What is the role of epoprostenol in the treatment of pulmonary veno-occlusive disease (PVOD)?

Updated: Oct 16, 2018
  • Author: Hakim Azfar Ali, MD; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
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Answer

The use of specific pulmonary arterial hypertension (PAH) therapies—eg, prostacyclin analogues, endothelin receptor antagonists, phosphodiesterase-5 inhibitors—in patients with pulmonary veno-occlusive disease (PVOD) is controversial.

Initial reports described the development of acute fulminant pulmonary edema and death [51] in association with infusions of intravenous epoprostenol. A 2008 report comparing PVOD patients with or without PCH to idiopathic patients reported pulmonary edema in 7 of 16 PVOD patients with vasodilator therapy. [52] However, in patients who do not have the option of lung transplantation, PVOD results in death within a few months to years after diagnosis. Consequently, continuous intravenous epoprostenol has been tried in PVOD patients, very cautiously and with relatively slow-dose up-titration. This treatment was met with some success. [47]

Epoprostenol has been reported to have some beneficial effects on hemodynamics in patients with pulmonary veno-occlusive disease (PVOD) and it has been demonstrated to reverse the increased vasomotor tone in pulmonary venules. [53] However, no structured clinical trials are available to support the use of any specific PAH therapies in PVOD patients.

Presently, treatment must be individualized to the patient after discussing the risks and benefits from the sparse data available. Pulmonary edema may occur, acutely or months after initiation of therapy, with the use of vasodilators. [39]


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