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Ann Thorac Surg 1999;67:1194-1202
© 1999 The Society of Thoracic Surgeons

Current Review

Congenital obstructive lesions of the right aortic arch

Doff B. McElhinney, MDa, Wayne Tworetzky, MDa, Frank L. Hanley, MDa, Abraham M. Rudolph, MDa

a Divisions of Cardiothoracic Surgery and Pediatric Cardiology, University of California, San Francisco, San Francisco, California, USA

Address reprint requests to Dr McElhinney, Children’s Hospital of Philadelphia, 34th St and Civic Center Blvd, Philadelphia, PA 19104


   Abstract
Top
 Abstract
Introduction
 Right-sided aortic arch
 Congenital obstructive lesions...
 Clinical implications
 References
 
Background. In the setting of normal cardiac situs, a right-sided aortic arch is uncommon. When a right arch does occur, it is typically in conjunction with other congenital cardiovascular anomalies, especially defects with abnormal right ventricular outflow. Congenital obstruction of a right arch, caused by coarctation, interruption, or cervical arch, is extremely rare.

Methods. We reviewed our experience and all reported cases of right aortic arch with coarctation of the aorta, interrupted arch, or obstruction of a cervical arch in the setting of normal cardiac situs and topology.

Results. Since 1992, 4 such patients have undergone repair at our institution, including 1 with interrupted arch, 1 with coarctation of a mirror image arch, and 2 with obstruction of a cervical arch. In addition to these 4 patients, 38 others have been described in the published reports: 15 with interrupted arch, 19 with coarctation, and 4 with obstruction of a cervical arch. Associated cardiac defects were uncommon, except for ventricular septal defect in patients with interrupted arch, but abnormalities of the brachiocephalic vessels were frequent. Except for most of the patients with interrupted right arch, the majority of patients described have undergone successful surgical repair.

Conclusions. Although obstructive arch lesions are often grouped together, the etiologies of coarctation of the aorta, interrupted arch, and cervical arch with obstruction almost certainly differ. The rarity of such lesions among patients with right aortic arch may be explained in part by the fact that the fetal hemodynamic conditions associated with persistence of a right arch do not facilitate flow-related arch obstruction. In this review, we discuss these issues in detail, along with specific surgical considerations in the management of obstruction lesions of the right aortic arch.


   Introduction
Top
 Abstract
 Introduction
Right-sided aortic arch
 Congenital obstructive lesions...
 Clinical implications
 References
 
A right aortic arch is uncommon in the setting of normal cardiac situs and almost always occurs in conjunction with other congenital cardiovascular anomalies. There have been only a handful of reports of surgical repair for congenital obstruction of a right arch. Although an obstructed right arch is unusual, it is nevertheless important to be familiar with the patterns of aortic arch branching and associated anomalies, which may have implications for diagnosis and repair. Over the past 6 years, 4 patients with situs solitus and obstruction of a single right aortic arch have undergone repair at our institution. In this review, we describe these patients, the collective reported experience, and pathogenetic and surgical considerations regarding obstructive lesions of the right aortic arch.


   Right-sided aortic arch
Top
 Abstract
 Introduction
 Right-sided aortic arch
Congenital obstructive lesions...
 Clinical implications
 References
 
Anatomic patterns
In autopsy and radiographic studies, a right aortic arch has been found in approximately 0.1% of cases [1–3]. Almost invariably, a right arch is accompanied by congenital anomalies of the cardiovascular system. The most commonly associated cardiac lesions are tetralogy of Fallot, pulmonary atresia with ventricular septal defect, and truncus arteriosus. In addition, brachiocephalic branching anomalies and other abnormalities of the central great arteries are relatively more common with a right aortic arch than with the usual left arch. Although a variety of brachiocephalic vascular arrangements are seen in patients with a right aortic arch, there are several basic patterns that are most characteristic [2, 4]. One of these is mirror image origin of the brachiocephalic vessels from the arch, with the left innominate artery originating as the most proximal branch of the arch, followed by the right common carotid and right subclavian arteries in succession. In this pattern, the ductus arteriosus is usually left-sided and runs from the left innominate artery to the left pulmonary artery. Less often, the ductus may be right-sided, running from the underside of the arch to the right pulmonary artery, completing a truly mirror image arch. In general, a mirror image right arch occurs with congenital heart defects, such as tetralogy of Fallot, pulmonary atresia with ventricular septal defect, and truncus arteriosus [2]. In the other typical branching pattern, the first branch of the right arch is the left common carotid artery, followed by the right common carotid and subclavian arteries, then an aberrant left subclavian artery from the descending aorta. This pattern is less frequently accompanied by intracardiac defects than is mirror image branching, and tetralogy of Fallot is the most common associated lesion when structural heart disease is present. In the absence of cardiac disease, this type of arch tends to come to clinical attention when the aberrant left subclavian artery passes behind the esophagus and gives off a left-sided ductus to the left pulmonary artery, producing a vascular ring that may cause airway or esophageal symptoms. Other important, albeit uncommon, variations of right arch are a circumflex arch and a cervical arch, which can be found with a wide range of brachiocephalic branching patterns [2, 4, 5].

