The Efficacy Of Beta Blockers In Patients With Duchenne Muscular Dystrophy Related Cardiomyopathy:Meta Analysis

Duchenne muscular dystrophy(DMD)is one of the most common hereditary degenerative muscle disease with the characteristic of long-term muscle deterioration.It has obvious effects of lung and heart dysfunction.End-stage heart failure(HF)of DMD is increasingly the leading cause of death in this population.The DMD guideline working group suggests that ACEI is a first-line therapy for DMD patients with left ventricular dysfunction,and the efficacy of ?-receptor blocker therapy for left ventricular(LV)dysfunction in DMD patients remains controversial.Objective: A meta-analysis of the efficacy of ?-receptor blocker in the treatment of children with DMD was performed to provide scientific references for the clinical application of ?-receptor blocker in the treatment of children with DMD.Method: Online electronic databases such as Pubmed,Embase,Cochrane Library,core collections of Web of science,clinical trial registration platform and CNKI were systematically searched in strict accordance with PRISMA guidelines and the specified retrieval strategies.The search was conducted in November 2018,and the update was rechecked in February 2019.All published domestic and foreign literatures related to DMD application of ?-receptor blockers were extracted.The RevMan 5.3 software was applied to evaluate the literatures and perform statistic analysis on the included data.Results: 5 researches were included in this study with 158 patients finally.There were 71 patients in ACEI+BB group and 87 in ACEI group.The results of meta-analysis showed that the addition of ?-receptor blocker had no significant short-term effect on the improvement of cardiac function in DMD children(P=0.19).The addition of ?-receptor blocker was significantly associated with decreased left ventricular end-diastolic diameter(P=0.03),and had significance in reducing heart rate(P<0.0001),reducing systolic blood pressure(P<0.0001),reducing left cardiac load,and had no clinical significance in improving BNP level(P=0.70).Conclusion: The application of ?-receptor blocker in DMD patients has no obvious clinical significance in improving cardiac function in the short term,while it has a beneficial effect on delaying further myocardial expansion and reducing left cardiac load in children with DMD.