Pulmonary hypertension
Long-term Follow-up After Conversion from Intravenous Epoprostenol to Oral Therapy With Bosentan or Sildenafil in 13 Patients With Pulmonary Arterial Hypertension

https://doi.org/10.1016/j.healun.2007.01.022Get rights and content

Background

Epoprostenol significantly improves function and survival in patients with pulmonary arterial hypertension (PAH) but is associated with many risks and side effects. Furthermore, effective oral therapy is now available. We report our long-term experience with 13 patients from among 118 treated with epoprostenol who were able to be weaned to oral therapy, including 6 with persistently abnormal hemodynamics (mean pulmonary artery pressure ≥35 mm Hg).

Methods

Oral therapy with bosentan (n = 11) or sildenafil (n =2) was started before weaning epoprostenol in all but 1 patient. Right heart catheterization was performed when patients reached a dose of 2 ng/kg/min, and epoprostenol was discontinued with hemodynamic monitoring. Functional class and 6-minute walk test were assessed at regular intervals. Repeat right heart catheterization was performed 1 year after discontinuation of epoprostenol.

Results

Nine patients remained on oral therapy alone for up to 46 months. Four patients deteriorated in functional class, and 2 of them resumed epoprostenol therapy. Inhaled iloprost was started in another patient. One additional patient died, unrelated to PAH. Twelve patients underwent right heart catheterization at the time of epoprostenol discontinuation. Hemodynamic evaluation 13.2 ± 0.9 months later showed that the 5 patients with normal or nearly normal hemodynamics at the time of discontinuation of epoprostenol had no deterioration, whereas 4 of the 7 patients with abnormal hemodynamics had worsened. The 6-minute walk test at last follow-up was not significantly changed from maximal distance on epoprostenol (420 ± 94 vs 412 ± 95 meters).

Conclusion

Weaning from epoprostenol to sildenafil or bosentan with sustained clinical improvement is possible, even with persistent pulmonary hypertension; however, patients with persistently abnormal hemodynamics are at risk for hemodynamic and clinical deterioration and require close follow-up.

Section snippets

Methods

We conducted a retrospective chart review and identified patients who had been successfully weaned from epoprostenol to oral therapy at our institution. Patients were weaned from epoprostenol as part of their clinical care, and the Vanderbilt University Institutional Review Board approved this study. We reviewed the clinical and hemodynamic data of all patients with PAH in whom we discontinued epoprostenol. All patients but 1 were initiated on epoprostenol before the availability of oral or

Results

Thirteen patients among 118 who have received epoprostenol at our institution over the last 4 years have been weaned from epoprostenol to oral therapy. Discontinuation of epoprostenol was unsuccessful in 2 additional patients. The diagnosis and baseline functional class of individual patients who discontinued epoprostenol can be found in Table 1, Table 2. None of the 10 patients who underwent vasodilator testing before receiving epoprostenol demonstrated an acute pulmonary vasodilator response.

Discussion

Using an outpatient weaning protocol, we were able to discontinue epoprostenol in 13 patients with PAH treated at our institution. The average duration of follow-up of nearly 2.5 years provides the longest follow-up, to our knowledge, of any series of patients who have been weaned off epoprostenol. In addition, our series reports the successful transition from epoprostenol to monotherapy with sildenafil. Most patients were treated with bosentan because sildenafil was not commercially available

Conclusion

We were able to wean 13 clinically stable, functional class I and II patients off long-term, continuous intravenous epoprostenol by using a protocol in which oral therapy was initiated before weaning. Patients with persistently abnormal hemodynamics despite treatment with epoprostenol are at risk for subsequent hemodynamic, and in some cases, clinical deterioration. Weaning of epoprostenol in these patients should be undertaken only in selective cases, usually where there are additional reasons

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    • Cited by (0)

    This work was supported by NIH T32 HL07123 and K23 RR015534.

    1

    Dr Robbins has received financial remuneration for serving on advisory board for Actelion Pharmaceuticals and Pfizer, Inc, and for speaking at meetings sponsored by these companies. He has also received research grants for participating in multi-center clinical trials sponsored by Actelion Pharmaceuticals and Pfizer, Inc.

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    Copyright © 2007 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.