HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Abstract Full Text (PDF) Online Discussion Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Wilkinson, J. L. Articles by Karl, T. R. Search for Related Content PubMed PubMed Citation Articles by Wilkinson, J. L. Articles by Karl, T. R.

Ann Thorac Surg 2000;69:S236-S248
© 2000 The Society of Thoracic Surgeons

Congenital Heart Surgery Nomenclature and Database Project: corrected (discordant) transposition of the great arteries (and related malformations)

^a Cardiac Surgical Unit, Royal Children’s Hospital, Melbourne, Australia
^b Department of Cardiology, Royal Children’s Hospital, Melbourne, Australia

Address reprint requests to Dr Karl, Department of Cardiothoracic Surgery, Children’s Hospital of Philadelphia, 34th & Civic Center Blvd, Philadelphia, PA 19104
e-mail: karl{at}email.chop.edu

Presented at the International Nomenclature and Database Conferences for Pediatric Cardiac Surgery, 1998–1999.

Abstract

The extant nomenclature for congenitally corrected transposition of the great arteries is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. All efforts were made to include all relevant nomenclature categories using synonyms where appropriate. Congenitally corrected transposition of the great arteries is a complex cardiac lesion that is associated with ventricular septal defect, left ventricular outflow tract obstruction, and tricuspid valve abnormalities. Nomenclature for this lesion has been variable and confusing. Herein we attempt to clarify the terminology, with an emphasis on identification of synonymous and nonsynonymous appellations. A comprehensive database set is presented that is based on a hierarchical scheme. Data are entered at various levels of complexity and detail that can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analyses. A minimum database set is also presented that will allow for data sharing and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.

I. Background

The anomaly referred to as corrected transposition of the great arteries describes a malformation in which the two ventricles are connected inappropriately to the atria and in which the great arteries are transposed. Thus, the systemic venous atrium (right atrium) is connected to the morphologically left ventricle (LV), and the pulmonary venous atrium (left atrium) is connected to the morphologically right ventricle (RV). The connection of the great arteries is also abnormal, with the aorta originating from the RV and the pulmonary artery (PA) from the left ventricle. As a result of the abnormal (discordant) connection at both levels, systemic venous blood flows to the pulmonary circulation and pulmonary venous blood passes to the systemic circulation (discordare, Latin for "to disagree"). Thus, the defect may be regarded as being "physiologically corrected."

The malformation may occur in patients with situs solitus (segmental arrangement: solitus/discordant/discordant) or in those with situs inversus (segmental arrangement: inversus/discordant/discordant). However, it does not occur in patients with situs ambiguus (atrial isomerism), because the arrangement of the atrial chambers is inevitably so disorganized in such cases that the atrioventricular (AV) connection can be described as neither discordant nor concordant.

The term "corrected transposition" was introduced by Rokitansky, who first described the malformation and noted the physiological correction [1]. A number of anecdotal descriptions were published over the next 50 years. In 1956, Cardell reviewed 25 cases (introducing one of his own), and in 1961, Lev and Rowlatt described the pathological appearances in a number of specimens [2, 3]. With the inception of surgical repair for transposition, the term congenitally corrected transposition was introduced by Schiebler and colleagues in 1961 [4]. The intent was to distinguish the naturally occurring condition from surgically repaired transposition (ie, cases with a naturally concordant AV connection made discordant with a Senning or Mustard operation). While the term "corrected transposition" has gained widespread use, it has been opposed by some cardiac morphologists and is not the favored nomenclature of others [3, 5, 6].

The use of the term "transposition" has itself been controversial, and various definitions of the term have been proposed. Some early workers used the term in a very broad sense, to refer to virtually any abnormality in the aorto-pulmonary relationship [1]. Other definitions used include antero-posterior reversal of the aorto-pulmonary relationship and the presence of a muscular subaortic conus [2, 7, 8]. Van Praagh and Van Praagh proposed the straightforward and currently favored definition, "right ventricular origin of the aorta and left ventricular origin of the pulmonary artery" in 1971 [9]. the term "transposition" is synonymous with either "ventriculoarterial discordance" or "discordant ventriculoarterial connection" [10, 11].

Left AV valve abnormalities
The frequent association of minor or major deformities and displacement of the left AV (tricuspid) valve, which may resemble Ebstein’s malformation, is well documented. Approximately 90% of cases reviewed in a postmortem series had abnormalities of the left AV valve [12]. Incompetence of the valve is less frequent and probably affects about one-third of patients, at least in early childhood [4]. Severe tricuspid incompetence is probably the single most important adverse prognostic feature in affected individuals [13].

The following terms have been used to describe the malformation (not necessarily in order of preference):

Ventricular inversion

L transposition

Double discordance

Mixed levocardia/mixed dextrocardia

Dextroversion

Atrioventricular discordance (with transposition)

Physiologically corrected transposition

Congenitally corrected transposition

Discordant transposition

Inverted transposition

II. Analysis: a unified nomenclature system for congenitally corrected transposition of the great arteries

Atrial situs
In considering the abnormal atrioventricular connection, which is universal in patients with corrected transposition, it is essential to clarify atrial arrangement (situs). Most authorities employ the terms "situs solitus" (solitus, Latin for "usual"), "situs inversus," and "situs ambiguus," according to the segmental approach proposed by Van Praagh and associates [6, 14]. Others have employed the terms "usual atrial arrangement," "mirror image atrial arrangement," and "atrial isomerism" or "isomerism of the atrial appendages" to imply the same thing [11]. For the purposes of this discussion, the terms, "solitus" and "inversus" (S & I) will be employed as being synonymous with usual and mirror image.

Ventricular inversion
The term "inversion" has been used widely to describe left-right reversal (mirror image) of paired structures [4]. This term can be applied to lungs, abdominal viscera, or cardiac structures (including atria, ventricles, or great arteries). When used to describe ventricular inversion, this normally implies that the morphological LV is right sided and the morphological RV is left sided. However, some have modified the use of the term to encompass the situation in which the ventricles are normally related in patients with situs inversus (atrial inversion) [15]. In this usage, this term "ventricular inversion" is synonymous with the more widely understood term "atrioventricular discordance" [10, 11, 14].

