Ann Thorac Surg 2003;75:422-429
© 2003 The Society of Thoracic Surgeons
Original article: cardiovascular
Sequential diagnosis of coronary arterial anatomy in congenitally corrected transposition of the great arteries
Ing-Sh Chiu, MD, PhDa*,
Shye-Jao Wu, MDa,
Shyh-Jye Chen, MDb,
Jou-Kou Wang, MD, PhDc,
Mei-Hwan Wu, MD, PhDc,
Hung-Chi Lue, MDc
a Department of Surgery, National Taiwan University Hospital, Taipei, Taiwan
b Department of Medical Imaging, National Taiwan University Hospital, Taipei, Taiwan
c Department of Pediatrics, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan
Accepted for publication July 22, 2002.
* Address reprint requests to Dr Chiu, Department of Surgery, National Taiwan University Hospital and National Taiwan University College of Medicine, No. 7 Chung-Shan S Rd, Taipei, Taiwan 100.
e-mail: ingsh{at}ha.mc.ntu.edu.tw
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Abstract
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BACKGROUND: The objective of this study was to analyze coronary arteries (CA) in congenitally corrected transposition (CCT) and to determine the influence of aortopulmonary rotation on its pattern systematically. Precise CA anatomy is surgically needed in the current era of double switch for CCT.
METHODS: We collected data on 62 patients who had CCT with situs solitus or inversus between 1981 and 1999. Coronary artery anatomy was analyzed as it related to apical position, atrial situs, ventricular looping, and aortopulmonary rotation. Five main types with similar variants of epicardial configuration at the base of the heart were categorized into five central patterns (patterns X, O, I, II, and IV).
RESULTS: The right CA coursed to the left in CCT with situs solitus, and to the right in CCT with situs inversus; and to the more posterior atrioventricular groove in both without apicocaval ipsilaterality. However, in CCT with more apicocaval ipsilaterality, the left circumflex might shift posterior to the right CA. With the same aortopulmonary rotation, the two groups had similar central patterns, and eta-square analysis showed that the evolution from patterns X, O, I, II, toward IV (n = 1, 36, 15, 9 to 1) was dependent on clockwise aortopulmonary rotation (p < 0.00000).
CONCLUSIONS: Peripheral CA pattern in the atrioventricular groove was dictated by apicocaval ipsilaterality anteroposteriorly and ventricular looping dextrosinistrally, irrespective of atrial situs. The central CA pattern near the aortic sinus depended on aortopulmonary rotation due to "marriage of convenience" between them, and thus was predictable from arterial relations irrespective of its disease category.
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Introduction
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Precise coronary artery (CA) anatomy in congenitally corrected transposition (CCT) is less well elucidated than that in complete transposition of the great arteries (TGA) [1, 2]. This information is surgically demanded, particularly for combined atrial and arterial switch operations [3, 4]. Previously, we reported that the CA pattern is related to the aortopulmonary rotation (APR) in TGA [2, 5], Fallot’s tetralogy [6] (Fig 1),
and persistent truncus arteriosus [7]. In this study we examined the influence of APR on the CA anatomy in CCT, which was frequently associated with situs inversus or dextrocardia. Sequential segmental analysis of such a complex congenital defect has been well advocated, but using this approach to analyze the CA in the complex heart has never been reported. We found that it was indispensable to analyze the peripheral CA pattern according to its apical position, atrial situs and ventricular looping, sequentially at first before reaching the conclusion of the influence of APR on the central CA pattern. Even more interestingly, this study confirmed that CCT occupied a particular portionof horizontal circle of short–axis APR (Fig 1), thus the gap between type 0 and type 10 of the six basic CA types were bridged together. It was not necessary to designate more numerical nomenclatures, such as type 1 or type 11, as we had planned before this study. Therefore, if one CA entering one facing sinus and the other two CAs entering the other, randomly speaking there would be only six possibilities for the basic arrangement of CA (not including the single CA and its variant), because only three main CA exist and usually they penetrate either of the aortic sinus facing the pulmonary trunk. Herein we described our findings.
