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Diagnostic accuracy and short-term implications of prenatally diagnosed vascular rings: a single center study

Abstract

Objective

To assess the diagnostic accuracy and postnatal outcomes of fetuses with a suspected vascular ring (VR).

Study design

Single-center retrospective study of patients with suspected VR by fetal echocardiography. Postnatal evaluation included echocardiography +/- cross-sectional imaging. Outcomes, including symptomatology and surgery, were recorded and compared between VR types.

Results

Of 68 suspected fetal VRs, 81% were confirmed postnatally. Concordance between fetal and postnatal anatomy was 76%. Five neonates required resuscitation unrelated to VR. Symptoms presented in 13 patients (median 45 days), independent of VR type. Surgical intervention was performed in 15 patients (median 99 days); 80% were symptomatic. At a median follow-up of 6 months, 4 patients were symptomatic including 3 postoperative.

Conclusions

Fetal echocardiography accurately identifies and characterizes VR anatomy in most instances. Specialized delivery planning in uncomplicated cases is unnecessary given absence of symptoms at birth. Fetal diagnosis may not indicate surgical disease, as symptoms do not occur in all and early surgery does not guarantee symptom resolution.

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Fig. 1: Representative fetal and postnatal imaging demonstrating the diagnosis of an isolated vascular ring.
Fig. 2
Fig. 3: Kaplan-Meier curve demonstrating freedom from symptoms in patients with a prenatally diagnosed isolated vascular ring.

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Data availability

The datasets generated during and/or analyzed during the current study are available from the corresponding author on reasonable request.

References

  1. Achiron R, Rotstein Z, Heggesh J, Bronshtein M, Zimand S, Lipitz S, et al. Anomalies of the fetal aortic arch: a novel sonographic approach to in-utero diagnosis. Ultrasound Obstet Gynecol. 2002;20:553–7.

    Article  CAS  PubMed  Google Scholar 

  2. Bronshtein M, Lorber A, Berant M, Auslander R, Zimmer EZ. Sonographic diagnosis of fetal vascular rings in early pregnancy. Am J Cardiol. 1998;81:101–3.

    Article  CAS  PubMed  Google Scholar 

  3. Yoo SJ, Lee YH, Kim ES, Ryu HM, Kim MY, Choi HK, et al. Three-vessel view of the fetal upper mediastinum: an easy means of detecting abnormalities of the ventricular outflow tracts and great arteries during obstetric screening. Ultrasound Obstet Gynecol. 1997;9:173–82.

    Article  CAS  PubMed  Google Scholar 

  4. Aly S, Papneja K, Mawad W, Seed M, Jaeggi E, Yoo SJ. Prenatal diagnosis of vascular ring: evaluation of fetal diagnosis and postnatal outcomes. J Am Soc Echocardiogr. 2022;35:312–21.

    Article  PubMed  Google Scholar 

  5. Stephens EH, Eltayeb O, Kennedy C, Rigsby CK, Rasatatter JC, Carr MR, et al. Influence of fetal diagnosis on management of vascular rings. Ann Thorac Surg. 2022;113:630–6.

    Article  PubMed  Google Scholar 

  6. Young AA, Hornberger LK, Haberer K, Fruitman D, Mills L, Noga M, et al. Prenatal detection, comorbidities, and management of vascular rings. Am J Cardiol. 2019;123:1703–8.

    Article  PubMed  Google Scholar 

  7. Peacock G, Kothari D, D’Orsogna L, Dickinson JE, Andrews D, Yim D. The impact of prenatal diagnosis on clinical outcomes of isolated vascular rings from a statewide paediatric cardiology tertiary service. Heart Lung Circ. 2023;32:735–44.

    Article  PubMed  Google Scholar 

  8. Milligan I, Border W, Sachdeva R, Michelfelder E. Contemporary outcomes in fetuses diagnosed with vascular rings. Pediatr Cardiol. 2024;45:1559–64.

  9. Donofrio MT, Moon-Grady AJ, Hornberger LK, Copel JA, Sklansky MS, Abuhamad A, et al. Diagnosis and treatment of fetal cardiac disease: a scientific statement from the American Heart Association. Circulation. 2014;129:2183–242.

    Article  PubMed  Google Scholar 

  10. Evans WN, Acherman RJ, Ciccolo ML, Lehoux J, Berthoty D, Mayman GA, et al. Isolated vascular rings in the era of high prenatal detection rates: Demographics, diagnosis, risk factors, and outcome. J Card Surg. 2021;36:1381–8.

    Article  PubMed  Google Scholar 

  11. Bakhru S, Koneti NR, Patil S, Dhulipudi B, Dash T, Kolar G, et al. Prenatal diagnosis of vascular rings and outcome. Ann Pediatr Cardiol. 2021;14:359–65.

    Article  PubMed  PubMed Central  Google Scholar 

  12. Tuo G, Volpe P, Bava GL, Bondanza S, De Robertis V, Pongiglione G, et al. Prenatal diagnosis and outcome of isolated vascular rings. Am J Cardiol. 2009;103:416–9.

    Article  PubMed  Google Scholar 

  13. Guo Q, Kong Y, Zeng S, Zhou J, Wang X, Shang Q, et al. Fetal double aortic arch: prenatal sonographic and postnatal computed tomography angiography features, associated abnormalities and clinical outcomes. BMC Pregnancy Childbirth. 2020;20:614.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  14. Afzal S, Fatima K, Ambareen M. Antenatal ultrasound diagnosis of congenital high airway obstruction syndrome: a case report and review of literature. Cureus. 2019;11:e4772.

    PubMed  PubMed Central  Google Scholar 

  15. Aslan H, Ekiz A, Acar DK, Aydiner B, Kaya B, Sezer S. Prenatal diagnosis of congenital high airway obstruction syndrome (CHAOS). Five case report. Med Ultrason. 2015;17:115–8.

    Article  PubMed  Google Scholar 

  16. D'Eufemia MD, Cianci S, Di Meglio F, Di Meglio L, Di Meglio L, Vitale SG, et al. Congenital high airway obstruction syndrome (CHAOS): discussing the role and limits of prenatal diagnosis starting from a single-center case series. J Prenat Med. 2016;10:4–7.

    Article  PubMed  PubMed Central  Google Scholar 

  17. Vigneswaran TV, Van Poppel MP, Griffiths B, James P, Jogeesvaran H, Rahim Z, et al. Postnatal impact of a prenatally diagnosed double aortic arch. Arch Dis Child. 2021;106:564–9.

    Article  PubMed  Google Scholar 

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Acknowledgements

Thank you to all the team members in the Children’s National Prenatal Cardiology Program and Echocardiology Laboratory for their contributions to this study, especially Megan Barbosa.

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Authors and Affiliations

Authors

Contributions

NMC, MLU, MHD, SJS, SRL, MTD contributed to the conception and design of the study. NMC, MLU, WH, and LB performed data acquisition. NMC and SJS performed data analysis. NMC wrote the original manuscript draft. All authors revised, reviewed, and approved the final manuscript.

Corresponding author

Correspondence to Mary T. Donofrio.

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Competing interests

The authors declare no competing interests.

Ethics approval and consent to participate

This study was approved and conducted under the Children’s National Hospital Institutional Review Board (Pro00011202) with a waiver of individual consent and in accordance with the Declaration of Helsinki.

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Ceneri, N.M., Udine, M.L., Desai, M.H. et al. Diagnostic accuracy and short-term implications of prenatally diagnosed vascular rings: a single center study. J Perinatol 45, 487–494 (2025). https://doi.org/10.1038/s41372-025-02244-x

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