Clinical spectrum and long-term outcome of ebstein's anomaly based on a 26-year experience in an asian cohort

Ya Mei Chang, Jou Kou Wang, Sheunn Nan Chiu, Ming Tai Lin, En Ting Wu, Chun An Chen, Shu Chien Huang, Yih Sharng Chen, Chung I. Chang, Ing Sh Chiu, Jiunn Lee Lin, Ling Ping Lai, Mei Hwan Wu

Research output: Contribution to journalArticle

18 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)685-690
Number of pages6
JournalEuropean Journal of Pediatrics
Volume168
Issue number6
DOIs
Publication statusPublished - Jun 1 2009
Externally publishedYes

Keywords

  • Ebstein's anomaly
  • Pulmonary atresia
  • Supraventricular tachyarrhythmia

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

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    Chang, Y. M., Wang, J. K., Chiu, S. N., Lin, M. T., Wu, E. T., Chen, C. A., Huang, S. C., Chen, Y. S., Chang, C. I., Chiu, I. S., Lin, J. L., Lai, L. P., & Wu, M. H. (2009). Clinical spectrum and long-term outcome of ebstein's anomaly based on a 26-year experience in an asian cohort. European Journal of Pediatrics, 168(6), 685-690. https://doi.org/10.1007/s00431-008-0820-0