Background Primary (idiopathic) pulmonary hypertension is aprogressive, fatal disease. Conventional therapy with anticoagulantand vasodilator drugs may improve symptoms and survival amongselected patients, but there is no evidence that the diseasecan be reversed.
Methods We evaluated the effects of long-term therapy (i.e.,for more than one year) with intravenous epoprostenol (prostacyclin)in patients with advanced primary pulmonary hypertension. Thebase-line evaluation included an assessment of pulmonary vasculardilation in response to intravenous adenosine. The epoprostenoldose was increased monthly to the maximum tolerated. Long-termtherapy was evaluated by measuring improvement in symptoms,exercise capacity, and hemodynamic variables.
Results We evaluated 27 patients with primary pulmonary hypertensionover a mean (±SD) period of 16.7±5.2 months. Intravenousadenosine had a variable effect on pulmonary vascular resistance(mean reduction, 27 percent; range, 0 to 56; P<0.001). Epoprostenoltherapy was initiated and the rate of infusion was increasedby an average of 2.4 ng per kilogram of body weight per minuteeach month. Twenty-six of the 27 patients had improvement insymptoms and hemodynamic measures, and overall, pulmonary vascularresistance declined by 53 percent to 7.9±3.8 resistanceunits (P<0.001) at the time of restudy. The long-term effectsof epoprostenol exceeded the short-term pulmonary vasodilatorresponse to adenosine in all but one patient. Seven of the eightpatients who had minimal pulmonary vasodilation in responseto adenosine (mean reduction in resistance units, <20 percent)still had a significant reduction in pulmonary vascular resistancewhen treated with epoprostenol (mean, 39±14 percent;P = 0.002).
Conclusions In primary pulmonary hypertension, long-term therapywith epoprostenol lowers pulmonary vascular resistance beyondthe level achieved in the short term with intravenous adenosine.Epoprostenol appears to have sustained efficacy in this disorder.
Source Information
From the Section of Cardiology, Rush Medical College, Rush–Presbyterian–St. Luke's Medical Center, Chicago.
Address reprint requests to Dr. Rich at the Rush Heart Institute, Center for Pulmonary Heart Disease, Rush–Presbyterian–St. Luke's Medical Center, 1725 W. Harrison St., Suite 020, Chicago, IL 60612-3824.
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SCHERMULY, R. T., KRUPNIK, E., TENOR, H., SCHUDT, C., WEISSMANN, N., ROSE, F., GRIMMINGER, F., SEEGER, W., WALMRATH, D., GHOFRANI, H. A.
(2001). Coaerosolization of Phosphodiesterase Inhibitors Markedly Enhances the Pulmonary Vasodilatory Response to Inhaled Iloprost in Experimental Pulmonary Hypertension . Maintenance of Lung Selectivity. Am. J. Respir. Crit. Care Med.
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Greidinger, E. L., Gaine, S. P., Wise, R. A., Boling, C., Housten-Harris, T., Wigley, F. M.
(2001). Primary Pulmonary Hypertension Is Not Associated With Scleroderma-Like Changes in Nailfold Capillaries. Chest
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Preston, I. R., Klinger, J. R., Landzberg, M. J., Houtchens, J., Nelson, D., Hill, N. S.
(2001). Vasoresponsiveness of Sarcoidosis-Associated Pulmonary Hypertension. Chest
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Beghetti, M, Berner, M, Rimensberger, P C
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86: e10-10
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HOSHIKAWA, Y., VOELKEL, N. F., GESELL, T. L., MOORE, M. D., MORRIS, K. G., ALGER, L. A., NARUMIYA, S., GERACI, M. W.
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Wort, S. J., Woods, M., Warner, T. D., Evans, T. W., Mitchell, J. A.
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