Skip to main content
SpringerLink
Log in

Complimentary Cardiac Computed Tomography Ventricular Volumetry-Derived Metrics of Severity in Patients with Ebstein Anomaly: Comparison with Echocardiography-Based Severity Indices

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

Abstract

Detailed three-dimensional cardiac segmentations using cardiac computed tomography (CT) data is technically feasible in patients with Ebstein anomaly, but its complementary role has not been evaluated. This single-center, retrospective study was aimed to evaluate the complementary role of cardiac CT ventricular volumetry in evaluating the severity of Ebstein anomaly. Preoperative cardiac CT ventricular volumetry was performed in 21 children with Ebstein anomaly. CT-based ventricular functional measures were compared between Carpentier types, and between definitive surgical repair types. The Celermajer severity index measured with echocardiography was correlated with CT-based functional parameters. Total right ventricle (RV) and functional RV (fRV) volumes, fRV fraction, fRV/left ventricle (LV) volume ratio, and end-diastolic CT severity index demonstrated statistically significant differences between Carpentier type A/B and Carpentier type C/D (p < 0.05). The Celermajer severity index measured with echocardiography showed a high positive correlation with the end-diastolic CT severity index (R = 0.720, p < 0.002). There were no statistically significant differences in both echocardiography- and CT-based functional measures between patients with biventricular repair and patients with one-and-a-half or univentricular repair (p > 0.05). Compared with echocardiography, cardiac CT ventricular volumetry can provide the severity of Ebstein anomaly objectively and may be used in select patients when echocardiographic results are inconclusive or inconsistent.

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

Similar content being viewed by others

Data Availability

Data are held in a secure, password protected format at the medical center.

References

  1. Alsaied T, Christopher AB, Da Silva J, Gupta A, Morell VO, Lanford L et al (2023) Multimodality imaging in Ebstein anomaly. Pediatr Cardiol 44:15–23. https://doi.org/10.1007/s00246-022-03011-x

    Article  PubMed  Google Scholar 

  2. Neumann S, Rüffer A, Sachweh J, Biermann D, Herrmann J, Jerosch-Herold M et al (2021) Narrative review of Ebstein’s anomaly beyond childhood: Imaging, surgery, and future perspectives. Cardiovasc Diagn Ther 11:1310–1323. https://doi.org/10.21037/cdt-20-771

    Article  PubMed  PubMed Central  Google Scholar 

  3. Carpentier A, Chauvaud S, Macé L, Relland J, Milhaileanu S, Marino JP et al (1988) A new reconstructive operation for Ebstein’s anomaly of the tricuspid valve. J Thorac Cardiovasc Surg 96:92–101

    Article  CAS  PubMed  Google Scholar 

  4. Celermajer DS, Bull C, Till JA, Cullen S, Vassillikos VP, Sullivan ID et al (1994) Ebstein’s anomaly: presentation and outcome from fetus to adult. J Am Coll Cardiol 23:170–176. https://doi.org/10.1016/0735-1097(94)90516-9

    Article  CAS  PubMed  Google Scholar 

  5. Cieplucha A, Trojnarska O, Bartczak-Rutkowska A, Kociemba A, Rajewska-Tabor J, Kramer L et al (2019) Severity scores for Ebstein anomaly: credibility and usefulness of echocardiographic vs magnetic resonance assessments of the Celermajer index. Can J Cardiol 35:1834–1841. https://doi.org/10.1016/j.cjca.2019.08.003

    Article  PubMed  Google Scholar 

  6. Geerdink LM, van Everdingen WM, Kuipers IM, Fejzic Z, du Marchie Sarvaas GJ, Frerich S et al (2023) Comprehensive evaluation of pediatric patients with Ebstein anomaly requires both echocardiography and cardiac magnetic resonance imaging. Pediatr Cardiol 44:75–85. https://doi.org/10.1007/s00246-022-02948-3

    Article  PubMed  Google Scholar 

  7. Hösch O, Sohns JM, Nguyen TT, Lauerer P, Rosenberg C, Kowallick JT et al (2014) The total right/left-volume index: a new and simplified cardiac magnetic resonance measure to evaluate the severity of Ebstein anomaly of the tricuspid valve: a comparison with heart failure markers from various modalities. Circ Cardiovasc Imaging 7:601–609. https://doi.org/10.1161/CIRCIMAGING.113.001467

