Management and long-term outcome of neonatal Ebstein anomaly

J Thorac Cardiovasc Surg. 2010 Feb;139(2):354-8. doi: 10.1016/j.jtcvs.2009.07.062. Epub 2009 Sep 22.


Objective: The objective of this study was to review the long-term results of symptomatic patients with Ebstein anomaly in the neonatal period.

Methods: The medical records of 40 neonates with a diagnosis of Ebstein anomaly who were admitted to our institution between January 1988 and June 2008 were retrospectively reviewed. Primary outcomes studied included patient survival and need for reintervention.

Results: No early intervention was required in 16 of the 40 patients with a hospital survival of 94% (15/16) and no late mortality. The remaining 24 patients underwent surgical intervention in the neonatal period. A shunt alone was performed in 9 patients with an actuarial survival of 88.9% at 1 year and 76.2% at 5 and 10 years. For the patients undergoing intervention on the tricuspid valve, survival estimates for the 11 patients with a right ventricular exclusion procedure were 63.6% at 1, 5, and 10 years and 47.7% at 15 years compared with 25.0% at 1, 5, and 10 years for the 4 patients with tricuspid valve repair. All long-term survivors were in New York Heart Association class I or II, and only 1 patient required antiarrhythmic medication.

Conclusion: Symptomatic neonates with Ebstein anomaly requiring no intervention or shunting alone have good long-term survival. For patients needing intervention on the tricuspid valve, overall survival is lower. For these patients, right ventricular exclusion may be superior to tricuspid valve repair.

MeSH terms

  • Clinical Protocols
  • Ebstein Anomaly / mortality
  • Ebstein Anomaly / surgery*
  • Hospital Mortality
  • Humans
  • Infant, Newborn
  • Retrospective Studies
  • Treatment Outcome