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Congenital Heart Disease for the Adult Cardiologist

Ebsteins Anomaly
Christine H. Attenhofer Jost, MD; Heidi M. Connolly, MD; Joseph A. Dearani, MD;
William D. Edwards, MD; Gordon K. Danielson, MD

bsteins anomaly is a rare congenital heart disorder


occurring in 1 per 200 000 live births and accounting for 1% of all cases of congenital heart disease.2 6
This anomaly was described by Wilhelm Ebstein in 1866
in a report titled, Concerning a very rare case of insufficiency of the tricuspid valve caused by a congenital
malformation.7,8 The patient was a 19-year-old cyanotic
man with dyspnea, palpitations, jugular venous distension,
and cardiomegaly.7,8 At autopsy, Ebstein described an
enlarged and fenestrated anterior leaflet of the tricuspid
valve. The posterior and septal leaflets were hypoplastic,
thickened, and adherent to the right ventricle. There was
also a thinned and dilated atrialized portion of the right
ventricle, an enlarged right atrium, and a patent foramen
ovale9 (Figure 1). By 1950, only 3 cases of this anomaly
had been published.8,10,11

Pathological Anatomy
In the normal heart, the tricuspid valve has 3 leaflets:
anterior, posterior, and septal.12,13 Ebsteins anomaly is a
malformation of the tricuspid valve and right ventricle
characterized by (1) adherence of the septal and posterior
leaflets to the underlying myocardium (failure of delamination, namely splitting of the tissue by detachment of the
inner layer during embryologic development); (2) downward (apical) displacement of the functional annulus
(septalposterioranterior); (3) dilation of the atrialized portion of the right ventricle, with various degrees of
hypertrophy and thinning of the wall; (4) redundancy,
fenestrations, and tethering of the anterior leaflet; and (5)
dilation of the right atrioventricular junction (true tricuspid
annulus).6,14
The apical displacement of the hinge point of the valve in
Ebsteins anomaly from the atrioventricular ring is shown in
Figure 2.12,1517 The point of maximal displacement is at the
commissure between the posterior and septal leaflets of the
tricuspid valve.16 In normal human hearts, the downward
displacement of the septal and posterior leaflets in relation to
the anterior mitral valve leaflet is 8 mm/m2 body surface
area.6 The spectrum of the malformation in Ebsteins anomaly may range from only minimal displacement of the septal
and posterior leaflets to an imperforate membrane or muscu-

lar shelf between the inlet and trabecular zones of the right
ventricle.
The anterior leaflet is generally redundant and may contain
several fenestrations.6 Its chordae tendineae are generally
short and poorly formed. Moreover, the anterior leaflet of the
tricuspid valve may be severely deformed, so that the only
mobile leaflet tissue is displaced into the right ventricular
outflow tract, where it may cause obstruction or form a large
sail-like intracavitary curtain. Typical autopsy examples of
Ebsteins anomaly are shown in Figures 3 and 4.
In Ebsteins anomaly, the right ventricle is divided into 2
regions: the part directly involved with the malformation (ie, the
inlet portion), which is functionally integrated with the right
atrium, and the part that is not involved by the anomaly, which
consists of the other 2 components of the right ventricle, namely
the trabecular and outlet portions, that constitute the functional
right ventricle. The atrialized portion of the right ventricle (ie,
the inlet component) can become disproportionately dilated and
may account for more than half of the right ventricular volume
in extreme cases instead of the usual one third of the total right
ventricular volume. There is often marked dilatation of the true
tricuspid valve annulus, which is not displaced, and a large
chamber separating this true annulus from the functional right
ventricle (atrialized portion of the right ventricle)6,12 (Figure 2).
The right coronary artery demarcates the level of the true
annulus and may become kinked during plication annuloplasty
procedures.
Two thirds of hearts with Ebsteins anomaly show
dilated right ventricles. Dilatation often involves not only
the atrialized inlet portion of the right ventricle but also the
functional right ventricular apex and outflow tract. In some
cases, right ventricular dilatation is so marked that the
ventricular septum bulges leftward, compressing the left
ventricular chamber.6 In such cases, the short-axis view
demonstrates a circular right ventricle and a crescentic left
ventricle. In extreme cases, episodic left ventricular outflow tract obstruction can occur.

