Fibrosing mediastinitis causing pulmonary arterial hypertension without pulmonary venous hypertension

Clinical and necropsy observations
      This paper is only available as a PDF. To read, Please Download here.


      Clinical and morphologic observations are described in two patients with severe pulmonary arterial hypertension without pulmonary venous hypertension from fibrosing mediastinitis. In one patient, both main pulmonary arteries and one major pulmonary vein were severely narrowed by dense fibrous tissue; in the second patient, only the right main pulmonary artery was severely narrowed. Both patients had normal intrapulmonary arteries and normal pulmonary parenchyma. Of nine previously described necropsy patients with pulmonary hypertension due to fibrosing mediastinitis, seven had severe narrowing of multiple large pulmonary veins and in six of them the pulmonary hypertension was entirely due to pulmonary venous obstruction. In one other patient, the pulmonary hypertension was due to obstruction of one main pulmonary artery and several large pulmonary veins. Each of these seven previously described patients had severe changes in the small intrapulmonary arteries. Of the other two previously described patients with pulmonary hypertension from fibrosing mediastinitis, one had severe narrowing of only the main right pulmonary artery, and the other, of both main pulmonary arteries. Thus, although pulmonary arterial hypertension in patients with fibrosing mediastinitis is usually due to obstruction of multiple large pulmonary veins and to severe secondary changes in small intrapulmonary arteries, fibrosing mediastinitis can cause severe pulmonary hypertension by obstructing the right or both main pulmonary arteries.
      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


      Subscribe to The American Journal of Medicine
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


        • Knox LC
        Chronic mediastinitis.
        Am J Med Sci. 1925; 169: 807
        • Keefer CS
        Acute and chronic mediastinitis. A study of sixty cases.
        Arch Intern Med. 1938; 62: 109
        • Peabody Jr, JW
        • Brown RB
        • Sullivan MB
        • et al.
        Mediastinal granulomas.
        J Thorac Surg. 1958; 35: 384
        • Lull GF
        • Winn Jr, DF
        Chronic fibrous mediastinitis due to histoplasma capsulatum (histoplasmal mediastinitis). Report of three cases with different presenting symptoms.
        Radiology. 1959; 73: 367
        • Marshall RJ
        • Edmundowicz AC
        • Andrews CE
        Chronic obstruction of the superior vena cava due to histoplasmosis. A hemodynamic and angiographic correlation.
        Circulation. 1964; 29: 604
      1. Clinicopathologic conference (JHH 664434). Bull Johns Hopkins Hosp. 119. 1966: 288
        • Hewlett TH
        • Steer A
        • Thomas DE
        Progressive fibrosing mediastinitis.
        Ann Thorac Surg. 1966; 2: 345
        • Schowengerdt CG
        • Suyemoto R
        • Main FB
        Granulomatous and fibrous mediastinitis: a review and analysis of 180 cases.
        J Thorac Cardiovasc Surg. 1969; 57: 365
        • Goodwin RA
        • Nickell JA
        • Des Prez RM
        Mediastinal fibrosis complicating healed primary histoplasmosis and tuberculosis.
        Medicine (Baltimore). 1972; 51: 227
        • Strimlan CV
        • Dines DE
        • Payne WS
        Mediastinal granuloma.
        in: Mayo Clin Proc. 50. 1975: 702
        • Ferguson TB
        • Burford TH
        Mediastinal granuloma. A 15-year experience.
        Ann Thorac Surg. 1965; 1: 125
        • McIntire FT
        • Sykes EM
        Obstruction of the superior vena cava a review of the literature and report of two personal cases.
        Ann Intern Med. 1949; 30: 925
        • Carton RW
        • Wong R
        Multifocal fibrosclerosis manifested by vena caval obstruction and associated with vasculitis.
        Ann Intern Med. 1969; 70: 81
        • Erganian J
        • Wade LJ
        Chronic fibrous mediastinitis with obstruction of the superior vena cava.
        J Thorac Surg. 1943; 12: 275
        • Hache L
        • Woolner LB
        • Bernatz PE
        Idiopathic fibrous mediastinitis.
        Dis Chest. 1962; 41: 9
        • Miller RE
        • Sullivan FJ
        Superior vena caval obstruction secondary to fibrosing mediastinitis.
        Ann Thorac Surg. 1973; 15: 483
        • Garcia J
        • Ramariz R
        • Bacos J
        • et al.
        Technique of superior vena cava reconstruction in fibrosing mediastinitis.
        J Thorac Cardiovasc Surg. 1973; 65: 547
        • Edwards JE
        • Burchell HB
        Multilobar pulmonary venous obstruction with pulmonary hypertension. “Protective” arterial lesions in the involved lobes.
        Arch Intern Med. 1951; 87: 372
        • Davis Jr, FW
        • Andrus EC
        Mitral stenosis in facsimile.
        N Engl J Med. 1954; 251: 297
        • Bindelglass IL
        • Trubowitz S
        Pulmonary vein obstruction an uncommon sequel to chronic fibrous mediastinitis.
        Ann Intern Med. 1958; 48: 876
        • Nelson WP
        • Lundberg GD
        • Dickerson RB
        Pulmonary artery obstruction and cor pulmonale due to chronic fibrous mediastinitis.
        Am J Med. 1965; 38: 279
      2. Clinicopathologic conference (JHH 600747). Bull Johns Hopkins Hosp. 118. 1966: 73
        • Cheris DN
        • Dadey JL
        Fibrosing mediastinitis. An unusual cause for cor pulmonale.
        Am J Roentgenol Radium Ther Nucl Med. 1967; 100: 328
        • Nasser WK
        • Feigenbaum H
        • Fisch C
        Clinical and hemodynamic diagnosis of pulmonary venous obstruction due to sclerosing mediastinitis.
        Am J Cardiol. 1967; 20: 725
        • Kittredge RD
        • Nash AD
        The many facets of sclerosing fibrosis.
        Am J Roentgenol Radium Ther Nucl Med. 1974; 122: 288
        • Vogel JHK
        • McNamara DG
        • Hallman G
        • et al.
        Effects of mild chronic hypoxia on the pulmonary circulation in calves with reactive pulmonary hypertension.
        Circ Res. 1967; 21: 661
        • Cohn LH
        • Sanders Jr, JH
        • Collins Jr, JJ
        Surgical treatment of congenital unilateral pulmonary arterial stenosis with contralateral pulmonary hypertension.
        Am J Cardiol. 1976; 38: 257
        • Andrews EC
        Five cases of an undescribed form of pulmonary interstitial fibrosis caused by obstruction of the pulmonary veins.
        Bull Johns Hopkins Hosp. 1957; 100: 28
        • Fowler NO
        • Black-Schaffer B
        • Scott RC
        • et al.
        Idiopathic and thromboembolic pulmonary hypertension.
        Am J Med. 1966; 40: 331
        • Arnett EN
        • Battle WE
        • Russo JV
        Intravenous injection of talc-containing drugs intended for oral use. A cause of pulmonary granulomatosis and pulmonary hypertension.
        Am J Med. 1976; 60: 711
        • Osler W
        Obliteration of the superior vena cava.
        Bull Johns Hopkins Hosp. 1903; 14: 169