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February 27, 1991, Vol 265, No. 8 >
ARTICLE | February 27, 1991

The Management of Primary Pulmonary Hypertension FREE

Harold I. Palevsky, MD; Alfred P. Fishman, MD
[+] Author Affiliations

This article is one of a series sponsored by the American Heart Association.

Reprint requests to Pulmonary and Critical Care Section, University of Pennsylvania Medical Center, 975 Maloney Bldg, 3600 Spruce St, Philadelphia, PA 19104-4283 (Dr Palevsky).


JAMA. 1991;265(8):1014-1020. doi:10.1001/jama.1991.03460080084038
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References

Primary pulmonary hypertension is a clinical syndrome characterized by pulmonary hypertension in the absence of sufficient underlying cardiac, parenchymal pulmonary, or systemic disease to account for it. The population of patients with primary pulmonary hypertension is a heterogeneous one, both clinically and histologically. As the etiologic mechanisms are unknown, therapy is directed toward the consequences of the pulmonary vascular process. Oxygen supplementation, the use of digoxin and diuretics for symptomatic heart failure, and anticoagulation all may have a role in treating primary pulmonary hypertension, although vasodilator therapy has been the main area of investigation. Screening for vasodilator responsiveness, defining a favorable vasodilator effect, predicting long-term effectiveness, and deciding who to treat have all been controversial. New approaches, such as use of high-dose calcium channel-blocking agents and continuous intravenous infusion of prostacyclin (an investigational agent), have recently been proposed. When medical therapies are exhausted, heart-lung or lung transplantation has increasingly become an option for selected patients.

(JAMA. 1991;265:1014-1020)

REFERENCES

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2 +
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3 +
Fishman AP, Pietra GG.  Primary pulmonary hypertension . Annu Rev Med. 1980;;31:421-431.
4 +
Weir EK.  Diagnosis and management of primary pulmonary hypertension . In: Weir EK, Reeves JT, eds. Pulmonary Hypertension . Mount Kisco, NY: Futura Publishing Co Inc; 1984;:115-168.
5 +
Hughes JD, Rubin LJ.  Primary pulmonary hypertension: an analysis of 28 cases and a review of the literature . Medicine. 1986;;65:56-72.
6 +
Rich S.  Primary pulmonary hypertension . Prog Cardiovasc Dis. 1988;;31:205-238.
7 +
Palevsky HI, Schloo BL, Pietra GG, et al.  Primary pulmonary hypertension: vascular structure, morphometry and responsiveness to vasodilator agents . Circulation. 1989;;80:1207-1221.
8 +
Rich S, Kieras K, Hart K, Groves BM, Stobo JD, Brundage BH.  Antinuclear antibodies in primary pulmonary hypertension . J Am Coll Cardiol. 1986;;8:1307-1311.
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Stupi AM, Steen VD, Owens GD, Barnes EL, Rodnan GP, Medsger TA Jr.  Pulmonary hypertension in CREST syndrome variant of systemic sclerosis (scleroderma) . Arthritis Rheum. 1986;;29: 515-524.
10 +
Fahey PJ, Utell MJ, Condemi JJ, Green R, Hyde RW.  Raynaud's phenomenon of the lung . Am J Med. 1984;;76:263-269.
11 +
Gurtner HP.  Aminorex pulmonary hypertension . In: Fishman AP, ed. The Pulmonary Circulation: Normal and Abnormal: Mechanisms, Management and the National Registry , Philadelphia, Pa: University of Pennsylvania Press; 1990;: 397-411.
12 +
Fishman AP.  Dietary pulmonary hypertension . Circ Res. 1974;;35:657-660.
13 +
Gomez-Sanchez MA, Mestre de Juan MJ, Gomez-Pajuelo C, Lopez JI, Diaz de Atauri MJ, Martinez-Tello FJ.  Pulmonary hypertension due to toxic oil syndrome: a clinicopathologic study . Chest. 1989;;95:325-331.
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Country-Specific Mortality and Growth Failure in Infancy and Yound Children and Association With Material Stature

Use interactive graphics and maps to view and sort country-specific infant and early dhildhood mortality and growth failure data and their association with maternal

