Canadian Anaesthetists’ Society Journal

, Volume 26, Issue 4, pp 322–327

Anaesthetic considerations in idiopathic orthostatic hypotension and the shy-drager syndrome

  • M. D. Malan
  • R. R. Crago


Orthostatic hypotension due to autonomic failure may occur secondary to systemic disease states (notably diabetes) or as a disease entity in its own right with a variable degree of neurological involvement that has resulted in a confused classification. The diagnosis, classification and treatment of these latter forms of orthostatic hypotension is reviewed. The pathology is in the central and efferent autonomic pathway, resulting in a disordered baro-receptor reflex, postural hypotension, abnormal responses to tilting and the Valsalva manœuvre, an inappropriately fixed heart rate and other autonomic features. Anaesthesia may be associated with profound hypotension and some of the signs of anaesthesia may be absent. The response to cardiac depressant drugs and reduction of circulating blood volume may be exaggerated due to absence of compensatory mechanisms. The response to vasoactive agents is unpredictable. The importance of preoperative evaluation, monitoring during operation and the careful selection of anaesthetic agents and techniques is discussed.


La dysfonction du système nerveux autonome avec hypotension orthostatique peut être primaire avec une atteinte neurologique variable ou secondaire à une atteinte systémique (le diabète est souvent en cause). Cet article présente la classification, le diagnostic et le traitement de la forme primaire. La pathologie du SNA se situe au niveau central et efférent avec atteinte des réflexes barorécepteurs et hypotension posturale, une réponse anormale aux manœuvres de Valsalva et de “tilting”, une fréquence cardiaque fixe et ďautres manifestations neuro-végétatives. Des épisodes ďhypotension grave sont susceptibles de se produire sous anesthésie et certains signes ďanesthésie peuvent être absents. Les effets des agents dépresseurs du myocarde et ceux de l'hypovolémie peuvent être exagérés par absence des mécanismes compensateurs. Les effets des vasopresseurs peuvent être imprédicti bles. Ľévaluation pré-opératoire, le monitoring adéquat et le choix judicieux des agents et des techniques ďanesthésie sont des aspects importants chez ces malades.


  1. 1.
    Cohen, C. A. Anaesthetic management of a patient with the Shy-Dragersyndrome. Anesthesiology35: 95–97 (1970).CrossRefGoogle Scholar
  2. 2.
    Katz, J. &Kadis, L.B. Anaesthesia and uncommondiseases. First edition. Philadelphia: Saunders (1973). p. 400.Google Scholar
  3. 3.
    Shy, C.M. &Drager, G.A. A neurologic syndrome associated with orthostatic hypotension. Arch. Neurol.2: 511–527 (I960).Google Scholar
  4. 4.
    Bannister, R. &Oppenhejmer, D.R. Degenerative diseases of the nervous system associated with autonomic failure. Brain95: 457–474 (1972).PubMedCrossRefGoogle Scholar
  5. 5.
    Bannister, R., Davies. B. &Sever, P. Indomethacin for Shy-Drager syndrome. Lancet1: 1312 (1978).CrossRefGoogle Scholar
  6. 6.
    Davies, B., Bannister, R. &Sever, P. Pressor amines and monoamine oxidase inhibitors for treatment of postural hypotension in autonomic failure. Lancet1: 172–175 (1978).PubMedCrossRefGoogle Scholar
  7. 7.
    Zieoler, M.G., Lake, C.R. &Kopin, I.J. The sympathetic-nervous-system defect in primary orthostatic hypotension. N. Engl. J. Med.296: 293–297 (1977).Google Scholar
  8. 8.
    Plum, F. inBeeson, P.B. &McDermott. W. Textbook of Medicine, 14th ed. Philadelphia: Saunders, p. 645(1975).Google Scholar
  9. 9.
    Bannister, R. Degeneration of the autonomic nervous system. Lancet2: 175–179 (1971).PubMedCrossRefGoogle Scholar
  10. 10.
    Meridy, H.W. &Crecghton. R.E. General anaesthesia in eight patients with familial dysautonomia. Can. Anaesth. Soc. J.18: 563 (1971).PubMedCrossRefGoogle Scholar
  11. 11.
    Bannister, R., Sever, P. &Gross, M. Cardiovascular reflexes and biochemical responses in progressive autonomic failure. Brain100: 327–344 (1977).PubMedCrossRefGoogle Scholar
  12. 12.
    Kontos, H.A., Richardson, D.W. &Norvell, J.E. Norepinephrine depletion in idiopathic orthostatic hypotension. Ann. Intern. Med.82: 336–341 (1975).PubMedGoogle Scholar
  13. 13.
    —— Mechanisms of circulatory dysfunction in orthostatic hypotension. Trans. Am. Clin. Climatol. Assoc.87: 26–35 (1975).Google Scholar
  14. 14.
    Ibrahim, M.M. Localization of lesion in patients with idiopathic orthostatic hypotension. Br. Heart J.37: 868–872 (1975).PubMedCrossRefGoogle Scholar
  15. 15.
    Diamond, M.A., Murray, R.H. &Schmid, P.G. Idiopathic postural hypotension: physiologic observations and report of a new mode of therapy. J. Clin. Invest.49: 1341–48 (1970).PubMedCrossRefGoogle Scholar
  16. 16.
    Sharpe, J., Marquez-Julio, A. &Ashby, P. Idiopathic orthostatic hypotension treated with levodopa and MAO inhibitor: a preliminary report. Can. Med. Assoc. J.107: 296–300 (1972).PubMedGoogle Scholar
  17. 17.
    Kochar, M.S. &Itskovitz, H.D. Treatment of idiopathic orthostatic hypotension (Shy-Drager syndrome) with indomethacin. Lancet1: 1011 (1978).PubMedCrossRefGoogle Scholar
  18. 18.
    Thomas, J.E. &Schirger, A. Idiopathic orthostatic hypotension. Arch. Neurol.22: 289–293 (1970).PubMedGoogle Scholar
  19. 19.
    Seller, R.H. Idiopathic orthostatic hypotension: a report of successful treatment with a new form of therapy. Am. J. Cardiol.23: 838–844 (1969).PubMedCrossRefGoogle Scholar
  20. 20.
    Gullner, H.G. Indomethacin for Shy-Drager syndrome. Lancet1: 1312 (1978).Google Scholar
  21. 21.
    Vickers, M.D.,Wood-Smith, F.G. &Stewart, H.C. Drugs in Anaesthetic Practice, 5th ed. But- terworihs(1978).Google Scholar
  22. 22.
    Lehrman, K.L., Guilleminault, C., Schroe-der, J.S., Tilkian, A. &Forno, L.N. Sleep apnoea syndrome in a patient with Shy-Drager syndrome. Arch. Intern. Med.138: 206–209 (1978),PubMedCrossRefGoogle Scholar

Copyright information

© Canadian Anesthesiologists 1979

Authors and Affiliations

  • M. D. Malan
    • 1
  • R. R. Crago
    • 2
  1. 1.Department of AnaesthesiaUniversity of Toronto Toronto Western HospitalToronto
  2. 2.Department of AnaesthesiaUniversity of Toronto, Toronto Western HospitalTorontoCanada

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