与三分支阻滞和QT间期延长相关的卡恩斯-塞尔综合征。

Kearns-Sayre syndrome associated with trifascicular block and QT prolongation.

作者信息

Karanikis Pavlos, Korantzopoulos Panagiotis, Kountouris Evaggelos, Dimitroula Vasiliki, Patsouras Dimitrios, Pappa Eugenia, Siogas Konstantinos

出版信息

Int J Cardiol. 2005 May 11;101(1):147-50. doi: 10.1016/j.ijcard.2004.01.027.

DOI:10.1016/j.ijcard.2004.01.027

PMID:15860400

Abstract

Patients suffering from Kearns-Sayre syndrome (KSS) often develop conduction defects that may lead to syncope or sudden cardiac death. The association of conduction abnormalities with prolonged QT interval in these patients is very rare. We describe a patient with KSS and diabetes mellitus who suffered a torsades de pointes-induced syncopal attack, in the presence of trifascicular block and QT prolongation (QTc: 574 ms). The patient was successfully treated with permanent pacing. This case highlights that torsades de pointes represents a potential mechanism of syncope or sudden cardiac death in patients with KSS.

摘要

患有卡恩斯-塞尔综合征（KSS）的患者常出现传导缺陷，这可能导致晕厥或心源性猝死。在这些患者中，传导异常与QT间期延长的关联非常罕见。我们描述了一名患有KSS和糖尿病的患者，该患者在存在三分支阻滞和QT延长（QTc：574毫秒）的情况下，发生了尖端扭转型室速诱发的晕厥发作。该患者通过永久起搏治疗成功。此病例强调了尖端扭转型室速是KSS患者晕厥或心源性猝死的一种潜在机制。