Skip to main content
Log in

Drug Treatment of Primary Pulmonary Hypertension

  • Practical Therapeutics
  • Published:
Drugs Aims and scope Submit manuscript

Summary

Primary pulmonary hypertension is a rare but difflcult-to-manage condition. Adequate clinical trials of agents used in its treatment have not been performed. A variety of drugs have been reported to provide benefit in patients with primary pulmonary hypertension, including β-adrenoceptor agonists, α-adrenoceptor antagonists, and vasodilators such as isosorbide dinitrate, diazoxide, hydralazine, angiotensin converting enzyme inhibitors, prostaglandins and calcium antagonists. Calcium antagonists appear to offer the most promise, although treatment failures have occurred with them as well as with all other drugs used in this condition. In the absence of a specific treatment for primary pulmonary hypertension, several of the agents listed above should be tried before accepting therapeutic failure. Invasive investigation is necessary to adequately monitor the acute response to therapy. Whether combination therapy with 2 or more drugs might improve the response to treatment is an area worthy of further research.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References

  • Bedford DE. Pulmonary heart disease. Proceedings of the Royal Society of Medicine 44: 597–600, 1951

    PubMed  CAS  Google Scholar 

  • Berkenboom G, Sobolski J, Stoupel E. Failure of nifedipine treatment in primary pulmonary hypertension. British Heart Journal 47: 511, 1982

    Article  PubMed  CAS  Google Scholar 

  • Berkov S. Hypoxic pulmonary vasoconstriction in the rat. The necessary role of angiotensin II. Circulation Research 35: 256–261, 1974

    Article  CAS  Google Scholar 

  • Bourdillon PDV, Oakley CM. Regression of primary pulmonary hypertension. British Heart Journal 38: 264–270, 1976

    Article  PubMed  CAS  Google Scholar 

  • Brooks N, Cattel M, Pidgeon J, Balcon R. Unpredictable response to nifedipine in severe cardiac failure. British Medical Journal 280: 1420, 1980

    Article  Google Scholar 

  • Camerini F, Albert E, Klugmann S, Salvi A. Primary pulmonary hypertension: Effects of nifedipine. British Heart Journal 46: 231, 1981

    Google Scholar 

  • Crevey BJ, Dantzker DR, Bower JS, Popat KD, Walker SD. Hemodynamic and gas exchange effects of intravenous diltiazem in patients with pulmonary hypertension. American Journal of Cardiology 49: 578–583, 1982

    Article  PubMed  CAS  Google Scholar 

  • Dalai JJ, Griffiths BE, Henderson AH. Primary pulmonary hypertension: Effects of nifedipine. British Heart Journal 46: 230–231, 1981

    Article  Google Scholar 

  • D’Alonzo GE, Bower JS, Dantzker DR. Differentiation of patients with primary and thromboembolic pulmonary hypertension. Chest 85: 457–461, 1984

    Article  PubMed  Google Scholar 

  • Daoud FS, Reeves JT, Kelly DB. Isoproterenol as a potential pulmonary vasodilator in primary pulmonary hypertension. American Heart Journal of Cardiology 42: 817–822, 1978

    Article  CAS  Google Scholar 

  • Daum S, Goerg R, Schlehe H. Isosorbiddinitrat (ISDN) in der therapie der primaren pulmonalen hypertonic Praxis und Klinik der Pneumologie 37: 67–70, 1983

    PubMed  CAS  Google Scholar 

  • De Feyter PJ, Kerkkamp HJJ, de Jong JP. Sustained beneficial effect of nifedipine in primary pulmonary hypertension. American Heart Journal 105: 333–334, 1983

    Article  PubMed  Google Scholar 

  • Douglas JS. Hemodynamic effects of nifedipine in primary pulmonary hypertension. Journal of the American College of Cardiology 2: 174–179, 1983

    Article  PubMed  Google Scholar 

  • Dresdale DT, Schultz M, Michtom RJ. Primary pulmonary hypertension. I. Clinical and hemodynamic study. American Journal of Medicine 11: 686–705, 1951

    Article  PubMed  CAS  Google Scholar 

  • Fujii A, Rabinovitch M, Matthews EC. A case of spontaneous resolution of idiopathic pulmonary hypertension. British Heart Journal 46: 574–577, 1981

    Article  PubMed  CAS  Google Scholar 

  • Gardiner JM. The effect of ‘priscol’ in pulmonary hypertension. Australian Annals of Medicine 3: 59, 1954

    CAS  Google Scholar 

  • Harris P, Heath D. The Human Pulmonary Circulation: Its Form and Function in Health and Disease, 2nd ed., Churchill Livingstone, London, 1977

    Google Scholar 

  • Hermiller JB, Bambach D, Thompson MJ, Huss P, Fontana ME, et al. Vasodilators and prostaglandin inhibitors in primary pulmonary hypertension. Annals of Internal Medicine 97: 480–489, 1982

    PubMed  CAS  Google Scholar 

  • Ikram H, Maslowski AH, Nicholls MG, Espiner EA, Hull FTL. Haemodynamic and hormonal effects of Captopril in primary pulmonary hypertension. British Heart Journal 48: 541–545, 1982

    Article  PubMed  CAS  Google Scholar 

  • James T. Thrombi in antrium atrii dextri of human heart as clinically important source for chronic microembolization to lungs. British Heart Journal 49: 122–132, 1983

