Transcript Double Outlet Right Ventricle

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
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:
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:
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
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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
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5.
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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
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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
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6 months of life
same as TOF
2. DORV with subpulmonary VSD
. Without PS
. With PS
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:
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
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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