Ebstein Anomaly in Adults.

The MRI Approach

Poster No.:

C-2493

Congress:

ECR 2012

Type:

Educational Exhibit

Authors:

C. Caparrs-Escudero , D. de Araujo Martins-Romeo , T. Ruiz

Garca , P. Gallego , J. A. Herrero Lara , Muoz Castillo ;

1

Seville/ES, Marbella/ES

Keywords:

Cardiac, MR, Diagnostic procedure, Education, Congenital

DOI:

10.1594/ecr2012/C-2493

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Learning objectives
The aim of this study is to:
Review the pathological spectrum of Ebsteins Anomaly (EA) in adults.
Illustrate by MR the morphologic aspects of EA.
Discuss MR findings of right ventricular overload including right ventricular dimension,
paradoxical ventricular septal motion, and myocardial segmental wall abnormalities.
Emphasize the role of MR in the assessment of ventricular and valvular function.
Demonstrate the use of delayed contrast-enhanced MR to rule out segments of
myocardial dysplasia.

Background
Ebstein's anomaly (EA) is a rare congenital malformation of the tricuspid valve and right
ventricle characterized by (Fig 1):
(1) Adherence of the septal and posterior leaflets to the underlying myocardium due to
failure of delamination.
(2) Redundancy, fenestrations, and tethering of the anterior leaflet;
(3) Apical displacement of the functional annulus dividing right ventricle into atrialized
right ventricle and functioning right ventricle;
(4) Dilation of the true tricuspid annulus;
(5) Dilation of the "atrialized" portion of the right ventricle, with various degrees of
hypertrophy and thinning of the wall.

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EA anatomic classification
The Carpentier classification is based on the extent of both leaflet displacement and right
ventricle enlargement (Fig 2):
(1) Type A: the volume of the true right ventricle is adequate;
(2) Type B: a large atrialized component of the right ventricle exists, but the anterior leaflet
of the tricuspid valve moves freely;
(3) Type C: the anterior leaflet is severely restricted in its movement and may cause
significant obstruction of the right ventricular outflow tract;
(4) Type D: almost complete atrialization of the ventricle except for a small infundibular
component.
Clinical presentation
The clinical presentation and hemodynamic consequences depends on:
(1) Age at presentation;
(2) Anatomic severity related to the grade of tricuspid regurgitation and dysfunction of
atrialized portion of the right ventricle;
(3) Presence and degree of right-to-left interatrial shunt;
Clinical features: Adults may present progressive cyanosis, exercise intolerance, fatigue,
right-sided heart failure, arrhythmias, and sudden cardiac death.
Diagnostic approach
Echocardiography: Is the non-invasive method of choice in the diagnostic of Ebsteins
anomaly. Allows evaluation of:

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(1) Anatomic characteristics of tricuspid valve leaflets and severity of tricuspid

regurgitation.
(2) Size and function of cardiac chambers in special the extension of atrialized portion
of right ventricle.
(3) Posible associated cardiac malformation. The most common is the atrial septal defect
presenting in 80% to 94% of patients.
Echocardiography limitation:
-Poor acoustic window.
-Lack of reliability in the follow-up studies.
Cardiac magnetic resonance imaging (CMR):
1- CMR allows detailed visualization of the pathological anatomy in patients with Ebstein
s anomaly. It is of great value when echocardiography image quality is inadequate due
to a poor acoustic window.
2- CMR also provides a method of accurate physiological assessment of already proven
superiority to echocardiography: It is a technique for precise volumetric analysis of
ventricular function and intracardiac blood flow, without any geometric assumptions.
3- CMR delayed contrast enhancement image is a potential tool to recognize areas of
right ventricle dysplasia.

Images for this section:

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Fig. 1: Fig 1a.- RA: Right Atrium; LA Left Atrium; LV: Left Ventricle; ARV: Atrialized Right
Ventricle; FRV: Functioning Right Ventricle Dotted line: atrioventricular junction. Fig1b.redundancy and tethering of the anterior leaflet. Apical displacement of the septal leaflet.
AL: Anterior Leaflet; SL: Septal Leaflet.

Fig. 2

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Imaging findings OR Procedure details

CMR imaging protocol for the EA assessment
All studies were performed on a 1.5T scanner (Magnetom Symphony Siemens).
The following acquisitions were performed parallel to the long axis of the heart (horizontal
and vertical long-axis planes) and perpendicular to it (short-axis planes).
(1) Balanced SSFP ( b-SSFP) sequences using retrospective ECG gating and parallel
imaging technique with an acceleration factor of two and reconstruction algorithm
GRAPPA (generalized auto calibrating partially parallel acquisition).
(2) Dark-blood techniques, being the single-shot inversion-recovery fast SE sequence
with breath holding the most commonly used.
(3) Phase contrast velocity map of the aorta and pulmonary arteries.
(4) At last delayed contrast enhanced images performed ten minutes after injection of
gadolinium-DTPA using a segmented inversion-recovery spoiled GRE sequence.
CMR Image findings
First determine atrial situs, atrioventricular and ventriculoarterial connections and
possible associated malformations.
1-Morphology findings
1.1- Tricuspid valve
- Degree of apical displacement of postero-septal leaflet: the grade of leaflet tethering
to the ventricular wall can be calculated according to their extension ( modified Becker
s dysplasia classification): grade I up to 25% of the distance from the atrioventricular
junction to the apex, grade II from 25-50%, grade III more than 50% of the distance. (Fig 2)

