corrected transposition of great arteries
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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 - PowerPoint PPT PresentationTRANSCRIPT
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 : 1st description in 1875 Schiebler : Clinical syndrome in 1961 Anderson, Lillehei : 1st repair in 1957 Ilbawi et al : Double switch operation in 1990
Congenitally Corrected TGA
Pathophysiology• Combined AV & ventriculo-arterial discordance r
esulting 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
• Diagrammatic models of four basic hearts
Morphologic characteristics
Congenitally Corrected TGA
Surgical morphology 1. Ventricle (conus, loop, position) Dextrocardia
2. Pulmonary outflow tract; Transverse plane & wedged
3. Atrial, ventricular septal position 4. Tricuspid, mitral, aortic valves 5. Ventricular septal defect 6. Atrioventricular node & bundle of His 7. Coronary arterial patterns 8. Associated anomalies
no coexistent cardiac anomalies in 1 ~ 2 %
Associated cardiac anomalies
• Ventricular septal defect : 86%
• Pulmonary stenosis : 64%
• Tricuspid regurgitation : 28%
• AV block : 12%
Congenitally Corrected TGA
Surgical morphology
Congenitally Corrected TGA
Congenitally Corrected TGASurgical morphology
Surgical pathology
Morphologic LV
Congenitally Corrected TGA
Morphologic RV
Congenitally Corrected TGA
Surgical pathology
VSD
Morphologic RV
Morphologic LV
Congenitally Corrected TGASurgical pathology
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
• Rt. sided AV valve & ASD through Rt. atriotomy
Congenitally Corrected TGASurgical view
• VSD through Rt. sided atrioventricular valve
Congenitally Corrected TGASurgical view
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
• Senning plus arterial switch operation
Congenitally Corrected TGA
Double switch operation
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 superi
or 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 re
main 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 pulmo
nary stenosis
• Movement of interventricular septum
Congenitally Corrected TGA
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%
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
Congenitally Corrected TGA
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 ConnectionMorphology• Ventricular architecture• Ventricular position & rotation Positional anomalies ; superior-inferior ventri
cles 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
• Isolated ventricular inversion (A), & anatomically corrected malposition of the great arteries (B)
Surgical morphology
AV Discordant Connection
Clinical features & diagnosis• Clinical features of AV discordant connection vary widel
y, 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 his
tory 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 ventricula
r 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 assu
med to be akin to that in congenitally corrected transposition of the great arteries in the anterosuperior edge of the septal defect
• Senning RepairNative 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 o
rigin 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
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
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 h
ypoplasia
Corrected Malposition of GAs
Clinical features & diagnosis• Clinical features depend on associated anomali
es.• 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 indicatio
n for operation. Coexisting cardiac anomalies may present an indication for operation.