    Article  PubMed  Google Scholar 

  8. Rydman R, Shiina Y, Diller GP, Niwa K, Li W, Uemura H et al (2018) Major adverse events and atrial tachycardia in Ebstein’s anomaly predicted by cardiovascular magnetic resonance. Heart 104:37–44. https://doi.org/10.1136/heartjnl-2017-311274

    Article  PubMed  Google Scholar 

  9. Goo HW (2019) Volumetric severity assessment of Ebstein anomaly using three-dimensional cardiac CT: a feasibility study. Cardiovasc Imaging Asia 3:61–67. https://doi.org/10.22468/cvia.2019.00052

    Article  Google Scholar 

  10. Celermajer DS, Cullen S, Sullivan ID, Spiegelhalter DJ, Wyse RK, Deanfield JE (1992) Outcome in neonates with Ebstein’s anomaly. J Am Coll Cardiol 19:1041–1046. https://doi.org/10.1016/0735-1097(92)90291-t

    Article  CAS  PubMed  Google Scholar 

  11. Negoi RI, Ispas AT, Ghiorghiu I, Filipoiu F, Negoi I, Hostiuc M et al (2013) Complex Ebstein’s malformation: defining preoperative cardiac anatomy and function. J Card Surg 28:70–81. https://doi.org/10.1111/jocs.12032

    Article  PubMed  Google Scholar 

  12. Goo HW (2010) State-of-the-art CT imaging techniques for congenital heart disease. Korean J Radiol 11:4–18. https://doi.org/10.3348/kjr.2010.11.1.4

    Article  PubMed  Google Scholar 

  13. Goo HW (2011) Individualized volume CT dose index determined by cross-sectional area and mean density of the body to achieve uniform image noise of contrast-enhanced pediatric chest CT obtained at variable kV levels and with combined tube current modulation. Pediatr Radiol 41:839–847. https://doi.org/10.1007/s00247-011-2121-4

    Article  PubMed  Google Scholar 

  14. Goo HW (2018) Comparison of chest pain protocols for electrocardiography-gated dual-source cardiothoracic CT in children and adults: the effect of tube current saturation on radiation dose reduction. Korean J Radiol 19:23–31. https://doi.org/10.3348/kjr.2018.19.1.23

    Article  PubMed  PubMed Central  Google Scholar 

  15. Hong SW, Goo HW, Maeda E, Choo KS, Tsai IC; Asian Society of Cardiovascular Imaging Congenital Heart Disease Study Group (2019) User-friendly, vendor-specific guideline for pediatric cardiothoracic computed tomography provided by the Asian Society of Cardiovascular Imaging (ASCI) Congenital Heart Disease Study Group: Part 1. Imaging Tech Korean J Radiol 20:190–204. https://doi.org/10.3348/kjr.2018.0571

    Article  Google Scholar 

  16. Goo HW (2018) Is it better to enter a volume CT dose index value before or after scan range adjustment for radiation dose optimization of pediatric cardiothoracic CT with tube current modulation? Korean J Radiol 19:692–703. https://doi.org/10.3348/kjr.2018.19.4.692

    Article  PubMed  PubMed Central  Google Scholar 

  17. Han BK, Grant KI, Garbrich R, Sedlmair M, Lindberg J, Lesser JR (2012) Assessment of an iterative reconstruction algorithm (SAFIRE) on image quality in pediatric cardiac CT datasets. J Cardiovasc Comput Tomogr 6:200–204. https://doi.org/10.1016/j.jcct.2012.04.008

    Article  PubMed  Google Scholar 

  18. Lee KB, Goo HW (2020) Comparison of quantitative image quality of cardiac computed tomography between raw-data-based and model-based iterative reconstruction algorithms with an emphasis on image sharpness. Pediatr Radiol 50:1570–1578. https://doi.org/10.1007/s00247-020-04741-x

    Article  PubMed  Google Scholar 

  19. Goo HW (2012) CT radiation dose optimization and estimation: an update for radiologists. Korean J Radiol 13:1–11. https://doi.org/10.3348/kjr.2012.13.1.1

    Article  PubMed  Google Scholar 

  20. Goo HW (2019) Semiautomatic three-dimensional threshold-based cardiac computed tomography ventricular volumetry in repaired tetralogy of Fallot: comparison with cardiac magnetic resonance imaging. Korean J Radiol 20:102–113. https://doi.org/10.3348/kjr.2018.0237

