Maki Hisataka, Yao Atsushi, Inaba Toshiro, Shiga Taro, Hatano Masaru, Kinugawa Koichiro, Yamashita Takeshi, Aizawa Tadanori, Nagai Ryozo
Department of Cardiovascular Medicine, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan.
Int Heart J. 2011;52(5):323-6. doi: 10.1536/ihj.52.323.
A 49-year-old woman suffering from rapidly progressing right-sided heart failure assessed as World Health Organization functional class (WHO-FC) IV is described. After treatment with oxygen and diuretics, she was in WHO-FC III on admission to our hospital, as confirmed by her poor exercise tolerance in cardiopulmonary exercise testing. Upon detailed examination, she was diagnosed as having idiopathic pulmonary arterial hypertension (IPAH). Right heart catheterization (RHC) revealed severe pulmonary hypertension (mPAP = 65 mmHg) with a markedly decreased cardiac index (CI = 1.0 L/minute/m(2)), and an acute vasoreactivity test with nitric oxide inhalation did not show any response. Due to her severe condition, we decided to attempt oral combination therapy consisting of bosentan, tadalafil, and beraprost, prescribed in the same order and titrated up to their maximum respective doses, instead of intravenous (IV) epoprostenol therapy. Her clinical symptoms improved day by day, and the hemodynamic parameters recovered to nearly normal ranges about 6 months after initiation of the combination therapy. Initial/programmed oral combination therapy for severe IPAH patients is not yet fully established, and there is less evidence concerning its efficacy than IV epoprostenol therapy. However, it has tremendous advantages for PAH patients when they respond well. It is very important to further identify what types of PAH patients will respond to this oral combination therapy and should be treated with it as the first-line therapy.
本文描述了一名49岁患有快速进展性右心衰竭的女性，其世界卫生组织功能分级（WHO-FC）为IV级。经吸氧和利尿剂治疗后，她入院时WHO-FC为III级，心肺运动试验显示其运动耐量较差，证实了这一点。经过详细检查，她被诊断为特发性肺动脉高压（IPAH）。右心导管检查（RHC）显示严重肺动脉高压（平均肺动脉压[mPAP]=65mmHg），心脏指数（CI）显著降低（CI=1.0L/分钟/m²），吸入一氧化氮的急性血管反应性试验未显示任何反应。由于她病情严重，我们决定尝试口服波生坦、他达拉非和贝拉普罗的联合治疗，按相同顺序给药并滴定至各自最大剂量，而不是静脉注射依前列醇治疗。联合治疗开始约6个月后，她的临床症状逐日改善，血流动力学参数恢复到接近正常范围。重度IPAH患者的初始/程序化口服联合治疗尚未完全确立，与静脉注射依前列醇治疗相比，其疗效的证据较少。然而，当PAH患者反应良好时，它对他们有巨大优势。进一步确定哪些类型的PAH患者会对这种口服联合治疗有反应并应将其作为一线治疗方法非常重要。
