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Ann Thorac Surg 1999;67:1125-1129
© 1999 The Society of Thoracic Surgeons

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Original Articles

Coarctation of the aorta with right aortic arch: surgical technique and new classification

^a Department of Cardiothoracic Surgery, Rabin Medical Center, Petach Tikva, Israel

Accepted for publication September 9, 1998.

Address reprint requests to Dr Vidne, Department of Cardiothoracic Surgery, Rabin Medical Center, Beilinson Campus, Petach Tikva 49100, Israel

	Abstract

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Background. The combination of right aortic arch and coarctation of the aorta has seldom been reported. This rare abnormality occurs as an isolated lesion or in association with other congenital defects, such as mixed gonadal dysgenesis and Turner’s syndrome.

Methods. The medical records of 2 patients who underwent operation for right aortic arch and aortic coarctation in our institution were reviewed together with case reports in the literature. Various surgical options have been reported: synthetic grafts, subclavian flap, and end-to-end anastomosis. End-to-end anastomosis is our method of choice for coarctation of the aorta and for right aortic arch with coarctation, even though an excessive amount of dissection is needed.

Results. Both patients from our institution are doing well, with no sign of recoarctation in either patient. Our experience and recent advances in the understanding of the anatomy of this lesion led us to develop an alternative simplified classification for right aortic arch, which is presented here.

Conclusions. Right aortic arch and coarctation of the aorta is a rare morphologic combination. On the basis of our experience with repair of coarctation of the aorta and our review of the literature, we think that end-to-end anastomosis is the operative technique of choice with the best long-term results. Our simplified classification is easy to understand when dealing with right aortic arch.

	Introduction

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Coarctation of the aorta is a congenital narrowing of the descending thoracic aorta usually occurring just distal to the left subclavian artery (LSA) and adjacent to the ductus arteriosus. The rate of aortic coarctation is 0.2 to 0.6 per 1,000 live births (5% to 8% of all congenital heart malformations) [1]. Radiographic and postmortem studies indicate that right aortic arch is found in approximately 0.1% of the population. Right aortic arch is usually accompanied by other congenital anomalies of the cardiovascular system, most commonly tetralogy of Fallot and truncus arteriosus [2, 3]. Coarctation of the aorta in patients with right aortic arch is extremely rare; only a few cases where it is the sole congenital malformation or where it is combined with other defects in the same patient have been reported [4–10].

	Material and methods

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Surgical experience in rabin medical center
Over the past 25 years, 2 patients with the combination of right aortic arch and aortic coarctation underwent operation in our department.

Patient 1
An 8-year-old boy complained of abdominal and leg pain during exercise. When he was 4 months old, the diagnosis of dextrocardia was made at routine medical examination. As his growth and development were normal, the diagnosis of aortic coarctation was made only several weeks before admission to the hospital.

Physical examination revealed a thin but well-developed boy. Blood pressure was 135/83 mm Hg in both arms. On auscultation, the most prominent finding was a long blowing systolic murmur in the right second interspace and along the spine in the midthoracic region. Peripheral pulses in both femoral arteries were weak.

Electrocardiogram and chest roentgenogram showed findings typical for dextrocardia. Brachial angiography revealed a dextrocardial aortic arch with mirror imaging of the aortic arch vessels and dilated subclavian and vertebral arteries. A 5-mm narrowing of the descending thoracic aorta 2 cm distal to the right subclavian artery with a poststenotic dilation was demonstrated. The diagnosis of coarctation of the aorta with mirror-image dextrocardia and situs inversus was made.

Resection of the coarctation with an end-to-end anastomosis was performed through a right thoracotomy. The pressure gradient between the upper and lower parts of the body disappeared, and the boy was discharged after an uneventful recovery.

Patient 2
A 3-year-old girl with right aortic arch was referred from another hospital for repair of aortic coarctation. Physical examination revealed a well-developed girl with a blood pressure of 133/87 mm Hg in the right arm and 102/66 mm Hg in the left. The echocardiogram showed remarkable left ventricular hypertrophy, and the roentgenogram revealed a normal cardiac silhouette with a right aortic arch. Angiography, right aortic arch with aberrant LSA was demonstrated; the location of the coarctation was just distal to the right subclavian artery. Dynamic spiral computed tomography showed a right aortic arch, a coarctation 1.8 cm long and 3 mm in diameter at the distal part of the arch, an aberrant LSA, and a left-sided descending aorta.

The child was operated on through a left thoracotomy. The retroesophageal part of the arch was freed, and ligation of the left-sided ligamentum arteriosum, resection of the coarctated segment, and end-to-end anastomosis between the descending aorta and the arch were performed. The pressure gradient between the right and left arms decreased to 7 mm Hg after the operation, and the echocardiogram revealed a good-sized anastomosis with normal flows. The postoperative hospitalization was prolonged because of chylothorax. However, after the child was placed on a special diet, secretion of chyle ceased, and she was released on postoperative day 15.