Most authorities would have problems with accepting the use of a well-established term, whose meaning is clearly understood, to cover a complex situation that includes a variety of additional or variable features. For this reason, the term "ventricular inversion" is probably an inappropriate term to describe corrected transposition.

L-transposition (L-TGA)
Van Praagh introduced this term in the 1960s [6]. It describes transposition with a left-sided (usually anterior) aorta. In most cases, such a situation coexists with a discordant AV connection and the morphologically left ventricle is right sided and the morphologically right ventricle is left sided. Van Praagh designated this ventricular arrangement as an "L loop," referring to the formation of the bulboventricular loop in the embryo, which rotates in the opposite direction (to the left) to that which normally occurs ("D loop") [6]. According to Van Praagh, the association of L-transposition with an L-loop is so close that the presence of L-transposition is, for practical purposes, indicative of corrected transposition. Since the term was introduced, many morphologists and clinicians have tended to use the terms as though they were synonymous. However, it is well recognized that some patients with corrected transposition, even in the presence of an L-loop, have an aorta that lies anterior and to the right of the pulmonary artery. This breaches the loop rule, as has also been noted in patients with transposition and a concordant AV connection [16]. Additionally, patients with situs inversus and corrected transposition exhibit a D-loop and D-transposition, implying that the aorta is right sided and the ventricles are normally related to noninverted (the reverse of the expected arrangement or loop for a patient with situs inversus). For these reasons, many experts have difficulty with the term "L-transposition" as a substitute for "corrected transposition."

Double discordance
This term is not widely used, though it does have the virtues of brevity and accuracy. According to most investigators, corrected transposition comprises the combination of AV discordance and ventriculoarterial discordance (or a discordant AV connection combined with a discordant ventriculoarterial connection). The term "double discordance" clearly caters adequately for the combined defects that characterize this malformation. Unfortunately, it is not in widespread use at the present time.

Mixed levocardia (and mixed dextrocardia)
This term, introduced by Lev, includes other malformations and is not in current use [17]. It refers to a situation in which the base-apex axis of the heart points to the left but in which the atria and ventricles do not correspond (AV discordance) [3]. Strictly speaking (according to Lev and Rowlatt), the appropriate designation for those cases that would fall within the category of corrected transposition is mixed levocardia with complete inverted transposition [3]. The related term "mixed dextrocardia" [17] caters similarly for patients with atrioventricular discordance and dextrocardia. However, the terms are not generally used/understood and are unhelpful.

Dextroversion (Fig 1)
This term is also of little use as a substitute for corrected transposition. It refers to hearts in patients with situs solitus, and rightward displacement or rotation of the ventricles, with or without ventricular inversion or corrected transposition [18–20]. As many patients have a left-sided heart, and the term clearly does not encompass hearts with atrial situs inversus, it is not a useful descriptive term for the malformation.

View larger version (31K): [in this window] [in a new window]   Fig 1. Levocardia/dextrocardia. (A) Variant of corrected TGA with apex to left. (B) Variant with apex to right (dextrocardia/dextroversion).

Physiologically corrected transposition
As alluded to in the introduction, this term reflects the fact that in corrected transposition, if other major defects are absent, pulmonary venous blood passes to the systemic circulation and systemic venous blood to the pulmonary circulation. With earlier concepts and nomenclatures for transposition (eg, anteroposterior reversal of the relationship between the aorta and pulmonary artery), the term "corrected transposition" was often used to describe the rare situation in which the aorta arises from the LV in an anterior position, with the PA from the RV, lying posteriorly. In such instances, the AV connection may be discordant but the ventriculoarterial connection is concordant and the physiology is therefore similar to that of transposition. This combination is not physiologically corrected, and was therefore described as "anatomically corrected" (to distinguish it from the physiologically corrected variety). However, in exceptional instances, the AV connection may also be concordant, so that the malformation is both anatomically and physiologically corrected. Fortunately, the currently favored definition of transposition, which is synonymous with ventriculoarterial discordance, solves this dilemma, as the anatomically corrected variants are no longer considered as examples of transposition (now being referred to instead as "malposition") [9].

In practice, because of the frequently associated defects (such as left ventricular outflow tract obstruction [LVOTO] or pulmonary atresia with a ventricular septal defect [VSD]) corrected transposition is all too often far from physiologically correct! This term (physiologically corrected transposition) is therefore of little practical utility.

Congenitally corrected transposition
This term, introduced in 1961 by Schiebler and colleagues, is sometimes used to help to maintain the important semantic distinction between congenitally corrected transposition and surgically repaired transposition [4]. The term is simple and accurate and is clearly acceptable, although often perceived as being a little cumbersome. This is probably the most accepted term in current use for this anatomic complex, although the acceptance is by no means universal.

Discordant transposition
This term is favored by some as a shorthand term for the malformation. In practice, it is no briefer than the more widely used "corrected transposition," and can be criticized as being a tautology, as the term "discordant" (at the arterial level) defines the term transposition. On the other hand, using Boolean logic, discordant transposition of the great arteries could only indicate combined AV and VA discordance, and in that sense is a precise and quite useful term. It has also been used frequently in important surgical journal articles and textbooks, and is preferred by the Royal Children’s Hospital surgeons, among others. Perhaps an expanded version of this nomenclature, such as "atrioventricular discordance with transposition," is more accurate and acceptable (see above), but it lacks the brevity and crispness.

Inverted transposition
This term has similar meaning to Van Praagh’s term "L-transposition" [3, 5]. It has not been widely used and is best avoided.

Ventricular spatial relationships (Fig 2)
Usually, the two AV inlets are aligned more or less in parallel, each being at approximately the same level (in the thorax). Under such circumstances, the ventricles are related in the anticipated manner, with one being to the right of the other, although sometimes one ventricle is more anterior or superior. The ventricular outlets are usually parallel as well, with the aorta on the same side as the RV. Rarely, one valve (inlet) may reside at a significantly higher level than its counterpart, and in extreme cases, the tricuspid valve (RV inlet) may be directly anterosuperior to the mitral valve (LV inlet). In such instances, the ventricles may be in a supero-inferior relationship (upstairs-downstairs) or, if the axes of the AV inlets cross one another in the transverse plane, an atrioventricular crisscross connection may be present [21]. This implies that the relationship of the ventricular inlets and apical trabecular zones are the opposite of that which would be anticipated. Thus, despite a discordant AV connection (with situs solitus), the RV may lie on the right side of the ventricular mass, with the LV being inferior and leftward, forming the cardiac apex (on the left). In such cases (fortunately very uncommonly), the loop direction (D or L) is debatable, and segmental description becomes almost meaningless. Thus, the proposed classification (see below) allows for a separate category for cases that cannot be adequately described using the usual conventions, both for those with situs solitus and with situs inversus.

