title: Clinical spectrum and long-term outcome of Ebstein's anomaly based on a 26-year experience in an Asian cohort.
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.
authors.affiliation: Department of Pediatrics, National Taiwan University Hospital and College of Medicine, National Taiwan University, No. 7, Chun-Shan Road, Taipei, Taiwan. Ssan924@gmail.com
authors.forename: Ya-Mei
authors.initials: YM
authors.lastname: Chang
authors.forename: Jou-Kou
authors.initials: JK
authors.lastname: Wang
authors.forename: Sheunn-Nan
authors.initials: SN
authors.lastname: Chiu
authors.forename: Ming-Tai
authors.initials: MT
authors.lastname: Lin
authors.forename: En-Ting
authors.initials: ET
authors.lastname: Wu
authors.forename: Chun-An
authors.initials: CA
authors.lastname: Chen
authors.forename: Shu-Chien
authors.initials: SC
authors.lastname: Huang
authors.forename: Yih-Sharng
authors.initials: YS
authors.lastname: Chen
authors.forename: Chung-I
authors.initials: CI
authors.lastname: Chang
authors.forename: Ing-Sh
authors.initials: IS
authors.lastname: Chiu
authors.forename: Jiunn-Lee
authors.initials: JL
authors.lastname: Lin
authors.forename: Ling-Ping
authors.initials: LP
authors.lastname: Lai
authors.forename: Mei-Hwan
authors.initials: MH
authors.lastname: Wu
mesh_terms: D000293:Adolescent; D000328:Adult; D017115:Catheter Ablation; D002648:Child; D002675:Child, Preschool; D004437:Ebstein Anomaly / Q000150:complications / Q000175:diagnosis* / Q000401:mortality / Q000601:surgery; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D008297:Male; D012189:Retrospective Studies; D013617:Tachycardia, Supraventricular / Q000209:etiology / Q000628:therapy; D013624:Taiwan; D016896:Treatment Outcome; D055815:Young Adult
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