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Ebsteins Anomaly
Christine H. Attenhofer Jost, MD; Heidi M. Connolly, MD; Joseph A. Dearani, MD;
William D. Edwards, MD; Gordon K. Danielson, MD
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.
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
277
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Attenhofer Jost et al
Ebsteins Anomaly
279
Physiology
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
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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Circulation
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
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
Attenhofer Jost et al
Ebsteins Anomaly
281
Surgical Options
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
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Circulation
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.
Attenhofer Jost et al
Ebsteins Anomaly
283
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.
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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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.
References
1. Attenhofer Jost CH, Connolly HM, Edwards WD, Hayes D, Warnes CA,
Danielson GK. Ebsteins anomaly: review of a multifaceted congenital
cardiac condition. Swiss Med Wkly. 2005;135:269 281.
2. Keith JD, Rowe RD, Vlad P. Heart Disease in Infancy and Childhood.
New York, NY: Macmillan Co; 1958.
3. Perloff JK. The Clinical Recognition of Congenital Heart Disease. 5th ed.
Philadelphia, Pa: WB Saunders; 2003.
4. Correa-Villasenor A, Ferencz C, Neill CA, Wilson PD, Boughman JA, for
the Baltimore-Washington Infant Study Group. Ebsteins malformation of
the tricuspid valve: genetic and environmental factors. Teratology. 1994;
50:137147.
5. Frescura C, Angelini A, Daliento L, Thiene G. Morphological aspects of
Ebsteins anomaly in adults. Thorac Cardiovasc Surg. 2000;48:203208.
6. Edwards WD. Embryology and pathologic features of Ebsteins anomaly.
Prog Pediatr Cardiol. 1993;2:515.
7. Mann RJ, Lie JT. The life story of Wilhelm Ebstein (1836 1912) and his
almost overlooked description of a congenital heart disease. Mayo Clin
Proc. 1979;54:197204.
8. van Son JA, Konstantinov IE, Zimmermann V. Wilhelm Ebstein and
Ebsteins malformation. Eur J Cardiothorac Surg. 2001;20:10821085.
9. Ebstein W. Ueber einen sehr seltenen Fall von Insufficienz der Valvula
tricuspidalis, bedingt durch eine angeborene hochgradige Missbildung
derselben. Arch Anat Physiol. 1866;238 255.
10. Engle MA, Payne TP, Bruins C, Taussig HB. Ebsteins anomaly of the
tricuspid valve: report of three cases and analysis of clinical syndrome.
Circulation. 1950;1:1246 1260.
11. Soloff LA, Stauffer HM, Zatuchni J. Ebsteins disease: report of the first
case diagnosed during life. Am J Med Sci. 1951;222:554 561.
12. Dearani JA, Danielson GK. Congenital Heart Surgery Nomenclature and
Database Project: Ebsteins anomaly and tricuspid valve disease. Ann
Thorac Surg. 2000;69(suppl):S106 S117.
13. Lamers WH, Viragh S, Wessels A, Moorman AF, Anderson RH. Formation of the tricuspid valve in the human heart. Circulation. 1995;91:
111121.
14. Dearani JA, Danielson GK. Ebsteins anomaly of the tricuspid valve. In:
Mavroudis C, Backer CL, eds. Pediatric Cardiac Surgery. 3rd ed. Philadelphia, Pa: Mosby; 2003:524 536.
15. Lev M, Liberthson RR, Joseph RH, Seten CE, Eckner FA, Kunske RD,
Miller RA. The pathologic anatomy of Ebsteins disease. Arch Pathol.
1970;90:334 343.
16. Anderson KR, Zuberbuhler JR, Anderson RH, Becker AE, Lie JT. Morphologic spectrum of Ebsteins anomaly of the heart: a review. Mayo Clin
Proc. 1979;54:174 180.
17. Zuberbuhler JR, Allwork SP, Anderson RH. The spectrum of Ebsteins
anomaly of the tricuspid valve. J Thorac Cardiovasc Surg. 1979;77:
202211.
