This document provides information on Ebstein's anomaly, including its anatomy, embryology, clinical presentation, diagnosis, and natural history. Some key points: - Ebstein's anomaly is a congenital defect involving downward displacement of the tricuspid valve into the right ventricle. This can cause dilation of the right atrium and dysfunction of the right ventricle. - Clinical presentation varies from neonatal congestive heart failure to later cyanosis, arrhythmias, and right heart failure in adults. Associated defects are common. - Diagnosis is made through echocardiogram demonstrating displacement of the tricuspid valve leaflets. Other tests like ECG, chest x-ray, and

1. Dr Awadhesh Kumar Sharma
Ebstein’s Anomaly

2. Sequence of presentation
 Work up
 Treatment
• Anatomy
• Embryology
• Clinical Features

3. Tricuspid Valve Anatomy

4. TV annulus
• The tricuspid valve is the most apically (or
caudally) placed valve with the largest orifice
among the four valves.
• The tricuspid annulus is oval-shaped and when
dilated becomes more circular.
• 20% larger than MV annulus .
• Normal TV annulus= 3.0 3.5 cm

5. Leaflets
 The tricuspid valve has three distinct leaflets
described as septal, anterior, and posterior.
 The septal and the anterior leaflets are larger.
 The posterior leaflet is smaller and appears to be
of lesser functional significance since it may be
imbricated without impairment of valve function.

6. Leaflets
 The septal leaflet is in immediate proximity of the
membranous ventricular septum, and its
extension provides a basis for spontaneous
closure of the perimembranous ventricular septal
defect.
 The anterior leaflet is attached to the anterolateral
margin of the annulus and is often voluminous
and sail-like in Ebstein’s anomaly.

7. Papillary Muscles & Chordae
 There are three sets of small papillary muscles,
each set being composed of up to three muscles.
 The chordae tendinae arising from each set are
inserted into two adjacent leaflets.
 The anterior set chordae insert into half of the
septal and half of the anterior leaflets.
 The medial and posterior sets are similarly related
to adjacent valve leaflets.

8. Transthoracic Views

9. Transesophageal Views

10. Embryologic aspect
 The leaflets and tensile apparatus of the atrio
ventricular valves seem to be formed by a process of
delamination of the inner layers of the inlet zone of
the ventricles.
 Delamination of the right ventricular free
wall, continues to the level of AV junction.
 In EA, delamination of these leaflets may have failed
to occur due to an incompletely understood
mechanism
 Process of delamination is incomplete and falls short
of reaching the level of the AV junction.
 The apical portions of the valve tissue fail to resorb
completely.
 Distortion and displacement of the tricuspid valve
leaflets, and a part of the right ventricle becomes

12. DEFINITION
 Congenital defect
 Origins of septal or posterior
leaflets, or both, are displaced
downward into RV
 Leaflets are variably deformed
 Atrialization of right ventricle
 Anterior leaflet is enlarged and sail-

13. Pathologic anatomy of TV
 Origin of TV from AV ring &
its chordal attachments are
malpositioned
 Leaflets are
malformed, dysplastic
(thickened &
distorted), enlarged or
reduced in size
 Septal leaflet always
affected, posterior leaflet
nearly always, and anterior
leaflet seldom
 Septal & posterior leaflets

14. Top, Normal tricuspid valve with anterior,
posterior,
and septal leaflets in 1 plane.
Middle, Tricuspid valve in right sided
Ebstein’s 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
Ebstein’s 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.

