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AUTHOR AND EDITOR INFORMATION

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Author: Julia Gates, MD, Department of Radiology, Consulting Staff and Assistant Residency Program Director, Baystate Medical Center

Julia Gates is a member of the following medical societies: Alpha Omega Alpha, American Heart Association, American Roentgen Ray Society, Association of University Radiologists, Massachusetts Medical Society, and Radiological Society of North America

Coauthor(s): George Hartnell, MB, Professor of Radiology, Tufts University School of Medicine, Director of Cardiovascular and Interventional Radiology, Department of Radiology, Baystate Medical Center

Editors: S Bruce Greenberg, MD, Professor of Radiology, University of Arkansas for Medical Sciences; Consulting Staff, Department of Radiology, Arkansas Children's Hospital; Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand; David S Levey, MD, PhD, Orthopedic/Spine MRI TeleRadiologist, Radsource, LLC; Robert M Krasny, MD, Consulting Staff, Department of Radiology, The Angeles Clinic and Research Institute; Eugene C Lin, MD, Consulting Staff, Department of Radiology, Virginia Mason Medical Center

Author and Editor Disclosure

Synonyms and related keywords: Ebstein's anomaly, arrhythmia, heart block, congenital heart disease, atrial septal defect, accessory conduction pathway, cardiac conduction block, ventricular septal defect, atrial fibrillation, heart disease, cardiovascular disease

INTRODUCTION

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Background

Professor Wilhelm Ebstein (1836-1912), who was a professor of medicine in Gottingen, Germany, first described Ebstein anomaly in 1866.1, 2, 3 The condition accounts for less than 1% of congenital heart diseases and occurs in 1 in 210,000 live births.4 Ebstein anomaly occurs with equal frequency in male individuals and female individuals; severely affected neonates can present on the first day of life. The condition may completely escape detection, or patients may not become symptomatic until late in life. In some individuals, Ebstein anomaly is not diagnosed until the eighth decade of life.5 As many as one half of patients have an atrial septal defect (ASD), and as many as one quarter have an accessory conduction pathway.

Pathophysiology

Numerous abnormalities are associated with Ebstein anomaly, including the following:

  • Ventricular septal defect (VSD)6, 7, 8
  • Aortic coarctation9, 10
  • Interatrial communication - ASD, especially secundum ASD, and patent foramen ovale7, 8, 11, 12
  • Pulmonary atresia with an intact ventricular septum7, 13
  • Partial atrioventricular canal8
  • Hypoplastic pulmonary arteries8
  • Patent ductus arteriosus8
  • Pulmonary stenosis14, 7
  • Parachute mitral valve8
  • Cleft anterior leaflet of the mitral valve8
  • Mitral valve prolapse7, 8
  • Left ventricular outflow obstruction11
  • Hypertensive pulmonary vascular disease11
  • Congenital deaf-mutism15
  • Corrected transposition of the great arteries16, 17, 18
  • Subaortic stenosis19
  • Tetralogy of Fallot20

Frequency

United States

Ebstein anomaly occurs in 1 of 210,000 live births.4

Mortality/Morbidity

Mortality depends on the severity of the lesion and other cardiac defects in each patient. Patients with severe disease may die in infancy if untreated, whereas patients with mild or moderate disease may not present until the eighth decade of life.

Sex

Ebstein anomaly occurs with equal frequency in male and female individual.

Age

Patients can present with Ebstein anomaly at any age.

Anatomy

The tricuspid valve is composed of 3 leaflets, including the anterior, posterior, and septal (also termed mural or medial) leaflets. In Ebstein anomaly, the leaflets are affected to various degrees. The septal leaflet is affected more than the posterior leaflet, which is affected more than the anterior leaflet.8 The septal and posterior leaflets are often displaced inferiorly toward the apex of the heart. The anterior tricuspid valve leaflet may be elongated,21 and it may adhere to the wall of the right ventricle.8, 12 The septal and posterior leaflets are displaced inferiorly at least 20 mm or 8 mm/m2 in adults.12, 21 This degree of inferior displacement of the septal leaflet is regarded as definitive.22

The leaflets may be tethered by muscular bands or by shortened chordae tendineae and shortened papillary muscles, or the leaflets may adhere directly to the ventricular wall.8 The valve annulus is not displaced; therefore, it is situated correctly between the atrium and ventricle. Similarly, the proximal attachment of the anterior leaflet to the annulus is normal.12 The mural leaflet always is present; if absent, the condition is termed unguarded tricuspid orifice rather than Ebstein anomaly.23