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Fig 1. Six basic coronary patterns are related to the horizontal circle of the aortopulmonary rotation. Each pattern (O, I, II, IV, IX, X) included a main type (0, 1, 2, 4, 9, 10) and its similar subtypes at the base of the heart; only main types were depicted here. Note the two main types facing each other are mirror images. Patterns O, I, II, IV to IX were reported in complete transposition (TGA). Patterns II, IV, IX to X in tetralogy of Fallot (TF) and patterns X, O, I, II to IV were described in congenitally corrected transposition (CCT) in this article as bridging the gap between pattern X and pattern O.
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Material and methods
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Study patients
Data from all patients who presented between 1981 and 1999 with CCT with a documented CA type and its relation to the great arteries were collected for analysis. Only patients with atrioventricular discordance and ventriculoarterial discordance (with or without pulmonary stenosis) or single outlet of the heart (pulmonary atresia) were included, but patients with double outlet right ventricle were excluded. The CA type and aortopulmonary relation were identified by angiography, electron beam computed tomographic scan, or during surgical intervention. The degree of APR of the heart was determined by the rotation of the aortic sinus (Fig 2),
as was previously reported [5, 6, 8]; this was especially useful in the hearts of patients with pulmonary atresia without a well-formed pulmonary trunk. Computed tomographic scan was used to define left–right and ventrodorsal relationship of the CA in the atrioventricular groove. All univentricular hearts and those with isomeric atrial situs were excluded. In each patient, the ventricular septum was either intact or with a defect. The patients were divided into two groups according to the atrial arrangement into situs solitus (CCTsol) or inversus (CCTinv). The position of the cardiac apex, atrial situs, and direction of ventricular looping were also recorded in each patient, and correlated with the peripheral CA pattern in the anterior, posterior, or lateral atrioventricular groove. The central CA pattern was defined as the pattern near the base of the heart.
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Fig 2. Varied aortopulmonary rotation on the horizontal plane (inner circle) results in different patterns of aortic sinus of Valsalva at its lateral projection (outer circle). From the directly anterior 0 degree, the left-hand sinus moves from a posterior position toward an anterior location on leftward aortopulmonary rotation, whereas the right-hand sinus moves in a rightward rotation. After 60 degrees in both directions, the nonfacing sinus moves posteriorly at the lateral projection. In cases of leftward rotation up to 120 degrees, the posterior sinus is the right-hand sinus, whereas it is the left–hand sinus in the other direction. (DA = directly anterior; DP = directly posterior; LA = left anterior; LH = left-hand facing sinus; LP = left posterior; N = nonfacing sinus; PT = pulmonary trunk; RA = right anterior; RH = right-hand facing sinus; RP = right posterior.)
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Central pattern
The previously reported CA types [9–16] in CCT were reorganized into five patterns (X, O, I, II, and IV) according to similarities of epicardial configuration at the base of the heart (Fig 3).
Pattern X, O, I, II, or IV included main type 10, 0, 1, 2, or 4 with their similar subtypes, respectively. The original classification of Shaher and Puddu [17] is not numerically randomized [2]. They named single CA as types 3, 5, and 7 and unusual morphologic left circumflex (LCX) as types 2, 4, 6, and 8 in between the usual type of TGA (type1) and normal heart (type 9). Among them, types 3, 5, 7, 6, and 8 have been grouped as subtypes to their similar main types (Table 1)
[2, 5, 6, 17, 18], therefore there were no patterns III, V, VI, VII, and VIII in our categorization. Pattern X had a retropulmonic LCX originating from the right-hand sinus with the morphologic right CA (RCA). Pattern O was defined as the original pattern in our previous article with TGA [2]. Type 0 (BA-1 of the de Groot classification) [19] was named one number before Shaher type 1 because it was found if the aorta was just more left anterior to the pulmonary trunk than that of type 1 [2, 20]. Type 10 was named one number after Shaher type 9 because its aorta was located just more right posterior than that of type 9 [6, 21]. Type 9 was not documented in CCT. Type 10, previously reported in the otherwise normal heart [21, 22] and tetralogy of Fallot [6, 23], was also documented in CCT [11]. A picture was taken in one heart of CCTsol with type 10 CA from Brompton Hospital, London.
