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Compassionate Use of Continuous Prostacyclin in the Management of Secondary Pulmonary Hypertension: A Case Series

Vallerie V. McLaughlin, MD; Diane E. Genthner, RN, BSN; Maureen M. Panella, RN, BSN; Dina M. Hess, RN; and Stuart Rich, MD

4 May 1999 | Volume 130 Issue 9 | Pages 740-743

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 s/cm⁵ to 575 dynes s/cm⁵, a decrease of 567 dynes s/cm⁵ (CI, 407 to 727 dynes 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.

Author and Article Information

From Rush-Presbyterian-St. Luke's Medical Center, Chicago, Illinois.

Acknowledgment: The authors thank Trude Cummens for secretarial assistance.

Requests for Reprints: Vallerie McLaughlin, MD, Rush Heart Institute, Center for Pulmonary Heart Disease, 1725 West Harrison Street, Suite 020, Chicago, IL 60612.

Current Author Addresses: Dr. McLaughlin, Ms. Genthner, Ms. Panella, Ms. Hess, and Mr. Rich: Rush Heart Institute, Center for Pulmonary Heart Disease, 1725 West Harrison Street, Suite 020, Chicago, IL 60612.

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