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Demulier L.,Universitair Ziekenhuis Gent 8K12 | Francois K.,Dienst Cardiochirurgie | Campens L.,Universitair Ziekenhuis Gent 8K12 | Campens L.,Universitair Ziekenhuis Gent | And 4 more authors.
Tijdschrift voor Geneeskunde | Year: 2011

The bicuspid aortic valve (BAV) is the most common congenital cardiac malformation in adults with a prevalence of 1 to 2%. BAV is increasingly recognized as part of a syndrome affecting the entire aortic root and ascending aorta including both valvular and vascular complications, which will develop in the majority of patients with BAV. In addition to these anatomic and functional abnormalities, histopathologic changes indicating intrinsic aortic wall defects are observed in BAVS. The exact mechanism underlying aortic dilatation in BAVS is not fully elucidated, but both hemodynamic and intrinsic defects appear to play an important role. An intriguing observation is that histopathologic and molecular changes in the wall show similarities with those observed in Marfan syndrome, an inheritable connective tissue disorder caused by mutations in the fibrillin-1 gene. Cardiovascular abnormalities include aortic root dilatation. Further investigation of these similarities may offer important new perspectives on follow-up and treatment of these patients.

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