Ebstein Anomaly

Jacek Pajak

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Ebstein anomaly (EA) is a rare congenital malformation of the heart that is characterized by apical displacement of the septal and posterior tricuspid valve leaflets, with atrialization of the right ventricle and variable degree of malformation and displacement of the anterior leaflet. Patients can have a variety of symptoms related to the anatomic abnormalities of EA and their hemodynamic effects or associated structural and conduction system disease. Most frequently cyanosis, palpitations, fatigue and dyspnea can be observed. In severe forms edema and ascites. Treatment of EA is complex and depends of the severity of the disease itself and the effect of accompanying congenital structural and electrical abnormalities. Options of treatment include medical therapy, radiofrequency ablation, and surgical therapy. Main aim of the surgical therapy is to correct the underlying tricuspid valve, right ventricular abnormalities and any associated intracardiac defects if exist. Palliative procedures and cardiac transplantation can be considered in most sever EA patients. JRCD 2013; 1 (4): 5–9


Ebstein anomalny, tricuspid valve plasty


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;36:417–27.

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;96:92–101.

W. Ebstein. Über einen sehr seltenen Fall von Insufficienz der Valvula tricuspidalis, bedingt durch eine angeborene hochgradige Missbildung derselben. Archiv für Anatomie, Physiologie und wissenschaftliche Medicin, Leipzig, 1866, 238–254.

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;54:197.

Soloff LA, Stauffer HM, Zatuchni J. Ebstein’s disease: report of the first case diagnosed during life. Am J Med Sci. 1951 Nov; 222:554–61

Van Lingen B, McGregor M, Kaye J et al. Clinical and cardiac catheterization findings compatible with Ebstein’s anomaly of the tricuspid valve: a report of two cases. Am Heart J. 1952;43:77–88

W.E. Medd, M.B. Matthews, W.R.R. Thursfield, Ebstein’s Disease Thorax 1954; 9:14.

Nora J, Nora A, Toews W. Lithium, Ebstein’s anomaly, and other congenital heart defects. Lancet 1974; 4:594–595.

Cohen L, Friedman J, Jefferson J, et al: A reevaluation of risk of in utero exposure to lithium. JAMA 1994; 271:146–150.

Emanuel R., O’Brien K., Ng R. Ebstein’s anomaly: Genetic study of 26 families. Br Heart J 1976; 38:5–7.

Rosenmann A, Arad I, Simcha A, Schaap T. Familial Ebstein’s anomaly. J Med Genet 1976; 13:532–535.

Postma AV, van Engelen K, van de Meerakker J et al. Mutations in the sarcomere gene MYH7 in Ebstein anomaly.Circ Cardiovasc Genet. 2011;4:43–50.

Digilio MC, Bernardini L, Lepri F et al. Ebstein anomaly: Genetic heterogeneity and association with microdeletions 1p36 and 8p23.1. Am J Med Genet A. 2011;155A:2196–202.

Radford DJ, Graff RF, Neilson GH. Diagnosis and natural history of Ebstein’s anomaly. Br Heart J. 1985;54:517–522.

Anderson KR, Zuberbuhler JR, Anderson RH, et al. Morphologic spectrum of Ebstein’s anomaly of the heart: a review. Mayo Clin Proc. 1979;54:174–80

Daliento L, Angelini A, Ho SY, et al. Angiographic and morphologic features of the left ventricle in Ebstein’s malformation. Am J Cardiol. 1997;15:1051–1059

Monibi AA, Neches WH, Lenox CC, et al. Left ventricular anomalies associated with Ebstein’s malformation of the tricuspid valve. Circulation. 1978;57:303–306

Cabin HS, Roberts WC Ebstein’s anomaly of the tricuspid valve and prolapse of the mitral valve. Am Heart J. 1981;101:177–180

Kirklin/Barratt‑Boyes. Cardiac Surgery. Morphology, Diagnostic Criteria, Natural History, Techniques, Results, and Indications. Third Edition. Churchill Livingstone. 2003.

