Double outlet right ventricle

Double Outlet Right Ventricle
Embryology, Anatomy,
Hemodynamics, Clinical presentation,
and Management
By: Himanshu Rana

DORV
‘the great Chameleon’

Introduction
 Newfield et al –
 Both great arteries arise exclusively from RV,
 neither semilunar valve in fibrous continuity with either AV
valve &
 VSD is usually present as the only outlet from LV
 Pulmonary valve or sub-pulmonary stenosis may be present
or absent.
 Lev et al. One complete 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
Definitions:

Introduction
 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) abnormal
ventriculoarterial connection with more than half of each great
artery originating above the morphologic RV.

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 DORV as a diagnostic term for a
partial transposition complex

HISTORY Cond…
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 and also provided the concept of Macgoon index.

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

Goor & Edwards: -
“DORV appears to represent a primitive
embryologic condition because of failure to
achieve cono-truncal rotation and left shift of the
conus.”
EMBRYOLOGY (Old theory…)

• CONUS: A circular tube of muscle, upon which semilunar valve sits up.
• One conus is absorbed normally.
• The remaining conus grows & pushes the great vessel superiorly and
anteriorly & is always connected to the RV.
• In DORV, absorption of both coni fails & both great vessels are connected
to RV.
• TGA – aortic conus persists
• DOLV – both coni absorbed
• TOF – Pulmonic conus persists but there is anterior deviation of the conal
septum. Van Praagh called it as Monology of Stenson.
EMBRYOLOGY Contd….

Van praagh theory of conal underdevelopment
• “ the distal or semilunar part of infundibulum or conus
arteriosus performs an arterial switch during cardiogenesis”
EMBRYOLOGY Cont….

Van Praagh classification of DORV
• Type I DORV:
– Isolated conotruncal anomaly
• Type II DORV:
– Conotruncal anomaly with malformations of AV valve
& ventricles
• Type III DORV:
– Heterotaxy syndromes (Polysplenia, asplenia)

DORV represent a part of the whole spectrum
of cono-truncal anomaly
Spectrum of cono-truncal anomalies
A
P
VSD TOF DORV TGA
(double coni)
A
P P
P P
A
A A
RL

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

Ventricular septal defect
• Often the only outflow tract of the left ventricle
• Mostly cono-ventricular VSD in peri-membranous
septum.
• Due to persistence of the bulbo-ventricular canal.
• Accordingly, DORV is classified w.r.t. VSD location as:
– Sub-aortic,
– Sub-pulmonary,
– Doubly committed &
– Non committed.

• Sub-aortic type(40%)
• Sub-pulmonary type
(Taussig Bing complex)(20%)
• Doubly committed(10%)
• Remote type(<10%)
POSITION OF VSD

GREAT ARTERY RELATIONSHIPS AT THE
LEVEL OF SEMILUNAR VALVES
 Right posterior aorta
Aortic valve & trunk originate from RV post.
& to the rt. of pulmonary valve.
 Right lateral aorta (side-by-side
relationship)
Aorta is to the rt. of PA., & the semilunar
valves lie approximately in the same
transverse & coronal plane. The classically
described great artery relationship in DORV.

• Right anterior aorta
(D-malposition) Aorta is to
the rt. & ant. to PA or may
be directly ant.
• Left anterior aorta
(L-malposition) Aorta is to
the lt. & ant. to PA. Least
common.

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

RELATIONSHIP OF GREAT ARTERIES AND VSD
Reationship
Of great
artereis
Location of VSD Total
Sub
aortic
Sub
pulmonary
Doubly
commited
remote
Normal 3% 0 0 0 3%
Side by
side
46% 8% 3% 7% 64%
Right
anterior
aorta
16% 10% 0 0 26%
Left
anterior
aorta
3% 4% 0 0 7%
TOTAL 68% 22% 3% 7% 100%

ASSOCIATED ANOMALIES
• Pulmonary Stenosis
- most common – 40-70% (50%),
- frequently co-exists with sub-aortic VSD (80%);
- bicuspid pulmonary valve;
- rarely seen in sub-pulmonary VSD type.

