ty - jour t1 - 慢性血换输出在毛细血管前肺动脉高血压的管理复杂性镰状细胞疾病JF - 欧洲呼吸杂志Jo - Eur Respir J Do - 10.1183/13993003.00272-2018 VL - 52是 - 4 SP - 1800272 Au -Turpin，Matthieu Au - Chantalat-Auger，Christelle Au - 父母们，Florence Au - Driss，FrançoiseAu - Lionnet，FrançoisAu - Habibi，Anoosha Au - Maître，Bernard Au - Huertas，Alice Au - Jaïs，Xavier Au - Weatherald，JasonAu - Montani，David Au - Sitbon，Olivier Au - Simonneau，GéraldAu - Galactéros，FrédericAu - Humbert，Marc Au - Bartolucci，Pablo Au - Savale，Laurent Y1 - 2018/10/01 UR - http：// Erj。ersjournals.com/content/52/4/1800272.Abstract n2 - 慢性血换输血（赌注）对毛细血管前肺动脉高血压复杂的长期影响使镰状细胞疾病（SCD）未知.13纯合的SS SCD患者痛苦从毛细血管前肺动脉高压和通过慢性妊娠治疗进行评估回顾性。评估包括血液动力学，纽约心脏协会功能级（NYHA FC），6分钟步行距离（6MWD）和血液测试。在启动投注中，所有患者都是NYHA FC III或IV，中位数（范围）6MWD为223（0-501）M和中位数（范围）肺血管抗性（PVR）为3.7（2-12.5）木单位。在4次下注的中位数，所有患者均已改善NYHA FC II或III。观察到血液力学的显着改善，包括PVR的降低（P = 0.01）。6MWD的趋势较高（P = 0.09）。 Median (range) follow-up time after initiation of BETs was 25 (6–53) months. During this period, two patients decided to stop BETs. One of them died from acute right heart failure and the other experienced worsening pulmonary hypertension. Two other patients died during follow-up at 25 and 54 months after BET initiation.Chronic BETs may be a potential therapeutic option in pre-capillary pulmonary hypertension complicating SCD, leading to significant clinical and haemodynamic improvements. These data must be confirmed in a prospective study.Chronic exchange transfusions may be a potential therapeutic option in pre-capillary pulmonary hypertension complicating sickle cell disease http://ow.ly/BrMj30loRBY ER -