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Fig 3. Coronary artery types are reorganized into five patterns (X, O, I, II, and IV) according to the similarity of their epicardial configuration at the base of the heart. Pattern O is the original pattern and includes type 0 and subtype 3c. Patterns I, II, and IV include main types 1, 2, and 4 of Shaher’s classification; subtype 3d belongs to pattern I and 3a to pattern II. Pattern X includes type 10 and subtype 10v. In hearts with levolooping of the ventricles, the morphologic right coronary artery (R) courses to the left lateral atrioventricular groove, instead of the morphologic left circumflex artery (C) as in dextrolooped hearts. Types from published reports other than the present study without specified aortopulmonary rotation are marked by a question mark in the center of the pulmonary trunk. (A = anterior descending artery; CCTinv = congenitally corrected transposition with situs inversus; CCTsol = CCT with situs solitus; TGAinv = complete transposition with situs inversus; TGAsol = TGA with situs solitus.)
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On the basis of similar epicardial configurations at the base of the heart, subtypes similar to the main type included type 3c and type 10v [6] and were grouped together with their main type 0 and type 10, respectively, to become patterns O and X, respectively. Type 3a was transitional between type 1 and type 4 and classified as a variant of type 2 in pattern II (Fig 3), whereas type 3d was transitional between type 0 and type 2, as was type 1 and grouped to pattern I (the details were depicted on Fig 3 in Ref [2].). The degree of APR of each subtype was checked whether it was similar to its main type, thus to categorize them into the same pattern can be justified. Previously in TGA, Shaher and Puddu [17] reported all the above subtypes, except for type 10v, which was reported by Uemura and colleagues [11] in CCT.
Our previous classification of CA types in TGA with either situs solitus (TGAsol) [2] or inversus (TGAinv) [5] was put together for convenience of comparison (Fig 3). The CA that coursed to the lateral atrioventricular groove in CCTinv and TGAsol (left of the dashed line) is reversed from that in CCTsol and TGAinv (right of the dashed line), which were grouped together as the same central pattern when the central epicardial configurations at the base of the heart were similar (in the same row in Fig 3). Inasmuch, the reversed peripheral patterns in the anterior or posterior atrioventricular groove, which were not depicted in Figure 3, were regarded as the same central pattern when the central epicardial configurations are similar.
Previous reports of documented CA type and arterial relations were reviewed. The 
2, Fisher’s exact test, and eta-square (correlation ratio) analyses were used to test statistical significance.
Terminology
The following terms are used throughout: right- or left-hand sinus is the sinus on either the right- or left-hand side if one is positioned according to the nonfacing cusp of the aortic valve [19]. Nonfacing sinus is the sinus other than the previous two. Apicocaval ipsilaterality means the discordance of apical position and atrial situs, either dextrocardia with situs solitus or levocardia with situs inversus.
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Results
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Sixty-two patients who met the selection criteria were identified (Table 2).
Mean age at the time of admission was 5 years and 4 months (range, 4 days to 46 years) and 41 were male and 21 were female patients. Situs solitus was found in 41 patients, whereas situs inversus appeared in 21 patients. Ventricular septal defect was seen in 59 patients except three, who had intact ventricular septum. Ventriculoarterial discordance was noted in 37 patients, and pulmonary atresia 25 patients.
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Table 2. Coronary Artery Patterns and Types in Relation to the Aortopulmonary Rotation in 62 Patients With Congenitally Corrected Transposition
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Peripheral pattern
Impact of atrial situs and apical position
In CCT without apicocaval ipsilaterality (concordance of apical position and atrial situs), irrespective of its situs, the CA in the more posterior atrioventricular groove is always the RCA (CCTsol, n = 25, Fig 4A;
CCTinv, n = 8, Fig 4D), as also shown in Figures 5F and 6F.
