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Ebstein Anomaly

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Abstract

Ebstein anomaly is a relatively rare lesion, which represents less than 1 % of all cases of congenital heart disease and has a prevalence of about 3–5 per 100,000 live births. Although Ebstein anomaly primarily relates to a malformation of the tricuspid valve with consequent effects on right ventricular development, there is, in fact, a wide variation of abnormalities and associated lesions which may have significant impact on disease prognosis. Ebstein anomaly is unique in that there is a broad spectrum of disease, varying from the severe neonatal form which continues to carry a dismal prognosis, even in contemporary series, to the milder form which may only become manifest in adult life, if at all.

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Author information

Authors and Affiliations

  1. Congenital Heart Disease Program, Department of Cardiology, University Hospital Basel, Petersgraben 4, Basel, CH-4031, Switzerland

    Daniel Tobler MD

  2. Department of Radiology, Sunnybrook Health Sciences Centre, University of Toronto, Toronto, ON, Canada

    Laura Jimenez Juan MD

  3. Department of Medicine and Medical Imaging, Toronto Congenital Cardiac Centre for Adults, Peter Munk Cardiac Centre, University Health Network, University of Toronto, Toronto, ON, Canada

    Andrew M. Crean BSc, BM, MRCP, MSc, FRCR

  4. Departments of Pediatrics, Medicine, Obstetrics/Gynecology and Medical Imaging, Toronto Congenital Cardiac Centre for Adults, Peter Munk Cardiac Centre, University Health Network, University of Toronto, Toronto, ON, Canada

    Rachel M. Wald MD, FRCPC

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  1. Daniel Tobler MD
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  2. Laura Jimenez Juan MD
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  3. Andrew M. Crean BSc, BM, MRCP, MSc, FRCR
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  4. Rachel M. Wald MD, FRCPC
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Corresponding author

Correspondence to Rachel M. Wald MD, FRCPC .

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Editors and Affiliations

  1. Department of Radiology, USC University Hospital Keck School of Medicine, Los Angeles, California, USA

    Farhood Saremi

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Tobler, D., Juan, L.J., Crean, A.M., Wald, R.M. (2014). Ebstein Anomaly. In: Saremi, F. (eds) Cardiac CT and MR for Adult Congenital Heart Disease. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-8875-0_15

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  • DOI: https://doi.org/10.1007/978-1-4614-8875-0_15

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