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Multiplanar review of three-dimensional echocardiography gives new insights into the morphology of Ebstein’s malformation

Published online by Cambridge University Press:  19 January 2010

Tara Bharucha*
Affiliation:
From Congenital Cardiac Centre (TB, ZSL, JJV), Southampton University NHS Trust, Southampton, United Kingdom, and Cardiac Unit (RHA), Institute of Child Health, London, United Kingdom
Robert H. Anderson
Affiliation:
From Congenital Cardiac Centre (TB, ZSL, JJV), Southampton University NHS Trust, Southampton, United Kingdom, and Cardiac Unit (RHA), Institute of Child Health, London, United Kingdom
Zek S. Lim
Affiliation:
From Congenital Cardiac Centre (TB, ZSL, JJV), Southampton University NHS Trust, Southampton, United Kingdom, and Cardiac Unit (RHA), Institute of Child Health, London, United Kingdom
Joseph J. Vettukattil
Affiliation:
From Congenital Cardiac Centre (TB, ZSL, JJV), Southampton University NHS Trust, Southampton, United Kingdom, and Cardiac Unit (RHA), Institute of Child Health, London, United Kingdom
*
Correspondence to: Dr Tara Bharucha, Congenital Cardiac Centre, Department of Paediatric Cardiology, Mailpoint 46, Southampton University NHS Trust, Tremona Road, Southampton, SO16 6YD, United Kingdom. Tel: +44 23 8079 8825; Fax: +44 23 8079 4526; E-mail: tarabharucha@doctors.org.uk

Abstract

Introduction

We aimed to assess the ability of the multiplanar review modality of three-dimensional echocardiography to examine the dynamic morphology and the functional characteristics of malformed tricuspid valves in patients previously identified as having Ebstein’s malformation. Based on these characteristics, we attempted to differentiate Ebstein’s malformation from tricuspid valvar dysplasia.

Methods

Using three-dimensional multiplanar review, analysed with either Qlab 6.0 or Tomtech Image Arena 3.0, we studied 23 patients, aged from 1 day to 70 years, previously diagnosed using cross-sectional echocardiography as having Ebstein’s malformation.

Results

Using the features of rotational abnormality, and the orientation, of the effective tricuspid valvar orifice as diagnostic features of Ebstein’s malformation, we reclassified 11 patients (48 per cent) as exhibiting tricuspid valvar dysplasia. In addition, we studied the dynamic morphology as well as the function of the tricuspid valve. Surgical treatment was undertaken on 10 patients, revealing good correlation with the findings obtained using three-dimensional multiplanar review. In those with Ebstein’s malformation, we found varying degrees of rotation, with the effective valvar orifice always directed towards the right ventricular outflow tract. The opening of the orifice of dysplastic tricuspid valves, in contrast, was towards the apex of the right ventricle. The degree of delamination, and abnormalities of subcordal apparatus, were similar in the two groups.

Discussion

Three-dimensional multiplanar review permits accurate definition of the dynamic morphology of Ebstein’s malformation, permitting clear differentiation from tricuspid valvar dysplasia.

Type
Original Articles
Copyright
Copyright © Cambridge University Press 2009

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