Double Outlet Right Ventricle

Seoul National University Hospital

Department of Thoracic & Cardiovascular Surgery

Double Outlet Right Ventricle

1. Definition
A congenital cardiac anomaly in which both great arteries
rise wholly or in large part from the right ventricle.
It is then, a type of ventriculoarterial connection.

2. History
Taussig-Bing heart
Braun
Mayo group
Lev

:
:
:
:

Described in 1949
Case of DORV+PS in 1952
1st repair in 1957
Clarified Taussig-Bing in 1972

Double Outlet Right Ventricle

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 overcirculation, pulmonary
hypertension, and congestive heart failure.
Pulmonary stenosis results in right-to-left
shunting and cyanosis.

Hemodynamics of DORV

Double Outlet Right Ventricle

Pathophysiology

DORV Angiography

Morphology of DORV
1. VSD; usually large
10 % small
rarely none
.
.
.
.

Subaortic
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.
5.
6.
7.

Pulmonary stenosis
Conduction system
Coronary arteries
Associated anomalies
PS, CoA, PDA, SubAS,
A-V canal, multiple VSD
in 30 %

Locations of VSD in DORV

a;
b;
c;
d;

Subaortic
Subpulmonic
Doubly committed
Noncommitted

DORV with
Subaortic VSD
Aorta
VSD

DORV with
Subpulmonic VSD

Aorta
PA
VSD

Taussig-Bing Heart

Aorta

PA

Double Outlet Right Ventricle

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

Clinical Features & Diagnosis

1. Incidence ; less than 1% of CHD
2. Pathophysiology
. Variable according to streaming of blood flow, PS, PVD
. Always some arterial desaturation
1) Streaming of blood flow
(relationship of semilunar valve to the VSD)
2) Pulmonary vascular disease
(more rapid onsets without PS)
3) Pulmonary stenosis
(severe cyanosis in important PS )

3. Examination
. No definite clinical signs with or without PS
. EKG, chest radiography : not diagnostic

4. Echocardiography
5. Cardiac catheterization & cineangiography

Double Outlet Right Ventricle

Natural History
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.

Operative Techniques
1. Intraventricular tunnel repair
.
.
.
.
.

Simple DORV
Subaortic VSD & PS
Doubly committed
Noncommitted
Taussig-Bing heart

2. Switch repair ( arterial, atrial )

. Taussig-Bing heart

3. Rastelli or Lecompte repair

. With intraventricular tunnel repair

4. Nikaidoh aortic translocation

5. Total cavopulmonary connection
. Noncommitted VSD with PS

6. Palliative operations

Surgical Strategy of DORV

Intraventricular Tunnel

Relationship between VSD , PV & Aorta

REV (Lecompte) Operation

Intraventricular Tunnel Repair

Taussig-Bing Heart

Rastelli Operation
Taussig-Bing Heart

Taussig-Bing Heart with COA

Combined aortic arch repair and arterial switch

without coronary reimplantation

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

Operative Indications
1. Simple DORV with subaortic VSD
. Without PS
. With PS

:
:

6 months of life
same as TOF

2. DORV with subpulmonary VSD

. Without PS
. With PS

:
:

1st month of life

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

Double Outlet Left Ventricle

Seoul National University Hospital
Department of Thoracic & Cardiovascular Surgery

Double Outlet Left Ventricle

Definition
A cardiac anomaly in which both great arteries arise
from the left ventricle.
DOLV may occur with atrioventricular concordant or
discordant connection.

History
Marechal : 1st description in 1819
Sakakibara : 1st intraventricular repair in 1967
Paul
: Unique case of DOLV+ IVS in 1970

Morphology of Double Outlet LV

VSD
Usually, large & most commonly subaortic
Some juxtaaortic, subpulmonic, juxtaarterial (double outlet both
ventricle) or some overriding

Conal pattern
Most often absent subaortic conus & subpulmonic conus is
displaced to LV
Rarely bilateral absent conus, Very rarely only subaortic conus

Pulmonary stenosis

Present most, either valvular or subvalvular

Right ventricle & TV ; tendency & somewhat hypoplastic
Left ventricle : normal
Conduction system: normal

Double Outlet Left Ventricle

Models of 4 basic hearts as they occur in double outlet LV

Double Outlet Left Ventricle

Clinical Features & Diagnosis

Pathophysiology
1. LV is a common mixing chamber, receiving
systemic & pulmonary venous blood
2. Clinical presentation is by severe cyanosis from
frequent occurrence of pulmonary stenosis
3. Tendency to develop cyanosis is more severe in
AV discordant connection

Diagnostic examination
1. Physical finding, chest X-ray, EKG; not diagnostic
2. Echocardiography
3. Cardiac catheterization & cineangiography

Double Outlet Left Ventricle

Cineangiograms of normally positioned heart and AV

concordant connection, DOLV, subaortic VSD and PS

Natural History
Incidence
Very rare

Natural history with DOLV without PS appears

to be similar to that of the patients with isolated large

VSD and progressive narrowing of VSD & closure has
not documented
Natural history with DOLV with PS appears to
be similar to that of patients with TOF and degree of
hypoxia and clinical course are related to severity of PS

Techniques of Operation
Identification of morphology
Repair of DOLV and atrioventricular
concordant connection
1. With pulmonary stenosis
2. Without pulmonary stenosis

DOLV with atrioventricular concordant

connection and important hypoplasia of
RV and TV

Repair of DOLV + VSD + PS

Operative Indications for DOLV

Diagnosis is an indication for operation
In the absence of pulmonary stenosis, corrective
operation should be performed in the first 6 months
of life, or pulmonary artery banding and
subsequent delayed repair at age 1-2 years or beyond 2
years, if morphology is more challenging
In the presence of pulmonary stenosis, this approach
are similar to those for Tetralogy of Fallot
When there is right ventricular or tricuspid valve
hypoplasia, Fontan operation should be considered