View larger version (31K): [in this window] [in a new window]   Fig 2. Criss-cross atrioventricular connection. (A) Usual arrangement with corrected TGA (SL). (B) Criss-cross arrangement: segmented formula unclear.

Related anomalies
Two other malformations have been included in the classification, being closely related morphologically to corrected transposition. These are:

1. AV discordance with double-outlet RV
This is a rare malformation, which frequently resembles corrected transposition with VSD. As the PA is usually closely related to the associated VSD and blood from the morphologically LV passes preferentially to the pulmonary artery, the physiology is also similar to corrected transposition with a VSD, as is the surgical treatment. However, as with other forms of double-outlet right ventricle, there is a wide variety of associated malformations and variations, which often profoundly affect the clinical presentation and management.

2. AV discordance with ventriculo-arterial concordance
This defect is sometimes called "isolated ventricular inversion" and is extremely uncommon. The term "isolated ventricular inversion" is neither universally used nor understood, and is best avoided. A better approach to these very rare defects is to utilize the segmental approach, describing atrial arrangement, AV connection, ventriculoarterial connection, and other aspects (including the spatial relationships of the various structures and associated malformations) in simple anatomic terms. The abbreviated segmental formula is of limited help in these situations, though in many instances with ventriculoarterial concordance the arterial relationship is either normal (when the ventricles are normally related) or mirror image with respect to normal (when the ventricles are in the mirror image relationship). The segmental formulas for such relationships are: D (D loop) S (solitus = normal arterial relationship) and L (L loop) I (inversus = mirror image arterial relationship). Thus, with AV discordance and VA concordance, the complete segmental formula is usually SLI or IDS. Unlike corrected transposition, the physiology of such defects resembles that of transposition, with deoxygenated blood passing to the systemic circulation via the morphologically LV (which is connected to the aorta), and oxygenated blood being channeled to the PA from the morphologically RV.

Corrected transposition and related anomalies hierarchy level 1

Discordant AV connection (AV discordance) with situs solitus (usual atrial arrangement)

Discordant AV connection (AV discordance) with situs inversus (mirror image atrial arrangement)

Corrected transposition and related anomalies hierarchy level 2

Discordant AV connection (AV discordance) with situs solitus (usual atrial arrangement)

With discordant VA connection (congenitally corrected transposition) (usually SLL)

With criss-cross AV connection and discordant VA connection (corrected transposition/criss-cross)

With concordant VA connection (isolated ventricular inversion) (usually SLI)

With double-outlet right ventricle

Discordant AV connection (AV discordance) with situs inversus (mirror image atrial arrangement)

With discordant VA connection (congenitally corrected transposition) (usually IDD)

With criss-cross AV connection and discordant VA connection (corrected transposition/criss-cross)

With concordant VA connection (isolated ventricular inversion) (usually IDS)

With double-outlet right ventricle

Corrected transposition and related anomalies hierarchy level 2

Discordant AV connection with situs solitus, not otherwise specified (NOS)

Discordant AV connection with situs solitus, Discordant VA connection (congenitally corrected transposition) (usually SLL)

Discordant AV connection with situs solitus, Crisscross AV connection and discordant VA connection (corrected transposition/crisscross)

Discordant AV connection with situs solitus, Concordant VA connection (isolated ventricular inversion) (usually SLI)

Discordant AV connection with situs solitus, Double-outlet right ventricle

Discordant AV connection with situs inversus, NOS

Discordant AV connection with situs inversus, Discordant VA connection (congenitally corrected transposition) (usually IDD)

Discordant AV connection with situs inversus, With criss-cross AV connection and discordant VA connection (corrected transposition/criss-cross)

Discordant AV connection with situs inversus, With concordant VA connection (isolated ventricular inversion) (usually IDS)

Discordant AV connection with situs inversus, With double-outlet right ventricle

Corrected transposition and related anomalies hierarchy level 3

Discordant AV connection (AV discordance) with situs solitus (usual atrial arrangement)

With discordant VA connection (congenitally corrected transposition) (usually SLL)

With intact ventricular septum and no left ventricular (LV) outflow tract obstruction

With intact ventricular septum and LV (subpulmonary/pulmonary) outflow tract obstruction

With VSD and no LV outflow tract obstruction

With VSD and LV (subpulmonary/pulmonary) outflow tract obstruction

With criss-cross AV connection and discordant VA connection (corrected transposition/criss-cross)

With intact ventricular septum and no LV outflow tract obstruction

With intact ventricular septum and LV (subpulmonary/pulmonary) outflow tract obstruction

With VSD and no LV outflow tract obstruction

With VSD and left ventricular (subpulmonary/pulmonary) outflow tract obstruction

With concordant VA connection (isolated ventricular inversion) (usually SLI)

With intact ventricular septum

With VSD

With double-outlet right ventricle

With aorta to left (or left anterior) of pulmonary artery (SLL)

With aorta anterior to pulmonary artery (SLA)

With aorta to right (or right anterior) of pulmonary artery (SLD)

Discordant AV connection (AV discordance) with situs inversus (mirror image atrial arrangement)

With discordant VA connection (congenitally corrected transposition) (usually IDD)

With intact ventricular septum and no LV outflow tract obstruction

With intact ventricular septum and LV (subpulmonary/pulmonary) outflow tract obstruction

With VSD and no LV outflow tract obstruction

With VSD and left ventricular (subpulmonary/pulmonary) outflow tract obstruction

With criss-cross AV connection and discordant VA connection (corrected transposition/criss-cross)

With intact ventricular septum and no LV outflow tract obstruction

With intact ventricular septum and LV (subpulmonary/pulmonary) outflow tract obstruction