1 +
Natano S, Strasser T, eds. Primary Pulmonary Hypertension: Report on a WHO Meeting . Geneva, Switzerland: World Health Organization; 1975;.
2 +
Voelkel NF, Reeves JT.  Primary pulmonary hypertension . In: Moser KM, ed. Pulmonary Vascular Disease . New York, NY: Marcel Dekker Inc; 1979:573-628.
3 +
Fishman AP, Pietra GG.  Primary pulmonary hypertension . Annu Rev Med. 1980;;31:421-431.
4 +
Weir EK.  Diagnosis and management of primary pulmonary hypertension . In: Weir EK, Reeves JT, eds. Pulmonary Hypertension . Mount Kisco, NY: Futura Publishing Co Inc; 1984;:115-168.
5 +
Hughes JD, Rubin LJ.  Primary pulmonary hypertension: an analysis of 28 cases and a review of the literature . Medicine. 1986;;65:56-72.
6 +
Rich S.  Primary pulmonary hypertension . Prog Cardiovasc Dis. 1988;;31:205-238.
7 +
Palevsky HI, Schloo BL, Pietra GG, et al.  Primary pulmonary hypertension: vascular structure, morphometry and responsiveness to vasodilator agents . Circulation. 1989;;80:1207-1221.
8 +
Rich S, Kieras K, Hart K, Groves BM, Stobo JD, Brundage BH.  Antinuclear antibodies in primary pulmonary hypertension . J Am Coll Cardiol. 1986;;8:1307-1311.
9 +
Stupi AM, Steen VD, Owens GD, Barnes EL, Rodnan GP, Medsger TA Jr.  Pulmonary hypertension in CREST syndrome variant of systemic sclerosis (scleroderma) . Arthritis Rheum. 1986;;29: 515-524.
10 +
Fahey PJ, Utell MJ, Condemi JJ, Green R, Hyde RW.  Raynaud's phenomenon of the lung . Am J Med. 1984;;76:263-269.
11 +
Gurtner HP.  Aminorex pulmonary hypertension . In: Fishman AP, ed. The Pulmonary Circulation: Normal and Abnormal: Mechanisms, Management and the National Registry , Philadelphia, Pa: University of Pennsylvania Press; 1990;: 397-411.
12 +
Fishman AP.  Dietary pulmonary hypertension . Circ Res. 1974;;35:657-660.
13 +
Gomez-Sanchez MA, Mestre de Juan MJ, Gomez-Pajuelo C, Lopez JI, Diaz de Atauri MJ, Martinez-Tello FJ.  Pulmonary hypertension due to toxic oil syndrome: a clinicopathologic study . Chest. 1989;;95:325-331.
14 +
Lopez-Sendon J, Gomez-Sanchez MA, Mestre de Juan MJ, Coma-Camella I.  Pulmonary hypertension in the toxic oil syndrome . In: Fishman AP, ed. The Pulmonary Circulation: Normal and Abnormal: Mechanisms, Management and the National Registry . Philadelphia, Pa: University of Pennsylvania Press; 1990;:385-395.
15 +
Fahlen M, Bergman H, Helder G, Ryden L, Wallentin I.  Zettergren L. Phenformin and pulmonary hypertension . Br Heart J. 1973;;35:824-828.
16 +
Douglas JG, Munro JF, Kitchen AH, Muir AL, Proudfoot AT.  Pulmonary hypertension and fenfluramine . BMJ. 1981;;283:881-883.
17 +
Miczoch J.  Drug and dietary induced pulmonary hypertension . In: Weir EK, Reeves JT, eds. Pulmonary Hypertension . Mount Kisco, NY: Futura Publishing Co Inc; 1984;:341-359.
18 +
Kay JM, Heath D. Crotalaria Spectabilis: Pulmonary Hypertension Plant . Springfield, Ill: Charles C Thomas Publisher Inc; 1969;.
19 +
Senior RM, Britton RC, Turino GM, Wood JA, Langer GA, Fishman AP.  Pulmonary hypertension associated with cirrhosis of the liver and with portacaval shunts . Circulation. 1968;;37:88-96.
20 +
McDonnell PF, Toye PA, Hutchins GM.  Primary pulmonary hypertension and cirrhosis: are they related? Am Rev Respir Dis. 1983;;127:437-441.
21 +
