Proclemer A, Facchin D, Feruglio G A, Nucifora R
Istituto di Cardiologia, Ospedale Civile S. Maria Della Misericordia, Udine.
G Ital Cardiol. 1993 Dec;23(12):1211-8.
In this report we describe a patient with a rare arrhythmic syndrome, recently reported and characterized by aborted sudden death, right bundle branch block and persistent ST segment elevation in precordial leads. The patient is a 65-year-old man, who had survived recurrent episodes of ventricular fibrillation (VF). The ECG during sinus rhythm revealed the coexistence of right bundle branch block and persistent ST segment elevation (1-4 mm) in leads V1-V3. The clinical and laboratory investigations, including echocardiography, cardiac catheterization and right endomyocardial biopsy, could not identify any significant morphological and functional cardiac abnormalities. The presence of late potentials (high gain ECG), and the inducibility of VF during the electrophysiological study, suggest a functional reentry as arrhythmogenic substratum. During a 60-month follow-up period, high-dose amiodarone per os, and subsequent AICD therapy successfully treated recurrent VF episodes. This clinical case confirms the existence of a new idiopathic malignant arrhythmic syndrome. Aggressive antiarrhythmic therapy seems fundamental in this type of patients.
在本报告中,我们描述了一名患有罕见心律失常综合征的患者,该综合征最近被报道并以心脏骤停、右束支传导阻滞和胸前导联持续性ST段抬高为特征。患者为一名65岁男性,曾多次从室颤(VF)发作中存活下来。窦性心律时的心电图显示右束支传导阻滞与V1-V3导联持续性ST段抬高(1-4毫米)并存。包括超声心动图、心导管检查和右心内膜活检在内的临床和实验室检查均未发现任何明显的心脏形态和功能异常。晚期电位(高增益心电图)的存在以及电生理研究期间室颤的可诱导性提示功能性折返是心律失常的发生基质。在60个月的随访期内,口服高剂量胺碘酮以及随后的植入式心律转复除颤器(AICD)治疗成功地治疗了反复发作的室颤。该临床病例证实了一种新的特发性恶性心律失常综合征的存在。积极的抗心律失常治疗在这类患者中似乎至关重要。