anatomy.
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)
Natural history
3 -Taussig Bing –severe PVOD develop
early
as in TGA
4 -Associated anomalies –COA
- LV hypoplasia
- poor prognosis
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

MEDICAL MANAGEMENT

Inadequate pulmonary blood flow –
maintain ductal patency.

Prostaglandin E1(ie, alprostadil) is the
standard of care until repair can take place.
MEDICAL MANAGEMENT
 Clinical
picture of congestive heart failure
–
diuresis,
 inotropic support


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




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

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
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-topulmonary shunt, such as a modified BlalockTaussig shunt, may be required if SpO2< 70%
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
Palliative Procedures
Taussig-Bing type –
Balloon /blade atrial septostomy,
- for better mixing
- for decompression the LA, which
causes PV congestion.
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.
SURGERY
GOALS OF REPAIR

Establish connection from LV to aorta

To connect RV to PA

To close the VSD
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)
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.
 During
intracardiac repair PS may require:
- Pulmonary valvotomy
- Infundibular resection
- Patch enlargement of RVOT
Carried out between 6 months and 2 yrs of age.
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
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%.
Anatomic repair of double outlet right ventricle with
subpulmonary ventriculoseptal defect
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.

IMPORTANCE OF TRICUSPID TO
PULMONARY VALVE DISTANCE
99
A.
B.
C.
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
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.

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

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.
.
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.
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.
Copyright ©1999 The American Association for Thoracic Surgery

Contraindications to performing a
biventricular repair

Significant left ventricular hypoplasia

Straddling of the atrioventricular valve
Timing for Univentricular repair

•
If the child is stable, mildly cyanosedDirect 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)
Timing for Univentricular repair
•
•
Significant cyanosis (SpO2< 70%) < 6 monthSystemic to pulmonary artery shunt followed by
Glenn at 9 months- 1 year, and Fontan at 3-4
years (ClassI).
Significant cyanosis (SpO2 <70%) > 6 monthsBD Glenn followed by Fontan at 3-4 years
(ClassI).
POTENTIAL DANGER AREAS DURING
SURGICAL CORRECTION
STATUS AFTER REPAIR

Most patient surviving repair are NYHA class
I

Early primary repair improve outcome
MAJOR CONCERNS AFTER REPAIR
PROGRESSIVE VENTRICULAR
DYSFUNCTION.
 Associated factors like Residual VSD

AV valve insufficiency

Prolonged circulatory arrest at intracardiac
repair.
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.
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: 1233-1239 doi: 10.1161/​01.CIR.96.4.1233
FOLLOW UP

Long term regular follow up

6-12 month interval

Subaortic VSD without PS – excellent
long term outcome
FOLLOW UP
-Ventricular arrythmia should be treated
- may cause sudden death
-20% require reoperation of
intraventricular tunnel
-Continue SBE prophylaxis
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.

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.