Department of Clinical Science, National Hospital Organization Okayama Medical Center, Okayama, Japan.
Circ J. 2012;76(7):1729-36. doi: 10.1253/circj.cj-11-0973. Epub 2012 Apr 5.
Pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis (PCH) are rare causes of pulmonary hypertension. There is no proven medical therapy to treat these diseases, and lung transplantation is thought to be the only cure. Administration of vasodilators including epoprostenol sometimes causes massive pulmonary edema and could be fatal in these patients.
Eight patients were treated with epoprostenol for 387.3±116.3 days (range, 102-1,063 days), who were finally diagnosed with PVOD or PCH by pathological examination. The maximum dose of epoprostenol given was 55.3±10.7 ng·kg(-1)·min(-1) (range, 21.0-110.5 ng·kg(-1)·min(-1)). With careful management, epoprostenol therapy significantly improved the 6-min walk distance (97.5±39.2 to 329.4±34.6 m, P<0.001) and plasma brain natriuretic peptide levels (381.3±136.8 to 55.2±14.4 pg/ml, P<0.05). The cardiac index significantly increased from 2.1±0.1 to 2.9±0.3 L·min(-1)·m(-2) (P<0.05). However, pulmonary artery pressure and pulmonary vascular resistance were not significantly reduced. For 4 patients, epoprostenol therapy acted as a bridge to lung transplantation. For the other patients who had no chance to undergo lung transplantation, epoprostenol therapy was applied for 528.0±216.6 days and the maximum dose was 63.9±19.0 ng·kg(-1)·min(-1).
This study data suggest that cautious application of epoprostenol can be considered as a therapeutic option in patients with PVOD and PCH.
肺静脉闭塞病（PVOD）和肺毛细血管瘤病（PCH）是肺动脉高压的罕见病因。目前尚无有效的医学治疗方法，肺移植被认为是唯一的治愈方法。血管扩张剂如依前列醇的使用有时会导致大量肺水肿，并可能使这些患者致命。
8 名患者接受了依前列醇治疗，治疗时间为 387.3±116.3 天（范围为 102-1063 天），最终通过病理检查诊断为 PVOD 或 PCH。给予的依前列醇最大剂量为 55.3±10.7ng·kg(-1)·min(-1)（范围为 21.0-110.5ng·kg(-1)·min(-1)）。经过精心管理，依前列醇治疗显著改善了 6 分钟步行距离（97.5±39.2 至 329.4±34.6m，P<0.001）和血浆脑钠肽水平（381.3±136.8 至 55.2±14.4pg/ml，P<0.05）。心指数从 2.1±0.1 显著增加到 2.9±0.3L·min(-1)·m(-2)（P<0.05）。然而，肺动脉压和肺血管阻力没有明显降低。对于 4 名患者，依前列醇治疗作为肺移植的桥梁。对于其他没有机会进行肺移植的患者，依前列醇治疗应用了 528.0±216.6 天，最大剂量为 63.9±19.0ng·kg(-1)·min(-1)。
本研究数据表明，谨慎应用依前列醇可作为 PVOD 和 PCH 患者的治疗选择。