ASSOCIATED ANOMALIES
• Subaortic stenosis– 3%; common in sub-pulmonic VSD(50%)
• Coarctation – 12%; common in sub-pulmonic VSD (50%).
• Mitral valve anomalies 10%; Common in remote VSD
• ASD – os type 25% of all DORV,
• TAPVC – 2%
• AV canal – 5%, Common in remote VSD.

CORONARY ANOMALIES (10%)
• Similar to TOF – LAD from RCA (mc)
• Similar to TGA – RCA from the right posterior
aortic cusp & LCA from the left posterior cusp
• Anomalous origin of the LCx from RCA,
• Single coronary ostium, origin of RCA from LCA.

DORV – MAJOR CLINICAL PATERNS
 Group 1 – Sub-aortic VSD with PS (resembles TOF) (MC-40%)
 Group 2 – Sub-pulmonary VSD, with or without PS (resembles
TGA). 20%; AKA T-B anomaly
 Group 3 – Sub-aortic VSD, no PS, (resembles VSD) (< 5%)
 Group 4 – Sub-aortic VSD with PVOD (Resembles
Eisenmenger–complex)

SUB AORTIC VSD WITH PS
RV aorta
LV aorta
Aortic saturation decreased
PBF decreased
Resemble TOF
50 % of sub-aortic VSD type have PS which is usually progressive
HEMODYNAMICS

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.
DORV with Sub pulmonic VSD AKA Taussig Bing anomaly &
resembles TGA
HEMODYNAMICS contd…

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

SUBAORTIC VSD – INCREASE PVR
-Increased blood to aorta
-Decreased flow to pulmonary trunk
-Aortic saturation falls
Resembles Eisenmengers syndrome

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

DEFECT AND PULMONARY STENOSIS..
• The precordium show evidence of a right ventricular impulse at
the left sternal border,
• prominent systolic thrill upper left sternal border due to obligatory
flow across the bulbo-ventricular foramen VSD
• 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

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.
.

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.

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.

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.

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

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 sub-pulmonary
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 first-degree AV conduction
delay .

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 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
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.
TOF

CHEST X RAY
In patients with PS
• Features may resemble TOF
• Mild degree of cardiomegaly
• Pulmonary vascularity is diminished (oligemia)
• MPA segment is absent, resulting in concave upper left border of
heart

DORV subaortic VSD pulmonary atresia

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

When PVR is high -Prominent MPA peripheral pruning

ECHO
4 OBSERVATIONS REQUIRED FOR DIAGNOSIS
• Origin of one great vessel and overlie of at least 50% 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
LA
LV
RV
Ao

VSD
AO.
LA
CONUS
RV
LV
DORV with Sub-aortic VSD

DORV with Sub-aortic VSD
VSD AO.
LA

DORV with doubly committed VSD

Echo Evaluation of DORV:
Role of Echocardiography in Planning Sx 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: 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 op.
- Additional muscular VSD which may require closure
at the time of sx
- 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.

Tear drop sign
SUB-AORTIC VSD WITH PS

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

• Inadequate pulmonary blood flow –
– Maintain ductal patency.
– Prostaglandin E1 (i.e. alprostadil) until repair.
• Congestive heart failure
– Diuresis,
– Inotropic support
– Digoxin
MEDICAL MANAGEMENT

SURGICAL MANAGEMENT
When DORV repair is planned – review :
 PS or PAH
 Single or Two ventricle repair
 Relationship of great vessels
 Location of the VSD and its size
 Associated lesions

Surgical Care - Palliative
• As 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
• Palliation
– Pulmonary artery banding or
– systemic-to-pulmonary shunt (Modified B-T shunt)
– Balloon/blade atrial septostomy
SURGICAL MANAGEMENT contd…