Nomenclature and Classification


We use 2 approaches in describing the anatomic severity of
Ebsteins anomaly. The first approach is based on the
echocardiographic appearance in which the abnormality is

From the Divisions of Cardiovascular Diseases (C.H.A.J., H.M.C.), Cardiovascular Surgery (J.A.D.), and Anatomic Pathology (W.D.E.), Mayo Clinic,
Rochester, Minn.
Dr Danielson is an emeritus member, Division of Cardiovascular Surgery, Mayo Clinic, Rochester, Minn.
This article is based in part on a previously published manuscript.1 Used with permission.
Reprint requests to Heidi M. Connolly, MD, Division of Cardiovascular Diseases, Mayo Clinic, 200 First St SW, Rochester MN, 55905.
(Circulation. 2007;115:277-285.)
2007 American Heart Association, Inc.
Circulation is available at http://www.circulationaha.org

DOI: 10.1161/CIRCULATIONAHA.106.619338

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Figure 2. Top, Normal tricuspid valve with anterior, posterior,


and septal leaflets in 1 plane. Middle, Tricuspid valve in rightsided Ebsteins anomaly showing displacement of posterior and
septal leaflets; maximal displacement is at the crux of the posterior and septal leaflets. Bottom, Tricuspid valve in left-sided
Ebsteins anomaly; the displacement of leaflets is similar to that
in the right-sided anomaly. From Anderson et al.16 Used with
permission of the Mayo Foundation for Medical Education and
Research.

Figure 1. Figure from Ebsteins original case report. The right


atrium and right ventricle are shown opened along the right border beginning at the superior vena cava. A, Right atrium; B,
right ventricle; b, valve; I, rudimentary septal leaflet of tricuspid
valve with its chordae tendineae, which insert on the endocardium of the ventricular septum; r, opening through which one
can get into the right conus arteriosus, and in the opposite
direction, one can get into the sac that is formed by membrane
h, h, and posterior part of endocardium of ventricular septum o.
From Mann and Lie.7 Used with permission of the Mayo Foundation for Medical Education and Research.

described as anatomically mild, moderate, or severe. The


amount of displacement and tethering of the leaflets and the
degree of right ventricular dilatation are assessed. This
classification is imprecise but simple. Our second approach is
to describe the exact anatomy of each of the involved
structures of the heart as visualized at operation. This nomenclature system emphasizes characteristics that surgeons find
important when considering repair versus replacement of the
tricuspid valve.12
In 1988, Carpentier et al18 proposed the following classification of Ebsteins anomaly: type A, the volume of the true
right ventricle is adequate; type B, a large atrialized component of the right ventricle exists, but the anterior leaflet of the
tricuspid valve moves freely; type C, the anterior leaflet is
severely restricted in its movement and may cause significant
obstruction of the right ventricular outflow tract; and type D,
almost complete atrialization of the ventricle except for a
small infundibular component.
Celermajer et al19 described an echocardiographic grading
score for neonates with Ebsteins anomaly, extended Glas-

gow Outcome Scale, with grades 1 to 4.19 The ratio of the


combined area of the right atrium and atrialized right ventricle is compared with that of the functional right ventricle and
left heart (ratio 0.5, grade 1; ratio of 0.5 to 0.99, grade 2;
ratio of 1.0 to 1.49, grade 3; ratio 1.5, grade 4).

Prevalence and Genetic Factors


The leaflets of the tricuspid valve develop equally from the
endocardial cushion tissues and the myocardium.13 The leaflets and tensile apparatus of the atrioventricular valves are
formed by a process of delamination of the inner layers of the
inlet zone of the ventricles. In Ebsteins anomaly, delamination of the tricuspid valve leaflets fails to occur, but the
mechanism for this is not entirely understood.16

Figure 3. Marked cardiomegaly caused by right-sided chamber


dilatation in a 67-year-old man with severe Ebsteins anomaly,
with normal heart at right for comparison (anterior view).