Because the annulus is sited anatomically as usual but because the septal and posterior valve attachments are displaced inferiorly, a region of upper right ventricle functionally becomes atrial; this is termed the atrialized portion of the right ventricle. As a result of its structural alteration, this region actually is thinned and hence prone to aneurysmal dilatation, particularly between the attachment of the posterior leaflet and the annulus.12 Furthermore, the right ventricle has a decreased number of myocardial fibers, which increases the risk of dilatation.23

Clinical Details

Clinical findings in Ebstein anomaly include the following:

  • Asymptomatic presentation
  • Dyspnea on exertion
  • Palpitations and/or ectopy
  • Exercise intolerance in patients
  • Atrial arrhythmias
  • Cyanosis
  • Paroxysmal supraventricular tachycardia
  • Atrial fibrillation - Paroxysmal or chronic
  • Atrial flutter - Paroxysmal or chronic
  • Accessory conduction pathways (Wolff-Parkinson-White syndrome, especially type B; Kent bundle; Mahaim fiber)
  • Cardiac conduction block - Arteriovenous block, left bundle-branch block, and right bundle branch block
  • Sinus node dysfunction
  • Congestive hepatosplenomegaly

Epstein anomaly classically falls into the gamut of chest radiograph findings termed cyanosis with decreased pulmonary vascularity, though chest radiographic findings are often characteristic (see Images 1-3).

In infants, diagnosis is a dilemma because of their inability to articulate the chief complaint. Approximately one fifth of infants presenting with supraventricular tachycardia have structural heart disease, most often Ebstein anomaly.24 The probability of a structural abnormality is increased in patients with Wolf-Parkinson-White syndrome type B.24, 25 Maternal use of lithium during pregnancy is thought to induce some accessory conduction pathways.7

Infants with severe Ebstein anomaly and marked cardiomegaly (see Image 2) may have further compromise due to excessive bilateral, pulmonary compression from the enlarged heart. Corrective surgery with cardiac reduction can help obviate ventilator dependence.8 Infants may benefit from inhaled nitric oxide therapy.26 Critically ill neonates can be given extracorporeal membrane oxygenation as a temporary measure.27

The severity of the abnormality can be assessed and graded 1-4 by calculating the ratio of the area of the right atrium and atrialized right ventricle to the area of the functional right ventricle and left heart chambers (on the 4-chamber view). The higher the grade, the worse the prognosis. This scheme was used in neonates in a retrospective study.14 When echocardiography is difficult, definition of the anatomy can be improved by using cardiac MRI and cine magnetic resonance angiography (MRA) when this is available.28, 29, 30

Related eMedicine topics:
Atrial Flutter
Tetralogy of Fallot

Related Medscape topics:
CME/CE Diagnosis and Management of Atrial Fibrillation: Focus on Guidelines
CME Supraventricular Tachycardia -- An Interactive Case

Preferred Examination

Chest radiography is the best initial study, though findings are often nonspecific.

Two-dimensional echocardiography is usually performed next and may be all that is required. Echocardiography can help confirm the diagnosis, assess the severity of the anatomic lesion, and determine the effects on cardiac function.

When available and of high quality, fast cardiac MRI with contrast-enhanced 3-dimensional (3D) MRA has the potential to replace cardiac catheterization for assessing pulmonary artery anatomy and for providing information that echocardiography cannot. MRI can depict anatomy and function with an unrestricted field of view; even the pulmonary arteries and tricuspid valve can be evaluated during a single study. Comprehensive imaging of this type provides a vehicle for surgical planning.31, 32

Limitations of Techniques

Traditional imaging modalities have included chest radiography, echocardiography, and cardiac catheterization with right ventriculography.

Plain radiographic findings are nonspecific despite occasional pathognomonic appearances. Normal chest radiographic findings do not exclude the diagnosis.

Echocardiography is usually performed after chest radiography; however, if echocardiography is inadequate, MRI can be used to assess anatomy and function with an unrestricted field of view. Echocardiography can be limited by restricted acoustic access, especially in patients who are obese, who underwent surgery, or who cannot lie still. Echocardiography cannot be used to measure the size of the left and right pulmonary arteries.

