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Ann Thorac Surg 2007;84:1014-1016
© 2007 The Society of Thoracic Surgeons

Case Reports

Hypoplastic Circumflex Retroesophageal Right-Sided Cervical Aortic Arch With Unusual Vascular Arrangement and Severe Coarctation

Gurpal S. Ahluwalia, MRCSa, Abbas G. Rashid, MRCSa, Massimo Griselli, MD, FRCSa, Michal Szczeklik, MSa, Michael L. Rigby, MD, FRCPb, Raad H. Mohiaddin, PhD, FRCRc, Daryl F. Shore, FRCSa,*

a Department of Cardiac Surgery, Royal Brompton Hospital, London, United Kingdom
b Department of Cardiology, Royal Brompton Hospital, London, United Kingdom
c Cardiovascular Magnetic Resonance Unit, Royal Brompton Hospital, London, United Kingdom

Accepted for publication April 18, 2007.

* Address correspondence to Dr Shore, Department of Cardiac Surgery, Royal Brompton Hospital, Sydney St, London, SW36NP, United Kingdom (Email: d.shore{at}rbh.nthames.nhs.uk).


   Abstract
Top
 Abstract
Introduction
 Comment
 Acknowledgments
 References
 
We report the case of a 12-year-old boy with a hypoplastic retroesophageal circumflex right-sided cervical aortic arch and coarctation. After the incidental finding of a heart murmur when the boy was 9 years old, cardiac magnetic resonance showed a right-sided cervical aortic arch, hypoplastic transverse arch, and separate origin of the left common carotid, right common carotid, right vertebral, and right subclavian arteries. The left subclavian artery arose from the proximal descending aorta next to the coarctation. An extra-anatomical ascending to descending aorta tube graft was inserted through a right lateral thoracotomy with good results.


   Introduction
Top
 Abstract
 Introduction
Comment
 Acknowledgments
 References
 
Hypoplastic circumflex retroesophageal right-sided cervical aortic arch with this vascular arrangement and severe coarctation is extremely rare. We describe our evaluation and successful surgical treatment.

A 12-year-old boy from Greece was admitted with coarctation of the aorta. This diagnosis was made by echocardiography when the boy was 9 years old after the incidental finding of a heart murmur. He was asymptomatic until 2005, when he complained of dysphagia and respiratory distress. Esophagography showed severe posterior indentation from the aortic arch.

Cardiac catheterization showed a right-sided aortic arch (rising above the level of the clavicles), hypoplastic transverse arch, and pre-ductal coarctation (gradient of 30 mm Hg). The origin and course of the head and neck vessels were unclear.

On examination the boy looked well. The systemic blood pressure was 140/75 mm Hg. He had a normal right radial pulse, but reduced left radial and femoral pulses and no other abnormal findings. Electrocardiogram showed sinus rhythm with features of a left ventricular hypertrophy.

Echocardiography showed normal intracardiac anatomy and good left ventricular function. Coarctation was found at the distal part of the isthmus (peak gradient, 50 mm Hg) with diastolic tail and reduced pulsatility of the abdominal aorta. As the aortic arch anatomy looked unusual, cardiac magnetic resonance imaging was requested.

This showed a circumflex retroesophageal right-sided aortic arch crossing the midline behind the esophagus on the left side of the spine. The first branch was the left common carotid artery, travelling horizontally in front of the trachea, followed by the right common carotid, right vertebral, and right subclavian arteries. The transverse arch was severely hypoplastic (7 mm), with ascending and descending aortas measuring 22 mm and 17 mm, respectively. The left subclavian artery arose from the proximal descending aorta next to the coarctation (Figs 1, 2). Go


Figure 1
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  		Fig 1. Preoperative cardiac magnetic resonance. (AA = ascending aorta; DA = descending aorta; LCA = left common carotid artery; LSA = left subclavian artery; RCA = right common carotid artery; RSA = right subclavian artery.)

 

Figure 2
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  		Fig 2. Diagram of aortic arch anatomy.

