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Dealing with Ebstein's anomaly

Published online by Cambridge University Press:  29 October 2013

Lianne M. Geerdink
Department of Pediatrics, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
Livia Kapusta
Pediatric Cardiology Unit, E. Wolfson Medical Centre, Holon, Israel Children's Heart Centre, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
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Ebstein's anomaly is a complex congenital disorder of the tricuspid valve. Presentation in neonatal life and (early) childhood is common. Disease severity and clinical features vary widely and require a patient-tailored treatment. In this review, we describe the natural history of children and adolescents with Ebstein's anomaly, including symptoms and signs presenting at diagnosis. Current classification strategies of Ebstein's anomaly are discussed. We report on diagnostic methods for establishing the severity of disease that might enhance decision on the timing of surgical intervention. Furthermore, we describe different surgical options for severely ill neonates and multiple surgical interventions after infancy. Only with ample knowledge and understanding of the above, this complex and diverse group of patients can be correctly treated in order to improve not only duration, but also quality of life.

Review Article
Copyright © Cambridge University Press 2013 

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1. Dearani, JA, Danielson, GK. Congenital Heart Surgery Nomenclature and Database Project: Ebstein's anomaly and tricuspid valve disease. Ann Thorac Surg 2000; 69 (Suppl 4): S106S117.CrossRefGoogle Scholar
2. Schreiber, C, Cook, A, Ho, SY, Augustin, N, Anderson, RH. Morphologic spectrum of Ebstein's malformation: revisitation relative to surgical repair. J Thorac Cardiovasc Surg 1999; 117: 148155.CrossRefGoogle Scholar
3. Carpentier, A, Chauvaud, S, Macé, L, et al. A new reconstructive operation for Ebstein's anomaly of the tricuspid valve. J Thorac Cardiovasc Surg 1988; 96: 92101.Google Scholar
4. Celermajer, DS, Bull, C, Till, JA, et al. Ebstein's anomaly: presentation and outcome from fetus to adult. J Am Coll Cardiol 1994; 23: 170176.CrossRefGoogle Scholar
5. Attenhofer Jost, CH, Connolly, HM, O'Leary, PW, Warnes, CA, Tajik, AJ, Seward, JB. Left heart lesions in patients with Ebstein anomaly. Mayo Clin Proc 2005; 80: 361368.CrossRefGoogle Scholar
6. Kapusta, L, Eveleigh, RM, Poulino, SE, et al. Ebstein's anomaly: factors associated with death in childhood and adolescence: a multi-centre, long-term study. Eur Heart J 2007; 28: 26612666.CrossRefGoogle ScholarPubMed
7. Flores Arizmendi, A, Fernández Pineda, L, Quero Jiménez, C, et al. The clinical profile of Ebstein's malformation as seen from the fetus to the adult in 52 patients. Cardiol Young 2004; 14: 5563.Google ScholarPubMed
8. Delhaas, T, Sarvaas, GJ, Rijlaarsdam, ME, et al. A multicenter, long-term study on arrhythmias in children with Ebstein anomaly. Pediatr Cardiol 2010; 31: 229233.CrossRefGoogle ScholarPubMed
9. Sharland, GK, Chita, SK, Allan, LD. Tricuspid valve dysplasia or displacement in intrauterine life. J Am Coll Cardiol 1991; 17: 944949.CrossRefGoogle ScholarPubMed
10. Shiina, A, Seward, JB, Edwards, WD, Hagler, DJ, Tajik, AJ. Two-dimensional echocardiographic spectrum of Ebstein's anomaly: detailed anatomic assessment. J Am Coll Cardiol 1984; 3 (Pt 1): 356370.CrossRefGoogle ScholarPubMed
11. Bharucha, T, Anderson, RH, Lim, ZS, Vettukattil, JJ. Multiplanar review of three-dimensional echocardiography gives new insights into the morphology of Ebstein's malformation. Cardiol Young 2010; 20: 4953.CrossRefGoogle ScholarPubMed
12. Driscoll, DJ, Mottram, CD, Danielson, GK. Spectrum of exercise intolerance in 45 patients with Ebstein's anomaly and observations on exercise tolerance in 11 patients after surgical repair. J Am Coll Cardiol 1988; 11: 831836.CrossRefGoogle ScholarPubMed
13. MacLellan-Tobert, SG, Driscoll, DJ, Mottram, CD, Mahoney, DW, Wollan, PC, Danielson, GK. Exercise tolerance in patients with Ebstein's anomaly. J Am Coll Cardiol 1997; 29: 16151622.CrossRefGoogle ScholarPubMed
14. Müller, J, Kühn, A, Vogt, M, Schreiber, C, Hess, J, Hager, A. Improvements in exercise performance after surgery for Ebstein anomaly. J Thorac Cardiovasc Surg 2011; 141: 11921195.CrossRefGoogle ScholarPubMed
15. James, FW, Kaplan, S, Glueck, CJ, Tsay, JY, Knight, MJ, Sarwar, CJ. Responses of normal children and young adults to controlled bicycle exercise. Circulation 1980; 61: 902912.CrossRefGoogle Scholar
