Ebstein anomaly

Ebstein’s anomaly
• Embryology
• Anatomy
• Physiology
• Natural history
• Clinical findings
• Laboratory findings
• Management

• Ebstein’s anomaly occurs in approximately 1 in 20,000
live births.
• Accounts for 0.3% to 0.7% of all cases of congenital heart
disease.
• Represents about 40% of congenital malformations of the
tricuspid valve.
• Originally described by Wilhelm Ebstein in 1866.
Patane et al Int J Cardiol. 2009;136:e6–e7.

Embryology:
• Failure of DELAMINATION of septal and posterior/inferior
/mural leaflets of TV.
• A layer of endomyocardium separates or delaminates,
from the underlying ventricular myocardium, and loses its
muscular character.
• This separation begins within the cavity and proceeds
back toward the atrioventricular junction, eventually
forming the valvar complex.
• An abnormality of both myocardial and valvular
development.
• Anterior and Apical Rotation of the Functional Orifice.
Cardiol Young 2006; 16 (Suppl. 3): 4–11

Normal Delamination (Separation) of the TV
from the RV Myocardium

Spectrum of Failed TV Delamination seen in
EbsteinAnomaly

The diagram shows the location of the orifice of the abnormal valve,
shown by the green ovals, as observed in the series of hearts examined. The valvar
orifice is displaced rotationally,
rather than in a downward fashion.
Circulation. 2007;
115: 277-285

Anatomy – normal tricuspid valve
• Normal tricuspid leaflets consist of basal attachments to
the annulus (right atrioventricular sulcus), peripheral
zones into which chordae tendineae insert, and clear
zones that lie between the basal attachments and the
peripheral zones.
• The semicircular or quadrangular anterior leaflet is the
largest of the three.
• The posterior leaflet is scalloped.

• The septal leaflet attaches chiefly to the ventricular
septum , but part of its basal attachment is to the posterior
wall of the right ventricle.
• The septal leaflet normally exhibits a slight but distinct
apical displacement of its basal attachment compared
with the mitral valve: 15 mm in children, and 20 mm in
adults.
Silver MD, et al .Morphology of the human tricuspid valve. Circulation.
1971;43:333–348.

Anatomy: Ebstein’s anomaly
1. Adherence of the septal and inferior leaflets to the
underlying myocardium.
2. Downward displacement of the functional annulus.
3. Dilation of the “atrialized” portion of the RV, with
variable degrees of thinning of the free wall.
4. Redundancy, fenestrations and tethering of the
anterior leaflet.
5. Dilation of the right AV junction (true tricuspid
annulus).
Attenhofer Jost et al. Circulation. 2007;115:277-285

Ebstein’sAnomaly
• The right ventricle is divided into 2 regions:
• the inlet portion – the part involved in malformaton,
• the trabecular
the functional right ventricle.
• outlet portions,
• 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.

• Marked dilatation of the true tricuspid valve annulus.
• A large chamber separates this true annulus from the
functional right ventricle i.e.(atrialized portion of the right
ventricle).
Danielson GK. : Ebstein’s anomaly and tricuspid valve disease. Ann Thorac
Surg. 2000;69(suppl):S106 –S117.

Figure from Ebstein’s original case report.. 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. Circulation. 2007; 115: 277-285

Severe Ebstein’s
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
Circulation. 2007; 115: 277-285

Severe Ebstein’s malformation
Alain Carpentier, et al. Carpentier's Reconstructive Valve Surgery: From Valve Analysis to Valve
Reconstruction CHAPTER 24 , 247-257
Chauvaud S, Carpentier A (2007). MMCTS. doi:10.1510/mmcts.2007.003038

Abnormalities in left side of heart
• Reported in 39% of patients with Ebstein’s anomaly.
• Derangements in left ventricular geometry, impairment of
systolic and diastolic function and noncompaction.
• Superior systolic displacement of the mitral valve
(prolapse) occurs because mitral leaflets with normal
areas and chordal lengths are housed in a small left
ventricular cavity.
• Abnormal shape,
• Impaired diastolic filling, systolic function
• Increased fibrous content of the free wall and the
ventricular septum.
Inai K, Nakanishi et al . Am J Cardiol. 2004;93:255-258.

