Ann Thorac Surg 2009;87:307-309. doi:10.1016/j.athoracsur.2008.05.050
© 2009 The Society of Thoracic Surgeons
Case Reports
Repair of Coarctation in Right Circumflex Retroesophageal Arch
Jinyoung Song, MDa,
Woong-Han Kim, MD, PhDc,*,
Heewoon Kim, MDa,
Jungkon Koh, MDb,
Jae Gun Kwak, MDc,
Ki Bum Kim, MDd,
Eun Jung Bae, MD, PhDd
a Department of Pediatrics, Handong University Sunlin Hospital, Kyungsangbukdo South Korea
b Department of Radiology, Handong University Sunlin Hospital, Kyungsangbukdo, South Korea
c Department of Thoracic & Cardiovascular Surgery, Clinical Research Institute, Seoul National University, College of Medicine, Seoul National University Children's Hospital, Seoul, South Korea
d Department of Pediatrics, Clinical Research Institute, Seoul National University, College of Medicine, Seoul National University Children's Hospital, Seoul, South Korea
Accepted for publication May 19, 2008.
* Address correspondence to Dr Kim, Department of Thoracic and Cardiovascular Surgery, Clinical Research Institute, Seoul National University, College of Medicine, Seoul National University Children's Hospital, 28 Yongon-Dong, Jongno-Gu, Seoul, 110-744, Korea (Email: woonghan{at}snu.ac.kr).
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Abstract
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A right-sided circumflex retroesophageal aortic arch combined with a ventricular septal defect and severe coarctation is an extremely rare condition. Moreover, surgical correction is significant when performing an anatomical reconstruction of the left aortic arch by end-to-side and native tissue-to-tissue anastomosis instead of using a bypass graft. The authors describe an unusual case of a 1 month-old neonate with a ventricular septal defect and coarctation of a right-sided circumflex retroesophageal aortic arch who underwent successful surgical correction.
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Introduction
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A right-sided circumflex retroesophageal aortic arch combined with a ventricular septal defect and severe coarctation is an extremely rare condition. The previously described surgical technique involves a bypass vascular graft. Here, the authors describe a new surgical technique involving anatomic reconstruction of a new left aortic arch by extended end-to-side and native tissue-to-tissue anastomosis.
A 1-month-old neonate weighing 4 kg was admitted with symptoms of respiratory distress. Physical examination revealed chest retraction with wheezing and rales in both lung fields, and a cardiac examination revealed a grade 2 or 3 systolic murmur at the left sternal border with increased S2 sound intensity. The femoral pulse was weak as compared with the radial pulse, and a chest roentgenogram showed marked cardiomegaly and increased pulmonary vascularity. Two-dimensional echocardiography demonstrated dilated pulmonary arteries and left cardiac chambers, and a large ventricular septal defect of the perimembranous type was detected from inlet to outlet extension with severe pulmonary hypertension. Furthermore, left ventricular contraction was attenuated, and severe tubular hypoplasia of the right aortic arch and focal coarctation in the isthmus were found. Computed tomographic images demonstrated a right-sided ascending aorta and a hypoplastic circumflex retroesophageal aortic arch crossing midline and joining the left-sided descending thoracic aorta with focal narrowing (Fig 1). The right aortic arch measured 2.8 mm in diameter, and the diameter of focal narrowing was 1.8 mm at the distal junction of the aortic arch and descending aorta.
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Fig 1. Preoperative cardiac computed tomographic images. (AA = ascending aorta; DA = descending aorta.)
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An operation was immediately conducted through a sternotomy supported by cardiopulmonary bypass. The arterial cannula was inserted into the proximal right aortic arch directly, and an aortic root cannular was inserted into the proximal ascending aorta and T-connected to the side hole of the arterial cannula. During cooling, the ascending aorta, aortic arch, aortic arch vessel, and descending aorta were fully mobilized. The left-sided ligamentum arteriosum was then divided. During coarctation repair, we adopted the regional perfusion technique. When the rectal temperature reached 28°C, the proximal portion of the right aortic arch was clamped after the division at the isthmic junction of the right aortic arch. The ascending aorta was clamped just distal to the root cannula, and simultaneous myocardial perfusion was maintained using a T-connected infusion line. We reconstructed a new left aortic arch from the right-sided retroesophageal arch by native tissue-to-tissue and extended end-to-side anastomosis, instead of using a bypass graft. The extensively dissected descending thoracic aorta was anastomosed directly to the side of the ascending aorta (Figs 2 and 3).