15. Pathologic anatomy of
RV
 Proximal
 Atrialized in one fourth of heartsdilated
 When thin moves paradoxically during systole
 Electrical potentials are ventricular, but pressure pulse is atrial
contoured
 Distal
 Smaller than normal RV
 RV dilatation
 Functional portion is infundibulum, trabeculated apex, portion
beneath anterior cusp
 Thinner walled with fewer muscles

16. 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

17. Epidemiology
 Ebstein’s anomaly occurs in 0.3-0.8% of all
congenital heart diseases
 1 in 20,000-50,000 live births
 Equal male: female occurrence
 Mortality in children presenting in the neonatal
period is 30-50%
 Mortality at all ages is 12.5%
Arch Anat Physiol 1866:238–25

18. Associated defects
 Commonly associated with:
 ASD or PFO (90%)
 VSD, AV canal defect
 Pulmonary stenosis/atresia (20-25%)
 Wolff-Parkinson-White
 Syndromes:
 Down, Marfan, Noonan
sumner RG, Jacoby WJ Jr, Tucker DH.
Ebstein’s anomaly associated with Car-
diomyopathy and Pulmonary Hypertension.
Circulation 1964;30:578–587.

20. Etiology
 Congenital disease of often uncertain
cause.
 Environmental factors
 Maternal ingestion of lithium in first
trimester
 Maternal benzodiazepine use
 Maternal exposure to varnishing
substances
 Maternal history of previous fetal loss
 Risk is higher in whites than in other
races.

21. Genetic factors
 Rare cases of cardiac transcription factor NKX2.5
mutations, 10p13-p14 deletion, and
1p34.3-p36.11 deletion have been described in
the anomaly .
 Recently, Postma et al. reported the results of a
mutational analysis in a cohort of 141 unrelated
probands with Ebstein anomaly.
 Eight were found to have a mutation in the gene
MYH7 and six of the eight patients also had left
ventricular noncompaction.
 This may warrant genetic testing and family
evaluation in this subset
emanuel R, O’Brien K, Ng R. Ebstein’s
anomaly: genetic study of 26 fami-lies. Br
Heart J 1976;38:5–7.

24. Origin
 1866 A.D.
 Hypothesis on single autopsy specimen
 He even did not see the patient, when later was
alive
 Word used in 1927 A.D.

25. A pencil sketch
of Wilhelm
Ebstein
published in the
Festschrift
celebrating
Ebstein’s 70th
birthday.
Published by
permission of
the Mayo Clinic
Proceedings,
where it was
published by
Mann RJ, Lie JT.
The life story
of Wilhelm
Ebstein (1836–
1912) and his
almost
overlooked
description of a
congenital heart

26. Clinical presentation

27. Presentation at various stages of
life
 Fetal life:
 Diagnosed incidentally by
echocardiography.
 Neonatal life and infancy:
 Cyanosis and/or severe
heart failure
 Improve as pulmonary
vascular resistance
decreases.
 Adult life:
 Fatigue, exertional
dyspnea, cyanosis, tricus
pid regurgitation and/or
right heart failure, and

28. Neonatal presentation
 Congestive heart failure
 Due to TR and RV dysfunction
 Cyanosis
 Decreased pulmonary blood flow due to R  L
shunt through ASD or PFO
 Increased pulmonary vascular resistance in the
neonatal period compounds this problem
 Murmur

29. Later presentation
 Cyanosis
 Due to R  L shunt at atrial level
 Fatigue and dyspnea
 Secondary to RV failure and decreased LV ejection
fraction
 Palpitations and sudden cardiac death
 Incidental murmur
 Paradoxical embolism

30. Cyanosis
 Fairly common
 Right-to-left shunt at the atrial level and/or severe heart
failure
 Transient in neonatal life with recurrence in adult life
 May appear for the first time in adult life
 Transient appearance/worsening of cyanosis in adult life
due to paroxysmal arrhythmias

31.  Fatigue and dyspnea
Poor cardiac output secondary to right
ventricular failure and decreased left
ventricular ejection fraction.
 Palpitations and sudden cardiac death
SVT in as many as one third of patients
 Fatal ventricular arrhythmias
accessory pathways

32. Arrhythmias
 Due to right atrial enlargement and high
prevalence of accessory pathways
 30-50% have evidence of WPW secondary to the
atrialized RV tissue
 Mapping and ablation are difficult
 Atrial dilation disrupts anatomic landmarks
 Accessory pathways are often multiple

33. Electrophysiologic studies
 25-30% have accessory pathways
 5-25% have evidence of preexcitation on the surface ECG.
 Right-sided pathways are more common.
 Fifty percent of the patients have multiple pathways.
 Guide ablative therapy.