1. With increased PBF and CHF–
– may first require pulmonary artery banding.
NOT RECOMMENDED for infants with
‘Sub-Aortic VSD /Doubly Committed VSD’
“Primary repair is a better choice”
Palliative Procedures

2. With pulmonary stenosis – systemic-to-pulmonary
shunt, (modified Blalock-Taussig shunt), if SpO2< 70%

Taussig-Bing type –
– Balloon/blade atrial septostomy –
• for better mixing
• for LA decompression, to relieve PV congestion.
Palliative Procedures

• Left ventricle to aorta baffle closure (intra-cardiac
rerouting)
– Simple VSD closure (subaortic VSD type)
– Patrick-McGoohan
– Kawashima (for original Taussig–Bing anomaly)
• Intraventricular repair with RVOT reconstruction
– Rastelli
– REV repair (with Lecompte manuever)
Definitive Procedures

• Switch operation
– Arterial switch operation (Jatene)
– Atrial switch operation (Senning, Mustard)
• Switch with RVOT reconstruction
– Switch + Rastelli (or REV)
– Aortic translocation (Nikaidoh, double root, truncal switch)
• Single-ventricle approach
– Bidirectional Glenn shunt
– Fontan operation
• Biventricular repair with DKS
– Yasui procedure

Essential Anatomic Determinants of
Method of Surgical Repair
• Position, size &no. of VSD(s) & its relation to conduction system
• Distance b/w tricuspid & pulmonary valve
• Straddling of AV valve
• Relationship of great arteries
• Presence of PS/AS
• Presence of RVOTO/LVOTO & weather fixed or dynamic
• Coronary artery anatomy

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

GOALS OF REPAIR
• Establish connection from LV to aorta
• To connect RV to PA
• To close the VSD
• To correct the associated anomaly

Principles of surgery
• Biventricular repair is performed if feasible
• Adequate LVOT must be created, even if it
compromises RVOT
• RVOT can be reconstructed by infundibulectomy,
valvotomy, gusset, or conduit

Contraindications to biventricular repair
• Significant left ventricular hypoplasia
• Straddling of the atrioventricular valve

Ten Commandments
• Age > 4 yrs
• Sinus rhythm
• Normal systemic venous return
• Normal Right atrial volume
• Mean pulmonary artery pressure < 15 mm Hg
• Pulmonary arteriolar resistance < 4 wood units/m2
• Pulmonary artery-aorta ratio >0.75
• Left Ventricular ejection fraction >0.60
• Competent mitral valve
• Absence of pulmonary artery distortion

Timing of Surgery
• VSD type
– One-stage repair as neonate or young infant because of pulmonary
over circulation
• Transposition type
– One-stage repair as a neonate before LV atrophies
• Tetralogy type
– One-stage repair within few months of birth, or
– Two stages with initial palliative shunt f/b complete ≥6 months
• Non-committed type
– Complete biventricular repair usually deferred beyond 6 months
because of complexity of the baffle.
– Initial palliation with either an aorto-pulmonary shunt or PA band may
be needed

Timing for Univentricular repair
• Stable, mildly cyanosed:
– Direct Fontan operation at 3-4 years of age (Class I)
– Glenn at 1 yr f/b Fontan at 3-4 year (Class IIa)
• Significant cyanosis (SpO2< 70%) Age < 6 month-
– Systemic to pulmonary artery shunt f/b
– Glenn at 9 months- 1 year, and
– Fontan at 3-4 years (Class I).
• Significant cyanosis (SpO2 <70%) Age > 6 months-
– BD Glenn followed by
– Fontan at 3-4 years (Class I).