The LCX shifts posteriorly in patients with apicocaval ipsilaterality (Fig 4 B,C,E, and F). The degree of posterior shifting of the LCX toward the RCA can indicate the index of apicocaval ipsilaterality. In those patients with less rotation of the cardiac apex (Fig 4B and E), the LCX is still located anterior to the RCA or at the same level (CCTsol, n = 8; CCTinv, n = 6). However, in extreme cases (Figs 4C, F, 5E, and 6E), the LCX is shifted even posterior to the slightly anteriorly shifted RCA (CCTsol, n = 8; CCTinv, n = 7), resulting in a similar anteroposterior atrioventricular groove arterial pattern as in TGA (Figs 5D and 6D).
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Fig 4. Electron beam computed tomogram of congenitally corrected transposition with situs solitus (A–C) and inversus (D–F). From the left column (A and D) to the right column (C and F), the degree of apical rotation and apicocaval ipsilaterality increases gradually. The ventricular mass was almost equal in the middle column (B and E, mesocardia), whereas it is more to the left in A and F (levocardia) and more to the right in C and D (dextrocardia). The left circumflex artery (C) was in front of the posteriorly located right coronary artery (R) in the hearts without apicocaval ipsilaterality (A and D), whereas it is almost at the same level anteroposteriorly in the hearts with mesocardia (B and E), and the left circumflex artery is located posterior to the right coronary artery in the hearts with extreme apicocaval ipsilaterality (C and F). The degree of posterior shifting of the left circumflex artery toward anterior shifting right coronary artery can indicate the index of apicocaval ipsilaterality. (LA = left atrium; LV = left ventricle; RA = right atrium; RV = right ventricle.)
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Fig 5. Type 1 coronary artery, usually seen in complete transposition of the great arteries with situs solitus (A and D), is also seen in congenitally corrected transposition with situs inversus and apicocaval ipsilaterality (B and E). The upper row is the frontal projection, and the lower row is the lateral projection. In congenitally corrected transposition without apicocaval ipsilaterality (C and F) the right coronary artery (R) is behind the left circumflex artery (C) (F). Note the right-hand sinus is located anteriosly to the left–hand sinus in hearts with a right anterior aorta (F). (A = anteriorly descending artery; LH = left-hand sinus; N = nonfacing sinus; RH = right-hand sinus.)
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Fig 6. Type 0 coronary artery usually seen in congenitally corrected transposition with situs solitus and apicocaval ipsilaterality (B and E) is also seen in complete transposition of the great arteries with situs inversus (A and D). The upper row is the frontal projection, and the lower row is the lateral projection. The right coronary artery (R) is behind the left circumflex artery (C) in hearts without apicocaval ipsilaterality (F). Otherwise, in the frontal projection (A to C), all are similar. Note in F with a left anterior aorta, the left-hand sinus is located anteriorly in the lateral projection. (A = anteriorly descending artery; LH = left-hand sinus; N = nonfacing sinus; RH = right-hand sinus.)
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Impact of ventricular looping
Ventricular looping determines the peripheral pattern dextrosinistrally.
In CCTinv with dextrolooping of the ventricle, the RCA is right-sided (n = 21) (Fig 3, second column from the left), as shown in upper panel of Figure 5.
The left-sided atrioventricular groove artery in CCTsol is the RCA (n = 41) (Fig 3, first column from the right), as shown in upper panel of Figure 6.
Central pattern
Impact of APR and aortic root position
The CA pattern near the aortic sinus depends on the APR irrespective of TGA, CCT, or their atrial situs. From the same APR one can recall a similar CA pattern near the aortic sinus in CCT as that in TGA, either with the same ventricular looping (left or right to the dashed line in Fig 3), or reversed ventricular looping (the same row in Fig 3). The number of patients in each type is listed in Table 2. Type 3c and 3a had a similar degree of APR to type 0 and 2, respectively, in all patients, and were categorized to pattern O and II, respectively. The evolution of central CA patterns from X, O, I, II, toward IV (n = 1, 36, 15, 9 to 1) was dependent on clockwise APR (Table 2, eta-square 0.875, p < 0.00000). The APR of pattern X is not specified in the previous report [11]. However, a heart with type 10 CA from the Brompton Hospital had its aortic valve to the left of the pulmonary trunk, at a slightly more posterior location than side-by-side (Fig 7).
