Abstract

Introduction: Patients with Marfan Syndrome have an increased risk of sudden death due to aortic dissection, mostly preceded by aortic dilatation.There has been considerable interest in evaluating the role of angiotensin blockade with Angiotensin Converting Enzyme (ACE) inhibitors or Angiotensin Receptor Blockers (ARB) on reducing the progression of aortic root dilatation in these patients. While earlier studies showed considerable benefit with ACE/ARB, recently large scale Randomized Control Trials showed conflicting results. The purpose of this meta-analysis was to assess the role of angiotensin blockade in reducing the rate of aortic root dilatation in patients with Marfan Syndrome. Methods: We searched PubMed, EMBASE, and Cochrane Central Register of Clinical Trials from the inception to May 30, 2015 using the terms “Marfan syndrome”, “angiotensin converting enzyme inhibitors” and “angiotensin II-receptor blockers”. Data was extracted using standardized protocol.The main outcome of the present analysis was the change in aortic root diameter with ACE/ABR therapy compared to control. The random effects model of DerSimonian and Laird was used. The statistical analysis was done in line with recommendations from the Cochrane Collaboration using Review Manager (RevMan) version 5.3. Results: Six studies enrolling a total of 1252 patients were included in the final analysis. Five were randomized controlled trials and one was an observation study. Analysis showed that there was no significant benefit with ACE/ARB in reducing aortic root dilatation rate compared to control [Mean Difference of -0.24 ; 95 % CI -0.36 to -0.13, P < 0.00001]. Conclusion: Based on the results of this meta analysis which includes data from the most recent trials, there is no significant benefit with ACE/ARB treatment on reducing the rate of aortic root dilatation in patients with Marfan Syndrome.

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