Differential Diagnoses

Workup

Hong YM, Moller JH. Ebstein's anomaly: a long-term study of survival. Am Heart J. 1993 May. 125(5 Pt 1):1419-24. [QxMD MEDLINE Link].
Ebstein W. Ueber einen sehr seltenen Fall von Insufficienz der Valvula Tricuspidalis bedingt durch eine angeborene hochgradige Missbildung derselben [German]. Arch Anat Physiol. 1866. 33:238.
van Son JA, Konstantinov IE, Zimmermann V. Wilhelm Ebstein and Ebstein's malformation. Eur J Cardiothorac Surg. 2001 Nov. 20(5):1082-5. [QxMD MEDLINE Link].
Watson H. Natural history of Ebstein's anomaly of tricuspid valve in childhood and adolescence. An international co-operative study of 505 cases. Br Heart J. 1974 May. 36(5):417-27. [QxMD MEDLINE Link].
Hunter SW, Lillehei CW. Ebstein's malformation of the tricuspid valve; study of a case together with suggestion of a new form of surgical therapy. Dis Chest. 1958 Mar. 33(3):297-304. [QxMD MEDLINE Link].
Hardy KL, May IA, Webster CA, Kimball KG. Ebstein's anomaly: A functional concept and successful definitive repair. J Thorac Cardiovasc Surg. 1964 Dec. 48:927-40. [QxMD MEDLINE Link].
Barnard CN, Schrire V. Surgical correction of Ebstein's malformation with prosthetic tricuspid valve. Surgery. 1963 Aug. 54:302-8. [QxMD MEDLINE Link].
Danielson GK, Maloney JD, Devloo RA. Surgical repair of Ebstein's anomaly. Mayo Clin Proc. 1979 Mar. 54(3):185-92. [QxMD MEDLINE Link].
Carpentier A, Chauvaud S, Mace L, et al. A new reconstructive operation for Ebstein's anomaly of the tricuspid valve. J Thorac Cardiovasc Surg. 1988 Jul. 96(1):92-101. [QxMD MEDLINE Link].
Hetzer R, Nagdyman N, Ewert P, et al. A modified repair technique for tricuspid incompetence in Ebstein's anomaly. J Thorac Cardiovasc Surg. 1998 Apr. 115(4):857-68. [QxMD MEDLINE Link].
Quaegebeur JM, Sreeram N, Fraser AG, et al. Surgery for Ebstein's anomaly: the clinical and echocardiographic evaluation of a new technique. J Am Coll Cardiol. 1991 Mar 1. 17(3):722-8. [QxMD MEDLINE Link].
Starnes VA, Pitlick PT, Bernstein D, Griffin ML, Choy M, Shumway NE. Ebstein's anomaly appearing in the neonate. A new surgical approach. J Thorac Cardiovasc Surg. 1991 Jun. 101(6):1082-7. [QxMD MEDLINE Link].
Itatani K, Miyaji K, Inoue N, Ohara K. Ebstein anomaly associated with double-orifice tricuspid valve. J Thorac Cardiovasc Surg. 2010 Jun. 139(6):e131-3. [QxMD MEDLINE Link].
Danielson GK, Driscoll DJ, Mair DD, Warnes CA, Oliver WC Jr. Operative treatment of Ebstein's anomaly. J Thorac Cardiovasc Surg. 1992 Nov. 104(5):1195-202. [QxMD MEDLINE Link].
Gentles TL, Calder AL, Clarkson PM, Neutze JM. Predictors of long-term survival with Ebstein's anomaly of the tricuspid valve. Am J Cardiol. 1992 Feb 1. 69(4):377-81. [QxMD MEDLINE Link].
Kumar AE, Fyler DC, Miettinen OS, Nadas AS. Ebstein's anomaly. Clinical profile and natural history. Am J Cardiol. 1971 Jul. 28(1):84-95. [QxMD MEDLINE Link].
Anderson KR, Danielson GK, McGoon DC, Lie JT. Ebstein's anomaly of the left-sided tricuspid valve: pathological anatomy of the valvular malformation. Circulation. 1978 Sep. 58(3 Pt 2):I87-91. [QxMD MEDLINE Link].
Zalzstein E, Koren G, Einarson T, Freedom RM. A case-control study on the association between first trimester exposure to lithium and Ebstein's anomaly. Am J Cardiol. 1990 Mar 15. 65(11):817-8. [QxMD MEDLINE Link].
Stulak JM, Dearani JA, Danielson GK. Surgical management of Ebstein's anomaly. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2007. 105-11. [QxMD MEDLINE Link].
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 May. 13(5):514-9. [QxMD MEDLINE Link].