18. Carpentier A, Chauvaud S, Mace L, Relland J, Mihaileanu S, Marino JP,
Abry B, Guibourt P. A new reconstructive operation for Ebsteins
anomaly of the tricuspid valve. J Thorac Cardiovasc Surg. 1988;96:
92101.
19. Celermajer DS, Bull C, Till JA, Cullen S, Vassillikos VP, Sullivan ID,
Allan L, Nihoyannopoulos P, Somerville J, Deanfield JE. Ebsteins
anomaly: presentation and outcome from fetus to adult. J Am Coll
Cardiol. 1994;23:170 176.
20. Cohen LS, Friedman JM, Jefferson JW, Johnson EM, Weiner ML. A
reevaluation of risk of in utero exposure to lithium. JAMA. 1994;271:
146 150.
21. Emanuel R, OBrien K, Ng R. Ebsteins anomaly: genetic study of 26
families. Br Heart J. 1976;38:57.
Attenhofer Jost et al
43. Shiina A, Seward JB, Edwards WD, Hagler DJ, Tajik AJ. Twodimensional echocardiographic spectrum of Ebsteins anomaly: detailed
anatomic assessment. J Am Coll Cardiol. 1984;3:356 370.
44. Eustace S, Kruskal JB, Hartnell GG. Ebsteins anomaly presenting in
adulthood: the role of cine magnetic resonance imaging in diagnosis. Clin
Radiol. 1994;49:690 692.
45. Fisher MR, Lipton MJ, Higgins CB. Magnetic resonance imaging and
computed tomography in congenital heart disease. Semin Roentgenol.
1985;20:272282.
46. Hebe J. Ebsteins anomaly in adults: arrhythmias: diagnosis and therapeutic approach. Thorac Cardiovasc Surg. 2000;48:214 219.
47. Ho SY, Goltz D, McCarthy K, Cook AC, Connell MG, Smith A,
Anderson RH. The atrioventricular junctions in Ebstein malformation.
Heart. 2000;83:444 449.
48. Anderson KR, Lie JT. The right ventricular myocardium in Ebsteins
anomaly: a morphometric histopathologic study. Mayo Clin Proc. 1979;
54:181184.
49. Watson H. Natural history of Ebsteins anomaly of tricuspid valve in
childhood and adolescence: an international co-operative study of 505
cases. Br Heart J. 1974;36:417 427.
50. Smith WM, Gallagher JJ, Kerr CR, Sealy WC, Kasell JH, Benson DW Jr,
Reiter MJ, Sterba R, Grant AO. The electrophysiologic basis and management of symptomatic recurrent tachycardia in patients with Ebsteins
anomaly of the tricuspid valve. Am J Cardiol. 1982;49:12231234.
51. Graham TP Jr, Bricker JT, James FW, Strong WB. Twenty-Sixth
Bethesda Conference: recommendations for determining eligibility for
competition in athletes with cardiovascular abnormalities: Task Force 1:
congenital heart disease. Med Sci Sports Exerc. 1994;26(suppl):
S246 S253.
52. Reich JD, Auld D, Hulse E, Sullivan K, Campbell R, for the Pediatric
Electrophysiology Society. The Pediatric Radiofrequency Ablation Registrys experience with Ebsteins anomaly. J Cardiovasc Electrophysiol.
1998;9:1370 1377.
53. Cappato R, Schluter M, Weiss C, Antz M, Koschyk DH, Hofmann T,
Kuck KH. Radiofrequency current catheter ablation of accessory atrioventricular pathways in Ebsteins anomaly. Circulation. 1996;94:
376 383.
54. Chetaille P, Walsh EP, Triedman JK. Outcomes of radiofrequency
catheter ablation of atrioventricular reciprocating tachycardia in patients
with congenital heart disease. Heart Rhythm. 2004;1:168 173.
55. Khositseth A, Danielson GK, Dearani JA, Munger TM, Porter CJ.
Supraventricular tachyarrhythmias in Ebstein anomaly: management and
outcome. J Thorac Cardiovasc Surg. 2004;128:826 833.