 
After multidisciplinary discussion, surgical management was decided and the patient underwent an extra-anatomical bypass grafting from the ascending to the descending aorta without cardiopulmonary bypass. Through a right lateral thoracotomy at the fifth intercostal space, segments of the aorta were prepared and two pairs of intercostal branches were ligated. A Gelatin-impregnated knitted vascular prosthesis (Gelseal [Vascutek Ltd, Inchinnian, Scotland]) of size 18 was prepared and an end-to-side anastomosis was performed on the descending aorta using 5-0 polypropylene sutures. The prosthesis was cut to size and end-to-side anastomosis was made with the ascending aorta using 4-0 polypropylene sutures (Fig 3). Postoperative echocardiography and cardiac magnetic resonance showed a patent graft and good pulsatile flow in the abdominal aorta (Fig 4).


Figure 3
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  		Fig 3. Ascending to descending aorta vascular graft.

 

Figure 4
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  		Fig 4. Postoperative cardiac magnetic resonance showing the conduit from ascending to the descending aorta. (AA = aortic arch; DA = descending aorta.)

 

   Comment
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 Abstract
 Introduction
 Comment
Acknowledgments
 References
 
Cervical aortic arch formation is the result of persistence of the right or left third branchial arch and regression of the fourth [1]. Often, there are associated developmental changes including abnormal laterality, branching, and structural anomalies [2, 3]. In our case there was a hypoplastic circumflex right-sided cervical aortic arch with aberrant origin of the left subclavian artery and severe coarctation.

Right circumflex retroesophageal aortic arch describes a right-sided arch that crosses the midline behind the esophagus, and it is often elongated and reaches a higher level in the thorax.

These anomalies may present in many ways including stridor, regurgitation, and dysphagia by compressing the trachea and the esophagus. This patient experienced an episode of dysphagia at 11 years of age. He had unequal upper limb systemic pressures due to the aberrant origin of the left subclavian artery next to the coarctation.

Barium studies, echocardiography, computerized tomography and cardiac catheterization are important diagnostic tools [4], but cardiac magnetic resonance is particularly useful in accurately defining the complex anatomy [5].

Treatment options vary from no intervention to stenting or surgery. The extensive hypoplasia of the transverse arch precluded ballooning and stenting, and surgical management seemed the best option. A left thoracotomy approach, as in common coarctation repair, would be very difficult due to the high course of the aortic arch and its abnormal branching [6]. Right lateral thoracotomy with extra-anatomical bypass from the ascending to the descending aorta is safe and effective for these complex cases [7]. However, the technique used must be designed for the specific underlying anatomy [2].


   Acknowledgments
Top
 Abstract
 Introduction
 Comment
 Acknowledgments
References
 
The authors thank Stephen Millward, BA (graphic designer, Royal Brompton Hospital, London) for Figure 2.


   References
Top
 Abstract
 Introduction
 Comment
 Acknowledgments
 References
 

  1. Van Son JA, Bossert T, Mohr FW. Surgical treatment of vascular ring including right cervical aortic arch J Card Surg 1999;14:98-102.[Medline]
  2. 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]
  3. Baravelli M, Borghi A, Rogiani S, et al. Clinical, anatomopathological and genetic pattern of 10 patients with cervical aortic arch Int J Cardiol 2007;114:236-240Epub 2006 June 15.[Medline]
  4. Tanju S, Ustuner E, Erden I, Aytac SK. Right cervical arch and pseudocoarctation of the aorta associated with aneurysms and steal phenomena: US, CTA, and MRA findings Cardiovasc Intervent Radiol 2007;30:146-149.[Medline]
  5. Almeida R, Alvares S, Fortuna A, Moreira J, Vieira A. Cervical aortic arch and 22q11 deletion—the role of MRI in diagnosis Rev Port Cardiol 2003;22:1241-1248.[Medline]
  6. Walker T, Heinemann MK, Nagy S, Steil E, Ziemer G. Right-sided cervical aortic arch with stenosis—treatment with an extra-anatomic bypass graft Thorac Cardiovasc Surg 2002;50:306-307.[Medline]
  7. Arakelyan V, Spiridonov A, Bockeria L. Ascending-to-descending aortic bypass via right thoracotomy for complex (re-) coarctation and hypoplastic aortic arch Eur J Cardiothorac Surg 2005;27:815-820.[Abstract/Free Full Text]



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