34. Complications
 Congestive heart failure
 Sudden cardiac death
 Bacterial endocarditis
 Brain abscess
 Paradoxical embolism
 Transient ischemic attacks
 Stroke

35. Pregnancy & Ebsteins
Anomaly
 Pregnancy seems to be well tolerated with adequate
supervision.

37. Prenatal presentation
 Difficult to diagnose prenatally
 Fetal presentation is variable: possible
features include cardiomegaly, RA
enlargement, tricuspid regurgitation or
dysplasia, arrhythmia, or fetal hydrops
 Prognosis for the fetus diagnosed in utero
with significant tricuspid valve disease is very
poor (20% survival)
 Progressive right heart dilatation
 Cardiac failure
 Lung hypoplasia
 Pulmonary stenosis/atresia

38. Cardiothoracic area ratio at 33 (A) and
35 (B) weeks gestational age. A
indicates anterior; P, posterior; R, right;
and L, left.
Circulation September 23, 2003

41. Prognosis & management
 Prenatal prognosis could be significantly
influenced by the ability of foramen ovale to
decompress the right atrium
 Arrythmia due to extreme dilatation of the RA
might be a cause of sudden intrauterine death
 Ebstein’s anomaly without cardiac failure or
hydrops: vaginal delivery
 Cesarean section

42. Physical Examination
 Cyanosis and clubbing - 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
 Jugular venous pulse
 May be normal
 Large a and v waves late in the course of the

43. Physical Examination
 Arterial pulses
 Usually normal
 Diminished volume
 Heart sounds
 First heart sound
 widely split with loud tricuspid component
 Mitral component may be soft or absent in the presence of prolonged
PR interval.
 Second heart sound
 usually is normal
 widely split when the pulmonary component is delayed due to RBBB.

45. Physical Examination
 Additional heart sounds and murmurs
 Third and fourth heart sounds
 commonly present, even in the absence of congestive heart failure
(CHF).
 Summation of third and fourth heart sounds, especially with prolonged
PR interval, can mimic an early diastolic murmur.
 The holosystolic murmur of tricuspid regurgitation
 At the lower left parasternal area and sometimes at the apex
 Murmur intensity and duration increase during inspiration.

46. Routine investigation protocal

47. 12-lead ECG
 Rhythm
 Usually normal sinus findings
 Intermittently
SVT, paroxysmal SVT, atrial
flutter, atrial
fibrillation, ventricular
tachycardia
 Abnormal P waves
consistent with right atrial
enlargement – “Himalayan
P waves”
• PR interval
– Most commonly prolonged
– May be normal or short in
patients with WPW (B)
syndrome
• QRS complex
– RBBB
• Low voltage in many
patients

48. Chest X-
Ray
 Cardiomegaly
( Rounded or Box-
like contour )
 Small aortic root
and main
pulmonary artery
shadow
 Decreased
pulmonary
vasculature

49. Echocardiogram
 Standard for diagnosis.
 M-mode
 Paradoxical septal motion
 Dilated right ventricle
 Delayed closure of tricuspid valve leaflets more
than 65 milliseconds after mitral valve closure
Roberson DA, Silverman NH. Ebstein’s
anomaly: echocardiographic and clinical
features in the fetus and neonate. J Am
Coll Cardiol 1989;14:1300–1307.

50. Echocardiogram
 Two-dimensional
 Apical displacement of the septal leaflet
of greater than 8 mm/m2 –
 Abnormalities in morphology and septal
attachment of the septal and anterior
tricuspid leaflets
 Eccentric leaflet coaptation
 Dilated right atrium
 Dilated right ventricle with decreased
contractile performance
 Various left heart structural abnormalities

