摘要目的:报道采用左心室辅助装置（LVAD）治疗Danon病2例，并进行文献复习。病例介绍:2例患者均为女性，年龄分别为51岁和42岁，临床均表现为扩张型心肌病样改变，均经基因检测证实为Danon病。虽经指南指导的药物治疗（GDMT），但心功能仍进行性恶化并快速发展至晚期心力衰竭阶段，拟行心脏移植手术，但群体反应性抗体（PRA）检测结果阳性，提示心脏移植配型困难，围手术期心脏排异风险极高，遂置入LVAD。术后均恢复良好，N末端B型利钠肽原（NT-proBNP）下降，心功能恢复至纽约心脏病协会（NYHA）心功能Ⅱ级，分别于术后54 d和25 d出院。文献检索及分析:检索PubMed和中国知网数据库。检索的英文关键词包括"Danon disease""glycogen storage disease type Ⅱ b""LAMP2""lysosome-associated membrane protein-2""LVAD"，检索的中文关键词包括"Danon"溶酶体相关膜蛋白质2"糖原贮积病Ⅱb型"。检索时间自建库起至2026年3月13日，共检索出文献87篇，其中符合标准的Danon病相关病例报告61篇，共95例患者。95例Danon病患者中，21例患者行心脏移植术，其中1例患者置入LVAD后34 d行心脏移植术，1例患者置入LVAD后990 d行心脏移植术，1例患者置入双心室辅助装置后791 d行心脏移植术；置入心脏辅助装置患者5例，除上述3例外，1例患者在置入LVAD术后1年内未发生任何不良事件，1例患者在置入LVAD术后101 d出院。结论:对于Danon病晚期心力衰竭患者，置入LVAD或可为一种新的治疗选择。

abstractsObjective:To report two cases of successful implantation of a left ventricular assist device (LVAD) in patients with Danon disease, and to review the relevant literature to summarize clinical experience.Clinical Feature:Two middle-aged female patients (aged 51 and 42 years) presented with dilated cardiomyopathy-like changes. Genetic testing confirmed Danon disease. Despite guideline-directed medical therapy (GDMT), cardiac function progressively deteriorated, rapidly advancing to advanced heart failure. Although heart transplantation was planned, abnormal panel-reactive antibody (PRA) test results indicated difficulty in finding a suitable donor and extremely high perioperative risk of cardiac rejection. Consequently, LVAD implantation was performed. Both patients recovered well postoperatively, with decreased N-terminal pro-B-type natriuretic peptide (NT-proBNP) levels and restored cardiac function to New York Heart Association (NYHA) class Ⅱ . They were discharged for 54 days and 25 days after implantation, respectively.Data Review:PubMed and CNKI databases were searched using the keywords of "Danon disease", "glycogen storage disease type Ⅱb", "LAMP2", "lysosome-associated membrane protein-2" and "LVAD". A total of 87 publications up to March 13, 2026 were identified. OF these, 61 were eligible case reports of Danon disease. Among 95 patients with Danon disease, 21 underwent heart transplantation. Among these, one patient received transplantation 34 days after LVAD implantation, another at 990 days post-LVAD, and a third at 791 days following biventricular assist device (BVAD) implantation. A total of 5 patients underwent LVAD implantation; of these, one experienced no adverse events within one year postoperatively, and another was discharged from hospital on postoperative day 101.Conclusion:For patients with advanced heart failure due to Danon disease, LVAD implantation may be selectively considered as a novel treatment option.