Knott-Craig CJ, Overholt ED, Ward KE, Razook JD. Neonatal repair of Ebstein's anomaly: indications, surgical technique, and medium-term follow-up. Ann Thorac Surg. 2000 May. 69(5):1505-10. [QxMD MEDLINE Link].
Dinckal MH, Davutoglu V, Soydinc S, Sezen Y. Radiofrequency current catheter ablation of accessory pathway in Ebstein's anomaly and a review of the literature. Turk Kardiyol Dern Ars. 2003 April. 31(4):239-43. [Full Text].
Lincoln T, Stewart C, Shah P. An unusual first presentation of Ebstein's anomaly in a 72-year-old patient. Ann Thorac Surg. 2012 Feb. 93(2):e19-20. [QxMD MEDLINE Link].
Ghosh S, Avari JN, Rhee EK, Woodard PK, Rudy Y. Noninvasive electrocardiographic imaging (ECGI) of epicardial activation before and after catheter ablation of the accessory pathway in a patient with Ebstein anomaly. Heart Rhythm. 2008 Jun. 5(6):857-60. [QxMD MEDLINE Link].
Healy C, Tanawuttiwat T, Viles-Gonzalez JF. Putting a name on it: Ebstein's anomaly. Am J Med. 2015 Apr. 128 (4):367-8. [QxMD MEDLINE Link].
Celermajer DS, Dodd SM, Greenwald SE, Wyse RK, Deanfield JE. Morbid anatomy in neonates with Ebstein's anomaly of the tricuspid valve: pathophysiologic and clinical implications. J Am Coll Cardiol. 1992 Apr. 19(5):1049-53. [QxMD MEDLINE Link].
Patel V, Nanda NC, Rajdev S, et al. Live/real time three-dimensional transthoracic echocardiographic assessment of Ebstein's anomaly. Echocardiography. 2005 Nov. 22(10):847-54. [QxMD MEDLINE Link].
Chauvaud S, Berrebi A, d'Attellis N, Mousseaux E, Hernigou A, Carpentier A. Ebstein's anomaly: repair based on functional analysis. Eur J Cardiothorac Surg. 2003 Apr. 23(4):525-31. [QxMD MEDLINE Link].
da Silva JP, Baumgratz JF, da Fonseca L,et al. The cone reconstruction of the tricuspid valve in Ebstein's anomaly. The operation: early and midterm results. J Thorac Cardiovasc Surg. 2007 Jan. 133(1):215-23. [QxMD MEDLINE Link].
Dearani JA, Said SM, O'Leary PW, Burkhart HM, Barnes RD, Cetta F. Anatomic repair of Ebstein's malformation: lessons learned with cone reconstruction. Ann Thorac Surg. 2013 Jan. 95(1):220-6; discussion 226-8. [QxMD MEDLINE Link].
Wu Q, Huang Z. A new procedure for Ebstein's anomaly. Ann Thorac Surg. 2004 Feb. 77(2):470-6; discussion 476. [QxMD MEDLINE Link].
Nakata T, Fujimoto Y, Hirose K, Tosaka Y, Ide Y, Sakamoto K. Norwood procedure combined with the Starnes procedure. Ann Thorac Surg. 2008 Oct. 86(4):1378-80. [QxMD MEDLINE Link].
Kawabata T, Kasahara S, Arai S, Sano S. Right ventricular exclusion for a neonatal patient with Ebstein anomaly: a free wall resection of the right ventricle. J Thorac Cardiovasc Surg. 2011 Dec. 142(6):1582-4. [QxMD MEDLINE Link].
Kotoulas C, Jones RP, Turkie W, Hasan R. Edge-to-edge repair of tricuspid valve in a corrected transposition of the great vessels. Hellenic J Cardiol. 2008 Nov-Dec. 49(6):434-6. [QxMD MEDLINE Link].
Barbara DW, Edwards WD, Connolly HM, Dearani JA. Surgical pathology of 104 tricuspid valves (2000-2005) with classic right-sided Ebstein's malformation. Cardiovasc Pathol. 2008 May-Jun. 17(3):166-71. [QxMD MEDLINE Link].
Brown ML, Dearani JA, Danielson GK, et al. Comparison of the outcome of porcine bioprosthetic versus mechanical prosthetic replacement of the tricuspid valve in the Ebstein anomaly. Am J Cardiol. 2009 Feb 15. 103(4):555-61. [QxMD MEDLINE Link].
Kiziltan HT, Theodoro DA, Warnes CA, O'Leary PW, Anderson BJ, Danielson GK. Late results of bioprosthetic tricuspid valve replacement in Ebstein's anomaly. Ann Thorac Surg. 1998 Nov. 66(5):1539-45. [QxMD MEDLINE Link].
