ISSN: 2161-0665
+44 1478 350008
Natasa Chrysodonta1, Athina Chrysodonta2 and Fotini Kalogirou3
1Hinchingbrooke Health Care NHS Trust, UK 2Comenius University in Bratislava, Slovakia 3Cyprus University of Technology, Cyprus
Scientific Tracks Abstracts: Pediat Therapeut
Marfan syndrome is a rare inherited connective tissue autosomal dominant disease. The vast majority of the affected population will develop cardiovascular complications, with aortic-root dissection being the leading cause of the death in this cohort. It also leads to premature death, with 50% mortality in adulthood if left untreated. Currently, beta-blockers are the standard therapy used in the management of aortic root dilation in Marfan��?s syndrome, as it has been shown to decrease the rate of the dilation. Within the last decade a number of studies have began to assess the effectiveness of angiotensin ��? II receptor blockers (Arb) vs. beta-blockers in the management of aortic root dilation in Marfan syndrome. The aim of this systematic review is to examine the use of Arb vs. Beta-blockers in the management of aortic root dilation in pediatric patients with Marfan syndrome. Four main databases were used for the article search ��? Cochrane, Medline, PubMed and EMBASE, using the following terms: ��?Aortic dilation�, or ��?aorticpathology�, or ��?aortic coarct�, and ��?marfan�, or ��?Marfan syndrome�, and beta-blockers, or b-blockers, or adrenergic beta antagonist, and arb, or angiotensin receptor blocker, or angiotensin-II receptor blocker. The primary outcome was defined as the normalised rates of aortic dimensions before treatment initiation compared with follow-up measurements after treatment initiation (z-score). A total of 7 studies were identified, out of which only 3 have published results and were included in the review. Two of the studies which compared the combination of beta-blockers and Arbs vs. beta-blockers alone, showed inconsistent results. The 3rd study, which compared Arbs vs. beta-blockers alone, revealed that the prophylactic use of either medication had a similar effect in both groups. Currently the evidence suggests that Arb and beta-blockers slow down the progression of aortic root dilations. However, more randomised control trials are needed in order to draw clear conclusions on whether Arbs are more effective than beta-blockers in the management of aortic root dilation in pediatric Marfan syndrome.
Natasa Chrysodonta has completed a medical degree in the University of Bristol, and a BSc in Medical Sciences and management in Imperial College London. Currently she is a foundation year acute medicine doctor at Hinchingbrooke Hospital in Cambridgeshire.
Email: nc8636@my.bristol.ac.uk