Skip to main content

Advertisement

SpringerLink
Log in

Narrowing Down the Symptomatology of Isolated Vascular Rings in Children

  • Research
  • Published:
Pediatric Cardiology Aims and scope Submit manuscript

Abstract

Vascular rings may cause respiratory or gastrointestinal symptoms due to compression of the trachea and/or esophagus. Advances in imaging have enabled early detection in asymptomatic patients posing new management dilemmas. Surgery is expected to relieve symptoms, although this has not been well studied. We sought to evaluate the presence and pattern of symptoms associated with vascular rings before surgical intervention and to detail symptom resolution after surgery. A 10-year retrospective review of patients diagnosed with an isolated vascular ring was performed between January 2010 and December 2019. 100 patients were identified; 35 double aortic arch (DAA) and 65 right aortic arch and left ligamentum arteriosum (RALL). 73 patients were symptomatic on presentation; 47 had respiratory, 5 had gastrointestinal, and 21 had both types of symptoms. Surgical repair was performed in 75 patients; 74 were symptomatic. Respiratory symptoms were more likely in patients with preoperative tracheal narrowing (p < 0.001). Moderate-severe respiratory symptoms led to surgery in RALL patients (OR 10.6, p = 0.0001). DAA patients were more likely to undergo surgery (p < 0.001) irrespective of symptom severity. At a median post-surgical follow-up of 4 months, there was a significant reduction in symptom burden (p < 0.001), except for asthma symptoms (p = 0.131). Symptom resolution was not dependent on the vascular ring anatomy (p = 0.331) or the age at operation (p = 0.158). Vascular rings are typically accompanied by respiratory symptoms and less commonly GI symptoms, both of which resolve in most patients after surgery. Those who present predominantly with asthma-like symptoms may not achieve resolution after surgery.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4

Similar content being viewed by others

References

  1. Gross RE (1945) Surgical relief for tracheal obstruction from a vascular ring. N Engl J Med 233:586–590. https://doi.org/10.1056/nejm194511152332003

    Article  CAS  PubMed  Google Scholar 

  2. Backer CL, Monge MC, Popescu AR, Eltayeb OM, Rastatter JC, Rigsby CK (2016) Vascular rings. Semin Pediatr Surg 25(3):165–175. https://doi.org/10.1053/j.sempedsurg.2016.02.009

    Article  PubMed  Google Scholar 

  3. Backer CL, Mavroudis C, Rigsby CK, Holinger LD (2005) Trends in vascular ring surgery. J Thorac Cardiovasc Surg 129(6):1339–1347. https://doi.org/10.1016/j.jtcvs.2004.10.044

    Article  PubMed  Google Scholar 

  4. Humphrey C, Duncan K, Fletcher S (2006) Decade of experience with vascular rings at a single institution. Pediatrics 117(5):e903–e908. https://doi.org/10.1542/peds.2005-1674

    Article  PubMed  Google Scholar 

  5. Woods RK et al (2001) Vascular anomalies and tracheoesophageal compression: a single institution’s 25-year experience. Ann Thorac Surg 72(2):434–438. https://doi.org/10.1016/s0003-4975(01)02806-5.

    Article  CAS  PubMed  Google Scholar 

  6. Subramanyan R, Venugopalan P, Narayan R (2003) Vascular rings: an important cause of persistent respiratory symptoms in infants and children. Indian Pediatr 40(10):951–957

    PubMed  Google Scholar 

  7. Braunstein P Jr, Sade R (1991) Vascular malformations with airway obstruction, The Pediatric Airways. WB Saunders, Philadelphia, pp 81–96

    Google Scholar 

  8. Nikaido H, Riker WL, Idriss FS (1972) Surgical management of “vascular rings.” Arch Surg 105(2):327–333. https://doi.org/10.1001/archsurg.1972.04180080173029

    Article  CAS  PubMed  Google Scholar 

  9. Marmon LM, Bye MR, Haas JM, Balsara RK, Dunn JM (1984) Vascular rings and slings: long-term follow-up of pulmonary function. J Pediatr Surg 19(6):683–692. https://doi.org/10.1016/s0022-3468(84)80353-x

