Skip to main content
SpringerLink
Log in

Clinical spectrum and long-term outcome of Ebstein’s anomaly based on a 26-year experience in an Asian cohort

  • Original Paper
  • Published:
European Journal of Pediatrics Aims and scope Submit manuscript

Abstract

Ebstein’s anomaly is a rare, congenital cardiac anomaly that may result in cyanosis, right heart failure, and tachyarrhythmia during the newborn stage or after adolescence. This study investigated the data of 77 patients diagnosed between 1980 and 2006 at a tertiary care center in Taiwan. Patients were grouped into either an early group or a late group. Survival declined rapidly within the newborn stage in the early group, but declined only during the third decade in the late group. Surgical results were poor (20% success rate) for neonatal systemic-to-pulmonary shunts in those cases with associated pulmonary atresia, but were satisfactory for other surgical modes. Supraventricular tachyarrhythmia occurred in 31 (41%) patients at a median age of 10 years and could be eliminated by radiofrequency ablation (81% success rate), though the recurrence rate was high (41%). In conclusion, other than those cases requiring shunts at the newborn stage, the long-term outcome was favorable.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6

Similar content being viewed by others

References

  1. Anderson KR, Zuberbuhler JR, Anderson RH, Becker AE, Lie JT (1979) Morphologic spectrum of Ebstein’s anomaly of the heart: a review. Mayo Clin Proc 54:174–180

    PubMed  CAS  Google Scholar 

  2. Attenhofer Jost CH, Connolly HM, Dearani JA, Edwards WD, Danielson GK (2007) Ebstein’s anomaly. Circulation 115(2):277–285. doi:10.1161/CIRCULATIONAHA.106.619338

    Article  PubMed  Google Scholar 

  3. Celermajer DS, Bull C, Till JA, Cullen S, Vassillikos VP, Sullivan ID et al (1994) Ebstein’s anomaly: presentation and outcome from fetus to adult. J Am Coll Cardiol 23:170–176

    Article  PubMed  CAS  Google Scholar 

  4. Chetaille P, Walsh EP, Triedman JK (2004) Outcomes of radiofrequency catheter ablation of atrioventricular reciprocating tachycardia in patients with congenital heart disease. Heart Rhythm 1:168–173. doi:10.1016/j.hrthm.2004.03.064

    Article  PubMed  Google Scholar 

  5. Dearani JA, Danielson GK (2005) Surgical management of Ebstein’s anomaly in the adult. Semin Thorac Cardiovasc Surg 17:148–154. doi:10.1053/j.semtcvs.2005.02.008

    Article  PubMed  Google Scholar 

  6. Endo M, Sawamura Y, Saiki Y, Shibuya T, Tanaka T, Tabayashi K (2004) Blalock–Taussig shunt for a neonatal case of Ebstein’s anomaly with unobstructed right ventricular outflow tract. Pediatr Cardiol 25:56–57. doi:10.1007/s00246-003-0382-x

    Article  PubMed  CAS  Google Scholar 

  7. Farooki ZQ, Henry JG, Green EW (1976) Echocardiographic spectrum of Ebstein’s anomaly of the tricuspid valve. Circulation 53:63–68

    PubMed  CAS  Google Scholar 

  8. Frescura C, Angelini A, Daliento L, Thiene G (2000) Morphological aspects of Ebstein’s anomaly in adults. Thorac Cardiovasc Surg 48:203–208. doi:10.1055/s-2000-6893

    Article  PubMed  CAS  Google Scholar 

  9. Kapusta L, Eveleigh RM, Poulino SE, Rijlaarsdam ME, du Marchie Sarvaas GJ, Strengers JL et al (2007) Ebstein’s anomaly: factors associated with death in childhood and adolescence: a multi-centre, long-term study. Eur Heart J 28:2661–2666. doi:10.1093/eurheartj/ehm398

    Article  PubMed  Google Scholar 

  10. Khositseth A, Danielson GK, Dearani JA, Munger TM, Porter CJ (2004) Supraventricular tachyarrhythmias in Ebstein anomaly: management and outcome. J Thorac Cardiovasc Surg 128:826–833. doi:10.1016/j.jtcvs.2004.02.012

    Article  PubMed  Google Scholar 

  11. Knott-Craig CJ, Overholt ED, Ward KE, Razook JD (2000) Neonatal repair of Ebstein’s anomaly: indications, surgical technique, and medium-term follow-up. Ann Thorac Surg 69:1505–1510. doi:10.1016/S0003-4975(00)01138-3

    Article  PubMed  CAS  Google Scholar 

  12. Knott-Craig CJ, Goldberg SP, Overholt ED, Colvin EV, Kirklin JK (2007) Repair of neonates and young infants with Ebstein’s anomaly and related disorders. Ann Thorac Surg 84:587–593. doi:10.1016/j.athoracsur.2007.03.061

    Article  PubMed  Google Scholar 

  13. Kugler JD, Danford DA, Deal BJ, Gillette PC, Perry JC, Silka MJ et al (1994) Radiofrequency catheter ablation for tachyarrhythmias in children and adolescents. The Pediatric Electrophysiology Society. N Engl J Med 330:1481–1487. doi:10.1056/NEJM199405263302103

    Article  PubMed  CAS  Google Scholar 

  14. Lupoglazoff, Denjoy I, Kabaker M, Benali K, Riescher B, Magnier S JM et al (1999) Cardiorespiratory exercise tolerance in asymptomatic children with Ebstein’s anomaly. Pediatr Cardiol 20:189–194. doi:10.1007/s002469900438