Edwards BS, Weir EK, Edwards WD, Ludwig J, Dykoski RK, Edwards JE.  Coexistent pulmonary and portal hypertension: morphologic and clinical features . J Am Coll Cardiol. 1987;; 10:1233-1238.
22 +
Rich S, Dantzker DR, Ayres SM, et al.  Primary pulmonary hypertension: a national prospective study . Ann Intern Med. 1987;;107:216-223.
23 +
Dawkins KD, Burke CM, Billingham ME, Jamieson SW.  Primary pulmonary hypertension and pregnancy . Chest. 1986;;89:383-388.
24 +
Elkayam U, Gleicher N.  Primary pulmonary hypertension in pregnancy . In: Elkayam U, Gleicher N, eds. Cardiac Problems in Pregnancy: Diagnosis and Management of Maternal and Fetal Disease . New York, NY: Alan R Liss Inc; 1982;: 153-160.
25 +
Oakley C, Somerville J.  Oral contraceptives and progressive pulmonary vascular disease . Lancet. 1968;;1:890-893.
26 +
Kleiger RE, Boxer M, Ingham RE, Harrison DC.  Pulmonary hypertension in patients using oral contraceptives: a report using six cases . Chest. 1976;;69:143-147.
27 +
Palevsky HI.  Pulmonary hypertension and thromboembolic disease in women . Cardiol Clin. 1989;;19:267-283.
28 +
Wagenvoort CA, Wagenvoort N.  Pulmonary vascular bed: normal anatomy and response to disease . In: Moser KM, ed. Pulmonary Vascular Diseases . New York, NY: Marcel Dekker Inc; 1979;: 1-109.
29 +
Edwards WD, Edwards JE.  Clinical primary pulmonary hypertension: three pathologic types . Circulation. 1977;;56:884-888.
30 +
Wagenvoort CA.  Lung biopsies and pulmonary vascular disease . In: Weir EK, Reeves JT, eds. Pulmonary Hypertension . Mount Kisco, NY: Futura Publishing Co Inc; 1984;:393-437.
31 +
Bjornsson J, Edwards WD.  Primary pulmonary hypertension: a histopathologic study of 80 cases . Mayo Clin Proc. 1985;;60:16-25.
32 +
Pietra GG, Edwards WD, Kay JM, et al.  Histopathology of primary pulmonary hypertension: a qualitative and quantitative study of pulmonary blood vessels from 58 patients in the National Heart, Lung and Blood Institute Primary Pulmonary Hypertension Registry . Circulation. 1989;; 60:1198-1206.
33 +
Pietra GG.  The histopathology of primary pulmonary hypertension . In: Fishman AP, ed. The Pulmonary Circulation: Normal and Abnormal: Mechanisms, Management and the National Registry . Philadelphia, Pa: University of Pennsylvania Press; 1990;:459-472.
34 +
Loyd JE, Atkinson JB, Pietra GG, Virmani R, Newman JH.  Heterogeneity of pathologic lesions in familial primary pulmonary hypertension . Am Rev Respir Dis. 1988;;138:952-957.
35 +
Newman JH, Loyd JE.  Familial pulmonary hypertension . In: Fishman AP, ed. The Pulmonary Circulation: Normal and Abnormal: Mechanisms, Management and the National Registry . Philadelphia, Pa: University of Pennsylvania Press; 1990;:301-313.
36 +
Palevsky HI, Fishman AP.  Chronic cor pulmonale: etiology and management . JAMA. 1990;; 263:2347-2353.
37 +
Fishman AP.  Pulmonary circulation . In: Fishman AP, Fisher A, eds. Handbook of Physiology: The Respiratory System: Circulation and Nonrespiratory Functions , Bethesda, Md: American Physiological Society. 1985;;3:93-165.
38 +
Palevsky HI.  Exercise and the pulmonary circulation . In: Leff A, ed. Cardiopulmonary Exercise Testing . New York, NY: Grune & Stratton; 1986;:89-106.
39 +
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