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Ebsteins anomaly.14,15,2730 We recently reported features


resembling noncompaction in 3 patients with the anomaly.31
Since then, we have analyzed 106 consecutive patients who
had Ebsteins anomaly and found left-sided heart abnormalities in 39%; 18% of these patients had left ventricular
dysplasia resembling noncompaction.32
Most patients with congenitally corrected transposition of
the great arteries have an abnormal systemic tricuspid valve,
which fulfills the criteria for Ebsteins anomaly in 15% to
50% of cases.16,3335 It is unclear whether the fundamental
nature of the anomaly is identical in concordant and discordant atrioventricular connections.36,37 The morphological
right ventricle is rarely dilated in congenitally corrected
transposition.14,36

Physiology

Figure 4. Severe Ebsteins malformation of tricuspid valve


(4-chamber view) showing marked downward displacement of
shelf-like posterior leaflet with attachment to underlying free wall
by numerous muscular stumps (arrows), markedly dilated atrialized portion of right ventricle (ARV), small functional portion of
right ventricle (RV), leftward bowing of ventricular septum, and
marked dilatation of right atrium (RA). LA indicates left atrium;
LV, left ventricle.

There are heterogeneous genetic factors in Ebsteins anomaly. Case-control studies suggest genetic, reproductive, and
environmental risk factors (eg, the anomaly is more common
in twins, in those with a family history of congenital heart
disease, and in those with maternal exposure to benzodiazepines).4 Maternal lithium therapy can rarely lead to Ebsteins
anomaly in the offspring.20 Most cases are sporadic; familial
Ebsteins anomaly is rare.
In a genetic study of 26 families with Ebsteins anomaly,
93 of 120 first-degree relatives were evaluated.21 No case of
the anomaly was found, but 2 first-degree relatives had
ventricular septal defects, and another, who died at 7 months,
was said to have had congenital heart disease. Rare cases of
cardiac transcription factor NKX2.5 mutations, 10p13-p14
deletion, and 1p34.3-p36.11 deletion have been described in
the anomaly.2224

Associated Cardiac Malformations in


Ebsteins Anomaly
An interatrial communication is present in 80% to 94% of
patients with Ebsteins anomaly.25,26 Additional associated
anomalies include bicuspid or atretic aortic valves, pulmonary atresia or hypoplastic pulmonary artery, subaortic stenosis, coarctation, mitral valve prolapse, accessory mitral
valve tissue or muscle bands of the left ventricle, ventricular
septal defects, and pulmonary stenosis.1
Abnormalities of left ventricular morphology and function,
as well as other left-sided heart lesions, also occur in

The functional impairment of the right ventricle and regurgitation of the tricuspid valve retard forward flow of blood
through the right side of the heart. In addition, during
contraction of the atrium, the atrialized portion of the right
ventricle balloons out and acts as a passive reservoir, decreasing the volume of ejected blood. The overall effect on the
right atrium is dilatation, increasing the size of an interatrial
communication. Tricuspid regurgitation increases by annular
dilatation.14 Associated heart disease in Ebsteins anomaly
has a further effect on physiology.