After repairing the aortic arch, the ventricular septal defect was closed through the right atrium in the conventional manner. The patient was extubated on postoperative day 6 and was discharged on postoperative day 15 without complications. Before discharge, a tracheal examination confirmed mild laryngomalacia, and a chromosomal study revealed a 22q11 deletion. Postoperative echocardiography and computed tomography showed an intact ventricular septum and a good pathway from the ascending aorta to the descending aorta (Fig 4).
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Fig 2. A diagram of the operation showing division of the vascular ring and reconstruction of the neo-left aortic arch. (LCCA = left common carotid artery; LSCA = left subclavian artery; RCCA = right common carotid artery; RSCA = right subclavian artery.)
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Fig 3. Preoperative (A) and postoperative (B) photographs showing the anastomosed and newly reconstructed left aortic arch (arrow).
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Fig 4. Postoperative cardiac computed tomographic images demonstrating that a good pathway was achieved from the ascending to the descending aorta by direct anastomosis.
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Comment
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A right aortic arch and a hypoplastic retroesophageal circumflex aorta is a rare condition and it is associated with developmental changes and genetic problems, such as, a 22q11 deletion [1, 2]. A literature search revealed cases of a right aortic arch and of a severe hypoplastic retroesophageal transverse arch with aortic coarctation [3–5], but no report has been issued on a case as complicated as that reported here. The present case presented vascular anomalies, including aortic coarctation and a bilateral superior vena cava with an intra-cardiac anomaly, which explains why our patient presented mainly with cardiac failure symptoms, unlike other cases without intra-cardiac anomalies. In many of these other reported cases, signs of airway or esophagus obstruction were the main presentation due to the formation of a vascular ring [3]. Computed tomographic images were helpful in terms of understanding the accurate vascular anatomy, which has been recommended by some authors in cases suspected of having an aortic arch anomaly [4, 5].
In some reports, an extra-anatomical bypass graft was used between the descending aorta and the ascending aorta (through a thoracotomy) to correct a hypoplastic retroesophageal circumflex arch [3, 4]. However, our surgical technique differed in that we reconstructed a new left aortic arch without using an artificial graft. Extensive dissection enabled us to mobilize the descending aorta to anastomose directly to the ascending aorta. Furthermore, the regional perfusion technique used maintains cerebral perfusion through a unilateral carotid arterial perfusion during aortic arch anomaly repair, which prevents neurologic complications in neonates and infants [6, 7]. The authors suggest that various corrective surgical techniques should be considered in cases with a right-sided ascending aortic arch and a hypoplastic circumflex retroesophageal aortic arch with coarctation.
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References
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- McElhinney DB, Thompson LD, Weinberg PM, Jue KL, Hanley FL. Surgical approach to complicated aortic arch: anatomic, developmental and surgical considerations Cardiol Young 2000;10:212-219.[Medline]
- Baravelli M, Borghi A, Rogiani S, et al. Clinical, anatomopathological and genetic pattern of 10 patients with cervical aortic arch Int J Cardiol 2007;114:236-240.[Medline]
- Juan-Miguel G, Marcos M, Arturo G, Luis M. Aortic coarctation, vascular ring, and right aortic arch with aberrant subclavian artery Ann Thorac Surg 2002;73:1640-1642.[Abstract/Free Full Text]
- Gurpal SA, Abbas GR, Massimo G, et al. Hypoplastic circumflex retroesophageal right-sided cervical aortic arch with unusual vascular arrangement and severe coarctation Ann Thorac Surg 2007;84:1014-1016.[Abstract/Free Full Text]
- Shelli B, Ziad S, Hitendra P, Olaf R, Frank LH. Coarctation of the aorta in the right aortic arch with left aberrant innominate artery Pediatr Cardiol 2006;27:621-623.[Medline]
- Cheong Lim, Woong-Han Kim, Soo-Cheol Kim, et al. Aortic arch reconstruction using regional perfusion without circulatory arrest Eur J Cardiothorac Surg 2003;23:149-155.[Abstract/Free Full Text]
- Hong-Gook Lim, Woong-Han Kim, Woo-Sung Jang, et al. One-stage total repair of aortic arch anomaly using regional perfusion Eur J Cardiothorac Surg 2007;31:242-248.[Abstract/Free Full Text]
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Abstract
Introduction
