Article
Intrinsic cardiomyopathy in Marfan syndrome: results from in-vivo and ex-vivo studies of the Fbn1C1039G/+ model and longitudinal findings in humans.
Pediatric research - 1 Sept 2015
Campens Laurence, Renard Marjolijn, Trachet Bram, Segers Patrick, Muino Mosquera Laura, De Sutter Johan, Sakai Lynn, De Paepe Anne, De Backer Julie
Abstract excerpt
BACKGROUND: Mild intrinsic cardiomyopathy in patients with Marfan syndrome (MFS) has consistently been evidenced by independent research groups. So far, little is known about the long-term evolution and pathophysiology of this finding. METHODS: To gain more insights into the pathophysiology of MFS-related cardiomyopathy, we performed in-vivo and ex-vivo studies of 11 Fbn1(C1039G/+) mice and 9 wild-type (WT)...
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