Discrete subaortic stenosis (DSS) accounts for 8 to 20% of all cases o
f congenital left ventricular outflow tract obstruction. There have be
en few scattered reports of left ventricular obstructive lesions occur
ring in immediate family members of patients with DSS. This is a repor
t of four cases of DSS in one family: one brother with a fibrous ring,
and two sisters and the son of one of the sisters with a fibrous memb
rane. The occurrence of multiple cases of DSS in this family suggests
an autosomal dominant mode of inheritance.