In the setting of a right aortic arch, ductal anatomy is often unusual as well. As mentioned earlier, a single ductus may arise as a left-sided or a right-sided structure from either of several brachiocephalic vessels. The ductus may also be absent or bilateral. There is generally no ductus in truncus arteriosus (except in cases with interrupted arch) or in tetralogy of Fallot with absent pulmonary valve, and a ductus may be absent with other forms of right arch as well [6–8]. Bilateral ductus in the setting of a right arch may occur with a variety of branching patterns of the central great arteries, including discontinuous pulmonary arteries and isolation of the left subclavian artery [9]. In isolation of the left subclavian artery, the left subclavian artery arises from the left pulmonary artery through a left ductus and, like bilateral ductus, is associated with a right arch more often than with a left arch [10]. The development of the variable branching patterns seen with an unobstructed right arch can be appreciated by relating these patterns to the hypothetic embryonic arch system (Fig 1).



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  		Fig 1. Schematic diagrams based on the hypothetic embryonic arch system showing differences in the development of normal great vessels (left) and three patterns of right aortic arch (RAA). The top row demonstrates the primitive arch system, with left (L) and right (R) third (III), fourth (VI), and sixth (VI) pharyngeal arches, distal pulmonary arteries (PA), seventh intersegmental arteries (VII), and dorsal aortas (DAo). Dark areas represent the segments that regress in the development of the corresponding mature arch anatomy, shown in the bottom row for each case. In normal development, the third pharyngeal arches contribute to the common carotid (CCA) and external carotid arteries; the fourth arches develop into the distal arch on the left (between the left common carotid and subclavian arteries) and the innominate artery on the right; the sixth arches contribute to the proximal branch pulmonary arteries and the ductus arteriosus (PDA); the seventh intersegmental arteries develop into the subclavian arteries (SCA); and the left dorsal aorta becomes the descending aorta, whereas the right dorsal aorta regresses. Three patterns of right arch branching are depicted in the diagrams to the right of the diagram of normal anatomy.

 
Etiologic considerations
In subjects with right aortic arch, there is a high prevalence of chromosome 22q11 microdeletions and the congenital anomaly syndromes that are now known to be associated with this genetic defect, such as the DiGeorge, velocardiofacial, and conotruncal anomaly face syndromes [11, 12]. The pathogenetic nature of the association between right aortic arch and del22q11 is not completely understood. Deletions of 22q11 are thought to affect neural crest cell migration or development and as a result to interfere with the formation of two specific components of the central cardiovascular system: the conotruncus and the embryonic pharyngeal arches, notably the fourth arch [13]. Although del22q11 most likely contributes significantly to persistence of the right arch in many cases, it is not the only factor. Rather, the association is likely to depend on both genetic and physiologic effects on morphogenesis.

