葡萄糖-6-磷酸脱氢酶缺乏症患者的心脏伴侣3型左心室辅助装置系统。

IF 1.1 4区 医学 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS
Elena Grasso, Roberto Lorusso, Ahmed Ibrahim, Mohamad Ibrahem Abdelhamed, Hassane Abdallah, Omer Ali Sayin
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引用次数: 0

摘要

葡萄糖-6-磷酸脱氢酶(G6PD)缺乏症是一种遗传性酶促疾病,影响着全世界数百万人,其特征是氧化应激下的溶血。左心室辅助装置(lvad)大大提高了晚期心力衰竭患者的生存率和生活质量。然而,它们的使用与溶血、血栓形成和栓塞事件的风险相关。这些风险在葡萄糖-6-磷酸脱氢酶(G6PD)缺乏的患者中可能会增加。鉴于该主题发表的研究有限,本研究的主要目的是评估接受心脏伴侣3 (HM3)左心室辅助装置(LVAD)植入的成人葡萄糖-6-磷酸脱氢酶(G6PD)缺乏症患者的溶血程度并确定相关因素。方法本研究是一项回顾性、观察性、单中心研究,研究对象为2017年至2022年期间接受HM3 LVAD植入的G6PD缺乏症成人(bb0 ~ 18岁)患者。对溶血相关调查和结果以及住院结果进行评估。结果左心室辅助装置(lvad)成功植入5例G6PD缺乏症成年患者,包括1例伴有镰状细胞特征(SCT)的患者。住院期间无重大并发症或死亡。平均随访时间30个月(平均30.4±13个月)。随访期间,2例患者死亡,2例左心室辅助装置被移植,1例患者接受心脏移植。在整个随访期间没有观察到宏观溶血的情况。虽然我们的研究规模有限,但lvad对g6pd缺陷患者似乎是安全的,并提供了显著的临床益处。需要更大规模的研究来证实这一点,并评估长期的相互作用。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Heartmate 3 left ventricular assist device system in patients with glucose-6-phosphate dehydrogenase deficiency.

BackgroundGlucose-6-phosphate dehydrogenase (G6PD) deficiency is a genetic enzymatic disorder that affects millions of people worldwide and characterized by hemolysis under oxidative stress. Left ventricular assist devices (LVADs) have substantially enhanced survival and quality of life for individuals with advanced heart failure. However, their use is associated with the risk of hemolysis, thrombosis, and embolic events. These risks may be heightened in patients with glucose-6-phosphate dehydrogenase (G6PD) deficiency. Given the limited published research on this subject, the primary objective of this study was to assess the degree of hemolysis and identify associated factors in adult patients with glucose-6-phosphate dehydrogenase (G6PD) deficiency who underwent Heartmate 3 (HM3) left ventricular assist device (LVAD) implantation.MethodsThis retrospective, observational, single-center study was conducted on adult (>18 years of age) patients with G6PD deficiency, who underwent LVAD implantation using the HM3 LVAD between 2017 and 2022. Hemolysis-related investigation and findings as well as in-hospital outcome were assessed.ResultsLeft ventricular assist devices (LVADs) were successfully implanted in five adult patients with G6PD deficiency, including one individual with associated sickle cell trait (SCT). There were no major complications or fatalities during the hospitalization period. The average follow-up duration was 30 months (mean 30.4 ± 13). During the follow-up period, two patients died, two LVADs were explanted, and one patient received a heart transplant. No instances of macroscopic hemolysis were observed throughout the follow-up period.ConclusionsWhile our study was limited in size, LVADs seem safe for G6PD-deficient patients and offer significant clinical benefits. Larger studies are needed to confirm this and assess long-term interactions.

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来源期刊
Perfusion-Uk
Perfusion-Uk 医学-外周血管病
CiteScore
3.00
自引率
8.30%
发文量
203
审稿时长
6-12 weeks
期刊介绍: Perfusion is an ISI-ranked, peer-reviewed scholarly journal, which provides current information on all aspects of perfusion, oxygenation and biocompatibility and their use in modern cardiac surgery. The journal is at the forefront of international research and development and presents an appropriately multidisciplinary approach to perfusion science.
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