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Communications to the Editor |

Hormone Replacement Therapy : A Possible Risk Factor in Carriers of Familial Primary Pulmonary Hypertension FREE TO VIEW

Jane H Morse, MD; Evelyn M Horn, MD; Robyn J Barst, MD
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Columbia University College of Physicians and Surgeon, New York, NY

Correspondence to: Jane H. Morse, MD, 630 West 168th St, PH 8 East-Suite 101, New York, NY 10032



Chest. 1999;116(3):847. doi:10.1378/chest.116.3.847
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To the Editor:

Pulmonary hypertension1and intimal vascular lesions2 have been associated with the use of oral contraceptives, alone or with other predisposing factors. A family history of pulmonary hypertension, congenital heart disease, collagen vascular disease, and the concomitant use of appetite suppressant drugs have all been reported as predisposing conditions for the development of pulmonary hypertension in women taking oral contraceptives.1 The familial form of primary pulmonary hypertension (PPH) has recently been linked to chromosome 2q31,32 using microsatellite markers followed by linkage analyses.34 Familial PPH (FPPH) is inherited as an autosomal-dominant disease with incomplete penetrance, but the gene, PPH1, has not yet been identified.

We report the onset of significant pulmonary hypertension in an obligate carrier of FPPH who took an estrogen-progesterone preparation for 6 months. This 64-year-old woman was known to be an obligate carrier: she has two daughters, two sisters, three nieces, a cousin, and three paternal aunts who have FPPH. Although this family also has male obligate carriers, pulmonary hypertension has been confined to women. Unknown to the patient’s cardiologist, hormone replacement therapy was prescribed as therapy for postmenopausal osteoporosis. As a part of serial monitoring for disease in an at-risk individual, a chest radiograph, an ECG, and an echocardiogram were performed and were interpreted as normal 8 months prior to hormone administration. Three months after starting hormone replacement therapy, the patient began complaining of exercise intolerance and dyspnea on exertion. A repeat chest radiograph, ECG, and echocardiogram were interpreted as consistent with pulmonary hypertension. The diagnosis of PPH was confirmed by cardiac catheterization.

This case report highlights the need to reexamine hormone use as a risk factor in those patients genetically at risk for FPPH and to avoid the use of predisposing agents in at-risk family members. The importance of these needs was highlighted by the recent summary of the World Health Organization World Symposium on Primary Pulmonary Hypertension,5which listed oral contraceptives and estrogens as “unlikely” risk factors for PPH. Genetic testing may identify family members who are at risk.6 This case report also provides support for alerting family members of their status as genetically at-risk for FPPH. This should reduce their exposure to factors that predispose them to the development of FPPH.

Kleiger, RE, Boxer, M, Ingham, RE, et al (1976) Pulmonary hypertension in patients using oral contraceptives.Chest69,143-147. [PubMed] [CrossRef]
 
Irey, N, Norris, H Intimal vascular lesions associated with female reproductive steroids.Arch Pathol1973;96,227-234. [PubMed]
 
Morse, JH, Jones, AC, Barst, RJ, et al Mapping of familial primary pulmonary hypertension locus (PPH1) to chromosome 2q31–32Circulation1997;95,2603-2606. [PubMed]
 
Nichols, WC, Koller, DL, Slovis, B, et al Localization of the gene for familial primary pulmonary hypertension to chromosome 2q31–32.Nat Genet1997;15,277-280. [PubMed]
 
World Health Organization. WHO World Symposium: Primary Pulmonary Hypertension 1998; summary. Available at: http://www.who.int/ncd/cvd/pph.html. Accessed: December 15, 1998.
 
Morse, JH, Barst, RJ Detection of familial primary pulmonary hypertension by genetic testing.N Engl J Med1997;337,202-203
 

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References

Kleiger, RE, Boxer, M, Ingham, RE, et al (1976) Pulmonary hypertension in patients using oral contraceptives.Chest69,143-147. [PubMed] [CrossRef]
 
Irey, N, Norris, H Intimal vascular lesions associated with female reproductive steroids.Arch Pathol1973;96,227-234. [PubMed]
 
Morse, JH, Jones, AC, Barst, RJ, et al Mapping of familial primary pulmonary hypertension locus (PPH1) to chromosome 2q31–32Circulation1997;95,2603-2606. [PubMed]
 
Nichols, WC, Koller, DL, Slovis, B, et al Localization of the gene for familial primary pulmonary hypertension to chromosome 2q31–32.Nat Genet1997;15,277-280. [PubMed]
 
World Health Organization. WHO World Symposium: Primary Pulmonary Hypertension 1998; summary. Available at: http://www.who.int/ncd/cvd/pph.html. Accessed: December 15, 1998.
 
Morse, JH, Barst, RJ Detection of familial primary pulmonary hypertension by genetic testing.N Engl J Med1997;337,202-203
 
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