In addition to the probable role of del22q11 in primary morphogenetic abnormalities, right arch predominance may be facilitated by flow-related factors in the developing embryo and fetus. The hydrodynamic conditions present in the normally developing conotruncus favor laminar flow of right ventricular output through the left ductus arteriosus and into the left descending aorta, which leads to predominance of the left arch [14]. With decreased pulmonary outflow in the developing heart, right-to-left ductal flow is absent or diminished, so the flow-related impetus for persistence of the left ductus and dorsal aorta is not present, allowing for a more stochastic distribution of right and left arch predominance. Although this process has not been proved, there are several empiric observations that support this contention. Lesions with right ventricular outflow obstruction (tetralogy, pulmonary atresia, and truncus arteriosus) are frequently accompanied by a right arch regardless of whether there are deletions of chromosome 22q11. In contrast, such lesions may be found with del22q11 and a normal left arch as well. In the setting of conotruncal defects with right ventricular outflow obstruction, the pathogenesis of right arch persistence can be difficult to sort out because conotruncal defects have a significant association with both del22q11 and right ventricular outflow obstruction, which means that the two factors most likely to cause right arch predominance often coexist, with neither clearly predominant. Additional support for the belief that del22q11 alone is probably not responsible for persistence of a right arch can be found in the fact that interrupted aortic arch, which is highly associated with deletions of chromosome 22q11, is rarely found with a right aortic arch. Unlike other lesions associated with del22q11, interrupted arch does not occur with right ventricular outflow obstruction, so the rarity of a right arch in this condition supports a role for the hydrodynamic factors discussed earlier. When interrupted arch does occur with a right-sided arch, however, it is almost invariably associated with del22q11. Ultimately, the role of chromosome 22q11 deletions in promoting right arch predominance will require a more in-depth understanding of the developmental perturbations it engenders.


   Congenital obstructive lesions of the right aortic arch
Top
 Abstract
 Introduction
 Right-sided aortic arch
 Congenital obstructive lesions...
Clinical implications
 References
 
University of California, San Francisco experience
Patients
Since 1992, 4 patients have undergone repair of congenital obstructive lesions of a right aortic arch associated with situs solitus and normal cardiac topology at the University of California, San Francisco. One patient had interrupted arch, 1 had coarctation with mirror image arch branching, and 2 had cervical arch with coarctation. The nature and location of obstruction in each patient is summarized in Table 1. Diagnosis was by echocardiography without catheterization in 2 patients; angiography was performed in 2 patients and magnetic resonance imaging in 1. Ages ranged from 7 days to 3 years. One patient had undergone previous repair of coarctation of a cervical right arch at another institution and presented with recurrent coarctation as well as vascular compression of the right mainstem bronchus. All patients had associated abnormalities of the great arteries, including aberrant left subclavian artery (n = 2), isolation of the left subclavian artery (n = 1), transverse arch aneurysm associated with a cervical arch (n = 1), and malposition of the great arteries with the main pulmonary artery anterior and to the left of the aorta (n = 1). Intracardiac defects were present in 2 patients, both of whom had a ventricular septal defect, associated with either interrupted arch (n = 1) or coarctation of the aorta (n = 1). Patient data are summarized in Table 1.


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  		Table 1. University of California, San Francisco Experience With Obstructive Lesions of Right Aortic Arch (1992 to 1998)a

 
Results
All 4 patients underwent repair using standard techniques. Repair was through a median sternotomy in 1 patient and a right lateral thoracotomy in 3. There were no early deaths. All patients are alive and well at a follow-up period ranging from 4 months to 5 years. No patient has required reoperation.

Review of published reports
Coarctation of the aorta with right aortic arch
Before the present report, 19 patients with coarctation of the aorta with right aortic arch have already been described in published reports, not including patients with coarctation of a right cervical arch (Table 2) [1, 2, 15–30]. All but 1 patient has undergone surgical repair. Including our patient, 12 of 20 patients had aberrant origin of the left subclavian artery from the descending aorta, with a left ductus or ductal remnant connecting to the left pulmonary artery in at least 4 (not specified in the others). One patient had isolation of the left subclavian artery, and 1 had isolation of the left innominate artery. Two patients had direct origin of the right vertebral artery from the arch. Only 6 patients had a right arch of the mirror image type [16, 19, 22]. In at least 3 patients with coarctation of a right-sided mirror image arch (the laterality of the ductus was not stated in the other 2), the coarctation was juxtaductal (to the right ductus). In contrast, in patients with coarctation of a right arch and aberrant origin of the left subclavian artery, the left ductus ran from the aberrant left subclavian artery to the left pulmonary artery, and thus the aortic coarctation could not possibly be juxtaductal. Among these 20 patients, there have been 6 with associated congenital heart defects, including ventricular septal defect (n = 4), transposition of the great arteries (n = 1), and anomalous origin of the circumflex coronary artery from the pulmonary artery (n = 1). There have also been at least 4 patients with coarctation of a right arch (cervical arch in 1) and cutaneous hemangiomas [17, 23, 25, 31]. The association of coarctation with cutaneous hemangiomas has been recognized in patients with a left arch as well [32] but appears to be stronger in conjunction with a right arch.


