Abstract

Primary pulmonary hypertension occurring in a 21-year-old woman, her father, and her father's sister is documented. The diagnosis was established by cardiac catheterization in all of the patients. Pulmonary arteriography was done in two cases and a postmortem examination in one. Origin of the clinical syndrome is discussed, and suggestions are made regarding its possible development as a congenital defect resulting in the absence of the normal transitional phase from a fetal pulmonary vascular bed.