corrected transposition of 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 : 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


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%


Surgical morphology


Congenitally Corrected TGASurgical morphology

Surgical pathology

Morphologic LV


Morphologic RV


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


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


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


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)


Morphologic characteristics


Surgical view

• Rt. sided AV valve through Rt. atriotomy


• 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


Apico-pulmonary artery conduit


Repair of VSD + PS

Standard repair of situs solitus congenitally corrected TGA, VSD, and PS


One & a half ventricle repair cc-TGA, VSD, PS

VSD closure, pulmonary valvotomy, and inparallel BCPC


Double switch operation

• Bidirectional superior cavopulmonary anastomosis and hemi-Mustard modification for double switch procedure


• Senning plus arterial switch operation


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


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


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


Problems of physiologic repair

• Progressive tricuspid regurgitation

• Right ventricular dysfunction

• Atrioventricular dysfunction

• Conduit related problems


Tricuspid regurgitation

• Volume load on the right ventricle

• Low incidence with naturally occurring pulmo

nary stenosis

• Movement of interventricular septum



Tricuspid valve abnormality• In IVS

Preop. 38% postop. 60%

• In VSD


• In VSD+PS


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 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


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


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.


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


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


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


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.

corrected transposition of great arteries

Documents