Haemmerli M, Bolens M, Friedli B
Pediatric Cardiac Unit, Hôpital Cantonal Universitaire, Geneva, Switzerland.
Int J Cardiol. 1990 May;27(2):167-73. doi: 10.1016/0167-5273(90)90155-x.
Overall, long-term survival after the Mustard or Senning operation is good, but late arrhythmias remain a concern. Whether postoperative electrophysiological investigations can identify patients at risk of developing serious arrhythmias is unknown. In this study, 16 children who underwent electrophysiological study after the Mustard or Senning operation for complete transposition (the combination of a concordant atrioventricular and a discordant ventriculo-arterial connexion) were followed up for one month to 9 years (mean 4.33 years) after this investigation. At the electrophysiological study, sinus nodal dysfunction was diagnosed in 9/16 patients, and abnormalities of atrial conduction and refractoriness in 7/13. During follow-up, 7 children developed clinical evidence of the sick sinus syndrome. When considering corrected sinus node recovery time as the only electrophysiological parameter, the sensitivity of predicting sick sinus syndrome from the electrophysiological study was 42%, and specificity 66%. The sensitivity increased to 71% if additional electrophysiological criteria of sinus node dysfunction were included, such as sinuatrial conduction time or sinus node entrance block, but specificity dropped to 55%. Atrioventricular conduction disturbances were rare. Thus, abnormalities at electrophysiological studies after the Mustard or Senning operations are frequent, but identification of patients at risk of developing sick sinus syndrome remains difficult.
总体而言,Mustard或Senning手术后的长期生存率良好,但晚期心律失常仍是一个令人担忧的问题。术后电生理检查能否识别有发生严重心律失常风险的患者尚不清楚。在本研究中,对16例因完全性大动脉转位(房室连接一致而心室-动脉连接不一致的组合)接受Mustard或Senning手术后进行电生理研究的儿童,在该检查后进行了1个月至9年(平均4.33年)的随访。在电生理研究中,16例患者中有9例被诊断为窦房结功能障碍,13例中有7例存在心房传导和不应期异常。在随访期间,7名儿童出现了病态窦房结综合征的临床证据。将校正窦房结恢复时间作为唯一的电生理参数时,电生理研究预测病态窦房结综合征的敏感性为42%,特异性为66%。如果纳入窦房结功能障碍的其他电生理标准,如窦房传导时间或窦房结入口阻滞,敏感性可提高到71%,但特异性降至55%。房室传导障碍很少见。因此,Mustard或Senning手术后电生理研究中的异常情况很常见,但识别有发生病态窦房结综合征风险的患者仍然困难。