MRI is not routinely performed because of its limited availability and expense; the limited availability of qualified personnel to perform the protocol, interpret the study, or both; the patient's inability to cooperate (eg, because of claustrophobia); unsuitability or contraindications (eg, clinically unstable condition, pacemaker, metal objects in an anatomic location where MRI is dangerous and therefore contraindicated); and physicians' unawareness of the uses of MRI.


DIFFERENTIALS

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Pulmonary Hypertension
Tetralogy of Fallot
Tricuspid Atresia
Ventricular Septal Defect

Other Problems To Be Considered

Patients who clinically present with tricuspid regurgitation and right-sided cardiac enlargement may have dysplasia of the tricuspid valve, an unguarded tricuspid orifice, tricuspid valve prolapse, arrhythmogenic right ventricular dysplasia, trauma, endocarditis, or dilatation of the tricuspid valve annulus secondary to pulmonary valve regurgitation.21, 23, 33 Acquired causes of tricuspid regurgitation and right-sided cardiac enlargement seen in adults also include carcinoid syndrome, pulmonary hypertension, and left heart failure, though the last 2 are unlikely to be mistaken for Ebstein anomaly on the basis of clinical or radiographic findings.

Ebstein anomaly classically falls into the gamut of chest radiographic findings termed cyanosis with decreased pulmonary vascularity, though chest radiographic findings often are characteristic (see Images 1-3).34 The differential diagnosis for this category includes Ebstein anomaly, tetralogy of Fallot, pulmonary atresia with VSD, pulmonary atresia with an intact ventricular septum, hypoplastic right ventricle and/or Uhl anomaly or parchment right ventricle, and tricuspid atresia with a restrictive VSD.


RADIOGRAPH

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Findings

Traditional imaging modalities include chest radiography, echocardiography, and cardiac catheterization with right ventriculography. Classic radiographic features of Ebstein anomaly include a squared-off cardiac contour and pulmonary oligemia. Cyanosis occurs as a result of a right-to-left shunt, most often at the atrial level, with reduced pulmonary perfusion resulting from ineffective right ventricular ejection. Normal chest radiographic findings with an unremarkable clinical presentation can occur with mild forms of Ebstein anomaly, but this manifestation is not typical.

Cardiomegaly may be seen on plain images of the chest (see Images 1-2). Decreased pulmonary vascularity may be seen in patients with right-to-left shunts, usually through an ASD (see Image 2). A distinctly enlarged right atrium may be identified on chest radiographs. All of these findings can be nonspecific, though in some patients, chest radiographic findings are characteristic (see Images 2-3).

On the lateral chest radiograph, distortion of the right ventricular outflow or displacement by the atrialized segment of the right ventricle may cause abnormal filling of the retrosternal space. This finding is of use in older children and adults. The presence of the thymus makes this sign irrelevant for the diagnosis of Ebstein anomaly in infants (see Image 3).


CT SCAN

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Findings

Although Ebstein anomaly can be evaluated by means of CT, MRI has largely supplanted CT.

On a chest CT scan, the right atrium and ventricle appear dilated. A variable degree of apical displacement of the tricuspid valve attachments can be seen relative to the atrioventricular junction. The atrialized and functional portions of the right ventricle may be discerned. The conspicuity of the myocardium may be greater in the functional portion of the right ventricle than in the atrialized portion. Depending on the image reconstruction, the patient's heart rate, and the CT scanner, leaflet thickening might be seen.


MRI

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Findings

MRI is a better noninvasive alternative to CT in evaluating Ebstein anomaly when echocardiography provides incomplete information. By using a combination of anatomic (spin-echo sequences and MRA) and functional imaging techniques (cine MRA), anatomy and function can be assessed reliably by imaging with an area larger than that studied with echocardiography. Even the pulmonary arteries and valve can be evaluated during a single study.35, 36 Comprehensive imaging of this type provides a vehicle for surgical planning. The presence of support devices or arrhythmias limits the use of this technique in sick patients.

All 4 types of Ebstein anomaly that Carpentier et al identified and classified can be imaged by using MRI. Both young and old patients tolerate MRI well.37 Critically ill neonates can be imaged in as few as 15 minutes without adverse sequelae.29 Imaging can be accomplished with 0.5- to 2-T machines.

