Role of electrophysiological studies and arrhythmia intervention in repairing Ebstein's anomaly.
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.
Huang
C J
CJ
Department of Surgery, National Taiwan University Hospital, Taipei.
Chiu
I S
IS
Lin
F Y
FY
Chen
W J
WJ
Lin
J L
JL
Lo
H M
HM
Wu
M H
MH
Chu
S H
SH
D000293:Adolescent; D000328:Adult; D001145:Arrhythmias, Cardiac / Q000209:etiology* / Q000601:surgery*; D002648:Child; D016757:Death, Sudden, Cardiac / Q000209:etiology; D004437:Ebstein Anomaly / Q000150:complications* / Q000503:physiopathology*; D004594:Electrophysiology; D005260:Female; D006801:Humans; D008297:Male; D011183:Postoperative Complications / Q000401:mortality; D012008:Recurrence; D016019:Survival Analysis; D016896:Treatment Outcome