Brancaccio G, Chauvaud S, Carpentier A
Servizio di Chirurgia Cardiovascolare, Hôpital Broussais, Parigi, Francia.
Ital Heart J Suppl. 2000 Sep;1(9):1173-9.
Patients with Ebstein's anomaly frequently have troublesome cardiac arrhythmias. In particular, this malformation is the most common congenital defect associated with the Wolff-Parkinson-White syndrome. The aim of this study was to investigate the postoperative evolution of arrhythmias without the aid of any surgical techniques for arrhythmia.
Between 1980 and 1999, 48 patients (22 males, 26 females), with a preoperatively documented arrhythmia, underwent an operation for the correction of Ebstein's anomaly at the Hôpital Broussais. Of these, 24 had paroxysmal supraventricular tachycardia, 12 had atrial fibrillation or flutter, 8 had Wolff-Parkinson-White syndrome, 1 had non-sustained ventricular tachycardia, and the remaining 3 patients had atrioventricular block.
The operative mortality was 8% (4/48). After operation 46% (20/44) of the patients regained permanent sinus rhythm (20/44 vs 2/48, p < 0.01), supraventricular tachyarrhythmia occurred in 16% of the patients (7/44), 8 patients (18%) had atrial fibrillation, and ventricular preexcitation syndrome was present in 3 patients (7%). The incidence of pacemaker implantation for complete heart block was 11% (5/44). Follow-up was achieved in 95% of patients (40/44) who survived the operation and the perioperative period. The mean follow-up was 63 +/- 54 months (range 4-226 months). During this time there were 6 additional deaths. Eight patients continued to have symptomatic arrhythmias (2 had paroxysmal supraventricular tachycardia, 6 had atrial fibrillation), but 55% of patients (20/36) reported no symptoms of arrhythmia (20/36 vs 2/48, p < 0.01).
Surgical repair improves the quality of life of these patients by reducing the incidence of arrhythmias, in fact less than one sixth of patients continued to have postoperative symptomatic arrhythmias. This can be explained by the interruption of accessory pathways that seem to be a major cause of arrhythmia in Ebstein's anomaly.
埃布斯坦畸形患者常伴有令人困扰的心律失常。特别是，这种畸形是与预激综合征相关的最常见先天性缺陷。本研究的目的是在不借助任何心律失常手术技术的情况下，调查心律失常的术后演变情况。
1980年至1999年间，48例术前记录有心律失常的患者（22例男性，26例女性）在布鲁赛医院接受了埃布斯坦畸形矫正手术。其中，24例有阵发性室上性心动过速，12例有心房颤动或扑动，8例有预激综合征，1例有非持续性室性心动过速，其余3例患者有房室传导阻滞。
手术死亡率为8%（4/48）。术后46%（20/44）的患者恢复了永久性窦性心律（20/44比2/48，p<0.01），16%的患者发生室上性快速心律失常（7/44），8例患者（18%）有心房颤动，3例患者（7%）有室性预激综合征。因完全性心脏传导阻滞植入起搏器的发生率为11%（5/44）。95%的患者（40/44）在手术后及围手术期存活下来并得到随访。平均随访时间为63±54个月（范围4-226个月）。在此期间又有6例死亡。8例患者仍有症状性心律失常（2例有阵发性室上性心动过速，6例有心房颤动），但55%的患者（20/36）报告没有心律失常症状（20/36比2/48，p<0.01）。
手术修复通过降低心律失常的发生率改善了这些患者的生活质量，事实上不到六分之一的患者术后仍有症状性心律失常。这可以通过中断旁路来解释，旁路似乎是埃布斯坦畸形中心律失常的主要原因。