16. Tobler, D, Yalonetsky, S, Crean, AM, et al. Right heart characteristics and exercise parameters in adults with Ebstein anomaly: new perspectives from cardiac magnetic resonance imaging studies. Int J Cardiol 2013; 165: 146150.CrossRefGoogle ScholarPubMed
17. 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: 767773.CrossRefGoogle ScholarPubMed
18. Attenhofer Jost, CH, Edmister, WD, Julsrud, PR, et al. Prospective comparison of echocardiography versus cardiac magnetic resonance imaging in patients with Ebstein's anomaly. Int J Cardiovasc Imaging 2012; 28: 11471159.CrossRefGoogle ScholarPubMed
19. Reemtsen, BL, Fagan, BT, Wells, WJ, Starnes, VA. Current surgical therapy for Ebstein anomaly in neonates. J Thorac Cardiovasc Surg 2006; 132: 12851290.CrossRefGoogle Scholar
20. Atz, AM, Munoz, RA, Adatia, I, Wessel, DL. Diagnostic and therapeutic uses of inhaled nitric oxide in neonatal Ebstein's anomaly. Am J Cardiol 2003; 91: 906908.CrossRefGoogle ScholarPubMed
21. Roten, L, Lukac, P, DE Groot, N, et al. Catheter ablation of arrhythmias in ebstein's anomaly: a multicenter study. J Cardiovasc Electrophysiol 2011; 22: 13911396.CrossRefGoogle ScholarPubMed
22. 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: 168173.CrossRefGoogle ScholarPubMed
23. Bockeria, L, Golukhova, E, Dadasheva, M, et al. Advantages and disadvantages of one-stage and two-stage surgery for arrhythmias and Ebstein's anomaly. Eur J Cardiothorac Surg 2005; 28: 536540.CrossRefGoogle ScholarPubMed
24. Knott-Craig, CJ, Goldberg, SP, Overholt, ED, Colvin, EV, Kirklin, JK. Repair of neonates and young infants with Ebstein's anomaly and related disorders. Ann Thorac Surg 2007; 84: 587592; discussion 592–593.CrossRefGoogle Scholar
25. Brown, ML, Dearani, JA, Danielson, GK, et al. The outcomes of operations for 539 patients with Ebstein anomaly. J Thorac Cardiovasc Surg 2008; 135: 11201136, e1–7.CrossRefGoogle ScholarPubMed
26. Sarris, GE, Giannopoulos, NM, Tsoutsinos, AJ, et al. Results of surgery for Ebstein anomaly: a multicenter study from the European Congenital Heart Surgeons Association. J Thorac Cardiovasc Surg 2006; 132: 5057.CrossRefGoogle ScholarPubMed
27. Palmen, M, de Jong, PL, Klieverik, LM, Venema, AC, Meijboom, FJ, Bogers, AJ. Long-term follow-up after repair of Ebstein's anomaly. Eur J Cardiothorac Surg 2008; 34: 4854.CrossRefGoogle Scholar
28. Badiu, CC, Schreiber, C, Hörer, J, et al. Early timing of surgical intervention in patients with Ebstein's anomaly predicts superior long-term outcome. Eur J Cardiothorac Surg 2010; 37: 186192.CrossRefGoogle ScholarPubMed
29. 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; 101: 10821087.Google Scholar
30. Watanabe, M, Harada, Y, Takeuchi, T, Satomi, G, Yasukouchi, S. Modified Starnes operation for neonatal Ebstein's anomaly. Ann Thorac Surg 2002; 74: 916917.CrossRefGoogle ScholarPubMed
31. Boston, US, Goldberg, SP, Ward, KE, et al. Complete repair of Ebstein anomaly in neonates and young infants: a 16-year follow-up. J Thorac Cardiovasc Surg 2011; 141: 11631169.CrossRefGoogle Scholar
32. Dearani, JA, Oleary, PW, Danielson, GK. Surgical treatment of Ebstein's malformation: state of the art in 2006. Cardiol Young 2006; 16 (Suppl 3): 1220.CrossRefGoogle ScholarPubMed
33. Wu, Q, Huang, Z. A new procedure for Ebstein's anomaly. Ann Thorac Surg 2004; 77: 470476; discussion 476.CrossRefGoogle ScholarPubMed
34. 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: 19751981; discussion 1981.CrossRefGoogle ScholarPubMed
35. Silva, JP, Baumgratz, JF, Fonseca, L, et al. Ebstein's anomaly: results of the conic reconstruction of the tricuspid valve. Arq Bras Cardiol 2004; 82: 212216.CrossRefGoogle ScholarPubMed
36. Hörer, J, Vogt, M, Wottke, M, et al. Evaluation of the Aristotle complexity models in adult patients with congenital heart disease. Eur J Cardiothorac Surg 2013; 43: 128134.CrossRefGoogle ScholarPubMed
37. Garatti, A, Nano, G, Bruschi, G, et al. Twenty-five year outcomes of tricuspid valve replacement comparing mechanical and biologic prostheses. Ann Thorac Surg 2012; 93: 11461153.CrossRefGoogle ScholarPubMed
38. Chang, YM, Wang, JK, Chiu, SN, et al. Clinical spectrum and long-term outcome of Ebstein's anomaly based on a 26-year experience in an Asian cohort. Eur J Pediatr 2009; 168: 685690.CrossRefGoogle Scholar
39. 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; 69: 377381.CrossRefGoogle ScholarPubMed
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