Reviewer: : Dr. İlyas Cetin

Name of the Study: Angiotensin receptor blockers (ARB) and ß-blockers in Marfan syndrome: a meta-analysis of individual patient data from randomized trials

Published Congress: ESC 2022

Link : https://www.thelancet.com/journals/lancet/article/PIIS0140-6736(22)01534-3/fulltext

Introduction:

Marfan syndrome is a disease that affects the connective tissue and has multisystemic effects. It is known that the mutation in the fibrillin-1 gene plays a role in the pathogenesis. It can lead to progressive and fatal aortic root dilatation if not controlled. At this stage, the main goal is to slow down aortic root growth. Although beta-blockers are widely preferred for this purpose, they affect the synthesis of TGF-B (transforming growth factor beta), which plays a role in the pathogenesis of aortic root dilatations. The use of angiotensin receptor blockers (ARB) in the treatment has also been demonstrated by studies to slow down aortic root dilatation. In this sense, a meta-analysis obtained by examining the data obtained from small observational and subsequent randomized studies has been published recently.

Aims:

The combined use of angiotensin receptor blockers and beta-blockers and their effects on reducing aortic root dilatation typical of Marfan syndrome were tried to be determined in the meta-analysis.

Methods:

The primary purpose of this meta-analysis is to reveal the positive effects of ARB and beta-blockers on aortic root enlargement in patients with aortic root dilatation with the diagnosis of Marfan syndrome and who did not undergo surgery. Accordingly, those who had undergone aortic root operation were not included in the analysis.

Results:

For meta-analysis, there were 10 studies involving a total of 1386 patients. Three studies involving 324 patients were recruited from packs of beta-blocker doses. The remaining 4.6% (70 patients) randomized were also excluded from the analysis for aortic surgery.

The mean age of the study participants was 29 (SD 14), 367 (54%) women and 507 (70%) were using beta-blockers at the beginning. (All first subjects decided to continue beta-blocker). 526 of 630 genotypes-1 (83% to variant. The mean Z score in the sinuses of aortic valsalva was 3.76 (SD 2.14) in the ARB and 3.64 (SD 1.94) in the control device. Follow-up ARB score at the end of the training 0.07 (SE 0.02) and control application was observed as 0.13 (SE 0.02). Reaching the mean difference of -0.07. It shows the advertisement in the store of the training aortic root Z in ARB, which can meet with the control group. Specifically, the mean midline in an aortic squash Z score was similar to that uptake by a ß-blocker in the patient.

Conclusion:

A meta-analysis was performed that the aorta is surgically small and even in beta-blocker Marfan syndrome, the average is halved. This ratio is actually greater in those with the fibrillin-1 variant. Beta-blockers are equivalent to ARB in all benefit. Therefore, as long as intolerance does not develop, the blocker and ARB can be cut in half for review and be humiliated from this picture.