Abstract
Persistent respiratory or feeding problems in children may be associated with a congenital vascular ring. Surgical management is fairly standardized, but long-term outcomes are not well described. This study aims to investigate clinical presentation, surgical treatment, and risk factors for early mortality and late outcome. Our database revealed 62 surgically treated vascular ring patients between 1993 and 2014. Double aortic arch was the most common diagnosis (53%). Median age at operation was 1 year. Symptoms were mainly respiratory (89%) and feeding problems (32%). Median extubation time and hospital stay were 4 h and 5 days. Mean follow-up was 7.8 ± 5.8 years. Early mortality was 8% and was related to anatomical diagnosis, concomitant anomalies, and a need for preoperative intubation. Freedom from residual symptoms at 1 and 6 months was 63 and 82%, respectively. Freedom from inhalation therapy at the last follow-up was 82% and was influenced by a type of vascular ring and preoperative ventilation. Dysphagia symptoms always disappeared.
Conclusion: Surgical relief of tracheoesophageal compression is commonly effective in vascular ring anomalies. Respiratory symptoms necessitating chronic inhalation therapy only persist in a minority of children. Patients with double aortic arch are at increased risk to remain symptomatic, particularly with infectious exacerbations.
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Abbreviations
- CT:
-
Computed tomography
- DAA:
-
Double aortic arch
- LAARSA:
-
Left aortic arch with right aberrant/retroesophageal subclavian artery
- MRI:
-
Magnetic resonance imaging
- PS:
-
Pulmonary artery sling
- RAALSA:
-
Right aortic arch with aberrant left subclavian artery
- GI:
-
Gastrointestinal
References
Backer CL, Mavroudis C (2000) Congenital heart surgery nomenclature and database project: vascular rings, tracheal stenosis, pectus excavatum. Ann Thorac Surg 69(Suppl.1):308–318
Cheng W, Manson DE, Forte V, Ein SH, MacLusky I, Papsin BC, Hechter S, Kim PC (2006) The role of conservative management in congenital tracheal stenosis: an evidence-based long-term follow-up study. J Pediatr Surg 41:1203–1207
Chiu PPL, Rusan M, Williams WG, Caldarone CA, Kim PCW (2006) Long-term outcomes of clinically significant vascular rings associated with congenital tracheal stenosis. J Pediatr Surg 41:335–341
Chun K, Colombani PM, Dudgeon DL, Haller JA (1992) Diagnosis and management of congenital vascular rings: a 22-year experience. Ann Thorac Surg 53:597–603
Dunn JM, Gordon I, Chrispin AR, de Leval MR, Stark J (1979) Early and late results of surgical correction of pulmonary artery sling. Ann Thorac Surg 28:230–238
Hartyánszky IL, Lozsadi K, Marcsek P, Hüttl T, Sápi E, Kovács AB (1989) Congenital vascular rings: surgical management of 111 cases. Eur J Cardiothorac Surg 3:250–254
Kir M, Saylam GS, Karadas U, Yilmaz N, Cakmakçi H, Uzuner N, Güzeloglu M, Ugurlu B, Oto O (2012) Vascular rings: presentation, imaging strategies, treatment and outcome. Pediatr Cardiol 33:607–617
Leijala M, Sairanen H, Mäkinen L, Maunuksela EL, Louhimo I (1989) Iatrogenic, unexpected and other vascular rings in children. Eur J Cardiothorac Surg 3:125–129
Moss AJ, Adams FH, Emmanouilides GC (1977) Heart disease in infants, children and adolescents. Williams and Wilkins, Baltimore
Sebening C, Jakob H, Tochtermann U, Lange R, Vahl CF, Bodegom P, Szabo G, Fleischer F, Schmidt K, Zilow E, Springer W, Ulmer HE, Hagl S (2000) Vascular tracheobronchial compression syndromes. Experience in surgical treatment and literature review. Thorac Cardiovasc Surg 48:164–174
Shanmugam G, Macarthur K, Pollock J (2005) Surgical repair of double aortic arch: 16-year experience. Asian Cardiovasc Thorac Ann 13:4–10
Stewart JR, Kincaid OW, Edwards JE (1964) Atlas of vascular rings and related malformations of the aortic arch system. Charles. C. Thomas, Springfield, IL
Suh YJ, Kim GB, Kwon BS, Bae EJ, Noh CI, Lim HG, Kim WH, Lee JR, Kim YJ (2012) Clinical course of vascular rings and risk factors associated with mortality. Korean Circ J 42:252–258
Turner A, Gavel G, Coutts J (2005) Vascular rings—presentation, investigation and outcome. Eur J Pediatr 164:266–270
Authors’ Contributions
K. François: concept of the study - database management - final patient follow-up - statistical analysis - writing of the manuscript. J. Panzer, K. De Groote, K. Vandekerckhove, D. De Wolf, H. De Wilde, F. Marchau, W. De Caluwe, A. Benatar: provider of patients - outpatient follow-up - review of the manuscript. T. Bové: statistical analysis - review and editing of the manuscript.
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None of the authors received financial support for this study.
Because this study was a retrospective study, the need for informed consent was waived by the local Ethical Committee of the University Hospital Ghent. All procedures performed in this study were in accordance with the ethical standards of the institutional research committee and with the 1964 Helsinki declaration and its later amendments.
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Communicated by Jaan Toelen
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François, K., Panzer, J., De Groote, K. et al. Early and late outcomes after surgical management of congenital vascular rings. Eur J Pediatr 176, 371–377 (2017). https://doi.org/10.1007/s00431-017-2850-y
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DOI: https://doi.org/10.1007/s00431-017-2850-y