Biard F, Philippe C, Berrut G, Lardy B, Slama R
Service de Cardiologie, Centre Hospitalier Général, Le Mans.
Ann Cardiol Angeiol (Paris). 1988 Nov;37(9):529-34.
The authors present the case of a new case of Kearns-Sayre syndrome, with early manifestations (7 months) and dramatic cardiac course. They document the ultimate and often fatal stage of the cardiac disorders of this syndrome, which was usually related to an asystole due to a total atrio-ventricular block and represented in this case by a ventricular hyperexcitability (ventricular tachycardia--"torsade de pointes"--ventricular fibrillation) occurring on an atrio-ventricular block; only one previous, undocumented case was found in the literature. The literature is reviewed in order to analyze the nature and chronology of the heart disorders in the course of the disease, and stress the screening modalities and therapeutic indications (continuous heart stimulation).
作者报告了一例新的凯-赛综合征病例，该病例有早期表现（7个月）且心脏病程严重。他们记录了该综合征心脏疾病的最终且往往致命的阶段，这通常与完全性房室传导阻滞导致的心搏停止有关，在本病例中表现为在房室传导阻滞基础上出现的心室过度兴奋（室性心动过速——“尖端扭转型室速”——心室颤动）；文献中仅发现一例先前未记录的病例。对文献进行了综述，以分析疾病过程中心脏疾病的性质和时间顺序，并强调筛查方式和治疗指征（持续心脏刺激）。
