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Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)

Compassionate use of continuous prostacyclin in the management of secondary pulmonary hypertension: a case series.

BACKGROUND: Treatment of patients with secondary pulmonary hypertension has been unsatisfactory. OBJECTIVE: To describe exercise capacity, functional class, and hemodynamic variables after long-term intravenous infusion of prostacyclin in patients with secondary pulmonary hypertension. DESIGN: Case series. SETTING: Academic referral center. PATIENTS: 33 patients with secondary, precapillary pulmonary hypertension (New York Heart Association class III or IV). INTERVENTION: Continuous intravenous prostacyclin administered by portable infusion pump on a compassionate-use basis. MEASUREMENTS: Functional class, treadmill time, and hemodynamic variables. RESULTS: Patients were followed for an average of 12.7 +/- 5.6 months. Exercise tolerance and New York Heart Association class improved in each patient. The duration of treadmill exercise increased from 186 seconds to 491 seconds, an increase of 305 seconds (95% CI, 194 to 417 seconds; P < 0.001). Mean pulmonary artery pressure decreased from 60 mm Hg to 46 mm Hg, a decrease of 14 mm Hg (CI, 9 to 19 mm Hg; P < 0.001). Cardiac output increased from 3.90 L/min to 6.30 L/min, an increase of 2.40 L/min (CI, 1.56 to 3.25 L/min; P < 0.001). The pulmonary vascular resistance decreased from 1143 dynes x s/cm5 to 575 dynes x s/cm5, a decrease of 567 dynes x s/cm5 (CI, 407 to 727 dynes x s/cm5; P < 0.001). Patients with collagen vascular disease, congenital heart disease, and portopulmonary hypertension were analyzed with other patients and separately. All groups had a statistically significant reduction in mean pulmonary artery pressure and a statistically significant increase in cardiac output. CONCLUSION: Intravenous prostacyclin may be effective in the treatment of patients with certain types of secondary pulmonary hypertension.[1]


  1. Compassionate use of continuous prostacyclin in the management of secondary pulmonary hypertension: a case series. McLaughlin, V.V., Genthner, D.E., Panella, M.M., Hess, D.M., Rich, S. Ann. Intern. Med. (1999) [Pubmed]
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