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Archiving Working Group of the International Society for Nomenclature of Pediatric and Congenital He

Contributors: Jeffrey P. Jacobs, MD; James St. Louis, MD; Jorge M. Giroud, MD; Charles W. Shepard, MD; Allen Everett, MD; Robert H. Anderson, MD; Vera D. Aiello, MD; Diane E. Spicer, BS

International Paediatric and Congenital Cardiac Code (IPCCC): 06.01.34 AEPC Derived Terms: Ebstein’s malformation of the Tricuspid Valve EACTS-STS Derived Terms: Tricuspid Valve Disease, Ebstein’s Anomaly ICD11 Derived Terms: Ebstein malformation of the Tricuspid Valve


The figures presented here illustrate the major morphological features of Ebstein’s in malformation of the tricuspid valve, both in anatomical specimens and in magnetic resonance imaging. The key for diagnosis is the apical and rotational displacement of the septal and mural (inferior) leaflets’ insertion, away from the atrioventricular junction. In contrast, the anterosuperior leaflet is usually redundant and retains its annular insertion (Figures 1, 2 and 3). The distinction between the anterosuperior and the mural leaflets is not so easy to determine, and in most cases, they appear combined as a curtain and the valve is bifoliate. Additional features include: variable degrees of leaflet dysplasia, with short and abnormally attached cords, obliteration of the intercordal spaces and irregular thickening. Complete understanding of the malformation requires recognition that the functional tricuspid orifice is displaced anteriorly inside the right ventricle, towards the transition between the inlet and the apical trabecular portion (Figures 4 and 5). In some severe cases, the functional orifice may open at the ventricular outlet.1,2 The pattern of cordal attachment is also highly variable, with tethering, linear or hyphenated attachments, leading to restriction in the leaflet motion. The commonest type of valvar dysfunction is insufficiency, but some combined morphological features may also result in valve stenosis or double dysfunction.

To read the full article, please go to the May 2018 Issue of CCT, where it was originally published.

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