Ulrich Silvia, Speich Rudolf, Domenighetti Guido, Geiser Thomas, Aubert John-David, Rochat Thierry, Huber Lars, Treder Ursula, Fischler Mannuel
Clinics of Internal Medicine, Clinic of Pulmonology, University Hospital Zurich, Zurich, Switzerland.
Swiss Med Wkly. 2007 Oct 20;137(41-42):573-80. doi: 10.4414/smw.2007.11819.
we performed an open-label national study to evaluate the effects of Bosentan on haemodynamics, exercise capacity, quality of life, safety and tolerability in patients with chronic thromboembolic pulmonary hypertension (CTEPH).
fifteen patients with CTEPH not eligible or waiting for surgery were enrolled. The primary endpoint was the change in pulmonary vascular resistance (PVR). Secondary endpoints included quality of life (measured by the Minnesota living with heart failure questionnaire, MLHF), 6 minute walk distance (6MWD), World Health Organization (WHO) functional class, Borg dyspnoea scale, plasma endothelin, serum values of disease severity such as uric acid, N-terminal-pro brain natriuretic peptide (NTproBNP), C-reactive protein measured by a highly sensitive method (CRPs) and other serum and haemodynamic parameters.
after six months of treatment with bosentan, the PVR decreased from 852 (319) to 657(249) dynsm-5 (p = 0.02). Quality of life considerably improved from a mean total score of 48(14) to 35(17) (p = 0.003) with improvements in the physical (from 25(5) to 17(7)) and emotional (from 11(6) to 6(5)) subscores (p = 0.005 and 0.011), respectively. The 6MWD improved from 389(78) to 443(79) meters (p = 0.005). 4 patients (27%) improved and 11 patients (73%) maintained their WHO class with no deterioration during the six months of bosentan treatment (p = 0.02). Uric acid serum levels declined from 525(145) to 453(151) micromol/l (p = 0.006), NTproBNP and CRPs declined insignificantly. Endothelin serum levels increased from 4.3(1.5) to 5.9(2.2) pg/ml (p = 0.025). Patients tolerated the treatment well, and there were no severe adverse events or deaths.
this open-label study suggests a beneficial effect of bosentan therapy not only on pulmonary haemodynamics, but also on quality of life and exercise capacity for patients with severe CTEPH.
我们开展了一项开放性全国性研究，以评估波生坦对慢性血栓栓塞性肺动脉高压（CTEPH）患者的血流动力学、运动能力、生活质量、安全性及耐受性的影响。
纳入15例不符合手术条件或等待手术的CTEPH患者。主要终点为肺血管阻力（PVR）的变化。次要终点包括生活质量（采用明尼苏达心力衰竭生活问卷，MLHF进行测量）、6分钟步行距离（6MWD）、世界卫生组织（WHO）功能分级、Borg呼吸困难量表、血浆内皮素、疾病严重程度的血清值，如尿酸、N末端脑钠肽前体（NTproBNP）、采用高敏方法测量的C反应蛋白（CRPs）以及其他血清和血流动力学参数。
波生坦治疗6个月后，PVR从852（319）降至657（249）dynsm-5（p = 0.02）。生活质量显著改善，平均总分从48（14）降至35（17）（p = 0.003），其中身体方面（从25（5）降至17（7））和情感方面（从11（6）降至6（5））的子评分分别改善（p = 0.005和0.011）。6MWD从389（78）米提高至443（79）米（p = 0.005）。4例患者（27%）病情改善，11例患者（73%）在波生坦治疗的6个月期间维持WHO分级且无恶化（p = 0.02）。血清尿酸水平从525（145）降至453（151）μmol/L（p = 0.006），NTproBNP和CRPs无显著下降。内皮素血清水平从4.3（1.5）升至5.9（2.2）pg/ml（p = 0.025）。患者对治疗耐受性良好，未发生严重不良事件或死亡。
这项开放性研究表明，波生坦治疗不仅对严重CTEPH患者的肺血流动力学有益，而且对其生活质量和运动能力也有益。
