Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Right Aortic Arch in Vascular Ring Defects Treatment & Management

  • Author: Doff B McElhinney, MD; Chief Editor: Howard S Weber, MD, FSCAI  more...
 
Updated: Jan 12, 2015
 

Medical Care

Medical care before surgical repair depends on the clinical presentation. In most cases, only supportive management is required.

Catheter interventions are not used in the management of vascular ring formed by a right aortic arch.

Routine postthoracotomy care is provided following division of a vascular ring. Patients are removed from mechanical ventilation as soon as possible, and tube thoracostomy is generally discontinued on the first postoperative day. The remainder of the inpatient stay is focused on determining and managing any residual symptoms, providing sufficient enteral nutrition, transitioning the patient to enteral analgesics, and educating the parents.

Aside from analgesic medications, pharmacologic therapy is generally not required after removal from mechanical ventilation. Relief from the pain of the thoracotomy incision may be achieved with age-appropriate narcotic and nonsteroidal anti-inflammatory medications.

Consultations

Unless specific associated anomalies or problems are identified, consultations are generally not necessary. As noted above, band 22q11 deletion is present in a substantial proportion of patients with vascular rings.

A consultation with a geneticist is indicated in patients with other characteristic features of the band 22q11 deletion syndrome and may be appropriate in young infants, in whom typical features of the syndrome may not yet be evident.

Transfer

After postoperative stabilization in the intensive care unit and removal from mechanical ventilatory support, the patient may be transferred to the regular inpatient care area for advancement of feedings and additional postoperative care.

Next

Surgical Care

Indications

Surgical division of the vascular ring is indicated in any patient with symptoms of airway or esophageal compression and in patients undergoing surgery for repair of associated cardiovascular or thoracic anomalies.

Techniques

The fundamental principle of surgical management of vascular rings is division of the ring to relieve compression of the trachea and esophagus.

In patients with all forms of vascular ring with a right aortic arch and a left-sided ductus arteriosus or ligamentum arteriosum, this is achieved by dividing the ductus/ligamentum through a left thoracotomy. Dissection should be carried down to the trachea and esophagus to ensure that no constricting fibrous bands remain. In patients with an aberrant subclavian artery in whom residual posterior compression of the trachea or esophagus is a concern, arteriopexy may be performed by suturing the retroesophageal diverticulum to the prevertebral fascia.

Video-assisted thoracoscopic division of vascular rings is performed at several centers and seems to be an effective approach in most cases, but data on outcomes are limited. Robotically assisted division of vascular rings has also been reported.

When associated intracardiac anomalies require surgery through a median sternotomy, division of the ductus/ligamentum is performed during the same procedure.

Results

In the current era, essentially no operative mortality is associated with repair of isolated vascular rings.[4] Outcomes in patients with associated anomalies depend on the coexisting condition.

Postoperative care and precautions

Postoperative care after division of a vascular ring is similar to that for patients undergoing other cardiovascular procedures through a thoracotomy. Most patients experience immediate relief after surgery, although persistent respiratory symptoms and signs may be present, especially in very young infants with severe symptoms preoperatively. Except in patients undergoing concurrent repair of associated anomalies, cardiopulmonary bypass is not used during the repair; therefore, postoperative cardiac function is generally not a problem.

Complications

Complications in the early postoperative period are uncommon after division of a vascular ring.

The major issue is persistent respiratory symptoms, especially in neonates who are more susceptible to tracheomalacia. Occasionally, the tracheomalacia is severe. However, this tends to improve with relief of the vascular ring over time.

Other rare operative complications include chylothorax due to injury to the thoracic duct, diaphragmatic paresis/paralysis secondary to phrenic nerve injury, and vocal cord paresis/paralysis resulting from injury to the recurrent laryngeal nerve.

Previous
Next

Diet

No special dietary considerations are indicated in patients with a vascular ring other than those dictated by associated conditions. Postoperatively, enteral feeding is resumed as soon as possible.

In patients with dysphagia or emesis as a presenting symptom, adequate oral intake should be verified and feeding therapy instituted if necessary.

In patients with band 22q11 deletion, velopharyngeal insufficiency or cleft palate is frequently present; oral feedings should be resumed with the aid of feeding specialists.

Previous
Next

Activity

Patients with vascular rings are not subject to specific restrictions on activity.

Prior to repair, their activity may be limited by symptoms. Following repair, any persistent respiratory symptoms due to tracheomalacia should dictate activity limitations.

Previous
 
 
Contributor Information and Disclosures
Author

Doff B McElhinney, MD Assistant Professor of Pediatrics, Harvard Medical School; Associate in Cardiology, Department of Cardiology, Children's Hospital of Boston

Doff B McElhinney, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology

Disclosure: Nothing to disclose.

Coauthor(s)

Gil Wernovsky, MD, FACC, FAAP Professor, Department of Pediatrics, University of Pennsylvania, Children's Hospital of Philadelphia

Gil Wernovsky, MD, FACC, FAAP is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Ameeta Martin, MD Clinical Associate Professor, Department of Pediatric Cardiology, University of Nebraska College of Medicine

Ameeta Martin, MD is a member of the following medical societies: American College of Cardiology

Disclosure: Nothing to disclose.

