- Amy L Juraszek, MD, FAAP, FACC
Amy L Juraszek, MD, FAAP, FACC
- Associate Professor of Pediatrics
- UT Southwestern Medical School
- David R Fulton, MD
David R Fulton, MD
- Section Editor — Pediatric Cardiology
- Associate Professor of Pediatrics
- Harvard Medical School
Vascular rings are congenital anomalies of the aortic arch that result in compression of the tracheobronchial tree and/or esophagus, leading to respiratory and gastrointestinal symptoms. They can be classified as either complete, when both the trachea and esophagus are fully encircled by a vascular anomaly, or incomplete without full encirclement of both structures.
The different forms of vascular rings and slings, and their clinical manifestations, diagnosis, and treatment are discussed here.
Beginning at the fourth week of embryogenesis, the aortic arch develops from six symmetrical paired aortic arch vessels and the paired dorsal aortae. During the next few weeks of embryogenesis, remodeling and rearrangement of these structures result in the formation of the normal left aortic arch [1,2].
●The right and left third arches persist as the right and left carotid arteries
●The left fourth arch persists as the transverse arch
- Davies M, Guest PJ. Developmental abnormalities of the great vessels of the thorax and their embryological basis. Br J Radiol 2003; 76:491.
- Licari A, Manca E, Rispoli GA, et al. Congenital vascular rings: a clinical challenge for the pediatrician. Pediatr Pulmonol 2015; 50:511.
- Shah RK, Mora BN, Bacha E, et al. The presentation and management of vascular rings: an otolaryngology perspective. Int J Pediatr Otorhinolaryngol 2007; 71:57.
- Humphrey C, Duncan K, Fletcher S. Decade of experience with vascular rings at a single institution. Pediatrics 2006; 117:e903.
- 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.
- Backer CL, Mavroudis C, Rigsby CK, Holinger LD. Trends in vascular ring surgery. J Thorac Cardiovasc Surg 2005; 129:1339.
- Turner A, Gavel G, Coutts J. Vascular rings--presentation, investigation and outcome. Eur J Pediatr 2005; 164:266.
- van Son JA, Konstantinov IE. Burckhard F. Kommerell and Kommerell's diverticulum. Tex Heart Inst J 2002; 29:109.
- Backer CL, Hillman N, Mavroudis C, Holinger LD. Resection of Kommerell's diverticulum and left subclavian artery transfer for recurrent symptoms after vascular ring division. Eur J Cardiothorac Surg 2002; 22:64.
- Powell AJ, Mandell VS. Vascular rings and slings. In: Nadas' Pediatric Cardiology, 2nd, Kean JF, Lock JE, Fyler DC (Eds), Saunders Elsevier, Philadelphia 2006. p.811.
- Bakker DA, Berger RM, Witsenburg M, Bogers AJ. Vascular rings: a rare cause of common respiratory symptoms. Acta Paediatr 1999; 88:947.
- 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; 108:E104.
- Hernanz-Schulman M. Vascular rings: a practical approach to imaging diagnosis. Pediatr Radiol 2005; 35:961.
- Fogel MA, Pawlowski TW, Harris MA, et al. Comparison and usefulness of cardiac magnetic resonance versus computed tomography in infants six months of age or younger with aortic arch anomalies without deep sedation or anesthesia. Am J Cardiol 2011; 108:120.
- Lillehei CW, Colan S. Echocardiography in the preoperative evaluation of vascular rings. J Pediatr Surg 1992; 27:1118.
- Tuo G, Volpe P, Bava GL, et al. Prenatal diagnosis and outcome of isolated vascular rings. Am J Cardiol 2009; 103:416.
- Jain S, Kleiner B, Moon-Grady A, Hornberger LK. Prenatal diagnosis of vascular rings. J Ultrasound Med 2010; 29:287.
- Li S, Luo G, Norwitz ER, et al. Prenatal diagnosis of congenital vascular rings and slings: sonographic features and perinatal outcome in 81 consecutive cases. Prenat Diagn 2011; 31:334.
- Yoo SJ, Min JY, Lee YH, et al. Fetal sonographic diagnosis of aortic arch anomalies. Ultrasound Obstet Gynecol 2003; 22:535.
- Patel CR, Lane JR, Spector ML, Smith PC. Fetal echocardiographic diagnosis of vascular rings. J Ultrasound Med 2006; 25:251.
- GROSS RE. Surgical relief for tracheal obstruction from a vascular ring. N Engl J Med 1945; 233:586.
- Backer CL, Mavroudis C, Dunham ME, Holinger LD. Pulmonary artery sling: results with median sternotomy, cardiopulmonary bypass, and reimplantation. Ann Thorac Surg 1999; 67:1738.
- Grillo HC, Wright CD, Vlahakes GJ, MacGillivray TE. Management of congenital tracheal stenosis by means of slide tracheoplasty or resection and reconstruction, with long-term follow-up of growth after slide tracheoplasty. J Thorac Cardiovasc Surg 2002; 123:145.
- Jones DT, Jonas RA, Healy GB. Innominate artery compression of the trachea in infants. Ann Otol Rhinol Laryngol 1994; 103:347.
- Koontz CS, Bhatia A, Forbess J, Wulkan ML. Video-assisted thoracoscopic division of vascular rings in pediatric patients. Am Surg 2005; 71:289.
- Al-Bassam A, Saquib Mallick M, Al-Qahtani A, et al. Thoracoscopic division of vascular rings in infants and children. J Pediatr Surg 2007; 42:1357.
- Suematsu Y, Mora BN, Mihaljevic T, del Nido PJ. Totally endoscopic robotic-assisted repair of patent ductus arteriosus and vascular ring in children. Ann Thorac Surg 2005; 80:2309.
- Kogon BE, Forbess JM, Wulkan ML, et al. Video-assisted thoracoscopic surgery: is it a superior technique for the division of vascular rings in children? Congenit Heart Dis 2007; 2:130.
- Marmon LM, Bye MR, Haas JM, et al. Vascular rings and slings: long-term follow-up of pulmonary function. J Pediatr Surg 1984; 19:683.
- Backer CL, Mongé MC, Russell HM, et al. Reoperation after vascular ring repair. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2014; 17:48.
- Left aortic arch
- Right aortic arch
- Complete vascular rings
- - Double aortic arch
- - R aortic arch with aberrant L subclavian artery and L ductus arteriosus/ligamentum
- - Other causes
- Incomplete vascular rings
- CLINICAL MANIFESTATIONS
- Presenting symptoms
- Associated anomalies
- Physical examination
- Our approach
- Antenatal diagnosis
- DIFFERENTIAL DIAGNOSIS
- Complete vascular rings
- Other vascular abnormalities (or anomalies)
- Alternative surgical techniques
- Pharmacologic treatment
- SUMMARY AND RECOMMENDATIONS