Familial Heart Block and Sick Sinus Syndrome of Adult Onset : Report of Two Families and Review of the Literature

概要

論文の詳細を見る
Two families presented here showed familial occurrence of both complete heart block and sick sinus syndrome of adult onset. In one family (A), a 40-year-old man had intermittent advanced infra-His block of 2 years duration and his uncle died of complete heart block which had lasted since he was 49 years of age, while his father suffered from sinus arrest (SA) and his mother (a cousin of his father) and 2 siblings had sinus bradycardia (SB) with first degree A-V block. In the other family (B), there were 8 affected members in 2 generations. They consisted of a 50-year-old woman with bradycardia-tachycardia syndrome, her cousin with complete A-V block and narrow QRS combined with SA and 6 other members with SB. One of the members with SB, a 26-year-old daughter, also had WPW syndrome with attacks of paroxysmal atrial fibrillation.In our review of the literature, 26 families with familial complete heart block (FCHB) of adult onset including 8 families associated with congenital FCHB and 12 families with familial sick sinus syndrome (FSSS) have been reported. Family A was similar to FCHB with SB and Family B was to FSSS. However, it is reasonable to designate these familial disorders as familial cardiac conduction disturbance (FCCD) because the affected region is not limited to a narrow area in the cardiac conduction system. Pathogenesis and clinical aspects of this familial disease were discussed.
International Heart Journal刊行会の論文