Ty -jour t1-毛细血管前肺动脉高压复杂性镰状细胞病JF的慢性血液交换输血 - 欧洲呼吸杂志Turpin, Matthieu AU - Chantalat-Auger, Christelle AU - Parent, Florence AU - Driss, Françoise AU - Lionnet, François AU - 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 -savale -savale，savale，laurent y1-2018/101-108/10/01 ur -http：// http：// http：// erj。erersjournals.com/content/52/4/1800272.Abstract N2-慢性血液交换输血（BET）对毛细血管前肺部高血压复杂的镰状细胞病（SCD）的长期影响是未知的。13肥大的SSSSSS SCD患者患者患者患者评估了毛细血管前肺动脉高压并通过慢性下注治疗追溯。评估包括血液动力学，纽约心脏协会功能类（NYHA FC），6分钟步行距离（6MWD）和血液测试。在开始赌注之前，所有患者均为NYHA FC III或IV，中位数（范围）6MWD为223（0 – 0 – 0–501）M和中值（范围）肺血管耐药性（PVR）为3.7（2-12.5）木单元。经过4个下注课程的中位数，所有患者均改善了NYHA FC II或III。观察到血流动力学的显着改善，包括PVR的减少（p = 0.01）。较高的6MWD趋势（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 -