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November 1966

Familial Occurrence of Primary Pulmonary Hypertension

Arch Intern Med. 1966;118(5):422-426. doi:10.1001/archinte.1966.00290170010003

A NUMBER of studies of primary pulmonary hypertension have suggested that this disease may occur in a familial form.1-8 In a previous communication from this laboratory, a family in which five members in three generations had primary pulmonary hypertension was described.9 It is the purpose of this report to describe a kindred in which primary pulmonary hypertension was present in two siblings and their father. Complete postmortem examinations were performed on the father and on one of the siblings.

Clinical Descriptions 

Case 1  —Patient 1 (No. 01-32-09) was referred to the National Heart Institute in 1956 at the age of 22 years with the chief complaint of progressive exertional dyspnea of two years' duration. He had immigrated to the United States at the age of 16 years, and at that time results of his physical examination was reported as being normal. One year prior to admission a chest