Clinical spectrum and long-term outcome of Ebstein's anomaly based on a 26-year experience in an Asian cohort.
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.
Chang
Ya-Mei
YM
Department of Pediatrics, National Taiwan University Hospital and College of Medicine, National Taiwan University, No. 7, Chun-Shan Road, Taipei, Taiwan. Ssan924@gmail.com
Wang
Jou-Kou
JK
Chiu
Sheunn-Nan
SN
Lin
Ming-Tai
MT
Wu
En-Ting
ET
Chen
Chun-An
CA
Huang
Shu-Chien
SC
Chen
Yih-Sharng
YS
Chang
Chung-I
CI
Chiu
Ing-Sh
IS
Lin
Jiunn-Lee
JL
Lai
Ling-Ping
LP
Wu
Mei-Hwan
MH
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
Circulation. 2008 Mar 11;117(10):1340-50
18332279
Mayo Clin Proc. 1979 Mar;54(3):174-80
431123
J Thorac Cardiovasc Surg. 2006 Jul;132(1):50-7
16798302
J Cardiovasc Electrophysiol. 1998 Dec;9(12):1370-7
9869537
Eur Heart J. 2007 Nov;28(21):2661-6
17895254
Thorac Cardiovasc Surg. 2000 Aug;48(4):203-8
11005593
Heart. 2008 Feb;94(2):237-43
18195130
Ann Thorac Surg. 2000 May;69(5):1505-10
10881831
N Engl J Med. 1994 May 26;330(21):1481-7
8164700
Circulation. 2007 Jan 16;115(2):277-85
17228014
Am J Cardiol. 2005 Aug 15;96(4):582-6
16098316
Am J Cardiol. 1995 Dec 15;76(17):1277-83
7503010
Ann Thorac Surg. 2007 Aug;84(2):587-92; discussion 592-3
17643640
J Thorac Cardiovasc Surg. 2004 Dec;128(6):826-33
15573066
J Thorac Cardiovasc Surg. 2006 Dec;132(6):1285-90
17140943
Circulation. 1976 Jan;53(1):63-8
1244257
Heart Rhythm. 2004 Jul;1(2):168-73
15851148
Semin Thorac Cardiovasc Surg. 2005 Summer;17(2):148-54
16087085
Int J Cardiol. 2006 Jul 28;111(1):92-7
16242194
J Postgrad Med. 1997 Jan-Mar;43(1):8-11
10740704
J Thorac Cardiovasc Surg. 2006 Feb;131(2):469-71
16434282
Pediatr Cardiol. 2004 Jan-Feb;25(1):56-7
14534759
J Am Coll Cardiol. 1994 Jan;23(1):170-6
8277076
Am J Cardiol. 1998 Mar 15;81(6):749-54
9527086
J Am Coll Cardiol. 1989 Nov 1;14(5):1300-7
2808987
Pediatr Cardiol. 1999 May-Jun;20(3):189-94
10089242