  • Axial spin-echo ECG-gated images are excellent for assessing valvular anatomy, particularly the septal and anterior leaflets (see Images 4-5), right atrial size, and morphology of the atrialized right ventricle.35
  • The posterior leaflet is imaged best in a coronal or oblique-coronal orientation.
  • Coronal and oblique-coronal views can help in assessing the functional right ventricle.
  • Coronal images can help in assessing the right atrial and size (see Image 6) and conformation of the atrialized right ventricle.35
  • Axial images help in identifying dilatation of the tricuspid valve annulus, which is achieved by calculating the ratio of the tricuspid ring to the mitral ring (normal ratio = 0.9-1.2).38
  • The degree of tricuspid-valve regurgitation or stenosis can be demonstrated by using cine techniques (see Image 7).
  • Right-to-left shunts through an ASD (see Image 8) and right ventricular function (see Image 6) can be analyzed by using cine techniques.35
  • Cine MRI can demonstrate right ventricular dysfunction and paradoxic motion of the ventricular septum. The abnormal septal motion can, in turn, cause abnormalities of left ventricular morphology and function.12
  • Cine MRI can demonstrate mitral-valve prolapse, which is known to be a result of left ventricular distortion.8
  • Multilevel time-of-flight sequences can be used to demonstrate pulmonary artery anatomy.
  • With new sequences, images can be acquired with breath-hold, contrast-enhanced, 3D MRA (see Image 9) more rapidly and reliably than before.

Degree of Confidence

When used properly and in suitable patients, MRI can be regarded as the standard for detecting Ebstein anomaly and for assessing several aspects of the disease when echocardiography is unable to provide all required information. However, MRI has limitations in evaluating the detail of tricuspid valve morphology, which is best ascertained by using echocardiography. In addition, image quality may be impaired in some patients with arrhythmias or large hearts (see Image 6).


ULTRASOUND

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Findings

Echocardiography can demonstrate elongated tricuspid valve leaflets (especially the anterior leaflet) and leaflet thickening, though the leaflets also may demonstrate a whipping motion.39, 40

The right ventricular cavity is dilated, abnormal septal motion may be observed, excursion of the anterior leaflet may be increased, and leaflet tethering may be seen (see Image 10). Low-velocity tricuspid regurgitation, which reflects the usual inability to generate high pressure in the right ventricle, is common (see Images 11-12).

The severity of the lesion can be estimated from the degree of right atrial and ventricular dilatation and from marked apical displacement of the tricuspid valve leaflets (see Images 13-14). The valve can be displaced inferiorly; such displacement is usually to the left.39

Degree of Confidence

Echocardiography is an excellent method for assessing Ebstein anomaly, and it may be the first and only cross-sectional imaging investigation that is required. If inadequate images are obtained, as in evaluating the pulmonary arteries before surgery, or if clinically significant chest deformity is present, MRI or transesophageal echocardiography can be used.


NUCLEAR MEDICINE

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Findings

Cross-sectional imaging has supplanted nuclear medicine study.


ANGIOGRAPHY

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Findings

Traditional imaging of Ebstein anomaly has included cardiac catheterization with right ventriculography. This method is performed less frequently now, except as part of an ablation procedure for arrhythmias. Relatively noninvasive testing with echocardiography or MRI has reduced the need for diagnostic cardiac catheterization.

Findings are identical to those in MRI and echocardiography, though the images are obtained at increased risk and with a decreased amount of information concerning tricuspid valve anatomy.

Contrast angiography demonstrates a large right atrium with a variable degree of tricuspid regurgitation (see Images 15-16). The exact position of the abnormal tricuspid valve may be difficult to determine.

A catheter with a tip electrode (eg, Zukor catheter) can be used to show that atrial pressures are recorded when a right ventricular ECG is detected, indicating displacement of the tricuspid valve towards the cardiac apex.