51. Echocardiogram
 Doppler studies
 Varying degrees of tricuspid regurgitation
 Excludes associated shunts

52. Echocardiogram
 Assessment of severity and surgical options
 Functional right ventricular area less than 35% of total right ventricular
area or an atrialized to functional right ventricular ratio greater than 0.5
associated with unfavorable prognosis
 Functional right ventricular size
 Degree of septal leaflet displacement
 Amount of leaflet tethering
 Magnitude of leaflet deformity and dysplasia
 Aneurysmal dilatation of right ventricular outflow tract (right ventricular
outflow tract-to-aortic root ratio of >2:1 on parasternal short axis view)
 Moderate-to-severe tricuspid regurgitation

53. Cardiac MRI
 CMR imaging has emerged as another tool for
evaluation of Ebstein’s patients
 It provides quantitative measurement of right
atrial and ventricular size and systolic function
even in the presence of significant distortion of
right ventricular anatomy.
Yalonetsky S, Tobler D, Greutmann M, et
al. Cardiac magnetic resonance imaging
and the assessment of Ebstein anomaly in
adults. Am J Cardiol 2011;107:767–773.

55. Cardiac Catheterization
 No longer required to make/confirm the diagnosis
 The most diagnostic characteristic- Typical
atrial pressure & ventricular intracardiac ECG in
the atrialized portion of the RV
 Elevated RAP
 R-L atrial shunting with systemic desaturation
 Elevated RVEDP

56. Natural history

57. Natural history
 It is not uncommon for Ebstein’s anomaly to be
undiagnosed until adulthood.
 However, late diagnosis is associated with
reduced survival.
 The mean age of diagnosis in a study of the
natural history of 72 unoperated patients, was
23.9 ± 10.4 years.
 In this group of patients, arrhythmias were the
most common clinical presentation (51%) .
 The estimated cumulative overall survival rates
were 89%, 76%, 53%, and 41% at 1, 10, 15, and
20 years of follow-up, respectively.

58. Natural history
 Predictors of cardiac-related death on univariate
analysis included:
 Cardiothoracic ratio of ≥ 0.65
 Increasing severity of TV displacement on
echocardiography
 New York Heart Association (NYHA) class III or IV
 Cyanosis
 Severe TR
 Younger age at diagnosis
 In a multivariate model, younger age at
diagnosis, male sex, cardiothoracic ratio of ≥
0.65, and the severity of TV leaflet displacement on
echocardiography were predictors of late cardiac
mortality.

60. Management
 Severity Assessment
 Guideline/Indications
 Medical management
 Surgical management
 Post operative functional status

61. Severity assessment

62. Celermajer Index
 Celermajer et al. described an echocardiographic
grading score for neonates with Ebstein’s
anomaly, the Great Ormond Street Echocardiography
(GOSE) score, with grades 1 to 4.
 The ratio of the combined area of the RA and
atrialized RV is compared to the functional RV and left
heart. This classification is particularly helpful with
neonatal Ebstein’s anomaly.
 Grade 1: ratio <0.5
 Grade 2: ratio of 0.5 to 0.99
 Grade 3: ratio of 1.0 to 1.49
 Grade 4: ratio ≥ 1.5 Celermajer DS, Bull C, Till JA, et al.
Ebstein’s anomaly: presentation and
outcome from fetus to adult J Am Coll

63. GOSE score
Grade Ratio Mortality
1 <0.5 8%
2 0.5-0.99 9%
3
(acyanotic)
1-1.49 10% (neonatal)
45% (later)
3
(cyanotic)
1-1.49 100%
4 >1.5 100%

64. Carpentier’s classification
 In 1988, Carpentier et al. proposed the following
classification of Ebstein’s anomaly -
 Type A: The volume of the true RV is adequate
 Type B: A 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 significant
obstruction of the RVOT
 Type D: Almost complete atrialization of the
ventricle except for a small infundibular
component.