1988 Carpentier et al.
• Classification:
• Type A: volume of true RV is adequate.
• Type B: large atrialized component of the RV exists, but
the anterior leaflet of the TV moves freely.
• Type C: the anterior leaflet is severely restricted in its
movement and may cause obstruction to RVOT.
• Type D: almost complete atrialization of the RV
except for small infundibular component.

Carpentier classification
TYPE A (M+ C+) TYPE B (M+ C -)
TYPE С (M- C -) TYPE D (“tricuspid suck”)
M – mobility C - contractility

Classification by anatomic findings at surgery
Dearani, et al. Ann Thorac Surg 2000; 69:S106. Moss & Adams' Heart Disease in Infants, Children,
and Adolescents: Including the Fetus and Young Adult , 8th edit. 889-912

Epidemiology
• 0.3-0.8% of all congenital heart diseases.
• 1 in 20,000-50,000 live births.
• Equal male: female occurrence.
• The relative risk of Ebstein’s anomaly is increased by 500-
fold in offspring exposed to in utero lithium carbonate
(Danish registery).
• Mortality in the neonatal period is 20%-40%. and less
than 50% survive to 5 years.
• Mortality at all ages is 12.5%
Nora JJ, et al Lancet. 1974;2:594–595.

Associations:
• ASD/PFO in 80-94%
• Additional associated anomalies :
• VSD +/- pulmonary atresia
• RVOT obstruction
• PDA
• Coarctation
• Left sided lesions in 39%: MVP, systolic dysfunction, Subaortic
stenosis, BAV, LV muscle bands, LV noncompaction in 18%.
• Accessory conduction pathway in 15-20%, usually near the orifice
of TV.
• CCTGA (15-50% of CCTGA patients meet criteria for EA).
• Brickner ME,et al N Engl J Med. 2000;342:334 –342.
• Attenhoferet al. Mayo Clin Proc. 2005;80:361–368 .
• Anderson et alCirculation. 1978;58(suppl):I-87–I-91.

Etiology and genetic factors
• More common in twins.
• Family history of congenital heart disease.
• Maternal exposure to benzodiazepines.
• Maternal lithium therapy.
• Most cases are sporadic.
• Familial Ebstein’s anomaly is rare.
Correa-Villasenor A et al Ebstein’s malformation of the tricuspid valve: genetic
and environmental factors. Teratology. 1994;50:137–147

Genetic factors
• Rare cases of cardiac transcription factor NKX2.5
mutations,
• 10p13-p14 deletion,
• 1p34.3-p36.11 deletion have been described in the
anomaly .

Physiology
RV
impairment
Regurgitation of
tricuspid valve
Forward flow
through right
side of heart
reduced
Atrial
contraction
Atrialized
portion of right
ventricle
balloons out
Acts as a
passive
reservoir
Decreases
volume of
ejected blood
RA dilatation and
increased
interatrial
communication

Clinical presentation
Most common presentation by age:
Celermajer DS, Bull C, Till JA, Cullen S, Vassillikos VP, Sullivan ID, et al.
Ebstein’s anomaly: presentation and outcome from fetus to adult. J Am Coll
Cardiol 1994;23:170–6.
• Fetus: abnormal routine scan (86%)
• Neonates: cyanosis (74%)
• Infants: heart failure (43%)
• Children: Incidental murmur (63%)
• Teens and adults: arrhythmia (42%), decreased exercise tolerance,
fatigue, right-sided failure, paradoxical embolism, brain abscess.

Conduction system
• The atrioventricular node is in the triangle of koch, but is
compressed and closer than usual to the coronary sinus
ostium.
• Downward displacement of the septal tricuspid leaflet is
accompanied by discontinuity between the central fibrous
body and the septal atrioventricular ring creates a
substrate for preexcitation.
• Accessory pathways, usually around the tricuspid
annulus: wolff-parkinson-white syndrome 5- 25% (right
ventricular free wall in 62%, the right septum in 30%, and
only 4% are left-sided) others are mahaim-like fibres.