    Article  CAS  PubMed  Google Scholar 

  10. van Son JA et al (1993) Surgical treatment of vascular rings: the Mayo Clinic experience. Mayo Clin Proc 68(11):1056–1063. https://doi.org/10.1016/s0025-6196(12)60898-2

    Article  PubMed  Google Scholar 

  11. Backer CL, Mavroudis C (2000) Congenital heart surgery nomenclature and database project: vascular rings, tracheal stenosis, Pectus excavatum. Ann Thorac Surg 69(4 Suppl):S308–S318. https://doi.org/10.1016/s0003-4975(99)01279-5

    Article  CAS  PubMed  Google Scholar 

  12. Bonnard A, Auber F, Fourcade L, Marchac V, Emond S, Revillon Y (2003) Vascular ring abnormalities: a retrospective study of 62 cases. J Pediatr Surg 38(4):539–543. https://doi.org/10.1053/jpsu.2003.50117

    Article  CAS  PubMed  Google Scholar 

  13. Stephens EH et al (2022) Influence of fetal diagnosis on management of vascular rings. Ann Thorac Surg 113(2):630–636. https://doi.org/10.1016/j.athoracsur.2021.01.025

    Article  PubMed  Google Scholar 

  14. Shah RK et al (2007) The presentation and management of vascular rings: an otolaryngology perspective. Int J Pediatr Otorhinolaryngol 71(1):57–62. https://doi.org/10.1016/j.ijporl.2006.08.025

    Article  PubMed  Google Scholar 

  15. Tola H et al (2017) Assessment of children with vascular ring. Pediatr Int 59(2):134–140. https://doi.org/10.1111/ped.13101

    Article  PubMed  Google Scholar 

  16. Fleenor JT, Weinberg PM, Kramer SS, Fogel M (2003) Vascular rings and their effect on tracheal geometry. Pediatr Cardiol 24(5):430–435. https://doi.org/10.1007/s00246-002-0385-z

    Article  CAS  PubMed  Google Scholar 

  17. Callahan CP, Merritt TC, Canter MW, Eghtesady P, Manning PB, Abarbanell AM (2020) Symptom persistence after vascular ring repair in children. J Pediatr Surg 10:2020. https://doi.org/10.1016/j.jpedsurg.2019.12.022

    Article  Google Scholar 

  18. Pereira Gonzalez MJ, Barranco P, Belver MT, Lopez-Serrano MC (2003) Vascular rings can give cough. Allergy 58(1):86–87. https://doi.org/10.1034/j.1398-9995.2003.23710_3.x

    Article  CAS  PubMed  Google Scholar 

  19. Licari A, Manca E, Rispoli GA, Mannarino S, Pelizzo G, Marseglia GL (2015) Congenital vascular rings: a clinical challenge for the pediatrician. Pediatr Pulmonol 50(5):511–524. https://doi.org/10.1002/ppul.23152

    Article  PubMed  Google Scholar 

  20. Valletta EA, Pregarz M, Bergamo-Andreis IA, Boner AL (1997) Tracheoesophageal compression due to congenital vascular anomalies (vascular rings). Pediatr Pulmonol 24(2):93–105. https://doi.org/10.1002/(sici)1099-0496(199708)24:2%3c93::aid-ppul4%3e3.0.co;2-j

    Article  CAS  PubMed  Google Scholar 

  21. Chun K, Colombani PM, Dudgeon DL, Haller JA Jr (1992) Diagnosis and management of congenital vascular rings: a 22-year experience. Ann Thorac Surg 53(4):597–602. https://doi.org/10.1016/0003-4975(92)90317-w

    Article  CAS  PubMed  Google Scholar 

  22. Ruzmetov M, Vijay P, Rodefeld MD, Turrentine MW, Brown JW (2009) Follow-up of surgical correction of aortic arch anomalies causing tracheoesophageal compression: a 38-year single institution experience. J Pediatr Surg 44(7):1328–1332. https://doi.org/10.1016/j.jpedsurg.2008.11.062

    Article  PubMed  Google Scholar 

  23. Binsalamah ZM et al (2020) Contemporary midterm outcomes in pediatric patients undergoing vascular ring repair. Ann Thorac Surg 109(2):566–572. https://doi.org/10.1016/j.athoracsur.2019.06.076

