Abstract：
Sickle cell disease (SCD) is associated with substantial morbidity and early mortality in afflicted adults. Cardiopulmonary complications that occur at increased frequency in SCD such as pulmonary embolism, pulmonary arterial hypertension, and acute chest syndrome can acutely worsen right ventricular function and lead to cardiogenic shock. Mechanical circulatory support including venoarterial extracorporeal membrane oxygenation (VA ECMO) is being increasingly utilized to treat hemodynamic collapse in various patient populations. However, a paucity of literature exists to guide the use of mechanical circulatory support in adults with SCD where disease-related sequela and unique hematologic aspects of this disorder may complicate extracorporeal therapy and must be understood. Here, we review the literature and describe three cases of adult patients with SCD who developed cardiogenic shock from acute decompensated right heart failure and were treated clinically with VA ECMO. Using an in vitro ECMO system, we investigate a potential increased risk of systemic fat emboli in patients with SCD who may be experiencing vaso-occlusive events with bone marrow involvement given the high-volume shunting of blood from venous to arterial systems with VA ECMO. The purpose of this study is to describe available extracorporeal life support experiences, review potential complications, and discuss the special considerations needed to further our understanding of the utility of VA ECMO in those with SCD.
摘要：
镰状细胞病（SCD）与患病成年人的大量发病率和早期死亡率有关。在SCD中发生频率增加的心肺并发症，如肺栓塞，肺动脉高压,急性胸部综合征可急性加重右心室功能，导致心源性休克。包括静脉动脉体外膜氧合（VAECMO）在内的机械循环支持越来越多地用于治疗各种患者人群的血液动力学崩溃。然而,目前缺乏相关文献来指导在SCD成人患者中使用机械循环支持,因为SCD患者的疾病相关后遗症和独特血液学方面可能会使体外治疗复杂化,因此必须加以了解.这里,我们回顾了文献,并描述了3例因急性失代偿性右心衰竭而发生心源性休克并接受VAECMO临床治疗的成年SCD患者.使用体外ECMO系统，我们调查了SCD患者的全身性脂肪栓塞的潜在风险增加,这些患者可能正在经历血管闭塞事件并伴有骨髓受累,考虑到VAECMO将血液从静脉系统大量分流至动脉系统.这项研究的目的是描述可用的体外生命支持经验，回顾潜在的并发症，并讨论需要进一步理解VAECMO在SCD患者中的效用的特殊考虑因素。
