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[3例强直性肌营养不良症(斯坦纳特病)心律失常的电疗法]

[The electrotherapy of the arrhythmias in 3 cases of myotonic dystrophy (Steinert's disease)].

作者信息

Gardini A, Zagami N, Marchini A, Morandi F, Bianchetti F, Curnis A, Politi A

机构信息

Servizio di Cardiologia, Ospedale Sacra Famiglia Fatebenefratelli, Erba, CO.

出版信息

Cardiologia. 1996 Dec;41(12):1209-14.

PMID:9064216
Abstract

Myotonic dystrophy is a progressive multisystem disease with autosomal dominant inheritance. Cardiac involvement is an integral part of the disorder, the most prominent manifestations being various conduction defects and rhythm disturbances sometimes related to syncope or sudden cardiac death. We describe 3 cases admitted to our Centres. Two patients received a permanent pacemaker for atrioventricular block of different degree and in the third case an implantable cardioverter defibrillator with antibradycardia pacing was inserted for malignant ventricular tachyarrhythmias and advanced atrioventricular block during atrial flutter.

摘要

强直性肌营养不良是一种具有常染色体显性遗传的进行性多系统疾病。心脏受累是该疾病不可或缺的一部分,最突出的表现是各种传导缺陷和节律紊乱,有时与晕厥或心源性猝死有关。我们描述了3例在我们中心就诊的病例。两名患者因不同程度的房室传导阻滞接受了永久性起搏器植入,在第三例病例中,为一名患有恶性室性心律失常以及心房扑动期间严重房室传导阻滞的患者植入了具备抗心动过缓起搏功能的植入式心脏复律除颤器。

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