[Skip to Content]
[Skip to Content Landing]
Article
November 1966

Familial Occurrence of Primary Pulmonary Hypertension

Author Affiliations
EUGENE BRAUNWALD, MD, BETHESDA, MD
From the Cardiology Branch and the Clinic of Surgery, National Heart Institute, Bethesda.
Arch Intern Med. 1966;118(5):422-426. doi:10.1001/archinte.1966.00290170010003
Abstract

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

References
1.
Clarke, R.C., et al:  On Certain Abnormalities, Congenital and Acquired, of Pulmonary Artery ,  Quart J Med 21:51-69, 1927.Crossref
2.
Lange, F.:  Die essentielle Hypertonie der Lungenstrombahn und ihr familiäres Vorkommen ,  Deutsche Med Wschr 72:322-326, 1948.Crossref
3.
Dresdale, D.T.; Michtom, R.J.; and Schultz, M.:  Recent Studies in Primary Pulmonary Hypertension Including Pharmacodynamic Observations on Pulmonary Vascular Resistance ,  Bull NY Acad Med 30: 195-207, 1954.
4.
Van Epps, E.F.:  Primary Pulmonary Hypertension in Brothers ,  Amer J Roentgen 78:471-482, 1957.
5.
Coleman, P.N.; Edmunds, A.W.B.; and Tregillus, J.:  Primary Pulmonary Hypertension in Three Sibs ,  Brit Heart J 21:81-88, 1959.Crossref
6.
Fleming, H.:  Primary Pulmonary Hypertension in Eight Patients Including Mother and Her Daughter ,  Aust Ann Med 9:18-28, 1960.
7.
Cahen, P., et al:  Hypertension pulmonaire primitive chez la mère et al fille ,  Arch Mal Coeur 54: 95-101, 1961.
8.
Van Bogaert, A., et al:  Familial Primary Pulmonary Hypertension ,  Arch Mal Coeur 54:1185-1203, 1961.
9.
Melmon, K.L., and Braunwald, E.:  Familial Pulmonary Hypertension ,  New Eng J Med 269:770-775, 1963.Crossref
10.
Heath, D., and Edwards, J.E.:  The Pathology of Hypertensive Pulmonary Vascular Disease: A Description of Six Grades of Structural Changes in the Pulmonary Arteries With Special Reference to Congenital Cardiac Septal Defects ,  Circulation 18: 533-547, 1958.Crossref
11.
Boiteau, G.M., and Libanoff, A.J.:  Primary Pulmonary Hypertension: Familial Incidence ,  Angiology 14:260-264, 1963.Crossref
12.
Parry, W.R., and Verel, D.:  Familial Primary Pulmonary Hypertension ,  Brit Heart J 28:193-198, 1966.Crossref
13.
Rosenberg, S.A.:  A Study of the Etiological Basis of Primary Pulmonary Hypertension ,  Amer Heart J 68:484-489, 1964.Crossref
14.
Farrar, J.F.:  Idiopathic Pulmonary Hypertension ,  Amer Heart J 66:128-135, 1963.Crossref
15.
Wood, P.:  Diseases of the Heart and Circulation , ed 2, Philadelphia: J. B. Lippincott Company, 1956, p 843.
16.
Levine, S.A., and Harvey, W.P.:  Clinical Auscultation of the Heart , ed 2, Philadelphia: W. B. Saunders Company, 1959, p 516.
17.
McCusick, V.A.:  Cardiovascular Sound in Health and Disease , Baltimore: The Williams & Wilkins Company, 1958, p 430.
×