Arrythmias:
• On average, 35–50% of patients had arrhythmias at some
time.
• In some studies there was an association between
arrhythmias and advancing age or increasing amount of
tricuspid regurgitation.

In this study by Attie et al. in a group of patients over 25 years of age
arrhythmias and degree of severity judged by an echocardiographic
index were unrelated.

• Over half of the arrhythmias are supraventricular
tachycardias of various types.
• About one-quarter are paroxysmal or sustained atrial
fibrillation or flutter.
• A few are high-grade conduction defects.
• A minority are ventricular tachycardia, ventricular
fibrillation.

• In the WATSON SERIES
505 cases of Ebstein's anomaly have been collected from 61 centres in 28 countries
• 72% of those under 1 year were in heart failure
• 71% of the children and adolescents had little or no disability
• 60% of adults >25 years had little or no disability

Of the 505, 77 (15.2%) died from natural causes

•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.

Natural history
Few patients have lived to older ages: 132 patients over 50 years of age and 87
patients over 60 years of age

Mortality
• Neonatal deaths - either to severe hypoxemia,
congestive heart failure or both.
• Younger patients - congestive heart failure.
• Older patirnts(20%) - with hemodynamically mild lesions
- from atrial or ventricular arrhythmias.
• Brain abscess or paradoxical embolism and infective
endocarditis - rare.

Pregnancy
Connolly HM, Warnes CA. Ebstein’s anomaly: outcome of pregnancy. J
Am Coll Cardiol. 1994;23:1194–8.
Donnelly JE, Brown JM, Radford DJ. Pregnancy outcome and Ebstein’s
anomaly. Br Heart J. 1991;66:368–71.
• 66 women had a total of 152 pregnancies. All did well with
no maternal deaths, serious arrhythmias or heart failure.
• Increased incidence of prematurity and probably of
spontaneous abortion. Four newborn infants died.
• The incidence of congenital heart disease in the offspring
was 6% in the study.

In a literature review of 127 pregnancies in women with EA, 3.9 % were
complicated by arrhythmias and heart failure was reported in 3.1 %.

Physical examination
• Cyanosis - Varying degrees of cyanosis at various times
in life and transient worsening with arrhythmias.
• Precordial asymmetry
• Usually left parasternal prominence and occasionally
right parasternal prominence.
• Absent left parasternal (ie, right ventricular) lift an
important negative sign.

• JVP –
• The jugular pulse is normal except for a prominent C
wave that coincides with mobility of the anterior tricuspid
leaflet.
• An attenuated X descent and a systolic venous V wave of
tricuspid regurgitation rarely appear despite severe
regurgitant flow because of ;
1. the damping effect of the commodious right atrium
2. the thin-walled toneless atrialized right ventricle
3. tricuspid regurgitation is low-pressure and hypokinetic

A and V waves in the right atrial pressure pulse are normal, and the X
descent is preserved despite severe tricuspid regurgitation. The C
wave is prominent because of a large mobile anterior tricuspid leaflet.

• Arterial pulses
• Usually normal
• Diminished volume
• Heart sounds
• First heart sound
• widely split with loud tricuspid component( THE SAIL SOUND )
• Mitral component - soft (long PR interval)
• Second heart sound
• usually is normal
• widely split when the pulmonary component is delayed due to RBBB.
Large size
Increased excursion of anterior leaflet

• Additional heart sounds and murmurs
• Third and fourth heart sounds
• commonly present.(QUADRUPLE RHYTHM)
• Summation of third and fourth heart sounds, especially with prolonged PR
interval, can mimic an early diastolic murmur.
• The systolic murmur of tricuspid regurgitation
• Medium frequency and decrescendo in nature (grade2/6 or 3/6).
• At the lower left parasternal area and sometimes at the apex .
• Murmur intensity and duration doesnot increase during inspiration.
• The timing and quality of systolic and diastolic murmurs
occasionally create the impression of a pericardial friction rub.

