Hostname: page-component-cd9895bd7-jn8rn Total loading time: 0 Render date: 2024-12-28T01:24:11.188Z Has data issue: false hasContentIssue false
  • English
  • Français

Surgical management of symptomatic neonates with Ebstein’s anomaly: choice of operation

Published online by Cambridge University Press:  24 September 2014

Christopher J. Knott-Craig ,
Thittamaranahalli Kariyappa S. Kumar ,
Alejandro R. Arevalo  and
Vijaya M. Joshi
Christopher J. Knott-Craig*
Affiliation:
Division of Pediatric Cardiovascular Surgery, Le Bonheur Children’s Hospital, University of Tennessee Health Science Center, Memphis, Tennessee, United States of America
Thittamaranahalli Kariyappa S. Kumar
Affiliation:
Division of Pediatric Cardiovascular Surgery, Le Bonheur Children’s Hospital, University of Tennessee Health Science Center, Memphis, Tennessee, United States of America
Vijaya M. Joshi
Affiliation:
Divison of Pediatric Cardiology, Le Bonheur Children’s Hospital, University of Tennessee Health Science Center, Memphis, Tennessee, United States of America
*
Corresponding to: Dr C. J. Knott-Craig, Division of Pediatric Cardiovascular Surgery, Le Bonheur Children’s Hospital, University of Tennessee Health Science Center, 50N Dunlap, Memphis, TN 38103, United States of America. Tel: +901 2875995; E-mail: cknottcr@uthsc.edu
Get access Rights & Permissions [Opens in a new window]

Abstract

Objective: Symptomatic neonates with Ebstein’s anomaly pose significant challenge. Within this cohort, neonates with associated anatomical pulmonary atresia have higher mortality. We review our experience with this difficult subset. Methods: A total of 32 consecutive symptomatic neonates with Ebstein’s anomaly underwent surgical intervention between 1994 and 2013. Of them, 20 neonates (62%, 20/32) had associated pulmonary atresia. Patients’ weights ranged from 1.9 to 3.4 kg. All patients without pulmonary atresia had two-ventricle repair. Of the 20 neonates, 16 (80%, 16/20) with Ebstein’s anomaly and pulmonary atresia had two-ventricle repair and 4 had single-ventricle palliation, of which 2 underwent Starnes’ palliation and 2 Blalock–Taussig shunts. Six recent patients with Ebstein’s anomaly and pulmonary atresia had right ventricle to pulmonary artery valved conduit as part of their two-ventricle repair. Results: Overall early mortality was 28% (9/32). For those without pulmonary atresia, mortality was 8.3% (1/12). For the entire cohort of neonates with Ebstein’s anomaly and pulmonary atresia, mortality was 40% (8/20; p=0.05). Mortality for neonates with Ebstein’s anomaly and pulmonary atresia having two-ventricle repair was 44% (7/16). Mortality for neonates with Ebstein’s anomaly and pulmonary atresia having two-ventricle repair utilising right ventricle to pulmonary artery conduit was 16% (1/6). For those having one-ventricle repair, the mortality was 25% (1/4). Conclusions: Surgical management of neonates with Ebstein’s anomaly remains challenging. For neonates with Ebstein’s anomaly and anatomical pulmonary atresia, single-ventricle palliation is associated with lower early mortality compared with two-ventricle repair. This outcome advantage is negated by inclusion of right ventricle to pulmonary artery conduit as part of the two-ventricle repair.

Keywords

neonatepulmonary valvetricuspid valvesurgical techniquesright ventricle
Type
Original Articles
Information
Cardiology in the Young , Volume 25 , Issue 6 , August 2015 , pp. 1119 - 1123
Copyright
© Cambridge University Press 2014 

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

Footnotes

Meeting Presentation: The Society of Thoracic Surgeons 50th Annual Meeting, 2014, Orlando, Florida, United States of America.

