Grazioli Alison, Rabin Joseph, Rector Raymond P, Wu Zhongjun J, Burke Allen P, Sharifai Nima, Shah Aakash, Taylor Bradley S, Gladwin Mark T
Department of Medicine, Division of Pulmonary and Critical Care Medicine, Program in Trauma, University of Maryland School of Medicine, Baltimore, MD, USA.
Department of Surgery, Program in Trauma, University of Maryland School of Medicine, Baltimore, MD, USA.
J Intensive Care Med. 2025 Sep;40(9):929-936. doi: 10.1177/08850666241260605. Epub 2024 Aug 14.
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.
镰状细胞病（SCD）与成年患者的高发病率和早期死亡率相关。SCD中发生频率增加的心肺并发症，如肺栓塞、肺动脉高压和急性胸综合征，可使右心室功能急剧恶化并导致心源性休克。包括静脉-动脉体外膜肺氧合（VA ECMO）在内的机械循环支持正越来越多地用于治疗各类患者群体的血流动力学衰竭。然而，缺乏文献来指导在患有SCD的成年患者中使用机械循环支持，因为该疾病相关的后遗症和这种疾病独特的血液学方面可能会使体外治疗复杂化，必须予以了解。在此，我们回顾文献并描述3例患有SCD的成年患者，他们因急性失代偿性右心衰竭而发生心源性休克，并接受了VA ECMO临床治疗。我们使用体外ECMO系统，研究了患有SCD且可能因骨髓受累而发生血管闭塞事件的患者发生全身性脂肪栓塞的潜在风险增加情况，这是由于VA ECMO导致大量血液从静脉系统分流至动脉系统。本研究的目的是描述现有的体外生命支持经验，回顾潜在并发症，并讨论进一步了解VA ECMO在SCD患者中的效用所需的特殊考虑因素。