Diagnosis
• ECG-
Himalayan p waves
Leads record right ventricular intracavitary potentials unusually far leftward
as a result of the large size of the right atrium.

Attenhofer Jost, CH, Connolly, HM, Dearani, JA, et al. Ebstein's anomaly. Circulation 2007; 115:277.

Conduction abnormalities Percentage
PR interval prolongation 42%
Right bundle branch block 75%-95%
Supraventricular tachycardia ,atrial
fibrillation ,atrial flutter
25%- 30%
WPW Syndrome (type B) 5%-25%

CXR
1. Cardiomegaly (Rounded or Box-like contour )
2. Decreased pulmonary vasculature
3. The infundibulum either straightens the left cardiac
border or forms a conspicuous convex shoulder.
4. The most consistent and dramatic radiologic feature is
the right atrial silhouette, which is almost always
enlarged.

Chest radiograph of a patient who had Ebstein’s 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.

Echocardiography
• Two-dimensional
a) Apical displacement of the septal leaflet of greater than 8 mm/m2
.
b) Abnormalities in morphology and septal attachment of the septal
and anterior tricuspid leaflets
c) Eccentric leaflet coaptation
d) Dilated right atrium
e) Dilated right ventricle with decreased contractile performance
f) Various left heart structural abnormalities

Schematic of anatomic abnormalities in ebstein anoamly
Armstrong et al, Feigenbaum’s Echocardiography, Lippincott Williams & Wilkins

Echo:Apical displacement index

Echocardiograms (apical four-chamber) from a 5-year-old girl with acyanotic Ebstein’s
anomaly. The septal tricuspid leaflet (stl) is displaced into the right ventricle and
tethered to the septum. The large anterior tricuspid leaflet (atl) was
highly mobile in real-time imaging and is shown here in diastole(A) and in systole (B).
A, The atrialized right ventricle (ARV) lies between the displaced septal tricuspid leaflet
and the anatomic tricuspid anulus. The functional right ventricle (RV) lies distally, and
the anatomic right atrium (RA) lies proximally. (LV ¼ left ventricle; LA ¼ left atrium.)

Echocardiogram from a patient with Ebstein’s anomaly. Color flow shows the jet of
tricuspid regurgitation (TR) originating at the junction of the functional right ventricle
(RV) and the atrialized right ventricle

Celermajer index score
• In neonate -
• RA area + aRV area
fRV area+ LA area + LV area
GRADE RATIO
1 <0.5
2 0.5 - .99
3 1 – 1.49
4 >1.5
Celermajer et al. J Am Coll Cardiol. 1994;23:170 –176.

Great Ormond Street Echocardiography (GOSE) score
Celermajer et al. J Am Coll Cardiol. 1994;23:170 –176.

Cardiac MRI
1. MRI is preferred for quantitative measurement of right
atrial and RV size and systolic function.
2. Provides complimentary information about TV anatomy .
3. Axial imaging provides the most reliable information
about atrialized RV volume.
4. The ability to create 3D images may also provide
greater delineation of disease severity.

Steinmetz et al (2012). J Clin Exp Cardiolog S8:008.

Yalonetsky, Sergey, et al. Am J Cardiol 2011;107:767–773

Cardiac Catheterization
• Routine hemodynamic catheterization is no longer
warranted in most patients with EA.
• In the rare patient with suspected pulmonary arterial
hypertension, hemodynamic catheterization can be used
for preoperative risk stratification.
• The presence and magnitude of intracardiac shunting can
also be determined by catheterization.

Fetal echo
• Prenatal diagnosis of Ebstein anomaly can be made by
fetal echocardiography at the 16 and 20 weeks of
gestation.
• Cardiac enlargement with right-sided dilation associated
with TR is usually the presenting clinical situation.
• Most fetuses tolerate Ebstein anomaly well during the
pregnancy, as left ventricular function is normal in most
cases but in rare cases, progression to fetal hydrops
portends a very poor prognosis.