    Article  PubMed  CAS  Google Scholar 

  15. Madiwale CV, Deshpande JR, Kinare SG (1997) Ebstein’s anomaly—an autopsy study of 28 cases. J Postgrad Med 43:8–11

    PubMed  CAS  Google Scholar 

  16. McElhinney DB, Salvin JW, Colan SD, Thiagarajan R, Crawford EC, Marcus EN et al (2005) Improving outcomes in fetuses and neonates with congenital displacement (Ebstein’s malformation) or dysplasia of the tricuspid valve. Am J Cardiol 96:582–586. doi:10.1016/j.amjcard.2005.04.024

    Article  PubMed  Google Scholar 

  17. Paranon S, Acar P (2008) Ebstein’s anomaly of the tricuspid valve: from fetus to adult: congenital heart disease. Heart 94:237–243. doi:10.1136/hrt.2006.105262

    Article  PubMed  Google Scholar 

  18. Reemtsen BL, Fagan BT, Wells WJ, Starnes VA (2006) Current surgical therapy for Ebstein anomaly in neonates. J Thorac Cardiovasc Surg 132:1285–1290. doi:10.1016/j.jtcvs.2006.08.044

    Article  PubMed  Google Scholar 

  19. Reich JD, Auld D, Hulse E, Sullivan K, Campbell R (1998) The Pediatric Radiofrequency Ablation Registry’s experience with Ebstein’s anomaly. Pediatric Electrophysiology Society. J Cardiovasc Electrophysiol 9:1370–1377

    Article  PubMed  CAS  Google Scholar 

  20. Roberson DA, Silverman NH (1989) Ebstein’s anomaly: echocardiographic and clinical features in the fetus and neonate. J Am Coll Cardiol 14:1300–1307

    Article  PubMed  CAS  Google Scholar 

  21. Sarris GE, Giannopoulos NM, Tsoutsinos AJ, Chatzis AK, Kirvassilis G, Brawn WJ et al (2006) Results of surgery for Ebstein anomaly: a multicenter study from the European Congenital Heart Surgeons Association. J Thorac Cardiovasc Surg 132:50–57. doi:10.1016/j.jtcvs.2005.10.062

    Article  PubMed  Google Scholar 

  22. Silverman NH, Gerlis LM, Horowitz ES, Ho SY, Neches WH, Anderson RH (1995) Pathologic elucidation of the echocardiographic features of Ebstein’s malformation of the morphologically tricuspid valve in discordant atrioventricular connections. Am J Cardiol 76:1277–1283. doi:10.1016/S0002-9149(99)80356-7

    Article  PubMed  CAS  Google Scholar 

  23. Sommer RJ, Hijazi ZM, Rhodes JF (2008) Pathophysiology of congenital heart disease in the adult: part III: complex congenital heart disease. Circulation 117:1340–1350. doi:10.1161/CIRCULATIONAHA.107.714428

    Article  PubMed  Google Scholar 

  24. Trojnarska O, Szyszka A, Gwizdała A, Siniawski A, Oko-Sarnowska Z, Chmara E et al (2006) Adults with Ebstein’s anomaly—cardiopulmonary exercise testing and BNP levels exercise capacity and BNP in adults with Ebstein’s anomaly. Int J Cardiol 111:92–97. doi:10.1016/j.ijcard.2005.07.019

    Article  PubMed  Google Scholar 

  25. Yetman AT, Freedom RM, McCrindle BW (1998) Outcome in cyanotic neonates with Ebstein’s anomaly. Am J Cardiol 81:749–754

    Article  PubMed  CAS  Google Scholar 

  26. Yun TJ, Lee SH, Ko JK (2006) Neonatal stenotic Ebstein’s anomaly: a novel technique of right ventricular exclusion. J Thorac Cardiovasc Surg 131:469–471

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Departments of Pediatrics, National Taiwan University Hospital and College of Medicine, National Taiwan University, No. 7, Chun-Shan Road, Taipei, Taiwan

    Ya-Mei Chang, Jou-Kou Wang, Sheunn-Nan Chiu, Ming-Tai Lin, En-Ting Wu, Chun-An Chen & Mei-Hwan Wu

  2. Department Pediatrics, Taipei City Hospital-Branch for Women and Children, Taipei, Taiwan

    Ya-Mei Chang

  3. Department of Surgery, National Taiwan University Hospital and College of Medicine, National Taiwan University, No. 7, Chun-Shan Road, Taipei, Taiwan

    Shu-Chien Huang, Yih-Sharng Chen, Chung-I Chang & Ing-Sh Chiu

  4. Department of Internal Medicine, National Taiwan University Hospital and College of Medicine, National Taiwan University, No. 7, Chun-Shan Road, Taipei, Taiwan

    Jiunn-Lee Lin & Ling-Ping Lai

Authors
  1. Ya-Mei Chang
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Jou-Kou Wang
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Sheunn-Nan Chiu
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Ming-Tai Lin
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. En-Ting Wu
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Chun-An Chen
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Shu-Chien Huang
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Yih-Sharng Chen
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Chung-I Chang
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. Ing-Sh Chiu
    View author publications

    You can also search for this author in PubMed Google Scholar

  11. Jiunn-Lee Lin
    View author publications

    You can also search for this author in PubMed Google Scholar

  12. Ling-Ping Lai
    View author publications

    You can also search for this author in PubMed Google Scholar

  13. Mei-Hwan Wu
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Mei-Hwan Wu.

Rights and permissions

Reprints and permissions

About this article

Cite this article

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 168, 685–690 (2009). https://doi.org/10.1007/s00431-008-0820-0

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00431-008-0820-0

Keywords

Search

Navigation