摘要
连续静脉内εε烯醇提高了严重初级肺动脉高压的运动能力,血液力学和生存。肺动脉高压也可以在结缔组织疾病的患者中危及生命。在一个未受控制的单子未受控制的学习中,对患有对患者对口腔血管血管(包括钙通道阻滞剂)反应有关的结缔组织疾病的严重肺动脉高血压患者对ε肺动脉高血压(包括钙通道阻滞剂)进行评估的影响。尽管常规医疗疗法,但功能级别III或IV。17名患者接受由与常规治疗(口腔抗凝剂,利尿剂,补充氧)相关的便携式输注泵给药的ePoprostenol。在治疗的前六周,两(12%)患者死亡,肺水肿(n = 1)和严重败血症(n = 1)。在十五个剩余的受试者中,临床和血液动力学参数在六周内显着提高。随后监测这些患者在开始Epoprostenol后的80 +/- 48(范围为14-154)周。五(33%)患者死亡,右心力衰竭(n = 2),严重的败血症(n = 2)或晕厥(n = 1),两名患者在开始疟原烯醇后成功移植24和52周。剩下的八名患者中有7名患有持续的临床改善。 Short-term epoprostenol therapy is effective in some patients with connective tissue diseases as demonstrated by better clinical status and haemodynamics at six weeks. However, this study reports several cases of early and late major complications including severe sepsis and pulmonary oedema. Additional information is needed to evaluate the benefit: risk ratio of long-term epoprostenol therapy in pulmonary hypertension secondary to connective tissue diseases.