With VSD and no LV outflow tract obstruction

With VSD and left ventricular (subpulmonary/pulmonary) outflow tract obstruction

With concordant VA connection (isolated ventricular inversion) (usually SLT)

With intact ventricular septum (IVS)

With VSD

With double-outlet right ventricle

With aorta to right (or right anterior) of pulmonary artery (IDD)

With aorta anterior to pulmonary artery (IDA)

With aorta to left (or left anterior) of pulmonary artery (IDL)

Corrected transposition and related anomalies hierarchy level 3

Discordant AV connection with situs solitus, NOS

Discordant AV connection with situs solitus, discordant VA connection (congenitally corrected transposition) (usually SLL), NOS

Discordant AV connection with situs solitus, discordant VA connection (congenitally corrected transposition) (usually SLL), IVS and no LVOTO

Discordant AV connection with situs solitus, discordant VA connection (congenitally corrected transposition) (usually SLL), IVS and LVOTO

Discordant AV connection with situs solitus, discordant VA connection (congenitally corrected transposition) (usually SLL), VSD and no LVOTO

Discordant AV connection with situs solitus, discordant VA connection (congenitally corrected transposition) (usually SLL), VSD and LVOTO

Discordant AV connection with situs solitus, crisscross AV connection and discordant VA connection (corrected transposition/crisscross), NOS

Discordant AV connection with situs solitus, crisscross AV connection and discordant VA connection (corrected transposition/criss-cross), IVS and no LVOTO

Discordant AV connection with situs solitus, crisscross AV connection and discordant VA connection (corrected transposition/criss-cross), IVS and LVOTO

Discordant AV connection with situs solitus, crisscross AV connection and discordant VA connection (corrected transposition/criss-cross), VSD and no LVOTO

Discordant AV connection with situs solitus, crisscross AV connection and discordant VA connection (corrected transposition/criss-cross), VSD and LVOTO

Discordant AV connection with situs solitus, concordant VA connection (isolated ventricular inversion) (usually SLI), NOS

Discordant AV connection with situs solitus, concordant VA connection (isolated ventricular inversion) (usually SLI), IVS

Discordant AV connection with situs solitus, concordant VA connection (isolated ventricular inversion) (usually SLI), VSD

Discordant AV connection with situs solitus, double-outlet right ventricle, NOS

Discordant AV connection with situs solitus, double-outlet right ventricle, With aorta to left (or left anterior) of pulmonary artery (SLL)

Discordant AV connection with situs solitus, double-outlet right ventricle, With aorta anterior to pulmonary artery (SLA)

Discordant AV connection with situs solitus, double-outlet right ventricle, With aorta to right (or right anterior) of pulmonary artery (SLD)

Discordant AVconnection with situs inversus, NOS

Discordant AV connection with situs inversus, discordant VA connection (congenitally corrected transposition) (usually IDD), NOS

Discordant AV connection with situs inversus, discordant VA connection (congenitally corrected transposition) (usually IDD), IVS and no LVOTO

Discordant AV connection with situs inversus, discordant VA connection (congenitally corrected transposition) (usually IDD), IVS and LVOTO

Discordant AV connection with situs inversus, discordant VA connection (congenitally corrected transposition) (usually IDD), VSD and no LVOTO

Discordant AV connection with situs inversus, discordant VA connection (congenitally corrected transposition) (usually IDD), VSD and LVOTO

Discordant AV connection with situs inversus, with criss-cross AV connection and discordant VA connection (corrected transposition/criss-cross), NOS

Discordant AV connection with situs inversus, with criss-cross AV connection and discordant VA connection (corrected transposition/criss-cross), IVS and no LVOTO

Discordant AV connection with situs inversus, with criss-cross AV connection and discordant VA connection (corrected transposition/criss-cross), IVS and LVOTO

Discordant AV connection with situs inversus, with criss-cross AV connection and discordant VA connection (corrected transposition/criss-cross), VSD and no LVOTO

Discordant AV connection with situs inversus, with criss-cross AV connection and discordant VA connection (corrected transposition/criss-cross), VSD and LVOTO

Discordant AV connection with situs inversus, with concordant VA connection (isolated ventricular inversion) (usually IDS), NOS

Discordant AV connection with situs inversus, with concordant VA connection (isolated ventricular inversion) (usually IDS), IVS

Discordant AV connection with situs inversus, with concordant VA connection (isolated ventricular inversion) (usually IDS), VSD

Discordant AV connection with situs inversus, with double-outlet right ventricle, NOS

Discordant AV connection with situs inversus, with double-outlet right ventricle, with aorta to right (or right anterior) of pulmonary artery (IDD)

Discordant AV connection with situs inversus, with double-outlet right ventricle, with aorta anterior to pulmonary artery (IDA)

Discordant AV connection with situs inversus, with double-outlet right ventricle, with aorta to left (or left anterior) of pulmonary artery (IDL)

Corrected transposition and related anomalies hierarchy level 4

GUEST EDITORS’ NOTE: Many of the issues described in Hierarchy level 3 and 4 may be coded in detail in the computer database as additional diagnoses. These include VSD, ventricular outflow tract obstruction, and AV valve abnormalities.

Discordant AV connection (AV discordance) with situs solitus (usual atrial arrangement)

With discordant VA connection (congenitally corrected transposition) (usually SLL)

With intact ventricular septum and no LV outflow tract obstruction

With no major left (tricuspid) AV valve abnormality/regurgitation

With significant left (tricuspid) AV valve abnormality/regurgitation

With intact ventricular septum and LV (subpulmonary/pulmonary) outflow tract obstruction

With no major left (tricuspid) AV valve abnormality/regurgitation

With significant left (tricuspid) AV valve abnormality/regurgitation

With VSD and no LV outflow tract obstruction

With no major left (tricuspid) AV valve abnormality/regurgitation

With significant left (tricuspid) AV valve abnormality/regurgitation

With VSD and left ventricular (subpulmonary/pulmonary) outflow tract obstruction

With no major left (tricuspid) AV valve abnormality/regurgitation

With significant left (tricuspid) AV valve abnormality/regurgitation

With criss-cross AV connection and discordant VA connection (corrected transposition/criss-cross)