Clinical Features
The cardinal symptoms in Ebsteins anomaly are cyanosis,
right-sided heart failure, arrhythmias, and sudden cardiac
death. The hemodynamic variations and clinical presentation
depend on age at presentation, anatomic severity, hemodynamics, and degree of right-to-left interatrial shunting.38
On examination, the jugular venous pulse rarely shows a
large V wave despite severe regurgitation of the tricuspid
valve because the large right atrium engulfs the increased
volume. A widely and persistently split second heart sound
and several added sounds are typical.38 A systolic murmur
may be audible. Digital clubbing depends on the degree of
cyanosis.38
Ebsteins anomaly is a common lesion referred for fetal
echocardiography because severe forms may lead to cardiomegaly, hydrops, and tachyarrhythmias.39,40
Neonates with Ebsteins anomaly may present with cyanosis, congestive heart failure caused by regurgitation of the
tricuspid valve, and marked cardiomegaly.39 Symptomatic
children with Ebsteins anomaly may have progressive rightsided heart failure, but most will reach adolescence and
adulthood.
Children 10 years of age and adults often present with
arrhythmias.19 Adults also present with progressive cyanosis,
decreasing exercise tolerance, fatigue, or right-sided heart
failure. In the presence of an interatrial communication, the
risk of paradoxical embolization, brain abscess, and sudden
death increases.19 Exercise tolerance is dependent on heart
size and oxygen saturation.41,42

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Figure 5. Example of an echocardiogram (4-chamber view,


apex down) of a patient with severe Ebsteins anomaly showing
a grossly displaced septal leaflet (arrow). The anterior leaflet is
severely tethered and nearly immobile. The functional right ventricle (RV) is small. ARV indicates atrialized right ventricle; LA,
left atrium; LV, left ventricle; and RA, right atrium.

Diagnostic Evaluation
Echocardiography
Echocardiography, the diagnostic test of choice for Ebsteins
anomaly, has largely obviated cardiac catheterization38,43
(Figure 5). Echocardiography allows accurate evaluation of
the tricuspid valve leaflets and the size and function of the
cardiac chambers.
The principal feature of Ebsteins anomaly is apical displacement of the septal leaflet of the tricuspid valve from the
insertion of the anterior leaflet of the mitral valve by at least
8 mm/m2 body surface area.6 Tethering of the tricuspid valve
is present if there are at least 3 accessory attachments of the
leaflet to the ventricular wall, causing restricted motion of the
leaflet.39 Marked enlargement of the right atrium and atrialized right ventricle is present when the combined area of the
right atrium and atrialized right ventricle is larger than the
combined area of the functional right ventricle, left atrium,
and left ventricle measured in the apical 4-chamber view at
end diastole.19 The site and degree of regurgitation of the
tricuspid valve and the feasibility of valve repair also are
assessed with echocardiography.14
Cine magnetic resonance imaging may be used to assess
ventricular size and function when echocardiographic image
quality is inadequate.44,45

Electrocardiography
The ECG is abnormal in most patients with Ebsteins anomaly. It may show tall and broad P waves as a result of right

atrial enlargement, as well as complete or incomplete right


bundle-branch block.38 The R waves in leads V1 and V2 are
small. Bizarre morphologies of the terminal QRS pattern
result from infra-Hisian conduction disturbance and abnormal
activation of the atrialized right ventricle.46 A typical ECG is
shown in Figure 6.
Complete heart block is rare in Ebsteins anomaly, but
first-degree atrioventricular block occurs in 42% of patients
because of right atrial enlargement and structural abnormalities of the atrioventricular conduction system.38,47 The atrioventricular node may be compressed and the central fibrous
body abnormally formed. The right bundle branch may be
abnormal or show marked fibrosis (or both).15,16,48
The downward displacement of the septal leaflet of the
tricuspid valve is associated with discontinuity of the central
fibrous body and septal atrioventricular ring with direct
muscular connections, thus creating a potential substrate for
accessory atrioventricular connections and pre-excitation.5,6
From 6% to 36% of patients with Ebsteins anomaly have 1
accessory pathways,26,38,46,49,50 and most accessory pathways
are located around the orifice of the malformed tricuspid
valve.25,27,47 Correct identification and treatment of accessory
pathways are essential and may help to prevent sudden
cardiac death. Paroxysmal tachyarrhythmias in Ebsteins
anomaly are based on typical, fast-conducting atrioventricular
accessory pathways with both antegrade and retrograde conduction properties in most patients.46 In addition, wide QRS
tachycardia over a septal accessory atrioventricular pathway,
ventricular tachycardia, or flutter, as well as ectopic atrial
tachycardia, atrial flutter, and atrial fibrillation, can occur.46,50
Atrial fibrillation and atrial flutter are most likely caused by
secondary alterations of the right atrial myocardium from
previous cardiac surgery or are postoperative as a result of
incisional atrial tachycardia.46

