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The purpose of this study is to evaluate the efficacy of Losartan versus Atenolol in the progression of aortic dilatation in patients with Marfan syndrome.
Marfan syndrome is a genetic disease of the connective tissue. Patients with Marfan syndrome experience an expansion of the aorta that can lead to dissection or rupture of it. This is the main cause of mortality in these patients.
The main objective of this study is to evaluate the efficacy of Losartan versus Atenolol in the progression of aortic dilation in patients with Marfan syndrome.
The measurement is made by echocardiography, assessing the diameter of the aorta in different zones: valve annulus, sinuses of Valsalva, sinotubular junction, ascending aorta, aortic arch, thoracic and abdominal aorta.
A total number of 150 subjects diagnosed with Marfan syndrome and who meet the diagnostic criteria of Ghent, of both sexes, 75 per treatment group, aged between 5 and 60, will be included in the study. The study is being conducted in two Spanish hospitals.
The treatment is maintained throughout the study period.
Allocation: Randomized, Endpoint Classification: Efficacy Study, Intervention Model: Parallel Assignment, Masking: Double Blind (Subject, Caregiver, Investigator, Outcomes Assessor), Primary Purpose: Prevention
Hospital 12 de Octubre
Active, not recruiting
Forteza, Albert, M.D.
Published on BioPortfolio: 2014-08-27T03:12:50-0400
To assess the efficacy of angiotensin II receptor blocker, Losartan, to prevent progressive dilation of aortic root in patients with Marfan syndrome.
Marfan syndrome is an inherited connective tissue disorder with morbidity and mortality from aortic dilation and dissection. The degree of aortic dilation and response to beta-blockade (s...
Marfan syndrome is a hereditary connective tissue disorder. Many individuals with this condition die because of the associated heart and blood vessel abnormalities. This study will compare...
Marfan syndrome is a genetic disease of our connective tissue, which provides material and support for our skeleton, muscles, blood vessels and other parts of our bodies. People with Marfa...
The major clinical problems in patients with Marfan Syndrome (MFS) are aortic root dilation (ARD), dissection and rupture. Although the available treatments (beta-blockers, BBs) improve th...
The aim of this study was to reevaluate dural ectasia criteria in Marfan syndrome patients fulfilling the revised Ghent criteria.
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An autosomal dominant disorder of CONNECTIVE TISSUE with abnormal features in the heart, the eye, and the skeleton. Cardiovascular manifestations include MITRAL VALVE PROLAPSE, dilation of the AORTA, and aortic dissection. Other features include lens displacement (ectopia lentis), disproportioned long limbs and enlarged DURA MATER (dural ectasia). Marfan syndrome is associated with mutations in the gene encoding fibrillin, a major element of extracellular microfibrils of connective tissue.
An autosomal dominant aneurysm with multisystem abnormalities caused by increased TGF-BETA signaling due to mutations in type I or II of TGF-BETA RECEPTOR. Additional craniofacial features include CLEFT PALATE; CRANIOSYNOSTOSIS; HYPERTELORISM; or bifid uvula. Phenotypes closely resemble MARFAN SYNDROME; Marfanoid craniosynostosis syndrome (Shprintzen-Goldberg syndrome); and EHLERS-DANLOS SYNDROME.
A fibrillin (FBN1) that functions as a structural support protein for MICROFIBRILS. It also regulates the maturation of OSTEOBLASTS by controlling the availability and concentration of TGF-BETA and BONE MORPHOGENETIC PROTEINS. Mutations in the FBN1 gene are associated with MARFAN SYNDROME.
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