Pulmonary artery assessment
• In patients with PS, degree of PA hypoplasia to be
assessed;
– McGoon ratio:- ratio of sum of immediate prebranching
RPA & LPA diameter to descending Ao diameter just above
the diaphragm.
• Normal = 2 – 2.5
• Good Fontan candidate >1.8
– Nakata Index: ratio of cross-sectional area of RPA & LPA to
BSA.
• Normal= 330±30 mm2/BSA
• Good Fontan candidate >250
• Good Rastelli candidate>200
• Patients < 200 – shunt surgery is better

Size of ventricles and AV valves are adequate
Yes No
VSD to aortic baffle can be created
without LVOT obstruction SV or 1½ Repair
Glenn/Fontan
Yes
No
Any obstruction to PV or RVOT
VSD to PV baffle can be
created without obstruction
Yes
No
DKS with RV-PA
Yasul proceedure
SV Repair
Glenn/FontanYesNo
Simple IVR IVR with RVOT
reconstructionSimple VSD closure
Patrick-MacGoohan
Kawashima
Rastelli
REV (Lecompte)
Any obstruction in
Neo PV or RVOT
Switch
Arterial switch
Atrial switch
(Senning/Mustard)
Yes
No
Switch with RVOT
reconstruction
Switch + Rastelli
Switch + REV
Nikaidoh (aortic translocation)
Double root translocation
Truncal switch (Yamagishi)
DORV
Surgical approach
Repair of DORV with subaortic VSD
• Intraventricular tunnel.
• If the VSD is smaller than the
aorta, VSD is enlarged.
• Mortality rate is <5%
(subaortic VSD)
REV Proceedure
Modified REV procedure: Pulmonary root translocation technique.
• A, Pulmonary root harvesting. The ascending aorta transected above the
sinus–tubular conjunction.
• B, Lecompte maneuver is done. Restoration of RV–PA continuity. A single-
valved bovine jugular vein patch to repair RVOT, enlarge the stenotic main
pulmonary artery, and restore the competence of the neo-pulmonary valve.
Repair of DORV with subaortic VSD and PS
• Similar to DORV and subaortic VSD without PS.
• During repair PS may require:
- Pulmonary valvotomy
- Infundibular resection
- Patch enlargement of RVOT
Yasui Proceedure
Single ventricle repairMustard/Senning procedure
Nikaidoh procedure

Taussig-Bing Anomaly: - 3 possible approaches.
Early surgery is recommended because of rapid development
of PVOD.
1. An Intraventricular tunnel between the VSD and PA with the
arterial switch operation. Preferred surgical method
Mortality 10-15%.
Anatomic repair of DORV with subpulmonary VSD

Anatomic repair of DORV with subpulmonary VSD

The second method –
• Consists of construction of a long intraventricular
tunnel to establish continuity between the LV and
the aorta and between the RV and PA.

The third method
• Closure of the VSD with baffling of the LVOT 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 to
undergo 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 DORV with doubly committed VSD
 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 DORV with noncommitted VSD
• 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 DORV with noncommitted VSD
 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.

POTENTIAL DANGER AREAS DURING SURGICAL
CORRECTION

MAJOR CONCERNS EARLY AFTER REPAIR
PROGRESSIVE VENTRICULAR DYSFUNCTION
• Residual VSD
• AV valve insufficiency
• Prolonged circulatory arrest at intracardiac repair.

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

Complications Associated with DORV
in Adults following repair
• LVOT/RVOT obstruction
• Pulmonary regurgitation/Aortic valve regurgitation (following arterial
switch)
• Failure of the RV-to-PA conduit (stenosis/regurgitation)
• Coronary artery stenosis (following coronary a. transfer)
• RV failure
• Arrhythmia (atrial or ventricular) and sudden death
• Endocarditis
• Thromboembolic event

FOLLOW UP
• Long term regular follow up
• 6-12 month interval
• Subaortic VSD without PS – excellent long term outcome
• Ventricular arrythmia should be treated, may cause SCD
• 20% require reoperation of intraventricular tunnel
• Continue SBE prophylaxis

Endocarditis Prophylaxis
• Routine endocarditis prophylaxis is not required
following surgery, except:
– prosthetic cardiac valve or device,
– previous endocarditis,
– for the first 6 months after intervention, and
– Residual defect(s) at/adjacent to the site of a
prosthetic patch or device

Double outlet right ventricle

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