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Early and late outcomes after surgical management of congenital vascular rings

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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

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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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Authors and Affiliations

  1. Department of Congenital Cardiac Surgery, Cardiac Center, University Hospital Ghent, Ghent, Belgium

    Katrien François & Thierry Bové

  2. Department Pediatric Cardiology, Cardiac Center, University Hospital Ghent, Ghent, Belgium

    Joseph Panzer, Katya De Groote, Kristof Vandekerckhove, Daniel De Wolf & Hans De Wilde

  3. Department of Pediatric Cardiology, University Hospital Antwerp, Antwerp, Belgium

    Fabienne Marchau

  4. Department of Neonatology, Sint-Jan Hospital, Bruges, Belgium

    Wim De Caluwe

  5. Department of Pediatric Cardiology, University Hospital Brussels, Brussels, Belgium

    Abraham Benatar

Authors
  1. Katrien François
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  2. Joseph Panzer
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  3. Katya De Groote
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  4. Kristof Vandekerckhove
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  5. Daniel De Wolf
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  6. Hans De Wilde
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  7. Fabienne Marchau
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  8. Wim De Caluwe
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  9. Abraham Benatar
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  10. Thierry Bové
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Corresponding author

Correspondence to Katrien François.

Ethics declarations

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.

Conflict of interest

The authors declare that they have no conflict of interest.

Additional information

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

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