Chief Editor

Howard S Weber, MD, FSCAI Professor of Pediatrics, Section of Pediatric Cardiology, Pennsylvania State University College of Medicine; Director of Interventional Pediatric Cardiology, Penn State Hershey Children's Hospital

Howard S Weber, MD, FSCAI is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, Society for Cardiovascular Angiography and Interventions

Disclosure: Received income in an amount equal to or greater than $250 from: St. Jude Medical.

Additional Contributors

Juan Carlos Alejos, MD Clinical Professor, Department of Pediatrics, Division of Cardiology, University of California, Los Angeles, David Geffen School of Medicine

Juan Carlos Alejos, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Medical Association, International Society for Heart and Lung Transplantation

Disclosure: Received honoraria from Actelion for speaking and teaching.

References
  1. Tuo G, Volpe P, Bava GL, et al. Prenatal diagnosis and outcome of isolated vascular rings. Am J Cardiol. 2009 Feb 1. 103(3):416-9. [Medline].

  2. McElhinney DB, McDonald-McGinn D, Zackai EH, Goldmuntz E. Cardiovascular anomalies in patients diagnosed with a chromosome 22q11 deletion beyond 6 months of age. Pediatrics. 2001 Dec. 108(6):E104. [Medline]. [Full Text].

  3. Chen X, Qu Y, Peng ZY, et al. Clinical value of multi-slice spiral computed tomography angiography and three-dimensional reconstruction in the diagnosis of double aortic arch. Exp Ther Med. 2014 Aug. 8(2):623-627. [Medline]. [Full Text].

  4. Ruzmetov M, Vijay P, Rodefeld MD, Turrentine MW, Brown JW. Follow-up of surgical correction of aortic arch anomalies causing tracheoesophageal compression: a 38-year single institution experience. J Pediatr Surg. 2009 Jul. 44(7):1328-32. [Medline].

  5. Achiron R, Rotstein Z, Heggesh J, et al. Anomalies of the fetal aortic arch: a novel sonographic approach to in-utero diagnosis. Ultrasound Obstet Gynecol. 2002 Dec. 20(6):553-7. [Medline].

  6. Anand R, Dooley KJ, Williams WH, Vincent RN. Follow-up of surgical correction of vascular anomalies causing tracheobronchial compression. Pediatr Cardiol. 1994 Mar-Apr. 15(2):58-61. [Medline].

  7. Angelini A, Dimopoulos K, Frescura C, et al. Fatal aortoesophageal fistula in two cases of tight vascular ring. Cardiol Young. 2002 Mar. 12(2):172-6. [Medline].

  8. Arciniegas E, Hakimi M, Hertzler JH, et al. Surgical management of congenital vascular rings. J Thorac Cardiovasc Surg. 1979 May. 77(5):721-7. [Medline].

  9. Backer CL, Ilbawi MN, Idriss FS, DeLeon SY. Vascular anomalies causing tracheoesophageal compression. Review of experience in children. J Thorac Cardiovasc Surg. 1989 May. 97(5):725-31. [Medline].

  10. Backer CL, Mavroudis C, Rigsby CK, Holinger LD. Trends in vascular ring surgery. J Thorac Cardiovasc Surg. 2005 Jun. 129(6):1339-47. [Medline].

  11. Bertrand JM, Chartrand C, Lamarre A, Lapierre JG. Vascular ring: clinical and physiological assessment of pulmonary function following surgical correction. Pediatr Pulmonol. 1986 Nov-Dec. 2(6):378-83. [Medline].

  12. Bonnard A, Auber F, Fourcade L, et al. Vascular ring abnormalities: a retrospective study of 62 cases. J Pediatr Surg. 2003 Apr. 38(4):539-43. [Medline].

  13. Burke RP, Rosenfeld HM, Wernovsky G, Jonas RA. Video-assisted thoracoscopic vascular ring division in infants and children. J Am Coll Cardiol. 1995 Mar 15. 25(4):943-7. [Medline].

  14. Cerillo AG, Amoretti F, Moschetti R, et al. Sixteen-row multislice computed tomography in infants with double aortic arch. Int J Cardiol. 2005 Mar 18. 99(2):191-4. [Medline].

  15. Chun K, Colombani PM, Dudgeon DL, Haller JA Jr. Diagnosis and management of congenital vascular rings: a 22-year experience. Ann Thorac Surg. 1992 Apr. 53(4):597-602; discussion 602-3. [Medline].

  16. Fleenor JT, Weinberg PM, Kramer SS, Fogel M. Vascular rings and their effect on tracheal geometry. Pediatr Cardiol. 2003 Sep-Oct. 24(5):430-5. [Medline].

  17. Giavini E, Prati M, Vismara C. Morphogenesis of aortic arch malformations in rat embryos after maternal treatment with glycerol formal during pregnancy. Acta Anat (Basel). 1981. 109(2):166-72. [Medline].

