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.
ABSTRACT
Background: Role of immunosuppression treatment in patients with inflammatory dilated cardiomyopathy is controversial. The aim of this review is to summarize current evidence for immunosuppressive therapy in inflammatory cardiomyopathy. Methods: A systematic literature search was performed using PubMed, Embase and MELDINE to identify trials comparing immunosuppressive therapy with either placebo or conventional medical therapy in adult patients with inflammatory cardiomyopathy. Combined primary outcome in our study was all cause mortality and heart transplantation. Secondary outcomes included improvement in left ventricular ejection fraction (LVEF) and left ventricular end diastolic dimension (LVEDD). Results: Five randomized controlled trials (RCTs) were identified and four trials with similar comparable groups, with a total of 359 adult patients were included for analysis. Pooled data demonstrated no reduction in all-cause mortality and heart transplantation amongst the immunosuppression or the placebo arm (OR 0.98, 95% CI 0.48-1.98). There was a significant improvement in LVEF (1.34%, 95% CI 0.37-2.30) in patients treated with immunosuppressive medications, however no difference was observed in LVEDD [-0.11mm (95% CI -1.92 – 1.71)] in the treatment arm. Conclusion: There was no survival benefit or reduction in heart transplantation events with a significant improvement in LVEF in inflammatory cardiomyopathy patients treated with immunosuppression therapy.