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Ann Thorac Surg 2001;71:729-731
© 2001 The Society of Thoracic Surgeons


Case report

A vascular ring: right aortic arch and descending aorta with left ductus arteriosus

Jae Jin Han, MDa, Sejung Sohn, MDa, Hae Soon Kim, MDa, Tae Hee Won, MDa, Jae Ho Ahn, MDa

a Departments of Cardiac Surgery and Pediatric Cardiology, Mokdong Hospital, Ewha Womans University, Seoul, South Korea

Accepted for publication April 15, 2000.

Address reprint requests to Dr Han, Department of Thoracic-Cardiovascular Surgery, Ewha Womans University Mokdong Hospital, 911-1, Mokdong, Yangcheongu, Seoul, 158-710, Korea
e-mail: jjhan{at}mm.ewha.ac.kr


    Abstract
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 Abstract
 Introduction
 Comment
 References
 
A case is presented of vascular ring caused by right aortic arch with mirror-image branching and left ductus arteriosus. In this case, the descending aorta was located right of the tracheoesophagus and the left ductus arteriosus connected to the descending aorta far below the arch, producing compression of the esophagus only. Through median sternotomy, the ligation and division of the ductus was performed with concomitant repair of ventricular septal defect.


    Introduction
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 Abstract
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 References
 
Congenital vascular ring that may cause compression of the airway and the esophagus, or both, usually includes a double aortic arch, right or left aortic arch with aberrant opposite-sided subclavian artery, and a right aortic arch with left ductus arteriosus [1, 2]. Other rare types of aortic arch anomalies have also been described [3]. We encountered a variant form of right aortic arch with left ductus arteriosus, accompanied with underlying ventricular septal defect.

An 18-month-old boy, weighing 9.2 kg, with frequent aspiration and vomiting during feeding, was admitted to the hospital. The esophagogram showed a posterior impression of esophagus without airway stenosis (Fig 1). Echocardiogram and aortic angiogram showed a ventricular septal defect and suggested a type of vascular ring such as right aortic arch, mirror-image branching and left ductus arteriosus. Through median sternotomy, a right aortic arch and left ligamentum arteriosum were identified. Under the extracorporeal bypass the descending aorta, located right to the trachea and esophagus, was dissected through the space between the superior vena cava and ascending aorta. A diverticulum of 10 mm in diameter originating from the descending aorta, traversed leftward all the way behind the esophagus, and was connected to the ligamentum arteriosum at the left side of trachea and esophagus. So the diverticulum eventually compressed the esophagus from behind as a pulsating blind-pouch (Fig 2). After ligation and division of the diverticulum and ligamentum of ductus, a ventricular septal defect was closed as usual. Postoperatively, the patient was free of dysphagia and vomiting. The esophagogram was repeated 2 months after the operation and showed the indentation had markedly decreased (Fig 3).



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Fig 1. Lateral view of preoperative esophagogram showing a posteriorly deep impression.

 


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Fig 2. Drawing of the vascular ring in the present case. A vascular ring is performed by the right aortic arch with right-sided descending aorta (DA), pulmonry artery (PA), and a left ductus arteriosus (arrow) originating from the descending aorta as a diverticulum, compressing the esophagus posteriorly, and connecting to the left pulmonary artery. (AA = ascending aorta.)

 


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Fig 3. Lateral view of postoperative esophagogram showing that the posteriorly deep impression was now significantly widened 2 months after the operation.

 

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The right–sided aortic arch results from regression of the left dorsal aortic root, and regression between the left ductus arteriosus and the descending aorta produces a mirror image branching of the aorta. A left-sided ductus arteriosus may connect the left pulmonary artery to the left subclavian portion of the innominate artery or to the descending aorta [4]. In the latter case, a complete vas-cular ring exists. The previous reports of this type of vascular ring usually described that tracheoesophagus was compressed by either a left dorsal aortic root or a left (posterior) circumflex descending aorta at the arch level [1, 5]. Others reported tracheoesophagus compressed at the junction between aortic arch and descending aorta by a diverticulum [6] or a ligamentum [7], and they are usually not associated with intracardiac defects. In this case, however, the ductus arteriosus arose below the aortic arch and a ventricular septal defect coexisted. The operation for the vascular ring of the right aortic arch with a mirror image branching and a left ductus arteriosus was usually done through the left thoracotomy [1, 2, 8]. For this patient, we performed a median sternotomy for the concomitant repair of ventricular septal defect.

In this particular patient, even without coexisted intracardiac defect, we believed that the conventional left thoracotomy would not be good for direct access to the originating portion of the diverticulum, which plays a major role in esophageal compression.


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 References
 

  1. Van Son J.A.M., Julsrud P.R., Hagler D.J., et al. Surgical treatment of vascular rings. The Mayo Clinic experience. Mayo Clin Proc 1993;68:1056-1063.[Medline]
  2. Roberts C.S., Othersen H.B., Jr, Sade R.M., Smith C.D.I.I.I., Tagge E.P., Crawford F.A., Jr Tracheoesophageal compression from aortic arch anomalies: analysis of 30 operatively treated children. J Pediatr Surg 1994;29:334-338.[Medline]
  3. Moes C.A.F., Freedom R.M. Rare types of aortic arch anomalies. Pediatr Cardiol 1993;14:93-101.[Medline]
  4. De Leval M. Vascular rings. Surgery for congenital heart defects, 2nd ed. Philadelphia: WB Saunders, 1994:307-316.
  5. D’Souza V.J., Velasquez G., Glass T.A., Formanek A.G. Mirror image right aortic arch. A proposed mechanism in symptomatic vascular ring. Cardiovasc Intervent Radiol 1985;8:134-136.[Medline]
  6. Garti I.J., Aygen M.M., Vidne B., Levy M.J. Right aortic arch with mirror image branching causing vascular ring. A new classificaton of the right aortic arch patterns. Br J Radiol 1973;46:115-119.[Abstract/Free Full Text]
  7. Schlesinger A.E., Mendeloff E., Sharkey A.M., Spray T.L. MR of right aortic arch with mirror image branching and a left ligamentum arteriosum: an unusual cause of a vascular ring. Pediatr Radiol 1995;25:455-457.[Medline]
  8. Hartyanszky I.L., Lozsadi K., Marcsek P., Huttl T., Sapi E., Kovacs A.B. Congenital vascular rings: surgical management of 111 cases. Eur J Cadiothorac Surg 1989;3:250-254.[Abstract/Free Full Text]




This Article
Right arrow Abstract Freely available
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Right arrow Author home page(s):
Jae Jin Han
Hae Soon Kim
Jae Ho Ahn
Right arrow Permission Requests
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Right arrow Articles by Han, J. J.
Right arrow Articles by Ahn, J. H.
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Right arrow Articles by Han, J. J.
Right arrow Articles by Ahn, J. H.
Related Collections
Right arrow Congenital - acyanotic


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