Isolated right ventricular hypoplasia is a rare congenital anomaly. This co
ndition is usually associated with a communication between the atria in the
form of a patent foramen ovale or secondum atrial septal defect. We descri
be a familial occurrence of this rare disease. A 1-day-old male child and h
is 34-year-old father were found to have isolated right ventricular hypopla
sia with atrial septal defect. An autosomal dominant mode of inheritance is
likely for this rare congenital anomaly, (C) 2000 Wiley-Liss, Inc.