12例家族性房室传导阻滞患者家系调查

A REPORT OF FAMILIAL ATRIOVENTRICULAR BLOCK IN 12 CASES

家族性完全性房室传导阻滞临床上较为少见,病情凶险,若不及时处理易致死亡。本文调查1家系4代76个成员中罹患本病者有12例。结果发现。(1)本家族具有遗传性连续传导,在连续4代中都有患者。在其同胞中,男女均有发病,可能属常染色体显性遗传。(2)致病原因为扩张型心肌病,其症状的个体差异较大,后代出现症状与死亡的年龄早于前代。

Twelve cases (7 males, 5 females) of atrioventricular(A-V) block were found in 76 members (4 generations) in a family. 3 patients died, among them 2 died suddenly, 1 died of refractory congestive heart failure. The survivors aged 34.5 years on the average. All had recurrent syncope with ECG of complete A-V block. Pacemakers were implanted in 4 patients, The survey revealed: (1) 12 cases occurred in 4 consecutive generations. Both male and female were affected. So the pedigree demonstrated autosomal dominant inheritance with direct transmission between generations. (2)Familial A-V block was probably caused by dilated cardiomyopathy. The severity and onset of A-V block showed individual variation. The average age of onset of symptoms of the coming generation was younger than that of the last generation, being 61, 52, 39 and 26 yrs, of the frist, second, third and fourth generation, respectively, so did the age of death. (3) Primary conduction abnormalities were likely the pathologic basis of familial entricular arrhythmias. (4) Pacemaker implantation is the only effective method for lifesaving of the patients.