Kuroda Tadashi, Hirota Hisao, Masaki Mitsuru, Sugiyama Shoko, Oshima Yuichi, Terai Kazuo, Ito Akihiko, Yamauchi-Takihara Keiko
Department of Molecular Medicine, Osaka University Graduate School of Medicine, 2-2 Yamadaoka, Suita, Osaka 565-0871, Japan.
Heart Lung Circ. 2006 Apr;15(2):139-42. doi: 10.1016/j.hlc.2005.07.002. Epub 2005 Oct 17.
Pulmonary veno-occlusive disease is refractory to medical treatment and is generally associated with a poor prognosis. Treatment with vasodilators, such as prostacyclin, of patients with PVOD is controversial because of concerns regarding hemodynamic deterioration. Although a preferential pulmonary vasodilatory effect of a specific phosphodiesterase-5 inhibitor, sildenafil, has recently been reported in patients with primary pulmonary hypertension, little information is available regarding the effect of sildenafil on patients with pulmonary veno-occlusive disease. In the present case, remarkable improvement of hemodynamics and of clinical course was produced by adjunctive use of oral sildenafil in association with intravenous high-dose epoprostenol. These findings suggest that sildenafil may be a therapeutic option in the medical treatment of pulmonary veno-occlusive disease.
肺静脉闭塞病药物治疗效果不佳，通常预后不良。由于担心血流动力学恶化，使用血管扩张剂（如前列环素）治疗肺静脉闭塞病患者存在争议。尽管最近有报道称，一种特定的磷酸二酯酶-5抑制剂西地那非对原发性肺动脉高压患者有优先的肺血管扩张作用，但关于西地那非对肺静脉闭塞病患者的影响，目前所知甚少。在本病例中，口服西地那非联合静脉注射高剂量依前列醇显著改善了血流动力学和临床病程。这些发现表明，西地那非可能是肺静脉闭塞病药物治疗的一种选择。