With intact ventricular septum and no LV outflow tract obstruction

With no major left (tricuspid) AV valve abnormality/regurgitation

With significant left (tricuspid) AV valve abnormality/regurgitation

With intact ventricular septum and LV (subpulmonary/pulmonary) outflow tract obstruction

With no major left (tricuspid) AV valve abnormality/regurgitation

With significant left (tricuspid) AV valve abnormality/regurgitation

With VSD and no LV outflow tract obstruction

With no major left (tricuspid) AV valve abnormality/regurgitation

With significant left (tricuspid) AV valve abnormality/regurgitation

With VSD and left ventricular (subpulmonary/pulmonary) outflow tract obstruction

With no major left (tricuspid) AV valve abnormality/regurgitation

With significant left (tricuspid) AV valve abnormality/regurgitation

Discordant AV connection (AV discordance) with situs inversus (mirror image atrial arrangement)

With discordant VA connection (congenitally corrected transposition) (usually IDD)

With intact ventricular septum and no LV outflow tract obstruction

With no major right (tricuspid) AV valve abnormality/regurgitation

With significant right (tricuspid) AV valve abnormality/regurgitation

With intact ventricular septum and LV (subpulmonary/pulmonary) outflow tract obstruction

With no major right (tricuspid) AV valve abnormality/regurgitation

With significant right (tricuspid) AV valve abnormality/regurgitation

With VSD and no LV outflow tract obstruction

With no major right (tricuspid) AV valve abnormality/regurgitation

With significant right (tricuspid) AV valve abnormality/regurgitation

With VSD and left ventricular (subpulmonary/pulmonary) outflow tract obstruction

With no major right (tricuspid) AV valve abnormality/regurgitation

With significant right (tricuspid) AV valve abnormality/regurgitation

With criss-cross AV connection and discordant VA connection (corrected transposition/criss-cross)

With intact ventricular septum and no LV outflow tract obstruction

With no major right (tricuspid) AV valve abnormality/regurgitation

With significant right (tricuspid) AV valve abnormality/regurgitation

With intact ventricular septum and LV (subpulmonary/pulmonary) outflow tract obstruction

With no major right (tricuspid) AV valve abnormality/regurgitation

With significant right (tricuspid) AV valve abnormality/regurgitation

With VSD and no LV outflow tract obstruction

With no major right (tricuspid) AV valve abnormality/regurgitation

With significant right (tricuspid) AV valve abnormality/regurgitation

With VSD and left ventricular (subpulmonary/pulmonary) outflow tract obstruction

With no major right (tricuspid) AV valve abnormality/regurgitation

With significant right (tricuspid) AV valve abnormality/regurgitation

Additional modifiers for corrected transposition and related anomalies

Discordant AV connection (AV discordance) with situs solitus (usual atrial arrangement)

With levocardia (left-sided ventricular mass/apex)

With dextrocardia (right-sided ventricular mass/apex)

With mesocardia (central/midline ventricular mass/apex)

Discordant AV connection (AV discordance) with situs inversus (mirror image atrial arrangement)

With levocardia (left-sided ventricular mass/apex)

With dextrocardia (right-sided ventricular mass/apex)

With mesocardia (central/midline ventricular mass/apex)

III. Nomenclature for treatment options for corrected transposition and related anomalies

Operations for congenitally corrected transposition fall basically into four categories, as follows and comprise hierarchy 1:

Palliative procedures

Definitive operations that leave the right ventricle in the systemic circulation and the left ventricle in the pulmonary circulation

Definitive operations that leave the left ventricle in the systemic circuit and the right ventricle in the pulmonary circuit

Definitive operations that leave both ventricles connected to the systemic circuit and that employ some type of cavopulmonary connection for the systemic venous to pulmonary arterial circuit.

Many of the palliative procedures used in corrected transposition have broad applicability in surgery for other types of complex heart lesions. However, the biventricular repairs are in many aspects unique to this lesion.

The classical repair for corrected transposition with VSD involves septation that leaves the right ventricle in the systemic circuit and the left ventricle in the pulmonary circuit (Fig 3). Although the initial outcome of such repairs have generally been good in properly selected patients, at 10 years the results are very disappointing, primarily due to problems with function of the tricuspid valve and right ventricle [22–24]. For this reason, there has been a great interest over the past decade in alternate strategies, with the goal of connecting the LV to the aorta. While much more complex, such operations are likely to have a better long-term result. Specifically, these include the Senning (or Mustard) operation + arterial switch + VSD closure, and the LV to aorta intraventricular baffle with RV to PA conduit (Rastelli) + Senning (or Mustard) procedure [24, 25].

View larger version (29K): [in this window] [in a new window]   Fig 3. "Classical" repair of corrected transposition versus double switch. (A) Septation leaving the LV in the pulmonary circulation and the RV in the systemic circulation, maintaining the AV and VA discordant connections. (B) Double switch, with restoration of concordant AV and VA connections.

A detailed list of possible surgical procedures for corrected transposition follows, and would be appropriate for a comprehensive database. In order to list the procedures applicable to a given patient, one only needs to provide the numbers in the outline key. The categories are not designed to be exclusive. For example a double-switch operation with VSD closure, LeCompte maneuver, and enlargement of the pulmonary venous atrium might be coded for database/computing purposes as 1c/4a,g,i/7a,c/9a, and a Senning Rastelli operation would be coded as 1c/4a,i/5c,i/7a,c. If the patient had a classical type septation plus an LV to PA homograft conduit and concurrent dual-chamber pacemaker insertion, the code would be 1c/4b,g,i/15a,b/16b. Thus, a series of numbers can be used to specify uniquely and in as much detail as is required which procedures were performed.

