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目的:汇总分析文献报道的室间隔肌部瘤（MuVSA）的临床特点与诊治情况。方法:从中国生物医学文献数据库（CBM）、中国知识资源总库（CCNKI，中国知网）、中国科技期刊数据库（VIP，维普网）、万方数据库以及PubMed、Wiley Online Library、Embase等数据库中检索从建库至2020年12月发表的关于MuVSA的文章，剔除不符合病例和重复病例后，对纳入文献中患者的性别、年龄、临床表现、合并疾病、诊断方法及辅助检查、治疗情况及转归等信息进行汇总分析。结果:共纳入24篇描述MuVSA文献，有37例患者纳入分析，多大多数（34例，占91.9%）是通过超声心动图确诊，个别通过心脏CT、心脏磁共振检查和心脏导管检查诊断。性别明确患者有30例，男性与女性人数比为1.5：1。描述初诊年龄的患者有37例，胎儿（妊娠8周以后的胎体）、年龄<1岁、1~17岁及≥18岁诊断的分别有6例（16.2%）、11例（29.7%）、7例（18.9%）和13例（35.2%）。37例患者中先天性MuVSA有29 例（占78.4%）。在33例描述症状的患者中，近半数（16例，占48.5%）无症状，其余17例（占51.5%）有症状患者的症状包括呼吸困难（7例）、心悸（2例）、呼吸急促（2例）、咳嗽（2例）、胸闷痛（2例）等。心电图检查22例，无特异性改变。对于无症状的MuVSA患者可不进行治疗，大多数预后良好。对于可能发生心力衰竭的大MuVSA，外科室间隔瘤切除术是一种有效的治疗方法，目前随访6例手术治疗的MuVSA患者术后恢复尚可。结论:MuVSA属罕见疾病，临床容易漏诊，需结合超声心动图、心脏磁共振或心脏血管造影检查协助诊断，无症状的MuVSA可不治疗，预后较良好。“,”Objective:to analyze and summarize the clinical features, diagnosis and treatment of muscular ventricular septal aneurysm (MuVSA) reported in the literature.Methods:Databases such as CBM, VIP, CNKI, Wanfang database, PubMed, Wiley Online Library and Embase database were searched on the case reports of MuVSA during the inception to December 2020. After eliminating suspected case and repetitive literature, gender, age, clinical manifestation, comorbidities, diagnostic method and auxiliary examination, therapy and outcome of patients in the included literature were extrated for pooled analysis.Results:a total of 24 literatures were included and 37 cases were involved. Most of the cases (n=34, 91.9%) were diagnosed by echocardiography, and a few were diagnosed by cardiac CT, cardiac magnetic resonance imaging, and cardiac catheterization. Among the 30 cases with documented gender, the ratio of male to female was 1.5∶1. According to the age at first diagnosis, there were 6 (16.2%), 11 (29.7%), 7 (18.9%) and 13 (35.2%) cases diagnosed before birth, at age of <1 year, 1~17 years and ≥18 years, respectively. Among the 33 patients with documented clinical manifestations, nearly half (n=16, 48.5%) were asymptomatic, while the other 17 cases (51.5%) showed symptoms including dyspnea (n=7), palpitations (n=2), tachypnea (n=2), cough (n=2), chest tightness and pain (n=2), etc. Electrocardiogram of 22 cases showed no specific changes. Asymptomatic MuVSA patients were not treated and most have a good prognosis. Surgical septal tumor resection is an effective treatment for large MuVSA patients with heart failure, and 6 cases with surgically treated MuVSA have recovered well.Conclusion:MuVSA is a rare disease, which is easy to be missed diagnosis in clinical practice. A combination of echocardiography, cardiac magnetic resonance imaging or cardiac angiography to assist in the diagnosis of MuVSA was preferred. Asymptomatic MuVSA patients may not need treatment with a relative good prognosis.