Marfan’s Syndrome and associated pathologies: An integrative systematic review of randomised controlled trials

Abstract

Background Marfan’s syndrome (MFS) is an autosomal dominant hereditary connective tissue disorder associated with numerous skeletal, ocular, cardiovascular, and respiratory pathologies which advance with age. Following multiple system involvement of body, the clinical presentation and management of MFS with associated pathologies are an area of cautious focus. Therefore, this review focused on highlighting various management options that are currently being supported for the MFS patients. Methodology The search methodology involved randomized controlled trials (RCTs) published between 2018 to 2022 from PubMed and Google Scholar databases. Preferred Reporting Items for Systematic Review and Meta-Analysis (PRISMA) were followed for reviewing the data extracted following the inclusion and exclusion criteria using the keywords “Marfan’s Syndrome”, “Irbesartan”, “Aortic Dilatation”, “Atenolol”, and “Lens Subluxation.” Results A total of eight articles were reviewed for the preferred distribution of population and the management options followed in the studies consisting of medical, psychological, and surgical interventions. Also, the associated pathologies related to MFS patients were analysed and found to have significant impact on health-related quality of life of MFS patients. Conclusions The review concluded that a combination of drugs atenolol, a β-blocker, and losartan which is an angiotensin receptor blocker, is beneficial in case of aortic root dilatation and higher aortic root stiffness. Additionally, ADRB1 testing may determine patients that are more likely to respond therapeutically to atenolol in comparison to losartan. Cionni modified capsular tension rings (MCTR) have been acknowledged as an effective device for delivering good capsular bag stabilization in cases of lens subluxation in MFS patients.