    Article  PubMed  CAS  Google Scholar 

  • Kambara H, Fujimoto K, Wakabayashi A, Kawai C. Primary pulmonary hypertension: Beneficial therapy of diltiazem. American Heart Journal 101: 230–231, 1981

    Article  PubMed  CAS  Google Scholar 

  • Klinke WP, Gilbert JAL. Diazoxide in primary pulmonary hypertension. New England Journal of Medicine 302: 91–92, 1980

    Article  PubMed  CAS  Google Scholar 

  • Kronzon I, Cohen M, Winer HE. Adverse effect of hydralazine in patients with primary pulmonary hypertension. Journal of the American Medical Association 247: 3112–3114, 1982

    Article  PubMed  CAS  Google Scholar 

  • Landmark K, Refsum AM, Simonsen S, Storstein O. Verapamil and pulmonary hypertension. Acta Medica Scandinavica 204: 299–302, 1978

    Article  PubMed  CAS  Google Scholar 

  • Lee TD, Roveti GC, Ross RS. The haemodynamic effects of isoproterenol on pulmonary hypertension in man. American Heart Journal 65: 361–367, 1963

    Article  PubMed  Google Scholar 

  • Leier CV, Bambach D, Nelson S, Hermiller JB, Huss P, et al. Captopril in primary pulmonary hypertension. Circulation 67: 155–161, 1983

    Article  PubMed  CAS  Google Scholar 

  • McLeod AA, Wise Jr JR, Daly K, Jewitt DE. Nifedipine in primary and secondary pulmonary hypertension. Circulation 64(Suppl. IV): 80, 1981

    Google Scholar 

  • Oakley CM. Management of primary pulmonary hypertension. British Heart Journal 53: 1–4, 1985

    Article  PubMed  CAS  Google Scholar 

  • Packer M, Greenberg B, Massie B, Dash H. Deleterious effects of hydralazine in patients with pulmonary hypertension. New England Journal of Medicine 306: 1326–1331, 1982

    Article  PubMed  CAS  Google Scholar 

  • Pietro DA, LaBresh KA, Shulman RM, Folland ED, Parisi AF, et al. Sustained improvement in primary pulmonary hypertension during six years of treatment with sublingual isoproterenol. New England Journal of Medicine 310: 1032–1034, 1984

    Article  PubMed  CAS  Google Scholar 

  • Rao BNS, Moller JH, Edwards JE. Primary pulmonary hypertension in a child: Response to pharmacologic agents. Circulation 40: 583–587, 1969

    Article  Google Scholar 

  • Rich S, Martinez J, Lam W, Rosen KM. Captopril as treatment for patients with pulmonary hypertension: Problem of variability in assessing chronic drug treatment. British Heart Journal 48: 272–277, 1982

    Article  PubMed  CAS  Google Scholar 

  • Rubin LJ, Groves BM, Reeves JT, Frosolono M, Handel F, et al. Prostacyclin-induced acute pulmonary vasodilatation in primary pulmonary hypertension. Circulation 66: 334–338, 1982

    Article  PubMed  CAS  Google Scholar 

  • Rubin LJ, Peter RH. Oral hydralazine therapy for primary pulmonary hypertension. New England Journal of Medicine 302: 69–73, 1980

    Article  PubMed  CAS  Google Scholar 

  • Rubino JM, Schroeder JS. Diazoxide in treatment of primary pulmonary hypertension. British Heart Journal 42: 362–363, 1979

    Article  PubMed  CAS  Google Scholar 

  • Rudolph AM, Paul MH, Sommer LS, Nadas AS. Effects of tolazoline hydrochloride (Priscoline) on circulatory dynamics of patients with pulmonary hypertension. American Heart Journal 55: 42432, 1958

    Article  Google Scholar 

  • Ruskin JN, Hutter AM. Primary pulmonary hypertension treated with oral phentolamine. Annals of Internal Medicine 90: 772–774, 1979

    PubMed  CAS  Google Scholar 

  • Saito D, Haraoka S, Yoshida H, Kusachi S, Yasuhara K, et al. Primary pulmonary hypertension improved by long term oral administration of nifedipine. American Heart Journal 105: 1041–1042, 1983

    Article  PubMed  CAS  Google Scholar 

  • Shettigar UR, Hultgren HN, Specter M, Martin R, Davies DH. Primary pulmonary hypertension — favourable effect of isoproterenol. New England Journal of Medicine 295: 1414–1415, 1976

    Article  PubMed  CAS  Google Scholar 

  • Wagenvoort CA, Wagenvoort N. Primary pulmonary hypertension: A pathologic study of the lung vessels in 156 clinically diagnosed cases. Circulation 42: 1163–1184, 1970

    Article  Google Scholar 

  • Wang SWS, Pohl JEF, Rowlands DJ, Wade EG. Diazoxide in treatment of primary pulmonary hypertension. British Heart Journal 40: 572–574, 1978

    Article  PubMed  CAS  Google Scholar 

  • Williams LT, Lefkowitz RJ. Receptor Binding Studies in Adrenergic Pharmacology, Raven Press, New York, 1978

    Google Scholar 

  • Wise JR. Nifedipine in the treatment of primary pulmonary hypertension. American Heart Journal 105: 693–694, 1983

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

McLeod, A.A., Jewitt, D.E. Drug Treatment of Primary Pulmonary Hypertension. Drugs 31, 177–184 (1986). https://doi.org/10.2165/00003495-198631020-00006

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.2165/00003495-198631020-00006

Keywords

Navigation