Tanaka M, Ohata T, Fukuda S, Kigawa I, Yamashita Y, Wanibuchi Y. Tricuspid valve supra-annular implantation in adult patients with Ebstein's anomaly. Ann Thorac Surg. 2001 Feb. 71(2):582-6. [QxMD MEDLINE Link].
Milgalter E, Laks H. Use of a pericardial patch to bridge the conduction tissue during tricuspid valve replacement. Ann Thorac Surg. 1991 Dec. 52(6):1337-9. [QxMD MEDLINE Link].
Glenn WW, Browne M, Whittemore R. Circulatory bypass of the right side of the heart: Cavo-pulmonary artery shunt-Indications and results (report of a collected series of 537 cases). Cassels DE, ed. The heart and circulation in newborn and infant. New York, NY: Grune & Stratton; 1966. 345-57.
Marianeschi SM, McElhinney DB, Reddy VM, Silverman NH, Hanley FL. Alternative approach to the repair of Ebstein's malformation: intracardiac repair with ventricular unloading. Ann Thorac Surg. 1998 Nov. 66(5):1546-50. [QxMD MEDLINE Link].
Liu J, Qiu L, Zhu Z, Chen H, Hong H. Cone reconstruction of the tricuspid valve in Ebstein anomaly with or without one and a half ventricle repair. J Thorac Cardiovasc Surg. 2011 May. 141(5):1178-83. [QxMD MEDLINE Link].
Celermajer DS, Bull C, Till JA, et al. Ebstein's anomaly: presentation and outcome from fetus to adult. J Am Coll Cardiol. 1994 Jan. 23(1):170-6. [QxMD MEDLINE Link].
Brown ML, Dearani JA, Danielson GK, Cetta F, Connolly HM, Warnes CA, et al. The outcomes of operations for 539 patients with Ebstein anomaly. J Thorac Cardiovasc Surg. 2008 May. 135(5):1120-36, 1136.e1-7. [QxMD MEDLINE Link].
Danielson GK. Ebstein's anomaly: Historical aspects and current management. AHA Cardiothoracic and Vascular Newsletter. 2000.
Brown ML, Dearani JA, Danielson GK, Cetta F, Connolly HM, Warnes CA, et al. Functional status after operation for Ebstein anomaly: the Mayo Clinic experience. J Am Coll Cardiol. 2008 Aug 5. 52(6):460-6. [QxMD MEDLINE Link].
Theodoro DA, Danielson GK, Kiziltan HT, et al. Surgical management of Ebstein’s anomaly: a 25-year experience. Circulation. 1997. 96 suppl I:1-507.
Chen JM, Mosca RS, Altmann K, et al. Early and medium-term results for repair of Ebstein anomaly. J Thorac Cardiovasc Surg. 2004. 127 (4):990-8. [QxMD MEDLINE Link].
Muller J, Kuhn A, Vogt M, Schreiber C, Hess J, Hager A. Improvements in exercise performance after surgery for Ebstein anomaly. J Thorac Cardiovasc Surg. 2011 May. 141(5):1192-5. [QxMD MEDLINE Link].
Boston US, Dearani JA, O'Leary PW, Driscoll DJ, Danielson GK. Tricuspid valve repair for Ebstein's anomaly in young children: a 30-year experience. Ann Thorac Surg. 2006 Feb. 81(2):690-5; discussion 695-6. [QxMD MEDLINE Link].
Kajihara N, Asou T, Takeda Y, et al. Rapid two-stage Starnes procedure for a symptomatic neonate with Ebstein anomaly. Ann Thorac Surg. 2010 Dec. 90(6):2073-5. [QxMD MEDLINE Link].
Shinkawa T, Polimenakos AC, Gomez-Fifer CA, et al. Management and long-term outcome of neonatal Ebstein anomaly. J Thorac Cardiovasc Surg. 2010 Feb. 139(2):354-8. [QxMD MEDLINE Link].
Luxford JC, Arora N, Ayer JG, et al. Neonatal Ebstein anomaly: a 30-year institutional review. Semin Thorac Cardiovasc Surg. 2017 Summer. 29(2):206-12. [QxMD MEDLINE Link].
Holst KA, Dearani JA, Said SM, et al. Surgical management and outcomes of Ebstein anomaly in neonates and infants: a Society of Thoracic Surgeons congenital heart surgery database analysis. Ann Thorac Surg. 2018 Sep. 106(3):785-91. [QxMD MEDLINE Link].
[Guideline] Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC guideline for the management of adults with congenital heart disease: executive summary: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol. 2018 Aug 10. [QxMD MEDLINE Link].