  18. Grathwohl KW, Afifi AY, Dillard TA, et al. Vascular rings of the thoracic aorta in adults. Am Surg. 1999 Nov. 65(11):1077-83. [Medline].

  19. Hartyanszky IL, Lozsadi K, Marcsek P, et al. Congenital vascular rings: surgical management of 111 cases. Eur J Cardiothorac Surg. 1989. 3(3):250-4. [Medline].

  20. Kocis KC, Midgley FM, Ruckman RN. Aortic arch complex anomalies: 20-year experience with symptoms, diagnosis, associated cardiac defects, and surgical repair. Pediatr Cardiol. 1997 Mar-Apr. 18(2):127-32. [Medline].

  21. Kogon BE, Forbess JM, Wulkan ML, Kirshbom PM, Kanter KR. Video-assisted thoracoscopic surgery: is it a superior technique for the division of vascular rings in children?. Congenit Heart Dis. 2007 Mar. 2(2):130-3. [Medline].

  22. Koontz CS, Bhatia A, Forbess J, Wulkan ML. Video-assisted thoracoscopic division of vascular rings in pediatric patients. Am Surg. 2005 Apr. 71(4):289-91. [Medline].

  23. Lillehei CW, Colan S. Echocardiography in the preoperative evaluation of vascular rings. J Pediatr Surg. 1992 Aug. 27(8):1118-20; discussion 1120-1. [Medline].

  24. McElhinney DB, Jacobs I, McDonald-McGinn DM, et al. Chromosomal and cardiovascular anomalies associated with congenital laryngeal web. Int J Pediatr Otorhinolaryngol. 2002 Oct 21. 66(1):23-27. [Medline].

  25. Mihaljevic T, Cannon JW, del Nido PJ. Robotically assisted division of a vascular ring in children. J Thorac Cardiovasc Surg. 2003 May. 125(5):1163-4. [Medline].

  26. Patel CR, Lane JR, Spector ML, Smith PC. Fetal echocardiographic diagnosis of vascular rings. J Ultrasound Med. 2006 Feb. 25(2):251-7. [Medline].

  27. Rimell FL, Shapiro AM, Meza MP, et al. Magnetic resonance imaging of the pediatric airway. Arch Otolaryngol Head Neck Surg. 1997 Sep. 123(9):999-1003. [Medline].

  28. van Son JA, Julsrud PR, Hagler DJ, et al. Imaging strategies for vascular rings. Ann Thorac Surg. 1994 Mar. 57(3):604-10. [Medline].

  29. Weinberg PM. Emmanouilides G, Reimenschneider T, Allen H, eds. Moss and Adams Heart Disease in Infants, Children and Adolscents. 5th ed. Baltimore, Md: Williams and Wilkins; 1995. 810-37.

  30. Yoo SJ, Min JY, Lee YH, et al. Fetal sonographic diagnosis of aortic arch anomalies. Ultrasound Obstet Gynecol. 2003 Nov. 22(5):535-46. [Medline].

 
Previous
Next
 
Left: Schematic diagram of the primitive pharyngeal arch system showing the left (L) and right (R) external carotid (EC) and internal carotid (IC) arteries, the fourth (IV) and sixth (VI) pharyngeal arches, distal pulmonary arterial segments (PA), dorsal aortas (DA), and seventh intersegmental arteries (VII). The proximal (p) sixth arches develop into the proximal pulmonary arteries, and the distal (d) sixth arches become the arterial ducts. The seventh intersegmental arteries develop into the subclavian arteries. Right: Schematic diagram depicting the segments of the pharyngeal arch system that regress (shown in black) in order for normal development of the great arteries and their thoracic branches (common carotid artery [CCA], left pulmonary artery [LPA], ductus arteriosus [PDA], right pulmonary artery [RPA], subclavian artery [SCA]).
Left: Schematic diagram depicting the segments of the pharyngeal arch system that regress (shown in black) in order for the development of a right aortic arch with aberrant left subclavian artery. Abbreviations are as in the first image. Right: Mature anatomy of a vascular ring formed by a right aortic arch with an aberrant left subclavian artery arising from a retroesophageal diverticulum with a left-sided ligamentum arteriosum to the left pulmonary artery.
Left: Schematic diagram depicting the segments of the pharyngeal arch system that regress (shown in black) in order for the development of a right aortic arch with mirror-image branching of the brachiocephalic vessels and a left-sided ductus arteriosus from the descending aorta to the left pulmonary artery. Abbreviations are as in the first image. Right: Mature anatomy of a vascular ring formed by a right aortic arch with mirror-image branching of the brachiocephalic vessels and a left-sided ductus arteriosus from the descending aorta to the left pulmonary artery.
Left: Schematic diagram depicting the segments of the pharyngeal arch system that regress (shown in black) in order for the development of a right aortic arch with aberrant retroesophageal left innominate artery. Abbreviations are as in the first image. Right: Mature anatomy of a vascular ring formed by a right aortic arch with an aberrant retroesophageal left innominate artery with a left-sided ligamentum arteriosum to the left pulmonary artery.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.