Danielson GK, Driscoll DJ, Mair DD, et al. Operative treatment of Ebstein’s anomaly. J Thorac Cardiovasc Surg. 1992;104:1195–11 202

Chauvaud SM, Brancaccio G, Carpentier AF, Cardiac arrhythmia in patients undergoing surgical repair of Ebstein’s anomaly. Ann Thorac Surg. 2001;71:1547–1552

Khositseth A, Danielson GK, Dearani JA, et al. Supraventricular tachyarrhythmia’s in Ebstein’s anomaly: management and outcome. J Thorac Cardiovasc Surg. 2004;128:826–833.

Giuliani ER, Fuster V, Brandenburg RO, Mair DD. Ebstein’s anomaly: the clinical features and natural history of Ebstein’s anomaly of the tricuspid valve.Mayo Clin Proc. 1979;54:163–173.

Shiina A., Seward J., Edwards W. Two‑dimensional echocardiographic

spectrum of Ebstein’s anomaly: Detailed anatomic assessment. J Am Coll

Cardiol 1984; 3:356–370.

Gussenhoven E, Stewart P, Becker A. Offsetting” of the septal tricuspid lealfet in normal hearts and in hearts with Ebstein anomaly: Anatomic and echographic correlation. Am J Cardiol 1984; 54:172–176.

Głowacki J, Miszalski‑Jamka K, Pawlak S et al. The new diagnostic tool – congenital heart diseases and the great arteries imaging using multislice computed tomography – case presentations. Kardiol Pol. 2009;67:459–63.

Celermajer DS, Dodd SM, Greenwald SE et al. Morbid anatomy in neonates with Ebstein’s anomaly of the tricuspid valve pathophysiologic and clinical implications. J Am Coll Cardiol 1992; 19: 1049–1053.

Barnard CN, Schrire V. Surgical Correction Of Ebstein’s Malformation With Prosthetic Tricuspid Valve. Surgery. 1963;54:302–308.

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;33:297–304

Dearani JA, O’Leary PW, Danielson GK. Surgical treatment of Ebstein’s malformation: state of the art in 2006. Cardiol Young. 2006; 16(suppl 3):12–20.

Wu Q, Huang Z. Anatomic correction of Ebstein anomaly. J Thorac Cardiovasc Surg. 2001;122:1237–1238.

Hetzer R, Nagdyman N, Ewert P, et al. A modified repair technique for tricuspid incompetence in Ebstein’s Anomaly. J Thorac Cardiovasc Surg. 1998;115:857–868.

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;133:215–223

Komoda T, Komoda S, Nagdyman N, et al. Combination of a Hetzer operation and a Sebening stitch for Ebstein’s anomaly. J Thorac Cardiovasc Surg 2007;55:355–359.

Malhotra SP, Petrossian E, Reddy VM et al. Selective right ventricular unloading and novel technical concepts in Ebstein’s anomaly. Ann Thorac Surg 2009;88:1975–1981.

Nagdyman N, Ewert P, Komoda T. et al. Modified repair in patients with Ebstein’s anomaly. J Heart Valve Dis 2010;19:364–370.

Ullmann MV, Born S, Sebening C, et al. Ventricularization of the atrialized chamber: a concept of Ebstein’s anomaly repair. Ann Thorac Surg 2004;78:918–925.

Perier P, Pająk J, Rycaj J, et al.Double leaflet reconstruction of tricuspid valve in Ebstein’s anomaly by use of autologous pericardium.Interact. Cardiovasc. Thorac. Surg. 2009; Suppl: 2, S79‑S80

Brown ML, Dearani JA, Danielson GK et al. The outcomes of operations for 539 patients with Ebstein anomaly. J Thorac Cardiovasc Surg. 2008;135:1120–1136.

Baumgartner H., Bonhoeffer Ph., De Groot N.M.S et al. Guidelines for the management of grown‑up congenital heart disease (new version 2010) European Heart Journal (2010) 31, 2915–2957.

DOI: http://dx.doi.org/10.20418%2Fjrcd.vol1no4.61


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