INTERVENTION

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Patients in whom medical treatment fails or disease progresses may later receive surgical or transcutaneous treatment. Patients with heart block can successfully undergo transvenous single- or dual-chamber pacing or epicardial pacing.5, 41 Pacing wires can be placed both in patients not undergoing surgery and in patients who underwent tricuspid valve replacement. Accessory conduction pathways can be treated with transvenous, radiofrequency, and catheter ablation therapy.42, 43, 44

Surgical Treatment

The surgical treatment of Ebstein anomaly is aimed at preventing arrhythmias, eliminating right-to-left intracardiac shunting, limiting cardiomyopathy, and improving exercise tolerance and functional class. Surgery may be performed in 1 or more stages. Reconstructive procedures include tricuspid valve repair, plication, resitting or replacement; ventricularization of the atrialized right ventricular chamber; closure of any interatrial communications; the Starnes operation with bidirectional Glenn shunt, and Fontan procedures. Surgical antiarrhythmia procedures are preformed to treat accessory pathway–mediated tachycardia (with ablation of an accessory pathway), atrioventricular nodal reentrant tachycardia (with surgical perinodal cryoablation), atrial flutter or fibrillation (with a right-sided Maze procedure or cryoablation of the atrial isthmus).45, 46, 47, 48, 49, 50, 51, 52, 53

Transcutaneous treatment

Percutaneous, transcatheter, electrophysiologic mapping of arrhythmias, particularly supraventricular tachycardia, can be subsequently treated with transcatheter radiofrequency ablation of an accessory conduction pathway. In addition, atrial defects can be closed by using percutaneous methods, transcatheter techniques, with an occlusion device. Transcatheter valvotomy can be performed in patients with pulmonary atresia and an intact ventricular septum.13, 54, 55

Medical/Legal Pitfalls

  • Some patients with Ebstein anomaly may not present until adulthood because of good compensation or mild disease. The complication is the development of irreversible pulmonary hypertension. Adults who have acute decompensation are poor surgical candidates.
  • Prenatal sonography can be helpful in identifying fetal cardiomegaly. Ebstein anomaly with pulmonary atresia has been diagnosed with fetal echocardiography. Hydrops fetalis and Down syndrome have been associated. Postnatal echocardiography can be performed in neonates with cyanosis.56, 57, 58, 59, 60, 61


MULTIMEDIA

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Media file 1:  Frontal chest radiograph in an infant with severe Ebstein anomaly shows a large heart that leaves little space for the lung. Although the appearance is relatively nonspecific, the large heart should suggest Ebstein anomaly in the differential diagnosis.
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Media type:  X-RAY

Media file 2:  Frontal chest radiograph in an adult with Ebstein anomaly shows the abnormal contour associated with distortion of the right ventricle. The pulmonary vasculature is underperfused.
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Media type:  X-RAY

Media file 3:  Lateral chest radiograph in an adult with Ebstein anomaly (same patient as in Image 2) shows prominent filling of the retrosternal space (arrow) related to distortion of the right ventricle.
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Media type:  X-RAY

Media file 4:  Axial ECG-gated spin-echo MRI in a patient with Ebstein anomaly. The patient presented with arrhythmias at the age of 49 years. Image shows enlargement of the right atrium and a dilated right ventricle (RV) compared with the left ventricle. The attachment of the septal leaflet (arrow) of the tricuspid valve is displaced toward the apex of the right ventricle. (Compare this with the level of the mitral valve attachment.)
Click to see larger pictureClick to see detailView Full Size Image
Media type:  MRI

Media file 5:  Axial ECG-gated spin-echo MRI in a patient with Ebstein anomaly (same patient as in Image 4) shows enlargement of the right atrium (RA). The attachment of the septal leaflet (arrow) of the tricuspid valve is seen displaced toward the apex of the right ventricle (RV). LV = left ventricle.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  MRI

Media file 6:  Coronal magnetic resonance angiogram acquired in an adolescent with severe Ebstein anomaly. Image quality is degraded because of resting tachycardia (heart rate >120 bmp). Image shows a large right atrium (RA), a displaced and distorted right ventricular outflow tract (RVOT), and apical displacement of the tricuspid valve leaflets (between the arrows).
Click to see larger pictureClick to see detailView Full Size Image
Media type:  MRI

Media file 7:  Axial cine magnetic resonance angiogram in a patient with Ebstein anomaly (same patient as in Images 4-5) shows enlargement of the right atrium and right ventricle, along with the loss of signal intensity resulting from tricuspid regurgitation (arrow). The origin (apex) of the area of signal loss, corresponding to the level of coaptation of the valve leaflet, indicates the apical displacement of the tricuspid-valve coaptation.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  MRI

