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Management and outcome of Ebstein's anomaly in children

Published online by Cambridge University Press:  15 March 2012

Angela Oxenius ,
Christine H. Attenhofer Jost ,
René Prêtre ,
Hitendu Dave ,
Urs Bauersfeld ,
Oliver Kretschmar ,
Burkhardt Seifert ,
Christian Balmer  and
Emanuela R. Valsangiacomo Buechel
Angela Oxenius*
Affiliation:
Division of Pediatric Cardiology, University Children's Hospital, Zurich, Switzerland Pediatric Research Centre (PRC), University of Zurich, Zurich, Switzerland
Christine H. Attenhofer Jost
Affiliation:
Cardiovascular Centre Zurich, Klinik im Park, Zurich, Switzerland
René Prêtre
Affiliation:
Pediatric Research Centre (PRC), University of Zurich, Zurich, Switzerland Cardiothoracic Surgery, University Children's Hospital, Zurich, Switzerland
Hitendu Dave
Affiliation:
Pediatric Research Centre (PRC), University of Zurich, Zurich, Switzerland Cardiothoracic Surgery, University Children's Hospital, Zurich, Switzerland
Urs Bauersfeld
Affiliation:
Division of Pediatric Cardiology, University Children's Hospital, Zurich, Switzerland Pediatric Research Centre (PRC), University of Zurich, Zurich, Switzerland
Oliver Kretschmar
Affiliation:
Division of Pediatric Cardiology, University Children's Hospital, Zurich, Switzerland Pediatric Research Centre (PRC), University of Zurich, Zurich, Switzerland
Burkhardt Seifert
Affiliation:
Division of Biostatistics, Institute of Social and Preventive Medicine, University of Zurich, Zurich, Switzerland
Christian Balmer
Affiliation:
Division of Pediatric Cardiology, University Children's Hospital, Zurich, Switzerland Pediatric Research Centre (PRC), University of Zurich, Zurich, Switzerland
Emanuela R. Valsangiacomo Buechel
Affiliation:
Division of Pediatric Cardiology, University Children's Hospital, Zurich, Switzerland Pediatric Research Centre (PRC), University of Zurich, Zurich, Switzerland
*
Correspondence to: Dr A. Oxenius, MD, Department of Pediatric Cardiology, University Children's Hospital, Steinwiesstrasse 75, 8032 Zurich, Switzerland. Tel: +41 44 266 70 22; Fax: +41 44 266 79 81; E-mail: [email protected]
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Abstract

Objectives

To assess clinical presentation, treatment, and outcome of children with Ebstein's anomaly.

Background

Data on long-term outcome of children with Ebstein's anomaly are scarce.

Methods

Retrospective analysis of all children with Ebstein's anomaly treated between February, 1979 and January, 2009 in a single tertiary institution. Primary outcomes included patient survival and need for intervention, either cardiac surgery or catheter intervention.

Results

A total of 42 patients were diagnosed with Ebstein's anomaly at a median age of 5 days ranging from 1 day to 11.7 years. Symptoms included cyanosis, heart murmur, and/or dyspnoea. Associated cardiac anomalies occurred in 90% of the patients. Average follow-up was 9.5 plus or minus 7.0 years. The overall mortality rate was 14%. Of the six patients, three died postnatally before treatment. Cardiac surgery and/or catheter-guided interventions were required in 33 patients (79%). Cardiac surgery was performed in 21 (50%) patients at a median age of 9.1 years (range 0.1–16.5 years), including biventricular repair in 13 (62%), one-and-a-half chamber repair in seven (33%), and a staged single-ventricle repair in one. Peri-operative mortality was 4%. Catheter-guided interventions consisted of device closure of an atrial septal defect in three cases and radiofrequency ablation of accessory pathways in nine patients. The estimated 10-year survival was 85.3 plus or minus 5.6%.

Conclusion

In children, Ebstein's anomaly is usually diagnosed in the first year of age. Even though children with Ebstein's anomaly often require an intervention, their peri-operative mortality is low and long-term survival is good. Symptomatic newborns requiring an intervention may have a worse outcome.