Chest Radiography
The cardiac silhouette may vary from almost normal to the
typical Ebsteins anomaly configuration consisting of a
globe-shaped heart with a narrow waist similar to that seen
with pericardial effusion (Figure 7). Vascularity of the pulmonary fields is either normal or decreased. A cardiothoracic
ratio 0.65 carries a poor prognosis.

Cardiac Catheterization
Diagnostic cardiac catheterization is rarely necessary in
patients with Ebsteins anomaly, other than for preoperative
coronary angiography. Right ventricular and pulmonary artery pressures are usually normal in patients with the anomaly, although the right ventricular end-diastolic pressure may
be increased. Right atrial pressure may be normal despite

Figure 6. ECG of a patient with severe


Ebsteins anomaly showing the typical
changes, with prolongation of the PR
interval (226 ms), right bundle-branch
block, and somewhat bizarre configuration of the QRS complex.

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in patients with the anomaly than in those with structurally


normal hearts, and the risk of recurrence is increased.46,5254
Supraventricular tachyarrhythmia associated with Ebsteins
anomaly also can be ablated at the time of operative
repair.55,56

Surgical Options

Figure 7. Chest radiograph of a patient who had Ebsteins


anomaly with severe tricuspid regurgitation and a small atrial
septal defect before tricuspid valve surgery. This typical image
shows cardiomegaly, a narrow waist, and a cardiothoracic ratio
of 0.56.

severe regurgitation of the tricuspid valve, especially if the


right atrium is markedly dilated. Oximetry may show systemic arterial desaturation in the presence of an interatrial
communication and right-to-left shunting.

Management
Medical
Any patient with Ebsteins anomaly needs to be evaluated
regularly by a cardiologist who has expertise in congenital
heart disease. Prophylaxis for endocarditis is recommended
despite its low risk in the anomaly.
Physical activity recommendations are summarized by
Task Force 1 on Congenital Heart Disease.51 Athletes with
mild Ebsteins anomaly, nearly normal heart size, and no
arrhythmias can participate in all sports. Athletes with severe
Ebsteins anomaly are precluded from sports unless the
anomaly has been optimally repaired, the heart size is nearly
normal, and no history of arrhythmias exists.
Patients with Ebsteins anomaly and cardiac failure who
are not candidates for surgery are treated with standard heart
failure therapy, including diuretics and digoxin. The efficacy
of angiotensin-converting enzyme inhibitors in patients with
Ebsteins anomaly who have right-sided heart failure is
unproved. Medical management of arrhythmias should be
individualized and combined with operative or catheter-based
intervention.

Catheter Ablation
Electrophysiological evaluation and radiofrequency ablation
of symptomatic accessory pathway(s) should be performed
when feasible in patients with Ebsteins anomaly who have
tachyarrhythmias. Catheter ablation has a lower success rate

