PURPOSE: The prevalence of most minor cardiovascular manifestations in Marfan syndrome (MFS) is unknown. We assessed the prevalence of minor cardiovascular manifestations in MFS to evaluate their usefulness in a diagnostic setting. METHODS: Seventy-seven patients with MFS (aged 4 months to 55 years) underwent echocardiography to assess the presence of mitral valve prolapse and the diameter of the main pulmonary artery. A subset of 29 adult patients with MFS also underwent magnetic resonance imaging evaluation of the diameters of the thoracoabdominal aorta. RESULTS: Mitral valve prolapse was encountered in 66% of patients with MFS, with an equal distribution of classic and nonclassic mitral valve prolapse. The main pulmonary artery diameter was significantly larger in patients with MFS at all ages when compared with controls. In the adult group (> or = 14 years), we were able to provide a cutoff value of 23 mm to define pulmonary artery dilatation. The descending aorta was enlarged, but with substantial overlap with controls, thus precluding the use of a cutoff value. CONCLUSIONS: Mitral valve prolapse and main pulmonary artery dilatation are common findings in MFS patients at all ages and are easy to assess with echocardiography. Cutoff values to define dilatation of the descending aorta are hard to define, making them of limited value in the diagnostic evaluation. We recommend echocardiographic evaluation of mitral valve prolapse and main pulmonary artery diameter in patients referred for cardiovascular diagnostic assessment for MFS.
PURPOSE: The prevalence of most minor cardiovascular manifestations in Marfan syndrome (MFS) is unknown. We assessed the prevalence of minor cardiovascular manifestations in MFS to evaluate their usefulness in a diagnostic setting. METHODS: Seventy-seven patients with MFS (aged 4 months to 55 years) underwent echocardiography to assess the presence of mitral valve prolapse and the diameter of the main pulmonary artery. A subset of 29 adult patients with MFS also underwent magnetic resonance imaging evaluation of the diameters of the thoracoabdominal aorta. RESULTS: Mitral valve prolapse was encountered in 66% of patients with MFS, with an equal distribution of classic and nonclassic mitral valve prolapse. The main pulmonary artery diameter was significantly larger in patients with MFS at all ages when compared with controls. In the adult group (> or = 14 years), we were able to provide a cutoff value of 23 mm to define pulmonary artery dilatation. The descending aorta was enlarged, but with substantial overlap with controls, thus precluding the use of a cutoff value. CONCLUSIONS: Mitral valve prolapse and main pulmonary artery dilatation are common findings in MFS patients at all ages and are easy to assess with echocardiography. Cutoff values to define dilatation of the descending aorta are hard to define, making them of limited value in the diagnostic evaluation. We recommend echocardiographic evaluation of mitral valve prolapse and main pulmonary artery diameter in patients referred for cardiovascular diagnostic assessment for MFS.
Authors: Josephina A N Meester; Aline Verstraeten; Dorien Schepers; Maaike Alaerts; Lut Van Laer; Bart L Loeys Journal: Ann Cardiothorac Surg Date: 2017-11
Authors: Veronika C Stark; Michael Huemmer; Jakob Olfe; Goetz C Mueller; Rainer Kozlik-Feldmann; Thomas S Mir Journal: Pediatr Cardiol Date: 2018-04-25 Impact factor: 1.655
Authors: Sara Sheikhzadeh; Julie De Backer; Neda Rahimian Gorgan; Meike Rybczynski; Mathias Hillebrand; Helke Schüler; Alexander M Bernhardt; Dietmar Koschyk; Peter Bannas; Britta Keyser; Kai Mortensen; Robert M Radke; Thomas S Mir; Tilo Kölbel; Peter N Robinson; Jörg Schmidtke; Jürgen Berger; Stefan Blankenberg; Yskert von Kodolitsch Journal: Orphanet J Rare Dis Date: 2014-12-10 Impact factor: 4.123