Table 3
shows the statistical results of the five main types. Because the aorta is rotated from a left posterior toward the left anterior position relative to the pulmonary trunk, the LCX moved from a retropulmonic location (type 10, 1 patient decreased to no patient; Fig 3) to an antepulmonic location to join with the left anterior descending to enter the left-hand sinus (type 0, 0 patient increased to 31 patients; p < 0.006). As the aorta rotated farther clockwise from a left anterior toward directly anterior position, the left anterior descending shifted to the left to have the same entry as RCA into the right-hand sinus (type 1, 3 cases increased to 12; type 0, 31 cases decreased to no cases; p < 0.00001). When the aorta rotated farther from directly anterior to right anterior less than 60 degrees (type 1, 12 patients decreased to no patient), the LCX tended to move retropulmonically and pierced into the left-hand sinus with the RCA (type 2, 1 patient increased to 4 patients; p < 0.002). When the aorta moved from right anterior to right lateral, the RCA shifted to the right-hand sinus anteaortically to join the left anterior descending (type 4, no patient increased to 1 patient; p = 0.833).
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Fig 7. A heart specimen of a congenitally corrected transposition showing that the aorta was to the left posterior of the pulmonary trunk. The aortic valve was located lower than the pulmonary valve. The left anterior descending (A) entered the anterior left-hand sinus. The inset, which was taken after ventroversion of the great arteries, showed the right-sided retropulmonic left circumflex (C) and the left-sided morphologic right coronary artery (R) pierced into the posterior right-hand sinus. The location of lower aortic valve and unusual ventricular septal defect was reminiscent of that in the so-called posterior complete transposition. (Ao = aorta; LA = left atrium; lpa = left pulmonary artery; LV = left ventricle; PDA = patent ductus arteriosus; PT = pulmonary trunk; RA = right atrium; rpa = right pulmonary artery; RV = right ventricle.)
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The dextroposed aorta is shown in Figure 3 (lower five rows) and Figure 8A and C.
With similar APR, the usual pattern of central CA (type 1) in TGAsol (Fig 5A and D) can be anticipated in CCTinv (Fig. 5B,E,C, and F). Type 2 of pattern II CA in TGAsol can also be seen in CCTinv with a more right anterior aorta (Fig. 8A and C). Type 4 of pattern IV in CCTinv has been reported [9] and seen in this series. The aortic root may be right anterior to the pulmonary trunk in CCTsol [9, 11, 12]. For a right anterior aorta in CCTsol, type 3d and 3a CA are reported [11–12], types 1 and 3a are seen in this series (Table 2).
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Fig 8. Type 2 coronary artery in patients with more right anterior aorta of a congenitally corrected transposition with situs inversus (A and C); the anterior aortic sinus is the right-hand sinus (for illustrations, see Fig 3). Type 3c with situs solitus and an almost left lateral aorta (B and D); the anterior sinus is the left-hand sinus. The upper panels are the frontal projection, and the lower panels are the lateral projection. (A = anteriorly descending artery; LH = left-hand sinus; N = nonfacing sinus; RH = right-hand sinus.)
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The levoposed aorta is shown in Figure 3 (upper four rows) and Figure 8B and D. With the same APR, the frequently seen type 0 in TGAinv (Fig 6A and D) [5] can be recalled in CCTsol (Figs. 6B,E,C, and F). Type 3c seen in TGAsol with a left anterior aorta [2] can be seen in CCTsol with a left anterior aorta (Fig 8B and D). In 1 patient with CCTsol, a small infundibular branch entering the right hand sinus is noted in addition to the type 3c pattern as reported previously [11].