In 1959, repair of the tricuspid valve was reported in 2


patients who had Ebsteins anomaly; both died.57 Successful
operative intervention for regurgitation of the tricuspid valve
in patients with the anomaly was first described in 1962; the
valve was replaced.58 The initial publication on patients with
Ebsteins anomaly undergoing tricuspid valve replacement
reported a surgical mortality of 54%.49 Similar high early
mortality and unsatisfactory late results for tricuspid valve
repair by the methods available at that time were
described.57,59
Between April 1972 and January 2005, 540 consecutive
patients with Ebsteins anomaly were operated on at Mayo
Clinic Rochester. The age at operation ranged from 2 months
to 79.1 years (median, 20 years). Of those having a tricuspid
valve procedure, valve reconstruction was possible in 34.4%,
and valve replacement (usually bioprosthesis) was performed
in 65.6%. There were 29 early deaths (5.4%). Late results of
the first 323 operations have been reviewed.60 There were 23
late deaths (7.6%) during a follow-up extending to 25 years
(mean, 7.1 years).
Our initial repair technique reported in 1979 consisted of
plication of the free wall of the atrialized portion of the right
ventricle, posterior tricuspid annuloplasty, and right reduction
atrioplasty.61 The repair is based on the construction of a
monocuspid valve using the anterior leaflet. Since the initial
report, we have incorporated various modifications of tricuspid valve repair, depending on the numerous variants encountered with the anatomy of Ebsteins anomaly.14,62 Our current
repair usually involves bringing the anterior papillary muscle(s) toward the ventricular septum, thus facilitating coaptation of the leading edge of the anterior leaflet with the
ventricular septum (Figure 8). Generally, an anteroposterior
tricuspid purse-string annuloplasty is used, and atrialized
right ventricular plication or resection is performed selectively. This results in a tricuspid valve repair at the level of
the functional annulus, in contrast to our original repair,
which brought the functional annulus up to the true annulus.
We believe the 2 most important features that enable a
successful, durable repair are a free leading edge of the
anterior leaflet and at least 50% delamination of the anterior
leaflet.
In 1988, Carpentier et al18 proposed a repair that used
mobilization of the anterior leaflet of the tricuspid valve. For
their types B and C, temporary detachment of the anterior
leaflet and adjacent part of the posterior leaflet was followed
by longitudinal plication of the atrialized ventricle and
adjacent right atrium, repositioning of the anterior and posterior leaflets to cover the orifice area at the normal level, and
remodeling and reinforcement of the tricuspid annulus with a
prosthetic ring. This repair was reported in 191 patients
(meanSD age, 2415 years).64 The early mortality rate was
9%, and the mean late survival rate at 20 years was 82%5%.

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Figure 8. Diagram of the tricuspid valve repair technique currently used for Ebsteins anomaly. A, Two papillary muscles
arise from the free wall of the right ventricle, with short chordal
attachments to the leading edge of the anterior leaflet. The septal leaflet is diminutive and only a ridge of tissue. The posterior
leaflet is not well formed and is adherent to the underlying
endocardium. A small patent foramen ovale is present. B, C,
The base of each papillary muscle is moved toward the ventricular septum at the appropriate level with horizontal mattress
sutures backed with felt pledgets. The patent foramen ovale is
closed by direct suture. D, The posterior angle of the tricuspid
orifice is closed by bringing the right side of the anterior leaflet
down to the septum and plicating the nonfunctional posterior
leaflet in the process. E, A posterior annuloplasty is performed
to narrow the diameter of the tricuspid annulus. The coronary
sinus marks the posterior and leftward extent of the annuloplasty. F, An anterior purse-string annuloplasty is performed to
further narrow the tricuspid annulus. This annuloplasty stitch is
tied down over a 25-mm valve sizer in an adult to prevent tricuspid stenosis. G, Completed repair that allows the anterior
leaflet to function as a monocuspid valve. From Dearani et al.63
Used with permission of Mayo Foundation for Medical Education and Research.

It is unclear whether late problems will develop because of


devitalized tricuspid valve tissue related to reattachment.
Another repair technique is characterized by reintegration
of the atrialized chamber into the right ventricular cavity
(called ventricularization). Ventricularization can be obtained
by orthotopic transposition of the detached septal and posterior leaflets of the tricuspid valve. The reimplanted septal