Media file 8:  Sagittal-oblique magnetic resonance angiogram in a patient with Ebstein anomaly (same patient as in Images 4, 5, and 7). A presaturation pulse (dotted line) is positioned through the right atrium to suppress signal from flow originating in that area. A small jet of low signal intensity 0(arrow) indicates a small right-to-left shunt through a patent foramen ovale, which was not visible on color flow Doppler echocardiography (not shown). More than one half of patients with Ebstein anomaly have a right-to-left shunt at the atrial level. The shunts can be large or, as shown here, so small that they are difficult to detect by using conventional techniques.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  MRI

Media file 9:  Coronal breath-hold, contrast-enhanced, 3-dimensional magnetic resonance angiogram (maximum intensity projection) in an adolescent with Ebstein anomaly (same patient as in Image 6). The study was performed to assess the size of the pulmonary arteries (arrows) before surgical palliation. Note the huge right atrium (RA), which is seen at the same level as the left ventricle (LV), and the displaced and distorted right ventricular outflow tract (RVOT).
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Media type:  MRI

Media file 10:  Apical 4-chamber, 2-dimensional echocardiogram in a patient with Ebstein anomaly shows displacement of the tricuspid valve toward the apex of the right ventricle (RV) and tethering of the septal leaflet to the interventricular septum (arrow). LA = left atrium; LV = left ventricle; RA = right atrium.
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Media type:  Image

Media file 11:  Apical color flow Doppler sonogram in a patient with Ebstein anomaly (same patient and same orientation as in Image 10) shows severe tricuspid regurgitation, with color filling the right atrium. Blue = flow away from the transducer; red and orange = aliased high-velocity flow or flow swirling anteriorly around the margins of the right atrium.
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Media type:  Image

Media file 12:  Apical continuous-wave (CW) Doppler image in a patient with Ebstein anomaly (same patient as in Images 10-11). Maximum velocity is only approximately 2 m/s, indicating a systolic pressure difference between the right atrium and the right ventricle of only approximately 16 mm Hg. This result indicates that the right ventricular pressure is not increased; this is the usual circumstance in Ebstein anomaly.
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Media type:  Image

Media file 13:  Apical 4-chamber image from 2-dimensional (2D) echocardiography (Echo) in a patient with severe Ebstein anomaly shows displacement of the tricuspid valve towards the apex of the right ventricle (RV) more extreme than that shown in Images 10-11. The atrialized part of the RV is more dilated and the tethering of the septal leaflet extends further toward the apex here than in Images 10-11. LV = left ventricle; RA = right atrium.
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Media type:  Image

Media file 14:  Short-axis image from a 2-dimensional echocardiogram in a patient with severe Ebstein anomaly shows extensive tethering of the septal leaflet to the interventricular septum (same patient as in Images 10-13). RA = right atrium; LV = left ventricle.
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Media type:  Image

Media file 15:  Right ventricular (RV) angiogram in a patient with Ebstein anomaly shows a dilated RV, opacification of a dilated right atrium (RA) resulting from severe tricuspid regurgitation, and relatively small pulmonary arteries. Contrast material in the right heart chambers obscures detailed information regarding the tricuspid valve anatomy.
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Media type:  X-RAY

Media file 16:  Comparison of conventional contrast-enhanced ventriculogram (see Image 15) and 3-dimensional contrast-enhanced magnetic resonance (MR) angiogram (see Image 9). Since MR angiography is performed in 3 dimensions, it can be interrogated with variable image thicknesses to reveal more intracardiac details than that observed with conventional angiography. Conventional contrast-enhanced angiography involves a 2-dimensional acquisition of a projectional image, which cannot be modified in the same way that 3-dimensional MR angiograms can be.
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Media type:  X-RAY

Media file 17:  The chest radiograph shows classic radiographic features of Ebstein anomaly, including a squared-off cardiac contour and pulmonary oligemia.
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Media type:  Radiograph

Media file 18:  The 2-dimensional echocardiogram shows many of the echocardiographic features of severe Ebstein anomaly: a large right atrium, extensive tethering of the septal leaflet of the tricuspid valve, and a consequently large atrialized segment of the right ventricle. The image demonstrates a large right atrium, which is larger functionally than anatomically because of marked atrialization of the right ventricle; this is a consequence of tethering of the septal tricuspid valve leaflet to the ventricular septum.
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Media type:  Echo


REFERENCES

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Ebstein Anomaly excerpt

Article Last Updated: Mar 6, 2008