1. Incisions
   

   Left thoracotomy
   

   Right thoracotomy
   

   Sternotomy 1°
   

   Sternotomy 2°
   

   Partial sternotomy
   

   Endoscopic
   

   
   
2. PA band (primary procedure)
   

   Via sternotomy
   

   Via right thoracotomy
   

   Via left thoracotomy
   

   Adjustable
   

   For left ventricular conditioning
   

   
   
3. Systemic PA-shunt
   

   Modified Blalock-Taussig shunt
   

   
   
4. Right
   
5. Left
   

   Classical Blalock-Taussig shunt
   

   
   
6. Right
   
7. Left
   

   Central shunt
   

   
   
8. Aorta-main pulmonary artery
   
9. Aorta-right pulmonary artery
   
10. Aorta-left pulmonary artery
    
11. VSD Closure
    
12. Trans RV
    

    Trans RA
    

    Trans LV
    

    Trans aorta
    

    Trans PA
    

    Trans LA
    

    de Leval technique
    

    Pericardial
    

    Dacron
    

    Polytetrafluoroethylene (PTFE)
    

    
    
13. Ventricle to PA connection
    

    Direct PA-RV
    

    
    
14. Patch augmentation
    
15. Monocusp valve
    

    Direct PA-LV
    

    
    
16. Patch augmentation
    
17. Monocusp valve
    

    RV-PA conduit insertion
    

    
    
18. Homograft valved conduit
    
19. Xenograft valved conduit
    
20. Valveless tube
    

    LV-PA conduit
    

    
    
21. Homograft valved conduit
    
22. Xenograft valved conduit
    
23. Valveless tube
    

    Pulmonary valvotomy
    

    LVOT patch enlargement
    

    Conduit replacement
    

    
    
24. Homograft valved conduit
    
25. Xenograft valved conduit
    
26. Valveless tube
    
27. Intraventricular baffle (LV in continuity with aorta)
    
28. Atrial baffle
    

    Senning
    

    Mustard
    

    Enlargement of pulmonary venous atrium
    

    
    
29. Valve revision
    

    Left AV valve repair
    

    Left AV valve replacement
    

    Right AV valve repair
    

    Right AV valve replacement
    

    Annuloplasty ring implant
    

    
    
30. Arterial switch
    

    LeCompte maneuver
    

    
    
31. Pulmonary artery reconstruction
    

    Pericardial patch
    

    PTFE patch
    

    Dacron patch
    

    Homograft tube
    

    Dacron tube
    

    Gore-Tex tube
    

    
    
32. Cavopulmonary connection
    

    Right superior vena cava-pulmonary artery
    

    Left superior vena cava-pulmonary artery
    

    Total cavopulmonary connection lateral tunnel
    

    Atriopulmonary fontan
    

    Extracardiac inferior conduit
    

    
    
33. VSD enlargement
    

    Trans LV
    

    Trans RV
    

    Transatrial
    

    
    
34. Intraoperative mapping
    
35. Ablation of accessory pathway
    

    Cryoablation, open
    

    Surgical interruption
    

    
    
36. Pacemaker lead insertion
    

    Atrial
    

    
    
37. Epicardial
    
38. Endocardial
    
39. Transvenous
    
40. Transatrial
    
41. Transventricular
    
42. Ventricular
    
43. Epicardial
    
44. Endocardial
    
45. Transvenous
    
46. Transatrial
    
47. Transventricular
    
48. Pacemaker insertion
    

    Single chamber
    

    Dual chamber
    

    Rate responsive
    

    Antitachycardia
    

    
    
49. Defibrillator implantation
    

    Transvenous endocardial
    

    Epicardial
    

    
    
50. ASD closure
    

    Direct suture
    

    Patch
    

    
    
51. Pericardial
    
52. Dacron
    
53. PTFE
    
54. Heart transplant
    

    Orthotopic
    

    Heterotopic
    

    
    

Additional comments regarding therapeutics
An alternative to the above outline key method for coding treatment options for corrected transposition and related anomalies involves coding the primary procedure from a list of procedures including those specific for corrected transposition presented below in the Procedure Short Lists. Then, several other therapeutic issues must be addressed and coded in other areas of the database. First, separate procedure options must cover palliative treatment of Corrected Transposition (pulmonary artery banding and debanding). Second, a separate part of the database must allow for coding of incisions for this and all other diagnoses (median sternotomy, submammary incision, right thoracotomy, left thoracotomy, minimally invasive incisions [partial sternotomy, parasternal incision, "mini-thoracotomy"], etc). Third, a separate part of the database must allow for coding of cardiac incisions for this and all other diagnoses (aortotomy, pulmonary arteriotomy, right atriotomy, right ventriculotomy, left ventriculotomy, etc). Finally, a separate module of the database must permit coding of patch materials (Dacron, Gore-Tex, bovine pericardium, autologous pericardium, gluteraldehyde fixated autologous pericardium, etc).

IV. Diagnosis and procedure short lists

Diagnosis Short List

Congenitally corrected TGA

Cardiac, Other

Procedure Short List

Congenitally corrected TGA repair, atrial switch and ASO (double switch)

Congenitally corrected TGA repair, atrial switch and Rastelli

Congenitally corrected TGA repair, VSD closure

Congenitally corrected TGA repair, VSD closure and LV to PA conduit

Congenitally corrected TGA repair, other

V. Potential diagnostic-related risk factors

Analysis of outcome
The following continuous and discrete variables are considered appropriate for entry into a comprehensive database for patients with corrected transposition. The relevance of each item to a given anatomic subset or type of operation will vary. For purposes of analysis, each variable could be tested in the univariate domain for potential predictive value for a given outcome. The most relevant variables could then be entered into multivariate analysis, which would identify surrogates and complex interactions. Many of the variables could be used as either independent or dependent variables for a multivariate logistic regression analysis.

Preoperative variables
Noncardiac

1. Age
   
2. Body surface area
   
3. Prematurity (no. of days)
   
4. Racial origin
   
5. Gender (m/f)
   
6. Weight
   

Cardiac

1. Aortic regurgitation (yes/no)
   
2. ASD (yes/no)
   
3. Cardiac position in the mediastinum
   
4. Clinical heart failure (yes/no)
   
5. Coronary anatomy other than expected (yes/no)
   
6. Great vessel position (side by side/anterior-posterior/oblique)
   
7. Heart block (none/1st degree/2nd degree/3rd degree)
   
8. LV dysfunction (none/mildly impaired/moderately impaired/severely impaired)
   
9. LV hypoplasia (yes/no)
   
10. LVOTO (none/stenosis/atresia)
    
11. LV/RV pressure ratio
    
12. Mitral (right AV valve) insufficiency (none/trivial/mild/moderate/severe)
    
13. New York Heart Association (NYHA) functional class (1/2/3/4)
    
14. Other arrhythmias (yes/no)
    
15. Preoperative inotrope requirement (yes/no)
    
16. Previous palliative procedures
    

    Blalock shunt (none/right/left)
    