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  		Table 2. Reported Cases of Right Aortic Arch With Coarctation of the Aortaa

 
According to the principle of flow-related development of the central great vessels [14], the rarity of coarctation with a mirror image right arch is to be expected. In the normal fetus, descending aortic flow is supplied through both left ventricular outflow, via the ascending aorta and arch, and right ventricular outflow across the left ductus. Coarctation, according to this theory, is most likely to develop in circumstances of relatively increased right-to-left ductal flow and decreased flow across the aortic arch and isthmus. However, a mirror image right arch is typically associated with congenital heart defects that involve decreased or absent right-sided outflow, as discussed earlier. Reduced flow through a pulmonary trunk is associated with diminished or reversed ductal flow and increased antegrade flow across the isthmus through the ascending aorta and arch. Conversely, ascending aortic and arch flow is increased relative to the situation in the normal fetus, and a greater portion of descending aortic flow traverses the aortic arch and isthmus, which makes isthmic hypoplasia or coarctation extremely unlikely to develop. In fact, there is a tendency toward "coarctation" of the left pulmonary artery rather than the aorta in patients with tetralogy or pulmonary atresia and a normal left arch, probably reflecting flow-mediated effects in the context of reversed ductal shunting [33]. In cases of right arch with mirror image branching and no pulmonary outflow obstruction, the ductus most often originates from the left innominate artery rather than the isthmus, and hence flow patterns are not as well suited for the development of aortic coarctation as they are when the ductus forms a more hydrodynamically efficient conduit that is short and merges with the arch at an acute angle. Thus, the rarity of coarctation of the aorta with a right arch and mirror image branching may be ascribed largely to the rarity of a truly mirror image right arch (including the ductus) occurring without abnormalities of right ventricular outflow.

If the genetic and hydrodynamic conditions of the developing conotruncus and arch vessels are the factors that lead to coarctation in a normal left arch or a mirror image right arch, what is the cause of coarctation in patients with a right arch and aberrant subclavian artery? The answer to this question is not known. However, a hemodynamic cause is potentially conceivable. The left ductus in these cases is typically connected to the subclavian artery, which arises from the descending aorta. Because there is no right ventricular outflow obstruction, antegrade flow rates through the left ductus should be normal and will most likely continue into the descending aorta through the left subclavian artery. The coarctation in such cases tends to appear as a hypoplastic segment of aorta between the right and left subclavian arteries, which might be a relatively low-flow region in the setting of normally distributed left and right ventricular outflow. There are no fetal echocardiographic characterizations of flow in an aberrant left subclavian artery, so this possibility is purely hypothetical.

When a coarctation is identified with a right-sided aortic arch, it is important to document the anatomy of the arch and the ductus, as well as the laterality of the proximal descending aorta. The likelihood of abnormal arch anatomy, such as a circumflex arch or retroesophageal aberrant subclavian artery, is higher with a right-sided arch than a normal left-sided arch, although symptoms caused by such patterns are still uncommon [2, 4]. When present, these or other variations may influence not only the surgical incision, but also decisions regarding whether to repair coexisting lesions at the same procedure. For example, a circumflex arch may be difficult to mobilize adequately through a right thoracotomy, especially when a technique that requires extensive mobilization of the descending aorta and entire arch is used. In such circumstances, repair may be facilitated by a left thoracotomy approach or a median sternotomy. When a ventricular septal defect is present and a median sternotomy is performed, single-stage repair will be the preferred option. Because the coarctation is not always juxtaductal in the setting of a right arch, there may be less need for concern about incorporating ductal tissue into the anastomosis, thus allowing greater flexibility in the technique of repair.

Interruption of a right aortic arch
In addition to our patient, 15 patients with interruption of a right aortic arch have been described (Table 3) [11, 34–41]. In all 16 patients, the interruption was the right-sided analog of a type B interruption [42], located between the right common carotid and right subclavian arteries. The left subclavian artery was anomalous in 6 patients overall, with aberrant origin from the descending aorta in 3 and isolation of the left subclavian artery in 3. In 9 of the 15 previous patients, and 11 of 16 including ours, DiGeorge syndrome has been documented. In the remaining 5 patients, absence of DiGeorge syndrome or del22q11 was not specifically established, so it is possible that DiGeorge syndrome was present subclinically in at least some of these patients. All patients had an associated ventricular septal defect. Only 2 of the previously described patients were reported to undergo complete repair, and another 4 had surgical palliation. The remaining 9 patients died without operation.


