Two siblings with sinus node disease are presented. The patients were
severely affected and required permanent pacing. A cardiac electrophys
iologic study was conducted in Case 2, which revealed an atrioventricu
lar conduction disturbance in addition to sinus node dysfunction. The
parents and other siblings showed no evidence of sinus node disorder.
The occurrence of this disease in these siblings suggests an autosomal
dominant mode of inheritance with variable penetrance.