56. Greason KL, Dearani JA, Theodoro DA, Porter CB, Warnes CA,
Danielson GK. Surgical management of atrial tachyarrhythmias associated with congenital cardiac anomalies: Mayo Clinic experience. Semin
Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2003;6:59 71.
57. Schiebler GL, Adams P Jr, Anderson RC, Amplatz K, Lester RG. Clinical
study of twenty-three cases of Ebsteins anomaly of the tricuspid valve.
Circulation. 1959;19:165187.
58. Barnard CN, Schrire V. Surgical correction of Ebsteins malformation
with prosthetic tricuspid valve. Surgery. 1963;54:302308.
59. Hardy KL, May IA, Webster CA, Kimball KG. Ebsteins anomaly: a
functional concept and successful definitive repair. J Thorac Cardiovasc
Surg. 1964;48:927940.
60. Theodoro DA, Danielson GK, Kiziltan HT, Driscoll DJ, Mair DD,
Warnes CA, Anderson BJ. Surgical management of Ebsteins anomaly: a
25-year experience. Circulation. 1997;96(suppl I):I507. Abstract.
61. Danielson GK, Maloney JD, Devloo RA. Surgical repair of Ebsteins
anomaly. Mayo Clin Proc. 1979;54:185192.
62. Dearani JA, Danielson GK. Tricuspid valve repair for Ebsteins anomaly.
Oper Tech Thorac Cardiovasc Surg. 2003;8:188 192.
63. Dearani JA, Danielson GK. Ebsteins anomaly. In: Sellke FW, del Nido
PJ, Swanson SJ, eds. Sabiston & Spencer Surgery of the Chest. 7th ed.
Philadelphia, Pa: Elsevier Saunders; 2005;2:22232235.
64. Chauvaud S, Berrebi A, dAttellis N, Mousseaux E, Hernigou A, Carpentier
A. Ebsteins anomaly: repair based on functional analysis. Eur J Cardiothorac Surg. 2003;23:525531.
65. Ullmann MV, Born S, Sebening C, Gorenflo M, Ulmer HE, Hagl S.
Ventricularization of the atrialized chamber: a concept of Ebsteins
anomaly repair. Ann Thorac Surg. 2004;78:918 924.
Ebsteins Anomaly
285
66. Hancock Friesen CL, Chen R, Howlett JG, Ross DB. Posterior annular
plication: tricuspid valve repair in Ebsteins anomaly. Ann Thorac Surg.
2004;77:21672171.
67. Chen JM, Mosca RS, Altmann K, Printz BF, Targoff K, Mazzeo PA,
Quaegebeur JM. Early and medium-term results for repair of Ebstein
anomaly. J Thorac Cardiovasc Surg. 2004;127:990 998.
68. Kiziltan HT, Theodoro DA, Warnes CA, OLeary PW, Anderson BJ,
Danielson GK. Late results of bioprosthetic tricuspid valve replacement
in Ebsteins anomaly. Ann Thorac Surg. 1998;66:1539 1545.
69. Ahel V, Kilvain S, Rozmanic V, Taylor JF, Vukas D. Right atrial
reduction for tachyarrhythmias in Ebsteins anomaly in infancy. Tex
Heart Inst J. 2001;28:297300.
70. Bockeria L, Golukhova E, Dadasheva M, Revishvili A, Levant A, Bazaev
V, Rzaev F, Kakuchaya T. Advantages and disadvantages of one-stage
and two-stage surgery for arrhythmias and Ebsteins anomaly. Eur J Cardiothorac Surg. 2005;28:536 540.
71. Knott-Craig CJ, Overholt ED, Ward KE, Ringewald JM, Baker SS,
Razook JD. Repair of Ebsteins anomaly in the symptomatic neonate: an
evolution of technique with 7-year follow-up. Ann Thorac Surg. 2002;
73:1786 1792.
72. Starnes VA, Pitlick PT, Bernstein D, Griffin ML, Choy M, Shumway NE.
Ebsteins anomaly appearing in the neonate: a new surgical approach.
J Thorac Cardiovasc Surg. 1991;101:10821087.