Review of literature
As stated earlier, the combination of right aortic arch and coarctation is rare, and there are only a few case reports in the literature. To facilitate analysis of the anatomical features and the associated lesions, these case reports have been summarized in Table 1 and include both right aortic arch with mirror-image arteries and right aortic arch with an aberrant LSA (types IIIA and IIIB according to Stewart and colleagues [11]). All diagnoses were made using angiography. The operative technique included a posterolateral thoracotomy in all patients but 1 [9]. In that patient, repair of aortic dissection was attempted through a median sternotomy but was unsuccessful. There were variations in the type of repair: synthetic tube, subclavian flap, or end-to-end anastomosis. Because we think end-to-end anastomosis provides the best results, it was used in both our patients.

The average age at repair in the case reports is higher than the age at which we operate today. This is not related to the special anatomy but rather to surgical attitudes at the time.

	Comment

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Right aortic arch is an abnormal regression of the left eighth dorsal segment resulting in an aortic arch that crosses the right bronchus. During the second month of fetal life, normal heart growth is in a caudal direction, so that the left fourth arch receives the major arterial blood flow. The brachiocephalic trunk then forms the left arch [11]. In their classification of aortic arch anomalies, Stewart and associates [11] designated right aortic arch as type III and further subdivided it according to the origin of the brachiocephalic trunk.

On the basis of their diagram, four different areas of involution can be identified on each arch, and these determine the type of vascular anomaly (Fig 1). We [12] proposed a simplified classification based on the areas of involution. In the case of right aortic arch, this can be a left or right descending aorta; the ductal (ligamentum) anatomy is often unusual as well. Single ductus can arise as a left-sided or right-sided structure from either the aorta or one of several brachiocephalic vessels; it can also be absent or bilateral.

View larger version (51K): [in this window] [in a new window]   Fig 1. Hypothetical double aortic arch system based on developmental pattern that provides the basis for understanding aortic arch anomalies. A, B, C, and D are the different areas of involution of the aortic arch. (AA = ascending aorta; DA = descending aorta; LCCA = left common carotid artery; LD = left ductus; LSA = left subclavian artery; RCCA = right common carotid artery; RD = right ductus; RSA = right subclavian artery.)

View larger version (50K): [in this window] [in a new window]   Fig 2. The different types of aortic arch according to our new classification. See text for details of Figure 2A through 2G. See Figure 1 for abbreviations.

Using only the flow-related theory to explain coarctation of the aorta and right aortic arch is unsatisfactory, and there are undoubtedly other factors that influence the process of aortic arch development. In the case of type C right aortic arch (right aortic arch and LSA), it is even harder to provide a logical explanation based on flow. A possible one might be that the ductus connected to the subclavian artery maintains a relatively normal flow in the descending aorta in the absence of right-sided obstruction.

In the past, diagnoses usually were made with angiography [3–8]. Today most centers use electrocardiography for the diagnosis of aortic coarctation alone or in the presence of aortic arch. Electrocardiography simplifies the diagnostic procedure and assists in the decision-making process. According to our policy, angiography is used only when special anatomic or hemodynamic questions exist.

Spiral computed tomography has become an important tool in the diagnosis of aortic arch malformation, and we tend to use it in such cases [14]. As in our patient 2, spiral computed tomography demonstrated the exact anatomy and location of the narrow segment as well as its length. We believe that knowing the exact anatomy of the lesion prior to operation is invaluable, especially in cases of type A or B right aortic arch where use of the subclavian flap is not an option. In such cases, a greater amount of dissection is usually necessary to obtain better exposure, and this facilitates an end-to-end anastomosis, which we consider the best technique for repair. Prior knowledge of anatomic details is also important to the surgeon when discussing the surgical risks with the patient’s family, such as the higher risk of chylothorax when extensive dissection is expected, and the possibility of using synthetic grafts for the repair when end-to-end anastomosis is not an option.

As stated earlier, the incision used is a left posterolateral thoracotomy, as it provides the best visualization. The location of the descending aorta can influence the level of technical difficulty, especially when it is on the right side. However, in our patient 2 and in one of the case reports reviewed [4], the descending aorta was on the right side and a left ligamentum was present. Division of the ligamentum is important to avoid the risk of a vascular ring and can be performed only through left thoracotomy.

In our experience, the best technique for repair of coarctation of the aorta alone or in combination with right aortic arch is end-to-end anastomosis. It gives the best hemodynamic results, especially in the long term [15]. To achieve good exposure for the repair, a large-scale dissection is made to free both the descending aorta and the aortic arch and vessels. The site of the proximal anastomosis in small babies in particular should be the caudal facing part of the aortic arch (extended end-to-end anastomosis) [16].

In summary, the combination of aortic coarctation and right aortic arch is rare, and only a few cases have been reported. The frequent association of other malformations, cardiovascular or extracardiac, is of importance, and every effort should be made to diagnose them. The operative technique is not different from the technique we use when repairing aortic coarctation with a left arch. We suggest a simplified classification for anomalies in the development of the aortic arch ramifications that is based on the possible areas of involution. There is considerable confusion in the medical literature concerning the nomenclature of aortic arch anomalies, and we believe that with use of our classification, this problem can be overcome.

	References

Top Abstract Introduction Material and methods Comment References

 

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This Article Abstract Full Text (PDF) 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 Author home page(s): Bernardo A. Vidne Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Ad, N. Articles by Vidne, B. A. Search for Related Content PubMed PubMed Citation Articles by Ad, N. Articles by Vidne, B. A.