扩张型心肌病合并心房静止与传导阻滞一家系

Familial dilated cardiomyopathy associated with atrial standstill and conduction block.

目的分析一家系扩张型心肌病(DCM)合并心房静止与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DCM合并心房静止与传导阻滞家系进行调查,并分析体表心电图及食管和心腔内电图。结果该家系3例患病(先证者,以及其姐和其兄),其母50岁猝死。2例表现为全心房静止,1例为部分心房静止,并合并左前分支和不完全性右束支阻滞,超声心动图示心房无收缩、房室扩大。2例因心房不能起搏,改为置入VVI起搏器,1例直接行VVI起搏。结论该家系符合遗传性DCM合并传导系统疾病的诊断。

Objective To analyze the clinical manifestation and electrocardiogram characteristics of the family patients with dilated cardiomyopathy （DCM） associated with atrial standstill and conduction block and discuss it＇s potential mecha- nism. Methods The members of the family affected by DCM associated with atrial standstill and conduction block were investigated. Their surface electrocardiogram, and transesophageal electrocardiogram and endocardial electrogram were ana- lyzed. Results Three affected individuals were identified, the proband, and his elder sister and elder brother. Their mother died suddenly at 50 year-old. There were two affected individuals with complete atrial standstill and one with partial atrial standstill, and associated with left anterior hemiblock and incomplete right bundle branch block. They showed biatrial and biventricular dilation and no atrial contraction on echocardiography. The right atrium could not be captured by pacing in two patients. A patient with VVI pacemaker had no P waves or f waves on surface electrocardiogram. Conclusions The clinical characteristics of the patients were consistent with the criteria for diagnosis of familial inherited DCM associated with conduction abnormalities. [ Chinese Journal of Cardiac Pacing and Electrophysiology ,2009,23 （1） ：34 -36 ]