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  		Table 3. Reported Cases of Right Aortic Arch With Interrupted Archa

 
Although interrupted arch and coarctation are often grouped together as obstructive arch lesions, there are significant pathogenetic differences between them. As with coarctation, flow-related vascular development is almost certainly important in the pathogenesis of interrupted aortic arch. However, genetic factors affecting primary morphogenesis of the aortic arch system are probably significant contributors to interruption in many cases as well. With respect to the issue of pathogenesis of interrupted arch, it is important to recognize the differences between the two characteristic patterns, in which the interruption is either between the left common carotid and left subclavian arteries (assuming a left arch; type B) or at the level of the aortic isthmus, distal to the origin of the left subclavian artery (type A) [42, 43]. Type B interruption is frequently associated with DiGeorge syndrome, or as has been recognized in recent years, with microdeletions of chromosome 22q11 [11, 12]. In addition, type B interrupted arch is characteristically associated with a bicuspid aortic valve; posterior deviation or malalignment of the conal outlet septum (and consequent subaortic stenosis), or both; a ventricular septal defect; and anomalous origin of the subclavian artery supplying the upper extremity contralateral to the aortic arch [43, 44]. In contrast, the less common type A interruption is almost never found in patients with DiGeorge syndrome or del22q11 [43]; the conal outlet septum is usually not deviated or malaligned, so subaortic stenosis is not present [45]; and ventricular septal defect or anomalous origin of the subclavian artery is unusual [43]; but there is a well-recognized association with aortopulmonary septal defect [46]. The disparity in findings associated with the two types of arch interruption suggests a difference in pathogenesis. Flow-related factors most likely predominate in type A interruption, which occurs at the isthmus of the arch. Such factors probably contribute to type B interruption as well, but unlike type A interruption, type B interruption is highly associated with chromosome 22q11 deletion in which there is often regression or underdevelopment of the fourth pharyngeal arches. In the fully developed human with a normal left arch, the derivatives of the primitive left and right fourth arches are the distal aortic arch (the segment between the left common carotid and left subclavian arteries) and the proximal segment of the right subclavian artery (which is necessary for normal formation of the innominate artery), respectively. In type B interruption of a left arch, the left fourth arch is always abnormal because this is the location of arch interruption, and the right fourth arch is frequently abnormal as well, as illustrated by the high frequency of aberrant right subclavian artery. Similarly, in interruption of a right arch, the fourth arches are often involuted bilaterally, with type B interruption and an aberrant or isolated left subclavian artery (Fig 2).



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  		Fig 2. This schematic depiction of cervical right arch and two patterns of interrupted right arch is based on the model described in Figure 1. The diagrams on the left and in the center demonstrate interruption of a right aortic arch with aberrant origin or isolation of the left subclavian artery, respectively. The diagram on the right shows one of the typical patterns of right cervical arch, with direct aortic origin of the right internal (ICA) and external (ECA) carotid arteries (obstruction not shown). Note that the fourth pharyngeal arches regress bilaterally in all three scenarios. (IAA = interruption of the aortic arch; other abbreviations are as in Fig 1.)

 
As discussed earlier, interruption of a right arch is rare, suggesting that flow-related factors are pathogenetically important. However, when an interrupted arch does occur with a right arch, del22q11/DiGeorge syndrome is almost always present, supporting the theory that primary morphogenetic abnormalities resulting from del22q11 are important as well. Type A interruption, which is almost never associated with del22q11, has not been reported in the setting of situs solitus with a right arch.

Although the standard approach to repair of interrupted arch at most centers is through a midline incision, which makes preoperative identification of arch laterality potentially less critical than in coarctation, it is nevertheless important to be aware of this condition. Relevant factors to consider are the possibility of a midline or left-sided descending aorta, as well as the potentially higher likelihood of bronchial compression in patients with a right arch, which may be in part a function of the shorter distance between the ascending and descending aorta in this configuration.