• Extreme cardiac enlargement can inhibit pulmonary
development and may lead to neonatal respiratory
compromise.
• Assessment of the RV outflow tract is extremely
important, as recognition of the anatomic obstruction or
functional pulmonary atresia guarantees that the fetus will
require postnatal ductal flow (and prostaglandin therapy)
to support the pulmonary circulation.

Management
• Guideline/Indications
• Medical management
• Surgical management
• Post operative functional status

EBSTEIN ANOMALY
Class I
The following situations warrant intervention:
• Limited exercise capacity (New York Heart Association class greater
than II) (Level of Evidence: B)
• Increasing heart size (cardiothoracic ratio greater than 65%) (Level of
Evidence: B)
• Cyanosis (resting oxygen saturations < 90%) (Level of Evidence: B)
• Severe tricuspid regurgitation with symptoms (Level of Evidence: B)
• Transient ischemic attack or stroke (Level of Evidence: B)
Consensus conference on management of adults with congenital heart disease.
Executive summary Can J Cardiol 2010;26(3):143-150

Neonatal Ebstein
• Neonatal Ebstein
Poor prognosis
Reported survival only 68%
• Indiactions for surgery-
Heart failure
Profound cyanosis
J Clin Invest 1999;104:1567–1573.

Initial management
• Prostaglandin infusion
• Initiation of mechanical ventilation
• Management of pulmonary hypertension
a) Nitric oxide
b) Sildenafil
• Diuretics for CHF

Figure 1. Initial medical management algorithm. O2 indicates oxygen; PGE1,
prostaglandin E1; PVR, pulmonary vascular resistance.

Figure 2. Algorithm for management of Ebstein’s anomaly with anatomic
pulmonary atresia. RV indicates right ventricle; TR, tricuspid
regurgitation; BTS, Blalock–Taussig shunt; RV-PA, right ventricle to
pulmonary artery.

Figure 3. Algorithm for management of Ebstein’s anomaly with functional pulmonary
atresia. LV indicates left ventricle; TR, tricuspid regurgitation;
iNO, inspired nitric oxide; BTS, Blalock–Taussig shunt; BDG, bidirectional Glenn
anastomosis.

The surgical options include-
(a) Biventricular repair (Knott-Craig approach)
(b) Single ventricle pathway with right ventricular
exclusion (Starnes’ approach)
(c) Cardiac transplantation.

Biventricular Repair (Knott-CraigApproach)
• TV is repaired and the atrial septum is partially closed.
• Repair typically a mono cusp type based on a
satisfactory anterior leaflet.
• Right atrial reduction done to reduce the size of the
markedly enlarged heart to allow room for the lungs.
• Although early mortality is high (about 25%), the
intermediate outcome appears to be promising.
• Survival to hospital discharge was 74% with no late
mortality.
Ann Thorac Surg 2002;73:1786–1793.

Right Ventricular Exclusion
StarnesApproach
• Starnes et al. pioneered the right ventricular exclusion
approach, which involves:
(a) fenestrated patch closure of the TV orifce,
(b) enlarging the interatrial communication,
(c) right atrial reduction, and
(d) placing a systemic-to-pulmonary artery shunt.
• Particularly useful when there is anatomic RVOT
obstruction.
Starnes VA, Pitlick PT, Bernstein D, et al. Ebstein’s anomaly appear-ing in
the neonate. A new surgical approach. J Thorac Cardiovasc Surg
1991;101:1082–1087.

Modified Starnes Repair (Total Ventricular Exclusion)
• Sano et al. modified the Starnes single-ventricle approach
by performing a total right ventricular exclusion in which
the free wall of the RV is resected and closed primarily or
with a poly tetrafluoroethylene patch .
• This simulates a large right ventricular plication, which
may improve the left ventricular filling and provide
adequate decompression to the lungs and LV.

Cardiac Transplantation
• With the improved results of the biventricular and single
ventricle approaches, transplantation rarely is performed
in the current era.
• Cardiac transplantation remains an option in the most
severe forms of Ebstein’s anomaly, particularly when
there is significant left ventricular dysfunction.

