摘要
目的分析一家系心脏传导阻滞患者的临床与心电表现,探讨其可能的发病机制。方法对一心脏传导阻滞家系进行调查,并分析临床及体表心电图特征和His束电图。结果此家系62人,直接接受调查的35人中有18人发病,为常染色体显性遗传。表现为完全性右束支传导阻滞6例[其中1例演变为Ⅲ度房室传导阻滞(AVB)]、Ⅰ度AVB 5例(其中1例演变为Ⅲ度AVB,2例His束电图检查AH间期延长)、左后分支阻滞4例、左前分支阻滞1例、Ⅱ度窦房阻滞和窦性心动过缓各1例。家系中另有5例猝死。有3例40岁以上的患者(其ECG表现为心脏传导阻滞)以前未作检查,不知道何时患病,其余均在40岁之前出现心脏传导阻滞。2例超声心动图示心房扩大,其余患者超声心动图均正常。2例Ⅲ度AVB植入心脏起搏器治疗。结论该家系诊断Lenegre病。但7例心电图特征提示Lenegre病可能存在第三型,即最初发病部位在心房内传导系统,以后病变逐步沿心脏特殊传导系统向His束发展,并演变为Ⅲ度AVB。
Objective To analyze the clinical manifestation and electrocardiogram characteristics of one familial patients with heart conduction block and to discuss its potential mechanism. Methods The members of the family affected by conduction block were investigated. Their clinical manifestation, surface electrogram and His bundle electrogram were analyzed. Results The family had sixty-two members, eighteen affected individuals with autosomal dominant transmission were identified in thirty-five physical examined cases. There were six affected individuals with complete right bundle branch block（ one of them evolved into Ⅲ degree atrioventricular block） ,five with Ⅰ degree atrioventricular block（ one of them evolved into Ⅲ degree atrioventricular block, two cases with AH interval prolongation ）, four with left posterior fascicular block, one with left anterior fascicular block,three with P-wave broadening, Ⅱ degree sinoatrial block and sinus bradycardia respectively. Five were sudden death. Three cases more than 40 years old were not found to be heart block until ECG examination ,the rest heart block were identified before 40-year-old. Except that two case was enlarged atrial found by echocardiography, the remaining patients were normal. Two cases of Ⅲ degree atrioventricular block were treated by implantation of cardiac pacemakers. Conclusions The family is diagnosed as the familial inherited Lenegre＇s disease. According to the pedigree analysis,the surface ECG and His-bundle electrogram characteristics in 7 patients are not consistent with two classical types of Lenegre＇s disease,but it may be another type of Lenegre＇s disease. It also may be intra-atrial conduction system evolution of original lesion gradually along the special bundle conduction system into Ⅲ degree atrioventricular block.
出处
《中国心脏起搏与心电生理杂志》
北大核心
2009年第6期513-516,共4页
Chinese Journal of Cardiac Pacing and Electrophysiology
