MASSIVE PULMONARY-EDEMA AND DEATH AFTER PROSTACYCLIN INFUSION IN A PATIENT WITH PULMONARY VENOOCCLUSIVE DISEASE

Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary hy pertension associated with fibrotic occlusion of the smaller pulmonary veins. Although vasodilator therapy is effective in many patients wit h primary pulmonary hypertension, the role of vasodilators in PVOD is unclear because of concerns about precipitating pulmonary edema. Recen tly, however, there have been reports of successful therapy with oral vasodilators or intravenous administration of prostacyclin in patients with PVOD. In contrast, a patient with PVOD is described who develope d acute pulmonary edema and respiratory failure during low-dose prosta cyclin infusion, leading to death. This report suggests that vasodilat ors, especially prostacyclin, must be used with extreme caution in pat ients with known PVOD.