Download Corrected Transposition of the Great Arteries

Corrected Transposition of
Great Arteries
Seoul National University Hospital
Department of Thoracic & Cardiovascular Surgery
Congenitally Corrected Transposition
of Great Arteries
Introduction
1. Definition
A cardiac anomaly with ventriculo-arterial discordant connection
(transposition of great arteries) & atrio-ventricular discordant
connection (right atrium connecting to left ventricle & left atrium
to the right ventricle).
The circulatory pathways are therefore in series.
2. History
Rokitansky
Schiebler
Anderson, Lillehei
Ilbawi et al
: 1st description in 1875
: Clinical syndrome in 1961
: 1st repair in 1957
: Double switch operation in 1990
Congenitally Corrected TGA
Pathophysiology
• Combined AV & ventriculo-arterial discordance
resulting in corrected transposition of systemic
and pulmonary circulations.
• There is high incidence of associated intracardiac
anomalies including VSD, pulmonary outflow
tract obstruction, tricuspid insufficiency, and AV
conduction anomalies.
Ventriculo-arterial Discordance
Morphologic characteristics
• Diagrammatic models of four basic hearts
Congenitally Corrected TGA
 Surgical morphology
1. Ventricle (conus, loop, position)
Dextrocardia
2. Pulmonary outflow tract;
Transverse plane & wedged
3.
4.
5.
6.
7.
8.
Atrial, ventricular septal position
Tricuspid, mitral, aortic valves
Ventricular septal defect
Atrioventricular node & bundle of His
Coronary arterial patterns
Associated anomalies
no coexistent cardiac anomalies in 1 ~ 2 %
Congenitally Corrected TGA
Associated cardiac anomalies
• Ventricular septal defect : 86%
• Pulmonary stenosis
: 64%
• Tricuspid regurgitation : 28%
• AV block
: 12%
Congenitally Corrected TGA
Surgical morphology
Congenitally Corrected TGA
Surgical morphology
Congenitally Corrected TGA
Surgical pathology
Morphologic LV
Congenitally Corrected TGA
Surgical pathology
Morphologic RV
Congenitally Corrected TGA
Surgical pathology
Morphologic RV
VSD
Morphologic LV
Congenitally Corrected TGA
Clinical features & diagnosis
1. Pathophysiology
* Determined by VSD & pulmonary stenosis ; usually mild symptom,
not severe pulmonary stenosis in infancy
* Most often, presentation is in childhood or in second decade ;
growth failure, exercise intolerance, cyanosis
* Left sided tricuspid valve incompetence seems to worsen with time
* Bradycardia, WPW syndrome
2. Physical findings
Not diagnostic
3. Additional investigations
1) Chest radiography : ascending aorta along left upper cardiac silhouette
2) EKG
3) Echocardiography
4) Cardiac catheterization & cineangiography
Congenitally Corrected TGA
Natural history
1. Incidence
0.5% - 1.4% of CHD, slightly male predominating
2. Heart block
1) Complete heart block
5 - 10% at birth, 10 - 15% in adolescence, 30% in adult
2) 1st or 2nd degree A-V block ; 40 - 50% at birth
3) 40% retain normal PR interval & QRS through their lives
3. Ventricular function
Not truly normal, but sufficiently good in most & tendency
to deteriorate after 2nd decade of life
4. Effect of coexisting cardiac anomalies
VSD, PS, left-sided A-V valve incompetence
Congenitally Corrected TGA
Operative Indications of cc-TGA
Conventional Repair
The presence of corrected TGA is not an
indication for a reparative operation.
