Skip to main content

Advertisement

Log in

Computed Tomography Measurement of the Aorta in Midaortic Syndrome in Children and Adolescents and Their Clinical Manifestations and Outcomes

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

Abstract

Midaortic syndrome (MAS) presents challenges in diagnosis due to the absence of well-defined diagnostic criteria in pediatric patients. This retrospective study aimed to aid in the diagnosis of MAS by employing computed tomography (CT) to measure the z-score of the aorta as well as to identify and understand its clinical features. CT images, echocardiography findings, and medical records of 17 patients diagnosed with MAS between 1997 and 2023 were reviewed, and z-scores were calculated. Aortic size on follow-up CT, blood pressure, and left ventricular function and hypertrophy at the last follow-up were analyzed, and possible prognostic factors were examined. Except for one patient, all individuals exhibited a z-score below − 2 at the level corresponding to stenosis. Left ventricular dysfunction occurred more frequently in patients aged < 5 years (p = 0.024). Patients with idiopathic MAS showed a better prognosis in terms of blood pressure and follow-up aortic size (p = 0.051 and 0.048, respectively). CT-measured aortic z-scores may be useful for the diagnosis and follow-up of MAS.

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

Access this article

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

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

Data Availability

All data generated or analyzed during this study are included in this published article.

References

  1. Rumman RK, Nickel C, Matsuda-Abedini M, Lorenzo AJ, Langlois V et al (2015) Disease beyond the Arch: a systematic review of Middle aortic syndrome in Childhood. Am J Hypertens 28:833–846. https://doi.org/10.1093/ajh/hpu296

    Article  PubMed  Google Scholar 

  2. O’Neill JA Jr., Berkowitz H, Fellows KJ, Harmon CM (1995) Midaortic syndrome and Hypertension in childhood. J Pediatr Surg 30:164–171 discussion 171 – 162. https://doi.org/10.1016/0022-3468(95)90555-3

    Article  PubMed  Google Scholar 

  3. Panayiotopoulos YP, Tyrrell MR, Koffman G, Reidy JF, Haycock GB et al (1996) Mid-aortic syndrome presenting in childhood. Br J Surg 83:235–240. https://doi.org/

    PubMed  CAS  Google Scholar 

  4. Tummolo A, Marks SD, Stadermann M, Roebuck DJ, McLaren CA et al (2009) Mid-aortic syndrome: long-term outcome of 36 children. Pediatr Nephrol 24:2225–2232. https://doi.org/10.1007/s00467-009-1242-6

    Article  PubMed  Google Scholar 

  5. Porras D, Stein DR, Ferguson MA, Chaudry G, Alomari A et al (2013) Midaortic syndrome: 30 years of experience with medical, endovascular and surgical management. Pediatr Nephrol 28:2023–2033. https://doi.org/10.1007/s00467-013-2514-8

    Article  PubMed  Google Scholar 

  6. Brunet-Garcia L, Prada Martínez FH, Lopez Sainz A, Sanchez-de-Toledo J, Carretero Bellon JM (2023) Mid-aortic syndrome in a Pediatric Cohort. Pediatr Cardiol 44:168–178. https://doi.org/10.1007/s00246-022-03036-2

    Article  PubMed  Google Scholar 

  7. Flynn JT, Kaelber DC, Baker-Smith CM, Blowey D, Carroll AE et al (2017) Clinical practice Guideline for Screening and Management of High Blood Pressure in children and adolescents. https://doi.org/10.1542/peds.2017-1904. Pediatrics 140

  8. Khoury PR, Mitsnefes M, Daniels SR, Kimball TR (2009) Age-specific reference intervals for indexed left ventricular mass in children. J Am Soc Echocardiogr 22:709–714. https://doi.org/10.1016/j.echo.2009.03.003

    Article  PubMed  Google Scholar 

  9. Hegde SV, Lensing SY, Greenberg SB (2015) Determining the normal aorta size in children. Radiology 274:859–865. https://doi.org/10.1148/radiol.14140500

    Article  PubMed  Google Scholar 

  10. Fillinger MF, Greenberg RK, McKinsey JF, Chaikof EL (2010) Reporting standards for thoracic endovascular aortic repair (TEVAR). J Vasc Surg 52:1022–1033 1033.e1015. https://doi.org/10.1016/j.jvs.2010.07.008

    Article  PubMed  Google Scholar 

  11. Patel RS, Nguyen S, Lee MT, Price MD, Krause H et al (2020) Clinical characteristics and long-term outcomes of midaortic syndrome. Ann Vasc Surg 66:318–325. https://doi.org/10.1016/j.avsg.2019.12.039

    Article  PubMed  Google Scholar 

  12. Kim SY, Kim MJ, Kim SJ, Lee JE, Chae JH et al (2021) A case of CHOPS syndrome accompanied with moyamoya Disease and systemic vasculopathy. Brain Dev 43:454–458. https://doi.org/10.1016/j.braindev.2020.11.004

    Article  PubMed  CAS  Google Scholar 

  13. Ahn KJ, Yoon JK, Kim GB, Kwon BS, Bae EJ et al (2016) Idiopathic midaortic syndrome with malignant Hypertension in 3-year-old boy. Korean J Pediatr 59:S84–S87. https://doi.org/10.3345/kjp.2016.59.11.S84

    Article  PubMed  PubMed Central  Google Scholar 

  14. Zhao L, Zhu L, Zhao QM, He L, Liang XC et al (2022) Mid-aortic syndrome: a rare cause of Heart Failure in infants. ESC Heart Fail 9:3619–3624. https://doi.org/10.1002/ehf2.14066

    Article  PubMed  PubMed Central  Google Scholar 

  15. Stiller B, Weng Y, Berger F (2006) Images in cardiology. Mid aortic syndrome: a rare cause of reversible cardiomyopathy. Heart 92:640. https://doi.org/10.1136/hrt.2005.073395

    Article  PubMed  PubMed Central  CAS  Google Scholar 

Download references

Funding

This study was not funded in any way and has no financial or non-financial interests.

Author information

Authors and Affiliations

Authors

Contributions

All authors contributed to the study conception and design. GBK suggested an idea regarding the difficulties of diagnosing midaortic syndrome due to lack of established references for aortic size in the CT, and proposed of conducting research. Data collection and analysis were performed by SMB. Computed tomography image and Doppler kidney sonography was reviewed by YSL and Echocardiography was reviewed by MKS and EJB. The first draft of the manuscript was written by SMB and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

Corresponding author

Correspondence to Gi Beom Kim.

Ethics declarations

Ethical Approval

This study was approved by the Institutional Review Board of the Seoul National University Hospital.

Competing Interests

The authors declare no competing interests.

Additional information

Publisher’s Note

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

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

Baek, S.M., Lee, Y.S., Song, M.K. et al. Computed Tomography Measurement of the Aorta in Midaortic Syndrome in Children and Adolescents and Their Clinical Manifestations and Outcomes. Pediatr Cardiol (2024). https://doi.org/10.1007/s00246-023-03399-0

Download citation

  • Received:

  • Accepted:

  • Published:

  • DOI: https://doi.org/10.1007/s00246-023-03399-0

Keywords

Navigation