Children and Adults
• Medical
Bacterial endocarditis prophylaxis may be required in the
presence of prosthetic materials or patches that were
used for the repair.
In mild Ebstein’s anomaly, with nearly normal heart size,
and in absence of arrhythmias, children can participate in
all sports.
However, in severe Ebstein’s anomaly, activity is
restricted unless it has been optimally repaired with near
normal heart size and no arrhythmias.

Surgery
• Indications for surgery
Presence of symptoms
Cyanosis
Paradoxical embolization
Patients who have decreased exercise performance
Progressive increase in cardiothoracic ratio
Progressive right ventricular dilatation and dysfunction
Onset or progression of arrhythmias
 Presence of class III or IV NYHA or significant symptoms
Moss & Adams' Heart Disease in Infants, Children, and
Adolescents:

PRINCIPLES OF SURGERY FOR EBSTEIN’S
ANOMALY
• The following principles are the goals of surgery:
(a) Closure of any intra cardiac communications
(b) TV repair or replacement
(c) Ablation of arrhythmias
(d) Selective plication of the atrialized RV from apex to
base
(e) Reduction right atrioplasty
(f) Repair of associated defects (e.g., closure VSD).
Moss & Adams' Heart Disease in Infants, Children, and Adolescents:

Tricuspid Valve Repair
The goal of operation is :
1. To obtain a competent TV,
2. Preserve right ventricular contractility,
3. Decrease the risk of late rhythm disturbances.

Danielson Repair
• Repair technique reported
in 1979
• Based on the creation of a
monocusp valve using the
anterior leaflet.
• This consisted of -
Plication of the free wall of
the atrialized RV
Posterior tricuspid
annuloplasty
Right reduction atrioplasty
Danielson GK, Maloney JD, Devloo RA. Surgical repair of Ebstein’s anom-aly. Mayo
Clin Proc 1979;54:185–192.

Modified Danielson Repair
• Modifications involves -
Bringing the anterior papillary muscle(s) toward the
ventricular septum, which facilitates coaptation of the
leading edge of the anterior leaflet with the ventricular
septum.
• Generally, an antero posterior tricuspid purse string or
ringed annuloplasty is used, and atrialized right ventricular
plication is performed selectively.
• This results in a TV repair at the level of the functional
annulus, in contrast to the original repair, which brought
the hinge point of the functional annulus up to the true
annulus.
Dearani JA, Danielson GK. Tricuspid valve repair for Ebstein’s anomaly.
Oper Tech Thorac Cardiovasc Surg 2003;8:188–192.

THE BRAZILEXPERIENCE (da SILVA APPROACH)
• The cone repair described by Dr. da Silva from Brazil
when the anatomy allows, as this technique is the most
anatomic of all the repair techniques described.
• Specially, some septal leaflet should be present, which
facilitates this repair technique.
• The cone technique represents the most anatomic repair
by completion of the delamination process of the TV,
providing 360 degrees of leafet tissue around the AV
junction with its hinge point at the AV groove (true
annulus).

Cone reconstruction ‘anatomic repair’

Surgical delamination – fibrous and muscular
attachments

Septal leaflet
delamination
Clockwise rotation to
septal leaflet

Relative contraindications to the cone reconstruction
technique
Age >50 years
Moderate pulmonary hypertension
Significant left ventricular dysfunction: ejection fraction
<30%
Complete failure of delamination of the septal and
posterior leaflets with poor delamination of the anterior
leaflet (<50%)
Severe right ventricular enlargement
Severe TV annular dilatation

THE VENTRICULIZATION PROCEDURE
• Ullmann et al. described the ventriculization procedure in
2004.
• This is characterized by reintegration of the atrialized
portion of the RV into the right ventricular cavity
(ventricularization).
• This can be obtained by orthotopic transposition of the
detached septal and posterior leaflets of the TV.
• The reimplanted septal leaflet serves as an opposing
structure for coaptation of the reconstructed AV valve.

