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- Title
Clinical spectrum and long-term outcome of Ebstein's anomaly based on a 26-year experience in an Asian cohort.
- Authors
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; Chang, Ya-Mei; Wang, Jou-Kou; Chiu, Sheunn-Nan; Lin, Ming-Tai; Wu, En-Ting; Chen, Chun-An; Huang, Shu-Chien
- 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.
- Subjects
TAIWAN; EBSTEIN'S anomaly; CONGENITAL heart disease; CYANOSIS in children; HEART failure; CATHETER ablation; TREATMENT effectiveness; RETROSPECTIVE studies; KAPLAN-Meier estimator; DISEASE complications; SUPRAVENTRICULAR tachycardia; DIAGNOSIS; THERAPEUTICS
- Publication
European Journal of Pediatrics, 2009, Vol 168, Issue 6, p685
- ISSN
0340-6199
- Publication type
journal article
- DOI
10.1007/s00431-008-0820-0