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Lacro, Ronald V; Dietz, Harry C; Sleeper, Lynn A; Yetman, Anji T; Bradley, Timothy J; Colan, Steven D; Pearson, Gail D; Selamet Tierney, E. Seda; Levine, Jami C; Atz, Andrew M; Benson, D. Woodrow; Braverman, Alan C; Chen, Shan; De Backer, Julie; Gelb, Bruce D; Grossfeld, Paul D; Klein, Gloria L; Lai, Wyman W; Liou, Aimee; Loeys, Bart L; Markham, Larry W; Olson, Aaron K; Paridon, Stephen M; Pemberton, Victoria L; Pierpont, Mary Ella; Pyeritz, Reed E; Radojewski, Elizabeth; Roman, Mary J; Sharkey, Angela M; Stylianou, Mario P; Wechsler, Stephanie Burns; Young, Luciana T; Mahony, Lynn
The New England journal of medicine, 11/2014, Volume: 371, Issue: 22Journal Article
In this study, children and young adults with Marfan's syndrome were randomly assigned to receive atenolol or losartan and were followed for 3 years. There was no significant difference between the two groups in the rate of aortic-root dilatation. Marfan's syndrome is an autosomal dominant disorder of connective tissue affecting approximately 1 in 5000 people. 1 Cardiovascular disease, mainly progressive aortic-root dilatation and dissection, is the leading cause of death in Marfan's syndrome. After an open-label, randomized trial comparing propranolol with no therapy, published in 1994, showed a reduced rate of aortic enlargement among treated patients, beta-adrenergic receptor antagonists (beta-blockers) became the mainstay of medical management. 2 Current management includes serial cardiac imaging, exercise restriction, administration of beta-blockers, and elective aortic-root replacement. 3 Although early diagnosis and refined medical and surgical treatment have improved survival, patients with Marfan's syndrome continue to have . . .
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