65. EBSTEIN’S ANOMALY
(Carpentier’s classification)
TYPE A (M+ C+) TYPE B (M+ C -)
TYPE С (M- C -) TYPE D (“tricuspid suck”)
M – mobility C - contractility

66. CANADIAN CARDIOVASCULAR
SOCIETYCANADIAN CARDIOVASCULAR SOCIETY
2009 CONSENSUS CONFERENCE
UPDATE ON THE GUIDELINES FOR THE
MANAGEMENT OF ADULTS WITH
CONGENITAL HEART DISEASE

67. 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)
 Important 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)
Patients who require operation for Ebstein anomaly should be
operated on by congenital heart surgeons who have substantial
specific experience and success with this operation. Every effort
should be made to preserve the native TV. (Level of Evidence: C)
Presentation at Annual CCS Meeting in Edmonton 2009

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

69. Initial management
 Prostaglandin infusion
 Placement of umbilical catheters
 Initiation of mechanical ventilation
 Minimum possible mean airway pressure
 Tidal volumes of 10-15 ml/kg to overcome
atelectasis
 Management of pulmonary hypertension
 Diuretics for CHF

70. Management of pulmonary
hypertension
 Nitric oxide
 Reduces after load of right ventricle
 Sedation
 Other pulmonary vasodilators

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

77. Biventricular Repair (Knott-Craig
Approach)
 TV is repaired and the atrial septum is partially
closed.
 This repair is typically a mono cusp type based
on a satisfactory anterior leaflet
 Routine right atrial reduction is important 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 dismissal was 74% with no
late
mortality. Ann Thorac Surg 2002;73:1786–1

81. Right Ventricular Exclusion
Starnes Approach
 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.
 Right ventricular decompression is required as it
passively fills from thebesian venous drainage, this is
usually accomplished with a 4- to 5-mm punch
fenestration in the TV patch
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–

82. 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.

84. 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.

85. Children & Adults

86. 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,
athletes 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.

87. 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

88. 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).

89. Tricuspid Valve Repair
The goal of operation is to obtain a competent
TV, preserve right ventricular contractility, and to
decrease the risk of late rhythm disturbances.

90. Danielson Repair
 This repair technique was reported in 1979 and
was 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.

91. 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.
 A more recent modification includes patch
augmentation of the mid-anterior leaflet with surgical
delamination of attachments to the anterior and/or
inferior leafets.
Dearani JA, Danielson GK. Tricuspid valve
repair for Ebstein’s anomaly. Oper Tech
Thorac Cardiovasc Surg 2003;8:188–192.

93. THE BRAZIL EXPERIENCE (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).
 Although not initially described with this technique,
some do supplement the repair with a flexible
anterior annuloplasty band from anteroseptal
commissure to inferseptal commissure whenever
possible.

94. Copyright ©2007 The American Association for Thoracic Surgery
da Silva J. P. et al.; J Thorac Cardiovasc Surg 2007;133:215-223
Operative steps for Ebstein's anomaly repair

97. 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

99. THE VENTRICULIZATION
PROCEDURE
 Ullmann et al. published their results with 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.

101. Tricuspid Valve Replacement
 Every effort should be made to repair the TV
rather than replacing it, but if TV repair is 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 .
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.

102. One and Half Ventricle Repair
 The BDCPA does two important things in the
setting of Ebstein’s anomaly.
 First, it reduces venous return to the enlarged,
dysfunctional RV by approximately one-third
 Second, it provides sufficient preload to the LV to
sustain adequate systemic perfusion when right-
sided output is low.
 Surgens usually prefer it if the left ventricular end-
diastolic pressure (LVEDP) is <12 mm Hg, the
transpulmonary gradient <10 mm Hg, and the
mean pulmonary arterial pressure <16 mm Hg,
before considering a BDCPA.
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.

103. Indications for the BDCPA
include
 Severe RV enlargement and/or dysfunction
 Squashed LV (D-shaped LV)
 Moderate degree of TV stenosis (mean gradient
>6 mm Hg) as a result of reduction in the valve
orifice area after repair
 RA:LA pressure ratio >1.5, which indicates poor
RV function.
 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

104. 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%).

105. Arrythmia management
 The most common atrial tachyarrhythmias in
Ebstein’s anomaly are atrial fibrillation and flutter.
 Most surgens 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.

108. FUNCTIONAL OUTCOME AFTER
SURGERY