leaflet serves as an opposing structure for coaptation of the


reconstructed atrioventricular valve.65
Posterior annular plication without plication of the atrialized right ventricle and prophylactic cavopulmonary connection are additional surgical options for nonneonatal Ebsteins
anomaly; however, no long-term follow-up data are available.66 The benefit of adding a bidirectional cavopulmonary
shunt after tricuspid valve repair or replacement to reduce
right ventricular volume load in selected patients with Ebsteins anomaly is unclear. We use the bidirectional cavopulmonary shunt when the right ventricle is markedly dilated and
functioning poorly. This allows a reduced volume load on the
right ventricle and improves preload to the left ventricle. Left
atrial and pulmonary artery pressures must be low for the
shunt to provide hemodynamic benefit. In our experience, a
modified Fontan procedure is very rarely required for patients
with Ebsteins anomaly who present after infancy.
It has not been shown whether tricuspid valve repair or
replacement has the better long-term outcome, nor is it known
whether bioprostheses are preferable to mechanical prostheses for tricuspid valve replacement in Ebsteins anomaly. One
study has suggested that valve repair is less durable in adults
than in children.67 It is known that tricuspid bioprostheses in
Ebsteins anomaly have greater durability than in other
cardiac positions, especially for pediatric patients; the mean
rate of freedom from bioprosthesis replacement was
80.67.6% in a study with up to 17.8 years of follow-up
(mean, 4.5 years).68 In a series of 294 patients with Ebsteins
anomaly, the difference in freedom from reoperation at 12
years for tricuspid valve repair versus replacement and for
bioprosthetic versus mechanical valve prostheses was not
significant.68 Currently, we prefer valve repair, when feasible,
over valve replacement because repair has the potential of
being more durable and avoids the potential complications of
valve prostheses. Some of our early patients are doing well,
free of reoperation 20 years after valve repair. We generally
prefer bioprostheses over mechanical prostheses for Ebsteins
anomaly, reserving the latter for those who are already taking
anticoagulants for another indication.14 The method we currently use for tricuspid valve replacement in Ebsteins anomaly is shown in Figure 9.
Although a decrease in atrial tachyarrhythmias after standard repair of Ebsteins anomaly generally has been noted,69
we prefer to combine repair with directed antiarrhythmia
procedures.14,53,56 This can be accomplished without an increase in operative mortality and with freedom from arrhythmia recurrence in 75% of patients with atrial flutter or
fibrillation and in up to 100% of patients with accessory
pathwaymediated tachycardia or atrioventricular nodal reentry tachycardia.55,70
Symptomatic neonates with Ebsteins anomaly have a poor
prognosis. Marked cardiac enlargement, advanced echocardiographic severity score, cyanosis, and severe regurgitation
of the tricuspid valve all predict neonatal death without
surgery.19,71 Biventricular repairs in combination with correction of all associated cardiac defects are feasible, and midterm
results are good.71 Conversion to a single-ventricle approach
for symptomatic neonates also has been advocated.72 Results
of tricuspid valve repair in young children have been reported

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Pacing
Permanent pacing is required for 3.7% of patients with
Ebsteins anomaly, most commonly for atrioventricular block
and rarely for sinus node dysfunction.75 In the presence of a
tricuspid valve prosthesis, the ventricular lead for permanent
DDD pacing usually is placed epicardially or through the
coronary sinus or a cardiac vein. Alternatively, a previously
placed transvenous ventricular lead may be sutured outside
the prosthesis sewing ring at the time of valve replacement.
Placement of a transvenous ventricular lead through a bioprosthesis is effective but less desirable because of the
possibility of propping open one of the valve cusps, thus
creating regurgitation of the tricuspid valve. This complication can be minimized by use of transesophageal echocardiographic monitoring to ensure that the lead lies safely in a
commissure between the valve cusps.

Natural History and Long-Term Sequelae


Figure 9. Diagram of technique for tricuspid valve replacement
in Ebsteins anomaly. A, The valve suture line is placed on the
atrial side of the membranous septum and atrioventricular (AV)
node to avoid injury to the conduction system. The suture line is
also deviated cephalad to the tricuspid annulus posterolaterally
when the tissues are thin to avoid injury to the right coronary
artery. When sufficient distance between the coronary sinus and
the AV node exists, the coronary sinus may be left on the atrial
side of the suture line. B, The sutures are tied with the heart
perfused and beating to ensure that a conducted rhythm is preserved. From Dearani et al.63 Used with permission of Mayo
Foundation for Medical Education and Research.

