Background: Repairing Ebstein's anomaly without correction of associated arrhythmia may result in sudden death. Catheter or surgical ablation is indicated for various symptomatic tachyarrhythmias in Ebstein's anomaly. Methods: Between October 1973 and October 1997, 30 patients with Ebstein's anomaly underwent surgical repair in our hospital. Tricuspid valve replacement was performed in 13 patients, tricuspid annuloplasty and valvuloplasty in the remaining 17 patients. Preoperative electrophysiological studies were performed in 11 patients after 1980. Concomitant arrhythmia ablation was done in 10 patients: 4 for Wolff-Parkinson-White syndrome, 2 for atrioventricular (AV) nodal reentrant tachycardia, one for ventricular tachycardia and 3 for paroxysmal atrial flutter and fibrillation. Results: No mortality or major morbidity occurred in those patients undergoing arrhythmia intervention. There were 7 deaths in total; 6 died suddenly, and the other died of purulent mediastinitis. None of the 6 sudden deaths underwent preoperative electrophysiological evaluation. The functional recovery was good in all survivals. Conclusions: We conclude that detailed preoperative electrophysiological evaluation in patients with Ebstein's anomaly is mandatory. Aggressive surgical intervention of the associated arrhythmias in addition to anatomic correction can reduce the sudden death in Ebstein's anomaly.
Key words:
Ebstein's anomaly - Electrophysiological study - Concomitant arrhythmia ablation
References
-
1
Oh J K, Holmes D R Jr, Hayes D L, Porter C B J, Danielson G K.
Cardiac arrhythmias in patients with surgical repair of Ebstein's anomaly.
J Am Coll Cardiol.
1985;
6
1351-1357
-
2
Mair D D.
Ebstein's anomaly: natural history and management.
J Am Coll Cardiol.
1992;
19
1047-1048
-
3
Danielson G K, Driscoll D J, Mair D D, Warnes C A, Oliver W C Jr.
Operative treatment of Ebstein's anomaly.
J Thorac Cardiovasc Surg.
1992;
104
1195-1202
-
4
Carpentier A, Chauvaud S, Macé L. et al .
A new reconstructive operation for Ebstein's anomaly of the tricuspid valve.
J Thorac Cardiovasc Surg.
1988;
96
92-101
-
5
Kupilik N, Simon P, Moidl R. et al .
Valve-preserving treatment of Ebstein's anomaly: Perioperative and follow-up results.
Thorac Cardiovasc Surg.
1999;
47
229-234
-
6
Gentles T L, Calder A L, Clarkson P M, Neutze J M.
Predictors of long-term survival with Ebstein's anomaly of the tricuspid valve.
Am J Cardiol.
1992;
69
377-381
-
7
Lin F Y, Huang J H, Lin J L, Chen W J, Lo H M, Chu S H.
Atrial compartment surgery for chronic atrial fibrillation associated with congenital heart defects.
J Thorac Cardiovasc Surg.
1996;
111
231-237
-
8
Lo H M, Lin F Y, Tseng C D, Jong Y S, Chern T H, Tseng Y Z.
Selective surgical ablation of the slow atrioventricular nodal pathway by posterior perinodal dissection.
Am J Cardiol.
1993;
71
1457-1459
-
9
Chiou C W, Chen S A, Chiang C E. et al .
Radiofrequency catheter ablation of paroxysmal supraventricular tachycardia in patients with congenital heart disease.
Int J Cardiol.
1995;
50
143-151
-
10
Pressley J C, Wharton J M, Tang A S L, Lowe J E, Gallagher J J, Prystowsky E N.
Effect of Ebstein's anomaly on short- and long-term outcome of surgically treated patients with Wolff-Parkinson-White syndrome.
Circulation.
1992;
86
1147-1155
-
11
Lo H M, Lin F Y, Jong Y S, Tseng Y Z, Wu T L.
Ebstein's anomaly with ventricular tachycardia: evidence for the arrhythmogenic role of the atrialized ventricle.
Am Heart J.
1989;
117
959-962
-
12
Cappato R, Schlüter M, Weiß C. et al .
Radiofrequency current catheter ablation of accessory atrioventricular pathways in Ebstein's anomaly.
Circulation.
1996;
94
376-383
-
13
Okishige K, Azegami K, Goseki Y. et al .
Radiofrequency ablation of tachyarrhythmias in patients with Ebstein's anomaly.
Int J Cardiol.
1997;
60
171-180
Dr. I.-S. Chiu
Department of Surgery National Taiwan University Hospital
No.7 Chung-Shan S. Road
Taipei, Taiwan 100
Phone: 886 2 2312-3456 Ext. 5091
Fax: 886 2 2393-8383
Email: ingsh@ha.mc.ntu.edu.tw
