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Multimodality Imaging in Ebstein Anomaly

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Abstract

Ebstein anomaly is the most common form of tricuspid valve congenital anomalies. The tricuspid valve is abnormal with different degrees of displacement of the septal leaflet and abnormal rotation of the valve towards the right ventricular outflow tract. In severe forms, it results in significant tricuspid regurgitation and requires surgical repair. There is an increased interest in understanding the anatomy of the tricuspid valve in this lesion as the surgical repair has evolved with the invention and wide adoption of the cone operation. Multimodality imaging plays an important role in diagnosis, follow-up, surgical planning and post-operative care. This review provides anatomical tips for the cardiac imagers caring for patients with Ebstein anomaly and will help provide image-based personalized medicine.

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Authors and Affiliations

  1. Heart Institute, UPMC Children’s Hospital of Pittsburgh, Division of Pediatric Cardiology, University of Pittsburgh School of Medicine, 4401 Penn Ave, Pittsburgh, PA, USA

    Tarek Alsaied, Adam B. Christopher, Aditi Gupta, Lizabeth Lanford, Jacqueline G. Weinberg, Brian Feingold, Thomas Seery, Arvind Hoskoppal, Bryan H. Goldstein, Jennifer A. Johnson & Laura J. Olivieri

  2. DaSilva Center of Ebstein Anomaly, UPMC Children’s Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA

    Jose Da Silva, Victor O. Morell & Luciana De Fonseca Da Silva

  3. Department of Cardiothoracic Surgery, UPMC Children’s Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA

    Jose Da Silva, Victor O. Morell & Luciana De Fonseca Da Silva

Authors
  1. Tarek Alsaied
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  2. Adam B. Christopher
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  3. Jose Da Silva
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  4. Aditi Gupta
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  5. Victor O. Morell
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  6. Lizabeth Lanford
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  7. Jacqueline G. Weinberg
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  8. Brian Feingold
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  9. Thomas Seery
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  11. Bryan H. Goldstein
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  12. Jennifer A. Johnson
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  13. Laura J. Olivieri
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  14. Luciana De Fonseca Da Silva
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Contributions

All authors contributed to conception, image acquisition and reviewed the final manuscript.

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Correspondence to Tarek Alsaied.

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Video 1: Color compare modified apical 5 chamber view of a patient with Carpentier type A and severe tricuspid regurgitation (MP4 2964 kb)

Video 2: An apical 4 chamber view of a patient with Carpentier type A with displacement and tethering of the sepal leaflet of the tricuspid valve (MP4 793 kb)

Video 3: An apical view 3D echocardiogram of a patient with Carpentier type A showing the tethering of the septal and inferior leaflets (MP4 1508 kb)

Video 4: An apical view 3D echocardiogram of a patient with Carpentier type A with color showing the tethering of the septal and inferior leaflets with severe tricuspid regurgitation (MP4 2105 kb)

Video 5: A modified apical view 3D echocardiogram of a patient with Carpentier type A showing the tethering of the septal and inferior leaflets. The tethering attachments can be appreciated better compared to 2D echocardiogram (MP4 1186 kb)

Video 6: Modified parasternal long axis echocardiogram in a patient with Carpentier type A and severe tricuspid regurgitation (MP4 1252 kb)

Video 7: Cardiac MRI 3 chamber view post cone operation of a patient with Carpentier type C showing the position of the repaired tricuspid valve at the anatomical annulus (MP4 293 kb)

Video 8: Cardiac MRI 4 chamber view post cone operation of a patient with Carpentier type C showing the position of the repaired tricuspid valve at the anatomical annulus (MP4 240 kb)

Video 9: An apical 4 chamber view of a patient with Carpentier type D Ebstein anomaly with severe displacement and tethering of the sepal leaflet and inferior leaflets of the tricuspid valve (MP4 1055 kb)

Video 10: Modified subcostal coronal view showing the thinning of the “atrialized” right ventricle in a patient with Carpentier type D Ebstein anomaly (MP4 964 kb)

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Alsaied, T., Christopher, A.B., Da Silva, J. et al. Multimodality Imaging in Ebstein Anomaly. Pediatr Cardiol 44, 15–23 (2023). https://doi.org/10.1007/s00246-022-03011-x

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  • DOI: https://doi.org/10.1007/s00246-022-03011-x

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