    Central shunt (none/right/left)
    

    
    
17. Aorta-LPA
    
18. Aorta-MPA
    
19. Aorta-RPA
    

    Modified Blalock shunt (none/right/left)
    

    Other shunt
    

    PA band (none/sternotomy/right thoracotomy/left thoracotomy/LV retraining)
    

    
    
20. Pulmonary artery distortion (yes/no)
    
21. Pulmonary pressure
    
22. Pulmonary regurgitation (yes/no)
    
23. Requirement for ventilation (yes/no)
    
24. RV hypoplasia (yes/no)
    
25. LV hypoplasia (yes/no)
    
26. Situs (solitus/inversus)
    
27. Straddling AV valve (none/mitral/tricuspid)
    
28. Tricuspid (right AV valve) insufficiency (none/trivial/mild/moderate/severe)
    
29. Ventricular position
    
30. VSD (yes/no)
    
31. WPW arrhythmia (yes/no)
    

Intraoperative variables

1. Atrial communication left open
   
2. Blood transfused post-cardiopulmonary bypass (CPB)
   
3. Circulatory arrest time
   
4. CPB time
   
5. Flow on CPB (L/min/m²)
   
6. Minimum intraoperative temperature
   
7. Myocardial ischemic time (cross-clamp time)
   
8. Number of operation (in the particular unit’s experience)
   
9. Operation performed (see codes for operations)
   

   Double switch
   

   Double switch and VSD closure
   

   Fontan operation
   

   Senning-Rastelli, Mustard-Rastelli
   

   VSD closure and direct relief of LVOTO
   

   VSD closure and LV-PA conduit
   

   
   
10. Pulmonary artery reconstruction (yes/no)
    
11. Repair of tricuspid valve
    
12. Recommencement of bypass after separation (yes/no)
    
13. Time from cessation of CPB to closure of chest
    
14. Type of cardioplegia (none, crystalloid, blood)
    
15. Use of LeCompte maneuver (yes/no)
    
16. Year of surgery
    
17. Operative mortality (yes/no)
    

Postoperative variables
Risk Factors for the Early Post-Operative Course

1. Atrial baffle obstruction (yes/no)
   
2. Atrial baffle leak (yes/no)
   
3. Complete Heart Block (CHB) (yes/no)
   
4. Chylothorax (yes/no)
   
5. Central nervous system injury (yes/no)
   
6. Conduit stenosis (yes/no)
   
7. Diaphragm paralysis (yes/no)
   
8. Excessive bleeding (none/medically managed/reexploration)
   
9. Myocardial ischemia (yes/no)
   
10. Fontan pathway obstruction
    
11. Hepatic impairment (yes/no)
    
12. Ventilated (days)
    
13. Intensive care unit stay (days)
    
14. Hospital stay (days)
    
15. Infection (none/mediastinum/pulmonary/urinary/septicaemia/endocarditis)
    
16. Left ventricular dysfunction (none/mild/moderate/severe)
    
17. Low cardiac output (yes/no)
    
18. Lung collapse/atelectasis (yes/no)
    
19. PA branch stenosis (yes/no)
    
20. Prolonged pleural effusion (>7 days) (yes/no)
    
21. Renal impairment (none/medically managed/peritoneal dialysis/hemofiltration)
    
22. Reoperation (yes/no)
    
23. Residual VSD (none/trivial/small/moderate/large)
    
24. Right ventricular dysfunction (none/mild/moderate/severe)
    
25. Left ventricular dysfunction (none/mild/moderate/severe)
    
26. Tachyarrhythmia (none/jet/supraventricular tachycardia/ventricular tachycardia)
    
27. Thrombo-embolic complications (Fontan pathway/pulmonary arteries/systemic ventricle)
    
28. Tricuspid (left AV valve) regurgitation (none/1/2/3/4+)
    
29. Early mortality (yes/no)
    

Late follow-up variables

1. Atrial arrhythmia (yes/no)
   
2. CHB (Late onset CHB) (yes/no)
   
3. Heart block (none, 1st degree, 2nd degree, 3rd degree)
   
4. Conduit obstruction (yes/no)
   
5. Functional class: NYHA (1/2/3/4)
   
6. LV dysfunction (yes/no)
   
7. Neoaortic valve regurgitation (yes/no)
   
8. PA branch stenosis (yes/no)
   
9. Protein losing enteropathy (yes/no)
   
10. Requirement for ACE inhibitor (yes/no)
    
11. Requirement for decongestive therapy (yes/no)
    
12. Residual VSD (yes/no)
    
13. RV dysfunction (yes/no)
    
14. LV dysfunction (yes/no)
    
15. Thromboembolic complications (yes/no)
    
16. Tricuspid valve (left AV valve) regurgitation (none/1/2/3/4+)
    
17. Endocarditis (yes/no)
    
18. Reoperation for any reason (yes/no)
    
19. Late mortality (yes/no)
    

Minimal dataset for corrected transposition and related anomalies

1. Age
   
2. Bypass time
   
3. Circulatory arrest time
   
4. Cross-clamp time
   
5. Heart block (none, 1st degree, 2nd degree, 3rd degree)
   
6. Hypoplastic ventricle: LV or RV
   
7. Late complications
   

   Atrial arrhythmias (yes/no)
   

   Atrial baffle obstruction (yes/no)
   

   AV valve regurgitation (yes/no)
   

   Endocarditis (yes/no)
   

   Late onset CHB (yes/no)
   

   Reoperation for any reason (yes/no)
   

   Ventricular dysfunction: LV or RV (yes/no)
   

   
   
8. Late Mortality (yes/no)
   
9. Length of stay (days)
   
10. LVOTO (none/stenosis/atresia)
    
11. Operative mortality (yes/no)
    
12. Postoperative complications
    

    CHB (yes/no)
    

    Myocardial ischemia (yes/no)
    

    Residual VSD (yes/no)
    

    
    
13. Previous palliative operation
    

    Pulmonary banding
    

    Shunt
    

    
    
