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

被引：19

作者：

Chang, Ya-Mei ^{[1
,2
,3
]}

Wang, Jou-Kou ^{[1
,2
]}

Chiu, Sheunn-Nan ^{[1
,2
]}

Lin, Ming-Tai ^{[1
,2
]}

Wu, En-Ting ^{[1
,2
]}

Chen, Chun-An ^{[1
,2
]}

Huang, Shu-Chien ^{[2
,4
]}

Chen, Yih-Sharng ^{[2
,4
]}

Chang, Chung-I ^{[2
,4
]}

Chiu, Ing-Sh ^{[2
,4
]}

Lin, Jiunn-Lee ^{[2
,5
]}

Lai, Ling-Ping ^{[2
,5
]}

Wu, Mei-Hwan ^{[1
,2
]}

机构：

[1] Natl Taiwan Univ, Natl Taiwan Univ Hosp, Dept Pediat, Taipei 10764, Taiwan

[2] Natl Taiwan Univ, Coll Med, Taipei 10764, Taiwan

[3] Taipei City Hosp, Branch Women & Children, Dept Pediat, Taipei, Taiwan

[4] Natl Taiwan Univ, Natl Taiwan Univ Hosp, Dept Surg, Taipei 10764, Taiwan

[5] Natl Taiwan Univ, Natl Taiwan Univ Hosp, Dept Internal Med, Taipei 10764, Taiwan

来源：

EUROPEAN JOURNAL OF PEDIATRICS | 2009年 / 168卷 / 06期

关键词：

Ebstein's anomaly; Pulmonary atresia; Supraventricular tachyarrhythmia; RADIOFREQUENCY CATHETER ABLATION; CONGENITAL HEART-DISEASE; TRICUSPID-VALVE; ADULT; FETUS; TACHYARRHYTHMIAS; MALFORMATION; MULTICENTER; FEATURES; CHILDREN;

D O I：

10.1007/s00431-008-0820-0

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

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.

引用

页码：685 / 690

页数：6

共 26 条

[1]

ANDERSON KR, 1979, MAYO CLIN PROC, V54, P174

[2] EBSTEINS-ANOMALY - PRESENTATION AND OUTCOME FROM FETUS TO ADULT [J].