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[成人家族性特发性双结区阻滞]

[Adult familial idiopathic binodal block].

作者信息

Fauchier J P, Latour F, Charbonnier B, Brochier M

出版信息

Arch Mal Coeur Vaiss. 1979 Oct;72(10):1059-68.

PMID:120710
Abstract

Four brothers with a maximum age difference of 20 years, the eldest of whom has been re-examined after a 10 year interval, have sinoatrial block, a supra-hisian atrioventricular block and paroxysmal atrial arrhythmias which have led to partial atrial standstill in the eldest: left anterior hemiblock is also present in the two youngest brothers. The condition is very well tolerated. This family is compared to the other 12 reported cases of familial idiopathic binodal block in the adult, an autosomal dominant condition with variable penetration. The diagnosis is reserved and justifies endocavitary investigation of the sinus node function and atrioventricular conduction in the four brothers and the most exposed members of their family. The mechanism of the condition is unknown. It seems to arise from variable degrees of nonspecific of the nodal and atrial tissues.

摘要

四兄弟年龄最大相差20岁,其中最年长者在间隔10年后接受了复查,他们均患有窦房阻滞、希氏束上房室阻滞以及阵发性房性心律失常,最年长者还出现了部分心房停搏:最年幼的两兄弟还伴有左前分支阻滞。病情耐受性良好。将这个家族与另外12例已报道的成人家族性特发性双结阻滞病例进行比较,后者为常染色体显性疾病,具有可变的外显率。诊断存疑,有必要对四兄弟及其家族中暴露风险最高的成员进行窦房结功能及房室传导的心腔内检查。该病的发病机制尚不清楚。似乎是由结区和心房组织不同程度的非特异性引起的。

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