Tricuspid Valve Replacement
• Every effort should be made to repair the TV rather than
replacing it.
• If not feasible, then porcine bioprosthetic valve
replacement remains a good alternative.
• Most prefer bioprostheses to mechanical valves due to
the relatively good durability and the lack of need for
anticoagulation .
• During valve replacement in Ebstein anomaly, the suture
line is deviated to the atrial side of the atrioventricular
node and membranous septum to avoid the conduction
tissue and right coronary artery injury. This results in an
‘intra-atrial’ position of the prosthesis
Kiziltan HT, Theodoro DA, Warnes CA, et al. Late results of biopros-thetic tricuspid valve
replacement in Ebstein’s anomaly. Ann Thorac Surg 1998;66:1539–1545.

Brown, Dearani, et al. JTCVS 2008

One and Half Ventricle Repair
• The BDCPA (bidirectional cavopulmonary shunt) does two
important things in the setting of Ebstein’s anomaly.
1. Reduces venous return to the enlarged, dysfunctional
RV by approx. one-third.
2. Provides sufficient preload to the LV to sustain
adequate systemic perfusion when right-sided output is
low.
• Preferred in following situations:
a) (LVEDP) is <12 mm Hg,
b) the transpulmonary gradient <10 mm Hg,
c) the mean pulmonary arterial pressure <16 mm Hg,
Kopf GS, Laks H, Stansel HC, et al. Thirty-year follow-up of superior vena cava-pulmonary artery
(Glenn) shunts. J Thorac Cardiovasc Surg 1990;117:662–670.

Indications for the BDCPA include
1. Severe RV enlargement and/or dysfunction
2. Squashed LV (D-shaped LV)
3. Moderate degree of TV stenosis (mean gradient >6 mm
Hg) as a result of reduction in the valve orifice area after
repair
4. RA:LA pressure ratio >1.5, which indicates poor RV
function.
5. Preoperative cyanosis at rest or with exercise
Chauvaud S, Fuzellier JF, Berrebi A et al. Bi-directional cavopulmonary shunt associated with
ventriculo and valvuloplasty in Ebstein’s anomaly: benefits in high risk patients. Eur J Cardiothorac
Surg 1998;13:514–519.

Atrial Septal Fenestration
• In the setting of RV dysfunction, an alternative to the
bidirectional cavopulmonary shunt.
• Involves either subtotal closure of an atrial septal defect
or leaving a patent foramen ovale open.
• An atrial level shunt affords right-to-left shunting, a ‘pop
off’, when RV dysfunction worsens following successful
tricuspid repair in the early postoperative period.

• Transient episodes of exacerbated RV dysfunction result
in right-to-left shunting that increases left-sided preload
that minimizes or avoids low cardiac output to the
systemic circulation.
• Helpful in the neonate undergoing biventricular repair
when increased pulmonary vascular resistance may still
be present or exacerbated by the pulmonary
vasoconstrictive effects of inotropic support.
Dearani JA et al.Anatomic repair of Ebstein malformation:lessons learned with cone
reconstruction. Ann Thorac Surg 2013;95(1):220-6;discussion 226-8

Post op care
• These strategies are reminder of management of RV
failure from pulmonary hypertension and include :
1. Optimal mechanical ventilation
2. Inotropic support
3. Fluid management
4. Avoidance of acidosis and arrhythmias.

• Epinephrine, milrinone and selective use of nitric oxide
optimizes oxygenation and minimizes RV afterload.
• When RV failure is significant, especially if hypoxia is
present during weaning from bypass, early initiation of
nitric oxide may help with gas exchange.
• The duration of nitric oxide is short term and is typically
weaned off within 24–48 h with or without a bridge to
another pulmonary artery vasodilator i.e.sildenafil.
Hyldebrandt JA, et al.Effects of milrinone and epinephrine or dopamine on
biventricular function and haemodynamics in right heart failure after pulmonary
regurgitation. Am J PhysiolHeart Circ Physiol 2015.