References

1. Epstein, ML. Congenital stenosis and insufficiency of the tricuspid valve. In: Allen HD, Clark EB, Gutgesell HP (eds). Moss and Adams’ Heart Disease in Infants, Children and Adolescents, 2nd edn. Lippincott Williams & Wilkins, Philadelphia, 2001: 810–819.Google Scholar
2. Dearani, JA, Danielson, GK. Ebstein’s anomaly. In: Sellke FW, Del Nido PJ, Swanson SJ (eds). Sabiston and Spencer Surgery of the Chest, 7th edn. Elsevier Saunders, Philadelphia, 2005: 2219–2231.Google Scholar
3. Dobell, ARC. Ebstein’s abnormality of the tricuspid valve. In: Arciniegas E (ed). Pediatric Cardiac Surgery. Year Book Medical Publishers, Chicago, 1985: 315–323.Google Scholar
4. Celermajer, DS, Cullen, S, Sullivan, ID, Spiegelhalter, DJ, Wyse, RK, Deanfield, JE. Outcome in neonates with Ebstein’s anomaly. J Am Coll Cardiol 1992; 19: 1041–1046.CrossRefGoogle ScholarPubMed
5. 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; 19: 1049–1053.Google Scholar
6. 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; 69: 1505–1510.Google Scholar
7. Goldberg, SP, Jones, RC, Boston, US, et al. Current trends in the management of neonates with Ebstein’s anomaly. World J Pediatr Congenit Heart Surg 2011; 2: 554–557.CrossRefGoogle ScholarPubMed
8. McElhinney, DB, Salvin, JW, Colan, SD, et al. Improving outcomes in fetuses and neonates with congenital displacement (Ebstein’s malformation) or dysplasia of the tricuspid valve. Am J Cardiol 2005; 96: 582–586.Google Scholar
9. 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: 1082–1087.CrossRefGoogle ScholarPubMed
10. Reemtsen, BL, Fagan, BT, Wells, WJ, Starnes, VA. Current surgical therapy for Ebstein anomaly in neonates. J Thorac Cardiovasc Surg 2006; 132: 1285–1290.Google Scholar
11. Shinkawa, T, Polimenakos, AC, Gomez-Fifer, C, et al. Management and long-term outcome of neonatal Ebstein anomaly. J Thorac Cardiovasc Surg 2010; 139: 354–358.CrossRefGoogle ScholarPubMed
12. Sano, S, Shino, K, Kawada, M, et al. Total right ventricular exclusion procedure: an operation for isolated congestive right ventricular failure. J Thorac Cardiovasc Surg 2002; 123: 640–647.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: 63–65.CrossRefGoogle Scholar
14. Knott-Craig, CJ, Overholt, ED, Ward, KE, Ringewald, JM, Baker, SS, Razook, JD. Repair of Ebstein’s anomaly in the symptomatic neonate: an evolution of technique with 7-year follow-up. Ann Thorac Surg 2002; 73: 1786–1792.Google Scholar
15. 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: 587–592.Google Scholar
16. Knott-Craig, CJ, Goldberg, SP. Management of neonatal Ebstein’s anomaly. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2007: 112–116.Google Scholar
17. Knott-Craig, CJ, Goldberg, SP, Ballweg, JA, Boston, US. Surgical decision making in neonatal Ebstein’s anomaly: an algorithmic approach based on 48 consecutive neonates. World J Pediatr Congenit Heart Surg 2012; 3: 16–20.Google Scholar
18. 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: 1163–1169.Google Scholar
19. Davies, RR, Pasquali, SK, Jacobs, ML, Jacobs, JJ, Wallace, AS, Pizarro, C. Current spectrum of surgical procedures performed for Ebstein’s malformation: an analysis of the Society of Thoracic Surgeons Congenital Heart Surgery Database. Ann Thorac Surg 2013; 96: 1703–1709.Google Scholar
20. Celermajer, DS, Bull, C, Till, JA, et al. Ebstein’s anomaly: presentation and outcome from fetus to adult. J Am Coll Cardiol 1994; 23: 170–176.Google Scholar
21. Augustin, N, Schmidt-Habelmann, P, Wottke, M, Meisner, H, Sebening, F. Results after surgical repair of Ebstein’s anomaly. Ann Thorac Surg 1997; 63: 1650–1656.Google Scholar
22. 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.Google Scholar
23. Tsukimori, K, Morihana, E, Fusazaki, N, Takahata, Y, Oda, S, Kado, H. Critical Ebstein anomaly in a fetus successfully managed by elective preterm delivery and surgical intervention without delay after birth. Pediatr Cardiol 2012; 33: 343–346.Google Scholar
24. 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.CrossRefGoogle Scholar
25. Dearani, JA, Danielson, GK. Surgical management of Ebstein’s anomaly in the adult. Semin Thorac Cardiovasc Surg 2005; 17: 148–154.Google Scholar
26. Danielson, GK, Maloney, JD, Devloo, RA. Surgical repair of Ebstein’s anomaly. Mayo Clin Proc 1979; 54: 185–192.Google ScholarPubMed
27. 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.Google Scholar
28. 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; 95: 220–226.Google Scholar