73. Boston US, Dearani JA, OLeary PW, Driscoll DJ, Danielson GK. Tricuspid valve repair for Ebsteins anomaly in young children: a 30-year
experience. Ann Thorac Surg. 2006;81:690 696.
74. Atiq M, Lai L, Lee KJ, Benson LN. Transcatheter closure of atrial septal
defects in children with a hypoplastic right ventricle. Catheter Cardiovasc
Interv. 2005;64:112116.
75. Allen MR, Hayes DL, Warnes CA, Danielson GK. Permanent pacing in
Ebsteins anomaly. Pacing Clin Electrophysiol. 1997;20:12431246.
76. Kumar AE, Fyler DC, Miettinen OS, Nadas AS. Ebsteins anomaly:
clinical profile and natural history. Am J Cardiol. 1971;28:84 95.
77. Bialostozky D, Horwitz S, Espino-Vela J. Ebsteins malformation of the
tricuspid valve: a review of 65 cases. Am J Cardiol. 1972;29:826 836.
78. Attie F, Rosas M, Rijlaarsdam M, Buendia A, Zabal C, Kuri J, Granados
N. The adult patient with Ebstein anomaly: outcome in 72 unoperated
patients. Medicine (Baltimore). 2000;79:2736.
79. McElhinney DB, Salvin JW, Colan SD, Thiagarajan R, Crawford EC,
Marcus EN, del Nido PJ, Tworetzky W. Improving outcomes in fetuses
and neonates with congenital displacement (Ebsteins malformation) or
dysplasia of the tricuspid valve. Am J Cardiol. 2005;96:582586.
80. Celermajer DS, Dodd SM, Greenwald SE, Wyse RK, Deanfield JE.
Morbid anatomy in neonates with Ebsteins anomaly of the tricuspid
valve: pathophysiologic and clinical implications. J Am Coll Cardiol.
1992;19:1049 1053.
81. Yetman AT, Freedom RM, McCrindle BW. Outcome in cyanotic
neonates with Ebsteins anomaly. Am J Cardiol. 1998;81:749 754.
82. Celermajer DS, Cullen S, Sullivan ID, Spiegelhalter DJ, Wyse RK,
Deanfield JE. Outcome in neonates with Ebsteins anomaly. J Am Coll
Cardiol. 1992;19:10411046.
83. Deanfield J, Thaulow E, Warnes C, Webb G, Kolbel F, Hoffman A,
Sorenson K, Kaemmerer H, Thilen U, Bink-Boelkens M, Iserin L, Daliento
L, Silove E, Redington A, Vouhe P, Priori S, Alonso MA, Blanc J-J, Budaj
A, Cowie M, Deckers J, Fernandez Burgos E, Lekakis J, Lindahl B, Mazzotta
G, Morais J, Oto A, Smiseth O, Trappe HJ, Klein W, Blmstrom-Lundqvist
C, de Backer G, Hradec J, Parkhomenko A, Presbitero P, Torbicki A, for the
Task Force on the Management of Grown Up Congenital Heart Disease of
the European Society of Cardiology, ESC Committee for Practice
Guidelines. Management of grown up congenital heart disease. Eur Heart J.
2003;24:10351084.
84. Therrien J, Dore A, Gersony W, Iserin L, Liberthson R, Meijboom F,
Colman JM, Oechslin E, Taylor D, Perloff J, Somerville J, Webb GD, for
the Canadian Cardiovascular Society. CCS Consensus Conference 2001
update: recommendations for the management of adults with congenital
heart disease: part I. Can J Cardiol. 2001;17:940 959.
85. Landzberg MJ, Murphy DJ Jr., Davidson WR Jr, Jarcho JA, Krumholz
HM, Mayer JE Jr, Mee RB, Sahn DJ, Van Hare GF, Webb GD, Williams
RG. Task Force 4: organization of delivery systems for adults with
congenital heart disease. J Am Coll Cardiol. 2001;37:11871193.
KEY WORDS: atrium heart defects, congenital
pathology pediatrics
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
Circulation is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231
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