Key result
Continuous IV epoprostenol is linked to progressive splenomegaly, worsening thrombocytopenia, and leukopenia in portopulmonary hypertension.
Why the study?
Patients with portopulmonary hypertension undergoing liver transplantation have high mortality, and continuous IV epoprostenol is used to reduce pulmonary artery pressure but its adverse effects are not well described.
Does continuous IV epoprostenol cause progressive splenomegaly and cytopenias in patients with portopulmonary hypertension?
Case Report (n=4)
Does continuous IV epoprostenol cause progressive splenomegaly and cytopenias in patients with portopulmonary hypertension?
Answers are grounded in this paper and cite their sources.
Continuous IV epoprostenol therapy in patients with portopulmonary hypertension may lead to progressive splenomegaly, thrombocytopenia, and leukopenia, potentially limiting its usefulness and affecting liver transplantation timing.
Patients with end-stage liver failure, portal hypertension, and associated pulmonary artery hypertension (portopulmonary hypertension [PPHTN]) have a high mortality when undergoing liver transplantation. Successful transplantation in these patients may depend on efforts to reduce pulmonary artery pressure (PAP). To this end, a number of centers are using a continuous intravenous (IV) infusion of epoprostenol, which has been shown to improve symptoms, extend life span, and reduce PAP in patients with primary pulmonary hypertension. We report four cases in which treatment of patients with PPHTN with continuous IV epoprostenol was followed by the development of progressive splenomegaly, with worsening thrombocytopenia and leukopenia. This finding may limit the usefulness of epoprostenol in PPHTN and influence the timing of transplantation in such patients.
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Findlay et al. (1999) conducted a case report in Portopulmonary hypertension (n=4). Epoprostenol was evaluated on Development of progressive splenomegaly, with worsening thrombocytopenia and leukopenia. Continuous IV epoprostenol therapy in four patients with portopulmonary hypertension was followed by the development of progressive splenomegaly, worsening thrombocytopenia, and leukopenia.
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