1. Ventricular septal defect
* same as normal heart
2. VSD & important PS
* same as TOF
3. Left-sided tricuspid incompetence
* same as mitral incompetence
4. Complete heart block
Congenitally Corrected TGA
Operative techniques
1. Repair of ventricular septal defect
2. Repair of coexisting VSD & PS
* Extracardiac conduit
* Without extracardiac conduit
* One & a half ventricle repair
3. Correction of incompetent tricuspid valve
* Repair (annuloplasty)
* Replacement
4. Fontan-type repair
Straddling, A-V canal , & hypoplastic ventricle
5. Anatomic correction (double switch operation)
Congenitally Corrected TGA
Morphologic characteristics
Congenitally Corrected TGA
Surgical view
• Rt. sided AV valve through Rt. atriotomy
Congenitally Corrected TGA
Surgical view
• Rt. sided AV valve & ASD through Rt. atriotomy
Congenitally Corrected TGA
Surgical view
• VSD through Rt. sided atrioventricular valve
Congenitally Corrected TGA
Repair of VSD
Congenitally Corrected TGA
Apico-pulmonary artery conduit
Congenitally Corrected TGA
Repair of VSD + PS
Standard repair of situs solitus congenitally corrected TGA,
VSD, and PS
Congenitally Corrected TGA
One & a half ventricle repair
cc-TGA, VSD, PS
VSD closure, pulmonary valvotomy, and inparallel BCPC
Congenitally Corrected TGA
Double switch operation
• Bidirectional superior cavopulmonary anastomosis and
hemi-Mustard modification for double switch procedure
Congenitally Corrected TGA
Double switch operation
• Senning plus arterial switch operation
Congenitally Corrected TGA
 BCPC in anatomic correction
• It may benefit the small or poorly functioning
RV
• It importantly reduces complexcity of the atrial
baffle procedure
• It eliminates complications related to the
superior limb of the atrial baffle
• It reduces flow across an RV-pulmonary trunk
conduit
• It likely increases conduit longevity
Congenitally Corrected TGA
Anatomic correction
• The evidence is strong that right ventricle should not
remain in the systemic circulation as it does after a
conventional repair
• A combined arterial switch and Senning operation
( double switch operation ) is an option for patients
with cc-TGA with two ventricles of adequate size for
biventricular repair and a normal pulmonary valve
• The timing of surgery is difficult to choose because this
is a long and complex operation of Rastelli and atrial
switch procedure in patients with cc-TDA & VSD , &
PS or atresia
Congenitally Corrected TGA
Results of conventional repair
1. Survival
* Early deaths
* Time-related survival
2. Modes of death
3. Incremental risk factors for death
* Abnormalities of conduction system
* Abnormalities of ventricular function
* Regurgitation of systemic tricuspid valve
4. Post-repair complete heart block
5. Left-sided tricuspid valve incompetence
6. Ventricular function & functional status
Congenitally Corrected TGA
 Problems of physiologic repair
•
Progressive tricuspid regurgitation
•
Right ventricular dysfunction
•
Atrioventricular dysfunction
•
Conduit related problems
Congenitally Corrected TGA
Tricuspid regurgitation
• Volume load on the right ventricle
• Low incidence with naturally occurring
pulmonary stenosis
• Movement of interventricular septum
Congenitally Corrected TGA
Tricuspid valve abnormality
• In IVS
Preop. 38%
postop. 60%
• In VSD
Preop. 90%
postop. 56%
• In VSD+PS
Preop. 36%
postop. 36%
Congenitally Corrected TGA
Causes of Tricuspid Regurgitation
• Structural alteration of tricuspid valve component
Congenitally abnormal tricuspid valve
Adherence of septal leaflet or chordae to VSD patch
Asynchronous papillary muscle contraction with RBBB
Supraventricular or ventricular arrhythmia
• Abnormal function of structurally normal valve
Dilated annulus
Distraction of papillary muscles
Right ventricular or papillary muscle dysfunction
Other Forms of Atrioventricular
Discordant Connection
Atrioventricular Discordant Connection
Introduction
• Definition
A congenital anomaly in which right atrium connects
to left ventricle (LV) and left atrium connects to right
ventricle (RV).
