Abstract PRIMARY pulmonary hypertension is an unusual disease of unknown etiology. An accurate diagnosis of this condition is now possible from the data obtained by catheterization of the right side of the heart. Wood found the characteristic unexplained pathologic changes in the pulmonary arterioles of only 17 of 10,000 patients examined postmortem.1 Although a familial incidence has already been emphasized by others,2-12 the purpose of this report is to call attention to the disease and to describe the autopsy findings in female twins. Report of Cases Case 1. —A 29-year-old white woman (403674) had been in good health until October 1964, when four months following her fourth uncomplicated pregnancy and delivery, she developed exertional dyspnea, slight dependent edema, and visual scotomata with activity. In February 1965, she was admitted to Hamilton Air Force Base Hospital and was soon transferred to Lettermen General Hospital, San Francisco. Cardiac catheterization studies were References 1. Wood, P.H.: " Primary Pulmonary Hypertension ," in Diseases of the Heart and Circulation , ed 2, Philadelphia: J. P. Lippincott Co., 1956, p 839. 2. Clarke, R.C., et al: On Certain Abnormalities, Congenital and Acquired of the Pulmonary Artery , Quart J Med 21:51-70, 1927-28.Crossref 3. Dresdale, D.T.; Michtom, R.J.; and Schulty, 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 Roentgenol 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 a Mother and Her Daughter , Aust Ann Med 9:18-28, 1960. 7. Van Bogaert, A., et al: Hypertension Pulmonaire primitive familiale , Arch Mal Coeur 54: 1185-1203, 1961. 8. Cahen, P., et al: Hypertension Pulmonaire primitive chez la mere et la fille , Arch Mal Coeur 54:95-101, 1961. 9. Melmon, K.L., and Braunwald, E.: Familial Pulmonary Hypertension , New Eng J Med 26: 770-775, 1963.Crossref 10. Boiteau, G.M., and Libanoff, J.A.: Primary Pulmonary Hypertension: Familial Incidence , Angiology 14:260-264, 1963.Crossref 11. Parry, W.R., and Verel, D.: Familial Pulmonary Hypertension , Brit Heart J 28:193-198, 1966.Crossref 12. Rogge, J.D.; Mishkin, M.E.; and Pasquale, D.G.: The Familial Occurrence of Primary Pulmonary Hypertension , Ann Intern Med 65:672, 1966.Crossref 13. Sleeper, J.; Orgain, E.S.; and McIntosh, H.D.: Primary Pulmonary Hypertension , Circulation 26: 1358-1369, 1962.Crossref 14. Kuida, H., et al: Primary Pulmonary Hypertension , Amer J Med 23:166-182, 1957.Crossref 15. Yu, P.N.: Primary Pulmonary Hypertension , Ann Intern Med 49:1138-1161, 1958.Crossref 16. Gedda, L.: " Anatomical Studies in Twins ," in Twins in History and Science , ed 1, Springfield, Ill: Charles C Thomas, Publisher, 1961. pp 155-214. 17. Shepherd, J.T., et al: Clinical, Physiological and Pathological Considerations in Patients With Idiopathic Pulmonary Hypertension , Brit Heart J 19:70-82, 1957.Crossref 18. Evans, W.; Short, D.S.; and Bedford, D.E.: Solitary Pulmonary Hypertension , Brit Heart J 19:93-116, 1957.Crossref 19. Burwell, C.S., and Metcalfe, J.: " Primary Pulmonary Hypertension ," in Heart Disease and Pregnancy, Physiology and Management , ed 1, Boston: Little, Brown & Co., 1958, p 310.
Archives of Internal Medicine – American Medical Association
Published: Aug 1, 1967