Keywords

Congenital heart defecttricuspid valveintervention
Type
Original Article
Information
Cardiology in the Young , Volume 23 , Issue 1 , February 2013 , pp. 27 - 34
Copyright
Copyright © Cambridge University Press 2012

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References

1. Attenhofer Jost, CH, Connolly, HM, Dearani, JA, Edwards, WD, Danielson, GK. Ebstein's anomaly. Circulation 2007; 115: 277–285.CrossRefGoogle ScholarPubMed
2. Brown, ML, Dearani, JA, Danielson, GK, et al. Functional status after operation for Ebstein anomaly: the Mayo Clinic experience. JACC 2008; 52: 460–466.CrossRefGoogle ScholarPubMed
3. Attenhofer Jost, CH, Connolly, HM, Warnes, CA, et al. Noncompacted myocardium in Ebstein's anomaly: initial description in three patients. J Am Soc Echocardiogr 2004; 17: 677–680.CrossRefGoogle ScholarPubMed
4. Celermajer, DS, Bull, C, Till, JA, et al. Ebstein's anomaly: presentation and outcome from fetus to adult. JACC 1994; 23: 170–176.Google Scholar
5. Attenhofer Jost, CH, Connolly, HM, Edwards, WD, Hayes, D, Warnes, CA, Danielson, GK. Ebstein's anomaly – review of a multifaceted congenital cardiac condition. Swiss Med Wkly 2005; 135: 269–281.Google Scholar
6. Danielson, GK, Maloney, JD, Devloo, RA. Surgical repair of Ebstein's anomaly. Mayo Clin Proc 1979; 54: 185–192.Google Scholar
7. 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.Google Scholar
8. 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.CrossRefGoogle ScholarPubMed
9. 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: 2661–2666.Google Scholar
10. Celermajer, DS, Cullen, S, Sullivan, ID, Spiegelhalter, DJ, Wyse, RK, Deanfield, JE. Outcome in neonates with Ebstein's anomaly. JACC 1992; 19: 1041–1046.Google Scholar
11. Yetman, AT, Freedom, RM, McCrindle, BW. Outcome in cyanotic neonates with Ebstein's anomaly. Am J Cardiol 1998; 81: 749–754.Google Scholar
12. Shinkawa, T, Polimenakos, AC, Gomez-Fifer, CA, et al. Management and long-term outcome of neonatal Ebstein anomaly. J Thorac Cardiovasc Surg 2010; 139: 354–358.Google Scholar
13. Bove, EL, Hirsch, JC, Ohye, RG, Devaney, EJ. How I manage neonatal Ebstein's anomaly. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2009; 12: 63–65.Google Scholar
14. 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: 112–116.Google Scholar
15. Munayer Calderon, JE, Maza Juarez, G, Carpio, JC, et al. Percutaneous closure of patent foramen ovale with amplatzer device. Report of two cases. Arch Cardiol Mex 2005; 75: 306–309.Google Scholar
16. Badiu, CC, Schreiber, C, Horer, 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: 186–192.CrossRefGoogle ScholarPubMed
17. Paranon, S, Acar, P. Ebstein's anomaly of the tricuspid valve: from fetus to adult: congenital heart disease. Heart 2008; 94: 237–243.CrossRefGoogle ScholarPubMed
18. Cantinotti, M, Bell, A, Razavi, R. Role of magnetic resonance imaging in different ways of presentation of Ebstein's anomaly. J Cardiovasc Med (Hagerstown) 2008; 9: 628–630.CrossRefGoogle ScholarPubMed
19. 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; 81: 690–695; discussion 695–696.Google Scholar
20. Wu, Q, Huang, Z, Pan, G, Wang, L, Li, L, Xue, H. Early and midterm results in anatomic repair of Ebstein anomaly. J Thorac Cardiovasc Surg 2007; 134: 1438–1440; discussion 1440–1432.Google Scholar
21. Knott-Craig, CJ, Goldberg, SP. Management of neonatal Ebstein's anomaly. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2007; 10: 112–116.Google Scholar
22. 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: 50–57.Google Scholar
23. Hebe, J. Ebstein's anomaly in adults. Arrhythmias: diagnosis and therapeutic approach. Thorac Cardiovasc Surg 2000; 48: 214–219.Google Scholar
24. Cappato, R, Schluter, M, Weiss, C, et al. Radiofrequency current catheter ablation of accessory atrioventricular pathways in Ebstein's anomaly. Circulation 1996; 94: 376–383.Google Scholar
25. 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.Google Scholar
26. 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.Google Scholar
27. Taguchi, K, Matsumura, M, Ishikawa, M, et al. Deterioration of Ebstein disease after closure of atrial septal defect. Jpn J Surg 1982; 12: 126–129.CrossRefGoogle ScholarPubMed

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