Summary
Overall speaking, one could recall a similar CA pattern, both peripheral and central, in CCT as in TGA with the same apical position, ventricular looping (CCTsol with dextrocardia as in TGAinv, CCTinv with levocardia as in TGAsol) and the same APR. It was worth reminding that both of these CCT patients had extreme apicocaval ipsilaterality (Figs 4C,F, 5B,E, and 6B,E). However, in CCT with less or without apicocaval ipsilaterality (CCTsol with meso- or levocardia, CCTinv with meso- or dextrocardia) (Figs 4A,B,D,E, 5C,F, and 6C,F) as seen in most patients (33 of 41 = 80% in CCTsol; 14 of 21 = 67% in CCTinv), the central CA pattern was still similar to TGA with the same APR, but the more posterior atrioventricular groove artery was the RCA instead of the LCX, and the lateral atrioventricular groove artery was dictated by ventricular looping.
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Comment
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The embryogenesis of the main CA originated from the formation of three vascular circles sequentially, that is, the atrioventricular circle, the interventricular circle, and the conotruncal circle [24]. The first two circles decided the peripheral pattern, and the third, the central pattern. Therefore, it is helpful to interpret the CA pattern in sequence, mostly on such a complex heart like CCT (nonlevocardia in 30 of 62 patients = 48% of this series). Herein we showed the RCA coursed to the posterior atrioventricular groove in CCT less with or without apicocaval ipsilaterality, because its ventricle connected with the left atrium, which was more posteriorly located than the right atrium. Second, the interventricular artery between the two ventricles was formed along with the ventricular looping; it was the loop rule that determined the ventricle and hence its appropriate CA in the lateral atrioventricular groove. Third, the APR dictated the types of CA in CCT as in TGA, because during embryogenesis CA pierced the nearest site of the aortic sinus after APR [2, 25, 26] (ie, marriage of convenience between the aortic sinus and the CA). By this systemic method, we were able to diagnose the CA anatomy in CCT in a segmental sequence. The similar CA type, both central and peripheral, could be anticipated in face of the same apical position, ventricular looping, and APR by sequential segmental analysis.
The exact preoperative interpretation and accurate intraoperative identification of the CA pattern is indispensable for a successful coronary transfer in the arterial switch operation. The LCX that courses through the right atrioventricular groove in CCTsol is frequently imagined to reach the most posterior surface of the heart [10, 16], especially in view of the subaortic right ventricle, which is usually leftward and anteriorly located in CCT according to the loop rule. However, this is true only in relatively rare cases of CCT with extreme apicocaval ipsilaterality (15 of 62 patients = 24%). The surgical implications of apicocaval ipsilaterality are discussed elsewhere [3, 4, 27]. Herein we also show the degree of posterior shifting of the LCX toward the RCA, which could indicate the index of apicocaval ipsilaterality and this is of great surgical implication during the double switch operation.
Although the numerical system of classification is not descriptive, and seems unfriendly and unlimitedly expandable at its first glance, type 0 (pattern O) and type 10 (pattern X) were now bridged together in the circle shown in Figure 1. Thus, it is not necessary to designate more numerical nomenclatures than 10 (type 11) or less than 0 (type -1), other than these six basic types. All so-called single CA can be categorized with one of these six basic main types to become six coronary artery patterns, as shown in Figure 3. It is time to unify and simplify the complex CA classification of all the normal and abnormal hearts according to the APR, as we have reported [2, 5–7].
In conclusion, apicocaval ipsilaterality dictates the peripheral atrioventricular groove artery anteroposteriorly, ventricular looping dextrosinistrally, whereas the APR determines the central CA pattern irrespective of the atrial situs or disease category. One might anticipate the CA types based on APR or aortic sinus pattern in CCT.
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Acknowledgments
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This study was supported with a grant from the National Science Council (NSC88–2314-B002–321). We are indebted to Chang-Ying Lin and Ju-Hsiu Cheng for secretarial assistance. We are grateful to Dr Yen Ho for permitting us to look at the heart specimens and take pictures at the Imperial College School of Medicine, National Heart & Lung Institute, Royal Brompton Hospital, London, UK.
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Abstract
Introduction