14. Repair of tricuspid valve
    
15. Gender
    
16. Situs (solitus/inversus)
    
17. Straddling AV valve (yes/no)
    
18. Tricuspid regurgitation: Grading (none/1/2/3/4+)
    
19. Type of repair
    

    Double switch
    

    Double switch and VSD closure
    

    Fontan operation
    

    Senning-Rastelli, Mustard-Rastelli
    

    VSD closure and direct relief of LVOTO
    

    VSD closure and LV-PA conduit
    

    
    
20. VSD (yes/no)
    
21. Weight
    

References

1. Rokitansky CFV. Die Defecte der Scheidewande des Herzens. Vienna: Braumuller, W., 1875:81–6.
2. Cardell B.S. Corrected transposition of the great vessels. Br Heart J 1956;18:186-192.
3. Lev M., Rowlatt U.F. The pathologic anatomy of mixed levocardia. A review of thirteen cases of atrial or ventricular inversion with or without corrected transposition. Am J Cardiol 1961;8:216-263.
4. Schiebler G.L., Edwards J.E., Burchell H.B., DuShane J.W., Ongley P.A., Wood E.H. Congenitally corrected transposition of the great vessels. A study of 33 cases. Pediatrics 1961;27:851-888.[Abstract/Free Full Text]
5. Spitzer A., Lev M., Vass A. The architecture of normal and malformed hearts. Springfield, MA: Thomas, 1951.
6. Van Praagh R., Van Praagh S., Vlad P., Keith J.D. Anatomic types of congenital dextrocardia. Diagnostic and embryologic implications. Am J Cardiol 1964;13:510-531.
7. Shaher R.M. Complete and inverted transposition of the great vessels. Br Heart J 1964;26:51-66.
8. Van Praagh R., Vlad P., Keith J.D. Complete transposition of the great arteries. In: Keith J.D., Rowe R.D., Vlad P., eds. Heart disease in infancy and childhood, 2nd ed. New York: Macmillan, 1967:683-744.
9. Van Praagh R., Perez-Trevino C., Lopez-Cuellar M., et al. Transposition of the great arteries with posterior aorta, anterior pulmonary artery, subpulmonary conus and fibrous continuity between aortic and atrioventricular valves. Am J Cardiol 1971;28:621-631.[Medline]
10. Van Praagh R., Van Praagh S. Isolated ventricular inversion. A consideration of the morphogenesis, definition and diagnosis of nontransposed and transposed great arteries. Am J Cardiol 1966;17:395-406.[Medline]
11. Shinebourne E.A., Macartney F.J., Anderson R.H. Sequential chamber localisation. Br Heart J 1976;38:327-340.[Abstract/Free Full Text]
12. Allwork S.P., Bentall H.H., Becker A.E., et al. Congenitally corrected transposition of the great arteries. Am J Cardiol 1976;38:910-923.[Medline]
13. Lundstrom U., Bull C., Wyse R.K., Somerville J. The natural and "unnatural" history of congenitally corrected transposition. Am J Cardiol 1990;65:1222-1229.[Medline]
14. Van Praagh R. The segmental approach to diagnosis in congenital heart disease. The Fourth Conference on the Clinical Delineation of Birth Defects, Part XV. The cardiovascular system. Birth Defects 1972;8:4-23.
15. De La Cruz M.V., Amoedo M., Rivera F. Atrioventricular relations and their classifications. Two specimens of atrioventricular discordance and review of published reports. Br Heart J 1974;36:539-553.[Free Full Text]
16. Carr I., Tynan M., Aberdeen E.O., Bonham Carter R.E., Graham G., Waterston D.J. Predictive accuracy of the "loop-rule" in 109 children with classical complete transposition of the great arteries. Circulation 1968;38:52.
17. Lev M. Pathological diagnosis of positional variations in cardiac chambers in congenital heart disease. Lab Invest 1954;3:71-82.[Medline]
18. Harris J.S., Farber S. Transposition of the great cardiac vessels, with special reference to the phylogenetic theory of Spitzer. Arch Pathol 1939;28:427-502.
19. Daves M.L., Prior R. Cardiac positions, a primer. Am Heart J 1970;79:408-421.[Medline]
20. Grant R.P. The syndrome of dextroversion of the heart. Circulation 1958;18:25-36.[Medline]
21. Anderson R.H., Shinebourne E.A., Gerlis L.M. Criss-cross atrioventricular relationships producing paradoxical atrioventricular concordance or discordance. Circulation 1974;50:176-180.[Abstract/Free Full Text]
22. Sano T., Riesenfield T., Karl T.R., Wilkinson J.L. Intermediate-term outcome after intracardiac repair of associated cardiac defects in patients with atrioventricular and ventriculoarterial discordance. Circulation 1995;92:II272-II278.
23. Cochrane A.D., Karl T.R., Mee R.B.B. Arterial switch for late failure of the systemic right ventricle. Ann Thorac Surg 1993;56:854-861.[Abstract]
24. Karl T.R., Weintraub R.G., Brizard C.P., Cochrane A.D., Mee R.B.B. Senning plus arterial switch operation for discordant (congenitally corrected transposition). Ann Thorac Surg 1997;64:495-502.[Abstract/Free Full Text]
25. Ilbawi M.N., DeLeon S.Y., Backer C.L., et al. An alternative approach to the surgical management for physiologically corrected transposition with ventricular septal defect and pulmonary stenosis or atresia. J Thorac Cardiovasc Surg 1990;100:410-415.[Abstract]

This article has been cited by other articles:

				P. D. Maldjian Diagnostic Imaging Approach to Dextrocardia: Self-Assessment Module Am. J. Roentgenol., June 1, 2007; 188(6_Supplement): S35 - S38. [Abstract] [Full Text] [PDF]

				P. D. Maldjian and M. Saric Approach to Dextrocardia in Adults: Review Am. J. Roentgenol., June 1, 2007; 188(6_Supplement): S39 - S49. [Abstract] [Full Text] [PDF]

This Article Abstract Full Text (PDF) Online Discussion Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Wilkinson, J. L. Articles by Karl, T. R. Search for Related Content PubMed PubMed Citation Articles by Wilkinson, J. L. Articles by Karl, T. R.