Cervical right arch with obstruction
Although both of our patients with cervical arch had forms of obstruction that might be characterized as coarctation, we discuss this as a separate category for several reasons (Table 4). Namely, the anatomy of a cervical arch may pose unique problems, obstruction may be due to causes other than simple coarctation, and the types of coarctation that do occur are often atypical [31, 47–49]. Before our 2 patients, 4 patients with a right cervical arch with obstruction were described. As is commonly the case with cervical arch, brachiocephalic branching patterns were highly variable, with an aberrant left subclavian artery in all but 1 of the 6 patients (ours included); separate aortic origin of the right internal and external carotid arteries (n = 1) or right vertebral artery (n = 2), or both; and tortuous or aneurysmal segments of arch in at least 3 patients. Operation was performed in all these patients.


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  		Table 4. Reported Cases of Cervical Right Aortic Arch With Obstructiona

 
Cervical aortic arch, regardless of laterality or obstruction, is a rare anomaly in itself. The frequency of a right-sided arch is much higher with cervical arch than with the normally located aortic arch [5]. In addition, a circumflex pattern is common, with the descending thoracic aorta crossing to the contralateral side of the vertebral column at the midthoracic level, and the branching pattern of the brachiocephalic vessels is highly variable [5]. Although there is some uncertainty about the developmental anatomy of a cervical arch, it is generally thought that a cervical arch represents persistence of the embryonic third arch and regression of the ipsilateral fourth arch. Cervical arch has been associated with deletions of chromosome 22q11 [50], so the theory of fourth arch involution and third arch persistence is quite plausible. The other standard theory is that a cervical arch is simply a high fourth arch, with failure of normal descent. However, the anomalous branching patterns frequently seen in patients with cervical arch, especially direct aortic origin of the external and internal carotid arteries ipsilateral to the arch, support the theory of third arch predominance, given that the embryonic third arch develops into the common carotid artery. Of course, the law of parsimony need not apply, as all cases of so-called cervical arch may not be expressions of the same developmental abnormality. In most cases, the associated obstruction has been a discrete narrowing or a hypoplastic segment at the apex of the arch, corresponding with the persistent third arch. There have also been a number of cases of cervical arch with aneurysm formation at the apex of the arch, similar to that found in our patient, which has prompted some to suggest that this tissue may be abnormal [51]. Although the theory of abnormal third arch tissue facilitating obstruction has not been substantiated, the fact that both aneurysms and coarctations have been observed to form at this site for no apparent hydrodynamic reason supports the notion that the third arch segment is susceptible to aberrant development. We have found small aneurysms of the vertebral arteries in conjunction with an obstructed right cervical arch and recommend that the anatomy and structure of the brachiocephalic vessels be inspected carefully during the repair to identify any coexisting abnormalities.


   Clinical implications
Top
 Abstract
 Introduction
 Right-sided aortic arch
 Congenital obstructive lesions...
 Clinical implications
References
 
There are several clinical implications of obstruction in the setting of a right aortic arch. Echocardiography, which is adequate for assessing most arch anomalies, is also sufficient in obstructive lesions of a right aortic arch. Magnetic resonance imaging may also be useful for characterization of arch and brachiocephalic vascular anatomy, especially when three-dimensional reconstruction is used [52]. With proper anatomic definition and awareness of the relatively high association between a right arch and both brachiocephalic branching abnormalities and a left-sided descending aorta, surgical repair of right arch obstruction should pose little difficulty in the majority of cases. As was seen in one of our patients with coarctation of a right cervical arch, bronchial obstruction caused by compression between the ascending and descending aorta may be more likely in the setting of a right-sided arch. The distance between the ascending and proximal descending aorta at the level of the right mainstem bronchus is shorter in patients with a right arch than in those with a left-sided arch, due in part to the normal location of the ascending aorta to the right of midline. As a result, the right mainstem bronchus may be at greater risk for vascular compression, especially if the descending aorta is not adequately mobilized during the repair, which it may not be, particularly if there is an aberrant left subclavian artery or a left-sided descending aorta or a midline approach is used. Preoperative magnetic resonance imaging may be valuable in these patients not only for clarification of vascular anatomy, but for defining the dimensions of the thoracic cavity and upper airways as well, which may aid in the intraoperative evaluation of risk for tracheobronchial obstruction.


   References
Top
 Abstract
 Introduction
 Right-sided aortic arch
 Congenital obstructive lesions...
 Clinical implications
 References
 

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