Vizza Carmine Dario, Badagliacca Roberto, Sciomer Susanna, Poscia Roberto, Battagliese Alessandro, Schina Mauro, Agati Luciano, Fedele Francesco
Department of Cardiovascular and Respiratory Sciences, University La Sapienza, Rome, Italy.
Cardiology. 2006;106(3):168-73. doi: 10.1159/000092920. Epub 2006 Apr 26.
Prostanoids are a well-established therapy for pulmonary arterial hypertension (PAH), and observational studies suggest their efficacy even in chronic thromboembolic pulmonary hypertension (CTEPH) patients.
To compare the effects of 6 months of treatment with beraprost, an orally-active prostacyclin analog, in patients with distal CTEPH and PAH.
Case-control study.
Sixteen patients with severe pulmonary hypertension (NYHA II-IV), eight with distal CTEPH matched with eight patients with idiopathic PAH for similar effort tolerance.
All patients were in stable clinical and hemodynamic condition for 3 months with maximal standard therapy. During the titration phase (4 weeks) beraprost was increased to maximal tolerated dose (mean daily dosage: CTEPH 275 +/- 47 microg, PAH 277 +/- 47 microg) in adjunction of standard therapy, patients were followed-up for 6 months.
World Heart Organization (WHO) functional class, exercise capacity measured by distance walked in 6 min, and systolic pulmonary pressure (echocardiography), were evaluated at baseline, and at 1-, 3- and 6-month interval.
At 6 months WHO class decreased significantly in both groups (CTEPH from 2.7 +/- 0.6 to 2.0 +/- 0.24, p < 0.05; PAH from 3.0 +/- 0.26 to 2.1 +/- 0.25, p < 0.05), similarly the 6-min walk distance increased significantly from baseline (CTEPH from 312 +/- 31 to 373 +/- 29 m, p < 0.003; PAH from 303 +/- 31 to 347 +/- 29, p < 0.0003). Systolic pulmonary artery pressure showed a trend toward lower value (CTEPH from 85 +/- 7 m to 81 +/- 6 mm Hg, p = NS; PAH from 89 +/- 7 to 82 +/- 5, p = NS). During the observation period we did not have any death. The drug was well-tolerated with minor side-effects.
In patients with CTEPH beraprost had similar mid-term clinical and hemodynamic improvements than in patients with PAH.
前列环素类药物是治疗肺动脉高压（PAH）的成熟疗法，观察性研究表明其对慢性血栓栓塞性肺动脉高压（CTEPH）患者也有效。
比较口服活性前列环素类似物贝拉普罗斯治疗6个月对远端CTEPH患者和PAH患者的效果。
病例对照研究。
16例重度肺动脉高压患者（纽约心脏协会II-IV级），其中8例远端CTEPH患者与8例特发性PAH患者匹配，运动耐力相似。
所有患者在接受最大标准治疗的情况下，临床和血流动力学状况稳定3个月。在滴定阶段（4周），在标准治疗的基础上，将贝拉普罗斯增加至最大耐受剂量（平均日剂量：CTEPH为275±47微克，PAH为277±47微克），对患者进行6个月的随访。
在基线以及1个月、3个月和6个月时，评估世界心脏组织（WHO）功能分级、通过6分钟步行距离测量的运动能力以及收缩期肺动脉压（超声心动图）。
6个月时，两组患者的WHO分级均显著降低（CTEPH组从2.7±0.6降至2.0±0.24，p<0.05；PAH组从3.0±0.26降至2.1±0.25，p<0.05），同样，6分钟步行距离较基线显著增加（CTEPH组从312±31米增至373±29米，p<0.003；PAH组从303±31增至347±29，p<0.0003）。收缩期肺动脉压呈下降趋势（CTEPH组从85±7毫米汞柱降至81±6毫米汞柱，p=无统计学意义；PAH组从89±7降至82±5，p=无统计学意义）。观察期内无死亡病例发生。该药物耐受性良好，副作用轻微。
对于CTEPH患者，贝拉普罗斯在中期的临床和血流动力学改善方面与PAH患者相似。
