Korean Circ J.  2012 Apr;42(4):252-258. 10.4070/kcj.2012.42.4.252.

Clinical Course of Vascular Rings and Risk Factors Associated With Mortality

• Suh YJ
• Kim GB
• Kwon BS
• Bae EJ
• Noh CI
• Lim HG
• Kim WH
• Lee JR
• Kim YJ

• KMID: 2225018
• DOI: http://doi.org/10.4070/kcj.2012.42.4.252

Abstract

BACKGROUND AND OBJECTIVES
Vascular rings refer to anomalies of the great arteries that cause respiratory or feeding problems. The purpose of this study was to analyze a series of patients with vascular rings and evaluate associated risk factors for mortality.
SUBJECTS AND METHODS
A retrospective review of all patients identified with vascular rings between 1997 and 2010 in the Seoul National University Children's Hospital.
RESULTS
Thirty-five patients were diagnosed with vascular rings (median age at diagnosis, 7 months). The vascular rings of 32 patients were confirmed by cardiac computed tomography or magnetic resonance imaging. The types of vascular rings were: a double aortic arch in ten patients, a right aortic arch with persistent left ligamentum arteriosum in seven, an aberrant subclavian artery in seven, a pulmonary sling in eight, and others types in three patients. Eleven patients were asymptomatic. Gastrointestinal and respiratory symptoms were seen in ten and sixteen patients, respectively. Associated cardiovascular anomalies were present in fifteen patients. Twenty patients with definite symptoms underwent surgical treatment. The median interval between diagnosis and operation was 6 days. Four patients eventually died; three deaths were associated with complex heart diseases, and one had pulmonary artery sling with severe tracheal stenosis. Only the presence of a complex heart disease significantly influenced mortality (p=0.002).
CONCLUSION
Vascular rings include several types of anomalies, each with a different prognosis and symptoms. The presence of a complex heart disease was significantly associated with mortality. Early diagnosis and timely surgery in symptomatic patients are essential.

Keyword

Vascular rings; Congenital heart disease; Pulmonary artery sling; Clinical characteristics; Mortality

MeSH Terms

Aneurysm
Aorta, Thoracic
Arteries
Cardiovascular Abnormalities
Deglutition Disorders
Early Diagnosis
Heart Diseases
Humans
Magnetic Resonance Imaging
Prognosis
Pulmonary Artery
Retrospective Studies
Risk Factors
Subclavian Artery
Tracheal Stenosis
Aneurysm
Cardiovascular Abnormalities
Deglutition Disorders
Subclavian Artery

Figure

• 

  Fig. 1 The three-dimensional structures of diverse types of vascular rings, reconstructed from cardiac computed tomography. A: double aortic arch; two aortic arches (white bold arrows) comprise the complete ring in this patient. B: right aortic arch with persistent left ligamentum arteriosum; Kommerell's diverticulum (arrowhead) and fibrotic band (white dotted arrows) comprise the complete ring in this patient. C: right aortic arch with aberrant left subclavian artery (red bold arrow). D: pulmonary sling; left pulmonary artery originates from right pulmonary artery (red dotted arrow), compressing the bronchus.

Reference

1. Kellenberger CJ. Aortic arch malformations. Pediatr Radiol. 2010. 40:876–884.

2. Hernanz-Schulman M. Vascular rings: a practical approach to imaging diagnosis. Pediatr Radiol. 2005. 35:961–979.

3. 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. 44:1328–1332.

4. Banka P, Geva T, Powell AJ, Geggel R, Lahiri T, Valente AM. Images in cardiovascular medicine: right aortic arch with aberrant left innominate artery: a rare vascular ring. Circulation. 2009. 120:264–265.

5. Eichhorn J, Fink C, Delorme S, Ulmer H. Rings, slings and other vascular abnormalities: ultrafast computed tomography and magnetic resonance angiography in pediatric cardiology. Z Kardiol. 2004. 93:201–208.

6. Humphrey C, Duncan K, Fletcher S. Decade of experience with vascular rings at a single institution. Pediatrics. 2006. 117:e903–e908.

7. Dodge-Khatami A, Tulevski II, Hitchcock JF, de Mol BA, Bennink GB. Vascular rings and pulmonary arterial sling: from respiratory collapse to surgical cure, with emphasis on judicious imaging in the hi-tech era. Cardiol Young. 2002. 12:96–104.

8. Park MK. Fletcher J, McGonigal C, editors. Vascular ring. Pediatric Cardiology for Practitioners. 2008. 5th ed. Philadelphia: Mosby Elsevier;303–306.

9. Binet JP, Longlois J. Aortic arch anomalies in children and infants. J Thorac Cardiovasc Surg. 1977. 73:248.

10. Gross RE. Surgical relief for tracheal obstruction from a vascular ring. N Engl J Med. 1945. 233:586–590.

11. Han BS, Kim CH, Oh BH, et al. A case of right sided aortic arch causing superior vena cava syndrome. Korean Circ J. 1989. 19:776–779.

12. Bonnard A, Auber F, Fourcade L, Marchac V, Emond S, Révillon Y. Vascular ring abnormalities: a retrospective study of 62 cases. J Pediatr Surg. 2003. 38:539–543.

13. Turner A, Gavel G, Coutts J. Vascular rings: presentation, investigation and outcome. Eur J Pediatr. 2005. 164:266–270.

14. Woods RK, Sharp RJ, Holcomb GW 3rd, et al. Vascular anomalies and tracheoesophageal compression: a single institution's 25-year experience. Ann Thorac Surg. 2001. 72:434–438.

15. Alsenaidi K, Gurofsky R, Karamlou T, Williams WG, McCrindle BW. Management and outcomes of double aortic arch in 81 patients. Pediatrics. 2006. 118:e1336–e1341.

16. 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. 18:127–132.