title: Effects of continuous IV prostacyclin in a patient with pulmonary veno-occlusive disease.
abstract: Pulmonary veno-occlusive disease (PVOD) is a rare but life-threatening disease. Although prostacyclin (PGI(2)) attenuates pulmonary hypertension and improves the prognosis in patients with primary pulmonary hypertension, little information is available regarding the effect of PGI(2) on patients with PVOD. This report describes a patient with severe PVOD who showed marked improvement in exercise capacity and pulmonary hemodynamics with continuous IV PGI(2) treatment. Furthermore, he experienced no clinical events for 12 months and survived for 25 months after the initiation of PGI(2) therapy. These results suggest that continuous IV PGI(2) therapy may serve as a bridge to transplantation in some cases of PVOD.
authors.affiliation: Department of Internal Medicine, National Cardiovascular Center, Osaka, Japan.
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Okumura
authors.forename: Noritoshi
authors.initials: N
authors.lastname: Nagaya
authors.forename: Shingo
authors.initials: S
authors.lastname: Kyotani
authors.forename: Fumio
authors.initials: F
authors.lastname: Sakamaki
authors.forename: Norifumi
authors.initials: N
authors.lastname: Nakanishi
authors.forename: Shinya
authors.initials: S
authors.lastname: Fukuhara
authors.forename: Chikao
authors.initials: C
authors.lastname: Yutani
mesh_terms: D000328:Adult; D004305:Dose-Response Relationship, Drug; D011464:Epoprostenol / Q000008:administration & dosage* / Q000009:adverse effects; D005080:Exercise Test / Q000187:drug effects; D017809:Fatal Outcome; D006801:Humans; D007262:Infusions, Intravenous; D008134:Long-Term Care; D008168:Lung / Q000473:pathology; D016040:Lung Transplantation; D008297:Male; D011652:Pulmonary Circulation / Q000187:drug effects; D011668:Pulmonary Veno-Occlusive Disease / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000473:pathology; D011669:Pulmonary Wedge Pressure / Q000187:drug effects; D011859:Radiography
chemical_list: D011464:Epoprostenol