    Article  PubMed  Google Scholar 

  21. Sugeng L, Mor-Avi V, Weinert L, Niel J, Ebner C, Steringer-Mascherbauer R et al (2010) Multimodality comparison of quantitative volumetric analysis of the right ventricle. JACC Cardiovasc Imaging 3:10–18. https://doi.org/10.1016/j.jcmg.2009.09.017

    Article  PubMed  Google Scholar 

  22. Yu S, Yang K, Chen X, Lu M, Zhao K, Yang S et al (2023) Cardiac remodeling after tricuspid valve repair in Ebstein’s anomaly: a magnetic resonance study. Eur Radiol 33:2052–2061. https://doi.org/10.1007/s00330-022-09190-8

    Article  PubMed  Google Scholar 

  23. Prota C, Di Salvo G, Sabatino J, Josen M, Paredes J, Sirico D et al (2019) Prognostic value of echocardiographic parameters in pediatric patients with Ebstein’s anomaly. Int J Cardiol 278:76–83. https://doi.org/10.1016/j.ijcard.2018.10.046

    Article  PubMed  Google Scholar 

  24. Homzova L, Photiadis J, Sinzobahamvya N, Ovroutski S, Cho MY, Schulz A (2021) Surgical management of Ebstein anomaly: impact of the adult congenital heart disease anatomical and physiological classifications. Interact Cardiovasc Thorac Surg 32:593–600. https://doi.org/10.1093/icvts/ivaa294

    Article  PubMed  Google Scholar 

  25. Zhu Y, Liu J, Weinsaft J, Spincemaille P, Nguyen TD, Prince MR et al (2015) Free-breathing 3D imaging of right ventricular structure and function using respiratory and cardiac self-gated cine MRI. Biomed Res Int 2015:819102. https://doi.org/10.1155/2015/819102

    Article  PubMed  PubMed Central  Google Scholar 

  26. Hanneman K, Kino A, Cheng JY, Alley MT, Vasanawala SS (2016) Assessment of the precision and reproducibility of ventricular volume, function, and mass measurements with ferumoxytol-enhanced 4D flow MRI. J Magn Reson Imaging 44:383–392. https://doi.org/10.1002/jmri.25180

    Article  PubMed  PubMed Central  Google Scholar 

  27. Goo HW (2018) Comparison between three-dimensional navigator-gated whole-heart MRI and two-dimensional cine MRI in quantifying ventricular volumes. Korean J Radiol 19:704–714. https://doi.org/10.3348/kjr.2018.19.4.704

    Article  PubMed  PubMed Central  Google Scholar 

  28. Alsaied T, Diaz-Castrillon C, Christopher A, Da Silva J, Morell VO, Lanford L et al (2022) Cardiac MRI predictors of right ventricular dysfunction after the Da Silva cone operation for Ebstein’s anomaly. Int J Cardiol Congenit Heart Dis 7:100342. https://doi.org/10.1016/j.ijcchd.2022.100342

    Article  Google Scholar 

  29. Tang XJ, Bao M, Zhao H, Wang LY, Wu QY (2017) Intraoperative transesophageal echocardiography in the operation of Ebstein’s anomaly: a retrospective Study. Chin Med J (Engl) 130:1540–1543. https://doi.org/10.4103/0366-6999.208233

    Article  PubMed  Google Scholar 

Download references

Funding

No funding was received to assist with the preparation of this manuscript.

Author information

Authors and Affiliations

  1. Department of Radiology and Research Institute of Radiology, University of Ulsan College of Medicine, Asan Medical Center, 88, Olympic-ro 43-gil, Songpa-gu, Seoul, 05505, Republic of Korea

    Hyun Woo Goo & Sang Hyub Park

Authors
  1. Hyun Woo Goo
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Sang Hyub Park
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

All named authors have been active participants in this research and have approved the manuscript for submission.

Corresponding author

Correspondence to Hyun Woo Goo.

Ethics declarations

Conflict of interest

The authors have no conflict of interest to declare that are relevant to the content of this article.

Ethical Approval

This retrospective study was approved by the local institutional Ethics Review Board and conducted with strict maintenance of patient anonymity.

Consent to Participate

The need for informed consent was waived by the local institutional Ethics Review Board.

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

Goo, H.W., Park, S.H. Complimentary Cardiac Computed Tomography Ventricular Volumetry-Derived Metrics of Severity in Patients with Ebstein Anomaly: Comparison with Echocardiography-Based Severity Indices. Pediatr Cardiol 45, 24–31 (2024). https://doi.org/10.1007/s00246-023-03342-3

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00246-023-03342-3

Keywords

Search

Navigation