• To minimize RV dilatation, increasing heart rates (100–120
beats per minute) with temporary atrial pacing may be needed.
• This helps minimize RV distention by reducing filling time and
minimizes tension on the tricuspid repair, annular and
ventricular suture lines.
• Optimal fluid management for these patients is challenging,
and relative hypovolemia helps avoid RV distention.
• Fluid administration is slow and goal directed, and transfusions
are avoided.
• In general, target right atrial pressures should be less than 10–
12 mmHg.
Handoko ML et al. Right ventricular pacing improves right heart function in experimental
pulmonary arterial hypertension: a study in the isolated heart. Am J Physiol Heart Circ Physiol
2009;297(5):1752-9

• Mechanical ventilation strategies
a) minimizing end expiratory pressure,
b) decreasing mean airway pressure,
c) decreasing inspiratory time,
d) choosing ventilation mode that promote reduction of
intrathoracic pressure.
• Atrial arrhythmias are common.
• Early and temporary use of amiodarone is usually
effective in most patients undergoing Ebstein surgery.

• Medical therapy at hospital discharge includes b-blocker
or angiotensin-converting enzyme inhibitor, or both.
• Sildenafil for 6–8 weeks in patients with poor RV function.
• Amiodarone therapy is used for 2–3 months when
transient atrial or ventricular arrhythmias are present.
• Porcine bioprosthesis – short term warfarin therapy for 3
months and life long aspirin 81 mg daily.
Philip MC,et al.Amiodarone versus procainamide for the acute treatment of
recurrent supraventricular tachycardia in pediatric patients. Circ Arrhythm
Electrophysiol 2010;3(2):134-40

Heart Transplantation
• Heart transplantation rarely is necessary for Ebstein’s
anomaly.
• Indication for transplantation is usually the presence of
severe biventricular dysfunction (left ventricular ejection
fraction <25%).

Arrythmia management
• The most common atrial tachyarrhythmias in Ebstein’s
anomaly are atrial fibrillation and flutter.
• Most surgeons used successfully the right-sided cut-and-
sew lesions of Cox-maze III procedure in Ebstein’s
anomaly.
• With the availability of newer devices such as
radiofrequency or cryoablation, the procedure time for
maze procedure is shortened significantly.
• A biatrial maze procedure, performed particularly when
there is chronic atrial fibrillation, left atrial dilation, or
concomitant mitral regurgitation.

Pacing
• Permanent pacing is required for 3.7% of patients with
Ebstein’s anomaly, most commonly for atrioventricular
block and rarely for sinus node dysfunction.
• 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.
Allen MR, Hayes DL, Warnes CA, Danielson GK. Permanent pacing in
Ebstein’s anomaly. Pacing Clin Electrophysiol. 1997;20:1243–1246.

• 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 can be minimized by use of TEE monitoring to
ensure that the lead lies safely in a commissure between
the valve cusps.

FUNCTIONAL OUTCOME AFTER
SURGERY

Recent advances
• The maturing field of cell-based ‘regenerative’
therapeutics may provide the necessary toolkit for
patients with congenital heart disease.
• Currently, allogeneic and autologous stem cells derived
from natural sources are being applied as tools to
augment the innate regenerative process of cardiac
tissues

Take home points
 Ebstein anomaly is a RV myopathy with failure of TV
delamination and highly variable TV morphology with
severe regurgitation.
 It is the only congenital heart lesion that has a range of
clinical presentation from the severely symptomatic
neonate to an asymptomatic adult.
 Neonatal operation has high operative mortality,
whereas operation performed in childhood and
adulthood has low early mortality.
 Late survival and quality of life are excellent for the vast
majority of patients in all age brackets.

 Atrial tachyarrhythmias are the most common late
complication and increase with age.
 The cone reconstruction can achieve nearly anatomic
restorations of TV anatomy and function, and early-to-
intermediate results are encouraging.
 Reduced RV function continues to be a challenge for
some patients, as is the need for reoperation for
recurrent TR.
 Innovative surgical and regenerative medicine
strategies to address poor RV function and associated
right-sided heart failure are evolving.

Ebstein anomaly

More Related Content

What's hot (20)

Similar to Ebstein anomaly (20)

More from Amit Verma (15)

Recently uploaded (20)

Ebstein anomaly