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- Location and dilation of the tricuspid anatomic valve ring: the degree of dilatation of the
tricuspid anatomic ring can be determine by the rate of the diameter of tricuspid valve to
that of the mitral valve at the level of annulus. (Fig 3)
- Anatomic characteristics of anterior valve leaflet : the proximal attachment of the anterior
valve leaflet is normally at the anatomic tricuspid valve ring, however distally it may
presents with abnormal attachment to the ventricular wall. The leaflet can be dysplastic,
redundant, and presents with abnormal motility.( Fig 4)
1.2- Right ventricle (RV) :
- Recognize the two regions of RV: The functional tricuspid annulus divides the RV in two
portions: the atrialized right ventricle (ARV), and the functional right ventricle (FRV). The
FRV is constituted of trabecular portion and outflow tract. (Fig 5 )
- Degree of dilation of the ARV. Dilation of ARV is associated with thinning of ventricular
wall. In some cases dilation is so extreme that the ventricular septum is displace leftward
and may compromise left ventricle movement and contractility(Fig 6). In some cases
there is also dilation of the FRV.
- Characteristics of the apical trabecular portion and the morphology of the right
ventricular infundibulum. (Fig7)
1.3- Morphology of the left heart chambers, mitral and aortic valves.
2-Functional findings:
1-Ventricular volumes and function as well as ejection fraction were calculated by manual
endocardial border detection on a stack of SSFP cine images orientated to the short axis
and four-chamber planes of the left ventricle. (Fig 8-10)
2-Right-to-left shunt was quantified by comparing pulmonary artery and aorta phase
contrast flow.
3-Tricuspid valve regurgitation fraction was determined by subtracting right ventricular
cardiac output obtained by volumetric quantification from phase contrast pulmonary
artery flow.

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4-Qualitative aspects such as abnormal septal motion, tricuspid regurgitation jet and
movement of the anterior valve (anterior leaflet) were evalued with cine MR images. (Fig
11-15)
3-Late enhancement contrast images
Myocardial late enhancement contrast image has the ability to precisely delineate
myocardial scar associated with fibrosis. In the EA scene this is an important tool to
recognize areas of dysplasia of right ventricle. (Fig 16)

Images for this section:

Fig. 3

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Fig. 4

Fig. 5

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Fig. 6

Fig. 7

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Fig. 8: 2D b-SSFP cine images in four-chamber view. The anatomic tricuspid ring is
normally situated at the ventricular junction and the functional opening is low .The anterior
tricuspid leaflets is dysplastic: thickening of the leaflet with abnormal attachment to the
anterior wall explains its reduced motility.

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Fig. 9: 2D b-SSFP cine images in four-chamber view. The anatomic tricuspid ring is
normally situated at the ventricular junction and the functional opening is low .The anterior
tricuspid leaflets is dysplastic: thickening of the leaflet with abnormal attachment to the
anterior wall explains its reduced motility.

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Fig. 10

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Fig. 11

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Fig. 12: A regurgitant jet can be seen between FRV and ARV.

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Fig. 13

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Fig. 14: 2D b-SSFP cine images in short-axis view at basal cavity level. The anterior
tricuspid leaflet is large and redundant. Motility is so extreme that the mobile leaflet tissue
is displaced into the right ventricle outflow tract at the end of diastole.

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Fig. 15: 2D balanced-SSFP (b-SSFP) cine image in coronal long-axis view through the
right atrium and right ventricle showing a redundant anterior leaflet with severely restrict
movement. Note the large atrialized portion of the right ventricle and the presence of a jet
due to tricuspid regurgitation. This is an example of type C of Carpentier classification.

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Fig. 16

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Conclusion
MR is an outstand method as it provides not only morphological detail of tricuspid valve
and right cavities, but also functional assessment including right ventricular volumes and
ejection fraction, global and segmental ventricular function, and tricuspid regurgitation
magnitude. Moreover it is an accurate method to determine the degree of right ventricular
dilatation associated to wall thinning and the presence of segments of myocardial
dysplasia.

Personal Information
References
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- Attenhofer Jost C, Connolly H, Dearani J et al. Ebsteins anomaly. Circulation
2007;115;277-285.
- C. H. Attenhofer Jo sta, H. M. Connolly, W. D. Edwards et al. Ebstein's anomaly - review
of a multifaceted congenital cardiac condition. SWISS MED WKLY 2005;135:269-281.
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of presentation of Ebstein's anomaly. J Cardiovasc Med 2008;9:628-630.
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Anatomo-echocardiographic correlation. Cardiovascular Ultrasound 2007, 5(1):43.
-Becker AE, Becker MJ, Edwards JE: Pathologic spectrum and dysplasia of the tricuspid
valve: features in common with Ebstein's malformation. Arch Pathol 1971, 91:167-178.

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