    Article  PubMed  Google Scholar 

  24. Yang HJ, Pyun BY (2010) Refractory asthma coexisting with a double aortic arch: a case report and literature review. J Asthma 47(10):1165–1167. https://doi.org/10.3109/02770903.2010.525676

    Article  PubMed  Google Scholar 

  25. Uchida DA (2009) Late presentation of double aortic arch in school-age children presumed to have asthma: the benefits of spirometry and examination of the flow-volume curve. Respir Care 54(10):1402–1404

    PubMed  Google Scholar 

  26. Stoica SC, Lockowandt U, Coulden R, Ward R, Bilton D, Dunning J (2002) Double aortic arch masquerading as asthma for thirty years. Respiration 69(1):92–95. https://doi.org/10.1159/000049378

    Article  PubMed  Google Scholar 

  27. Lone GN, Rathore SS, Malik JA, Ashraf HZ, Qadri AA (2012) Double aortic arch masquerading as bronchial asthma for five decades. Asian Cardiovasc Thorac Ann 20(3):338–340. https://doi.org/10.1177/0218492311435818

    Article  PubMed  Google Scholar 

  28. Payne DN, Lincoln C, Bush A (2000) Lesson of the week: right sided aortic arch in children with persistent respiratory symptoms. BMJ 321(7262):687–688

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  29. Parker JM, Cary-Freitas B, Berg BW (2000) Symptomatic vascular rings in adulthood: an uncommon mimic of asthma. J Asthma 37(3):275–280. https://doi.org/10.3109/02770900009055450

    Article  CAS  PubMed  Google Scholar 

  30. Backer CL, Ilbawi MN, Idriss FS, DeLeon SY (1989) Vascular anomalies causing tracheoesophageal compression. Review of experience in children. J Thorac Cardiovasc Surg 97(5):725–731

    Article  CAS  PubMed  Google Scholar 

  31. Roesler M, De Leval M, Chrispin A, Stark J (1983) Surgical management of vascular ring. Ann Surg 197(2):139–146. https://doi.org/10.1097/00000658-198302000-00004

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  32. Bertolini A et al (1987) Vascular rings and slings. Diagnosis and surgical treatment of 49 patients. J Cardiovasc Surg (Torino) 28(3):301–312

    CAS  PubMed  Google Scholar 

Download references

Acknowledgements

The authors would like to thank graphical illustrator Sofia S. Hanabergh for her creation of the figures.

Funding

No funding was received for this study.

Author information

Authors and Affiliations

  1. Division of Cardiology, Children’s National Hospital, Washington, DC, 20010, USA

    Nicolle M. Ceneri, Eleanor F. Gerhard & Mary T. Donofrio

  2. Division of Cardiovascular Surgery, Children’s National Hospital, Washington, DC, 20010, USA

    Manan H. Desai & Karthik Ramakrishnan

  3. Division of Cardiology, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA

    Adam B. Christopher

  4. Departments of Anesthesiology and Surgery, Harvard Medical School, Boston Children’s Hospital, Boston, MA, USA

    Steven J. Staffa & David Zurakowski

Authors
  1. Nicolle M. Ceneri
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Manan H. Desai
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Adam B. Christopher
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Eleanor F. Gerhard
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Steven J. Staffa
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. David Zurakowski
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Karthik Ramakrishnan
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Mary T. Donofrio
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

NMC, MHD, ABC, KR, and MTD contributed to the study conception and design. NMC, MD, ABC, and EFG performed the data acquisition. NMC, MHD, ABC, SJS, and DZ performed the statistical analyses. NMC, MHD, ABC, and MTD designed the figures. NMC, SJS, and DZ created the tables. NMC, MHD, SJS, DZ, and MTD drafted the manuscript text. All authors were involved in revising and reviewing the manuscript for accuracy and intellectual content.

Corresponding author

Correspondence to Mary T. Donofrio.

Ethics declarations

Conflict of interest

The authors have no relevant financial or non-financial interests to disclose for this study.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Supplementary Information

Rights and permissions

Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Ceneri, N.M., Desai, M.H., Christopher, A.B. et al. Narrowing Down the Symptomatology of Isolated Vascular Rings in Children. Pediatr Cardiol 45, 416–425 (2024). https://doi.org/10.1007/s00246-023-03301-y

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00246-023-03301-y

Keywords

Search

Navigation