and demonstrate low early mortality and good durability at


late follow-up.73

Indications for Operation


Observation alone is advised for asymptomatic patients with
no right-to-left shunting and only mild cardiomegaly. Children who have survived infancy generally do well for several
years, and surgery can be postponed until symptoms appear,
cyanosis becomes evident, or paradoxical emboli occur.
Deliberations about an operation should begin if evidence of
deterioration exists, such as progressive increase in right heart
size, reduction in systolic function, or appearance of ventricular or atrial tachyarrhythmias. However, once symptoms
progress to New York Heart Association functional class III
or IV, medical management has little to offer, surgical risks
increase, and operation is clearly indicated. A biventricular
reconstruction is feasible for most patients. A 1.5 ventricle
repair can be applied to the failing right ventricle. Heart
transplantation is reserved for patients with severe biventricular dysfunction.
Some patients with cyanosis on exercise who have a shunt
at the atrial level but only mild or moderate regurgitation of
the tricuspid valve may benefit from device closure to
alleviate cyanosis and to prevent paradoxical emboli. Some
centers commonly perform such procedures either as a staged
approach or for long-term palliation.74 The degree of tricuspid
valve regurgitation must be assessed carefully, however,
because closure of an atrial septal defect alone may worsen
right ventricular dysfunction.

Several studies have reported on the natural history of


Ebsteins anomaly.49,76 78 The largest of these studies, reporting on 505 patients with Ebsteins anomaly, was published
30 years ago.49 The study consisted primarily of patients
between 1 and 25 years of age, with 67 patients 25 years
and only 35 patients 1 year of age. Of the infants 1 year
of age, 72% were in heart failure, but for 81% of the others,
growth and development during infancy were average or
good. In addition, 71% of children and adolescents and 60%
of adults were classified as New York Heart Association
functional class I or II.49 A high mortality rate from congestive heart failure was noted during the first few months of life;
subsequently, mortality plateaued at an average of 12%
scattered uniformly throughout childhood and adolescence.
Of those who had surgical treatment, 54% did not survive the
operation. This study was published before the echocardiographic era and thus does not reflect the current Ebsteins
anomaly population.
Of all neonates with the diagnosis of Ebsteins anomaly,
20% to 40% do not survive 1 month, and 50% survive to 5
years.79 82
Celermajer et al19 reviewed 220 cases of Ebsteins anomaly
with 1 to 34 years of follow-up. Actuarial survival for all
live-born patients was 67% at 1 year and 59% at 10 years.
Predictors of death were echocardiographic grade of severity
at presentation (relative risk increased by 2.7 for each
increase in grade), fetal presentation, and right ventricular
outflow tract obstruction.
In rare cases, patients with Ebsteins anomaly live 70
years, but 1 reported patient died at 85 years of age.38 A
reassessment of the prognosis of Ebsteins anomaly is appropriate in the current era of refined cardiovascular
intervention.

Conclusions
Ebsteins anomaly is a complex congenital anomaly with a
broad anatomic and clinical spectrum. Management is complex and must be individualized. Precise knowledge about the
different anatomic and hemodynamic variables, associated
malformations, and management options is essential. Thus, it
is important that patients with Ebsteins anomaly be evalu-

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284

Circulation

January 16, 2007

ated regularly by a cardiologist who has expertise in congenital heart disease.83 85 With better management strategies, it is
hoped that survival of patients with this anomaly of all ages
will continue to improve.

Acknowledgment
Editing, proofreading, and reference verification were provided by
the Section of Scientific Publications, Mayo Clinic.

Disclosures
None.

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KEY WORDS: atrium heart defects, congenital
pathology pediatrics

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hemodynamics

Ebstein's Anomaly
Christine H. Attenhofer Jost, Heidi M. Connolly, Joseph A. Dearani, William D. Edwards and
Gordon K. Danielson
Circulation. 2007;115:277-285
doi: 10.1161/CIRCULATIONAHA.106.619338
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