Role of Beta-Blockers on Aortic Root Growth Rate in Marfan syndrome: Meta -Analysis of 243 Patients

ABSTRACT

Background: Marfan syndrome (MS) is inherited autosomal dominant connective tissue disorder caused by mutations in the FBN1 gene encoding fibrillin-1. Aortic dilatation is present in about 80% patients with MS and is the major cause of premature mortality. The objective of our study was to determine the effect of beta-blockers on aortic root growth rate in patients with MS. Methods: We performed a systematic review of all randomized controlled trials and prospective cohort studies that evaluated the efficacy of beta-blockers in patients with MS. The primary outcome of the study was aortic root growth rate. Secondary outcome was composite of death, aortic regurgitation, congestive heart failure, aortic dissection or cardiovascular surgery. Results: Five prospective trials were identified with similar comparable groups, with a total of 243 patients. In our study mean patient age was 12 years with a mean follow-up 86.5 months. There was a significant reduction in aortic root growth rate (SMD -0.86, 95% CI -1.23 to -0.48, p <0.001) with the use of beta-blockers. No significant difference was observed in secondary outcomes in the beta-blocker group as compared to placebo (OR = 1.80, 95% CI 0.21-15.53). Conclusion: Beta-blockers were associated with a significant reduction in aortic root growth rate with reduction in morbidity and mortality.