• History
Ruttenberg ; AV discordant with DORV in 1964
Brandt
; AV discordant with DOLV in 1966
Van Praagh ; Isolated ventricular inversion in 1966
Isolated atrial inversion in 1972
Brandt
; Surgery for AV discordant with DOLV
in 1966
AV Discordant Connection
Morphology
• Ventricular architecture
• Ventricular position & rotation
Positional anomalies ; superior-inferior
ventricles
Rotational anomalies ; criss-cross pathway
• Ventricular size
• Cardiac position
• Ventriculoarterial connection
• AV node & bundle of His
• Accessory conduction pathways
• Coronary arteries
• Atrioventricular valves
Isolated Atrial Inversion
Surgical morphology
Isolated Ventricular Inversion
Surgical morphology
• Isolated ventricular inversion (A), & anatomically
corrected malposition of the great arteries (B)
AV Discordant Connection
Clinical features & diagnosis
• Clinical features of AV discordant connection vary
widely, depending on ventriculo-arterial connection
and associated cardiac anomalies
• Congenitally corrected TGA
VSD, PS
• DORV and DOLV
VSD, PS
• Isolated ventricular or atrial inversion
Similar to TGA, PS add additional cyanosis
AV Discordant Connection
Natural history
• Most of the information concerning natural
history drawn from patients with corrected
TGA should be expected, & other morphologic
findings may affect natural history
• Patients with situs inversus are more likely to
have DORV and TOF physiology, but less likely
to have systemic AV valve regurgitation and
heart block then patients with situs solitus
AV Discordant Connection
Surgicai indications
The diagnosis of DORV, DOLV, and isolated ventricular
or atrial inversion in patients with AV discordant
connection are indications for operation, but each has
special considerations.
Technique
•
•
•
•
•
Congenitally corrected TGA
DORV+PS
DOLV
Isolated ventricular or atrial inversion
Placing epicardial pacemaker leads
Isolated A-V Discordance
Surgicai procedures
• VSD closure
The position of the conduction bundle location was
assumed to be akin to that in congenitally corrected
transposition of the great arteries in the anterosuperior
edge of the septal defect
• Senning Repair
Native interatrial septum sufficed for the intra-atrial
baffle utilized to separate the pulmonary veins from the
mitral valve in all four Senning repairs.
AV Discordant Connection
Results of surgical treatment
• Survival
Early death
Time-related survival
• Mode of death
• Incremental risk factors for death
AV discordant connection ; probably major
VA discordant connection ; probably not recently
• Postrepair heart block
• Other outcome events
Others as in cc-TGA ( TR, Block, Function, etc)
Use of valved conduit
Anatomically Corrected Malposition
of Great Arteries
Anatomically Corrected Malposition
of Great Arteries
Introduction
• Definition
Anatomically corrected malposition is an anomaly in the position of the great
arteries and not in cardiac connections.
The aortic origin lies to the left and usually anterior to the pulmonary trunk
origin when there is situs solitus and the circulatory pathways remain in
series.
• History
Theveanin
; 1st report in 1985
Harris & Farber ; Termed anatomically corrected malposition
Raghib
; Described isolated bulbar inversion in corrected
transposition
Van Praagh
; Described in 1967 using the term of anatomically
corrected transposition of great arteries
Corrected Malposition of GAs
Morphology
• Structure of sinus portions of both ventricle is normal
• There are abnormalities of the outlet, or infundibulum,
in both ventricle.
• The LV probably always exhibits a subaortic conus
• The RV may also have an infundibulum, but it may be
less well developed and in some case is absent
• The aorta lies to the left and usually anterior to the
pulmonary trunk
Corrected Malposition of GAs
Associated anomalies
•
•
•
•
Commonly large VSD, usually conoventricular
Pulmonary stenosis is usual, often infundibular
Subaortic stenosis may occur
Tricuspid atresia or hypoplasia in half & RV
hypoplasia
Corrected Malposition of GAs
Clinical features & diagnosis
• Clinical features depend on associated
anomalies.
• Characteristic appearance of L-malposition in
chest radiograph
• The natural history is affected as typical for the
associated cardiac anomalies
Corrected Malposition of GAs
Technique of operation
Determined by associated cardiac anomalies, such as
Fontan operation in hypoplastic ventricle
VSD closure & PS relief when necessary
Indications for operation
Anatomically corrected malposition is not an indication
for operation. Coexisting cardiac anomalies may
present an indication for operation.