[Guideline] Cotts TB. 2018 AHA/ACC guideline for adults with congenital heart disease. American College of Cardiology. Available at https://www.acc.org/latest-in-cardiology/ten-points-to-remember/2018/08/13/16/26/2018-aha-acc-guideline-for-the-management-of-achd. August 16, 2018; Accessed: October 25, 2018.
Dearani JA, Mora BN, Nelson TJ, Haile DT, O'Leary PW. Ebstein anomaly review: what's now, what's next?. Expert Rev Cardiovasc Ther. 2015 Oct. 13(10):1101-9. [QxMD MEDLINE Link].
Freud LR, Escobar-Diaz MC, Kalish BT, et al. Outcomes and predictors of perinatal mortality in fetuses with Ebstein anomaly or tricuspid valve dysplasia in the current era: a multicenter study. Circulation. 2015 Aug 11. 132(6):481-9. [QxMD MEDLINE Link].

Media Gallery

Anatomic features of Ebstein anomaly. Note the atrialized portion of the right ventricle and displacement of the tricuspid valve. AV = atrioventricular.
Characteristic chest radiograph of a neonate with Ebstein anomaly. The heart shadow demonstrates cardiomegaly, with evidence of severe right atrial enlargement.
Surgical repair of Ebstein anomaly as described by Danielson. (A) The right atrium is incised and reduced, and the atrial septal defect is closed with a patch. The arrow identifies the large anterior leaflet. (B) Mattress sutures with felt pledgets are used to pull the tricuspid annulus and valve together in a horizontal plane, obliterating the atrialized right ventricle. (C) Sutures are tied after all have been inserted. The arrow identifies the septal leaflet. (D) A posterior annuloplasty is used to narrow the orifice of the tricuspid annulus. (E) Completed repair, resulting in a competent tricuspid valve.
Surgical repair of Ebstein anomaly as described by Carpentier. The anterior and posterior leaflets are detached from the tricuspid annulus. In type D lesions, fenestrations are used to create interchordal spaces for the passage of blood into the right ventricle outflow tract (insert). Mattress sutures with pledgets are placed in a vertical plane to plicate the atrialized portion of the right ventricle (top right). The anterior leaflet is reattached at the level of the true annulus with a continuous running suture (bottom left). An annuloplasty ring is inserted to reinforce the repair (bottom right).
Surgical repair of Ebstein anomaly in the neonate as described by Starnes. The atrial septal defect is enlarged by excising the remaining septum. The tricuspid valve orifice is closed with a Gore-Tex patch, effectively creating tricuspid atresia. A Gore-Tex shunt (not shown) is then placed to connect the innominate artery to the right pulmonary artery. PTFE = polytetrafluoroethylene.
Surgical replacement of the tricuspid valve in Ebstein anomaly. (A) The atrialized right ventricle is plicated in a horizontal plane. (B) Sutures are placed on the atrial side of the coronary sinus and atrioventricular node to avoid injury to the conduction system. (C) Sutures are tied with the heart beating and perfused to ensure the conduction system is intact.
Surgical replacement of the tricuspid valve using a pericardial patch to avoid injury to the conduction system. The valve insertion is begun anterior to the coronary sinus using a continuous running suture. A glutaraldehyde-treated pericardial patch is sutured to the septal portion of the prosthetic valve sewing annulus. The free margin of the patch is then sutured to the atrial tissue beyond the area of the conduction tissue. AV = atrioventricular.
Operative steps for Ebstein anomaly repair. (A) Opened right atrium showing displacement of the tricuspid valve. ASD = atrial septal defect, CS = coronary sinus, TTA = true tricuspid annulus. (B) Detached part of the anterior and posterior leaflet as a single piece. (C) Clockwise rotation of the posterior leaflet edge to be sutured to the anterior leaflet septal edge and plication of the true tricuspid annulus to bring the valve to a uniform level. (D) Completion of valve attachment to the true tricuspid annulus and closure of the atrial septal defect. Used with permission from Elsevier (Fig 1 from Da Silva JP, Baumgratz JF, da Fonseca L, et al. The cone reconstruction of the tricuspid valve in Ebstein's anomaly. The operation: early and midterm results. J Thorac Cadiovasc Surg. 2007 Jan;133(1):215-23).
The cardiac silhouette is usually enlarged in Ebstein anomaly. A chest radiograph will usually show an enormous shadow of the right atrium.
Pathology of Ebstein anomaly: Dilated right atrium (RA) with inferior displacement of an abnormal tricuspid valve (TV) septal leaflet, which results in a small right ventricle (RV) that is atrialized. ASD = atrial septal defect, LA = left atrium, LV = left ventricle, PA = pulmonary artery, R-L = right to left.

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