Patients with Marfan syndrome had decreased aortic distensibility and increased flow wave velocity in the normal-sized aorta compared with matched controls (P <.05).
Case-Control (n=101)
101 participants, including 78 patients with Marfan syndrome with aortic root dilatation and 23 matched control subjects, evaluated with MR flow mapping.
Marfan syndrome vs Matched control subjects
Aortic distensibility and flow wave velocity, p=<.05
p-value: p=<.05
PURPOSE: To investigate the feasibility of magnetic resonance (MR) flow mapping in the assessment of aortic biophysical properties in patients with Marfan syndrome and to detect differences in biophysical properties in the normal-sized aorta distal to the aortic root between these patients and matched control subjects. MATERIALS AND METHODS: Seventy-eight patients with Marfan syndrome with aortic root dilatation and 23 matched control subjects underwent MR flow mapping in four locations in the normal-sized aorta (1, ascending aorta; 2, thoracic descending aorta; 3, descending aorta at the level of the diaphragm; and 4, abdominal descending aorta). Distensibility at each location and flow wave velocity between locations were calculated. RESULTS: Compared with the control subjects, patients with Marfan syndrome had decreased aortic distensibility at three of the four locations (levels 1, 2, and 4; P <.05) and increased flow wave velocity between all locations (P <.05) in the aorta. In patients with Marfan syndrome, flow wave velocity was also significantly increased along the entire aortic tract beyond the aortic root (from level 1 to level 4). CONCLUSION: MR imaging reveals abnormal biophysical properties of the normal-sized aorta in patients with Marfan syndrome. Monitoring of these properties is relevant for evaluating disease progression and treatment options.
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Maarten Groenink
Adult Congenital Heart Disease
Albert de Roos
Cardiac Imaging
Barbara J.M. Mulder
Adult Congenital Heart Disease
Radiology
Amsterdam UMC Location University of Amsterdam
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Groenink et al. (Tue,) conducted a case-control in Marfan syndrome (n=101). Marfan syndrome vs. Matched control subjects was evaluated on Aortic distensibility and flow wave velocity (p=<.05). Patients with Marfan syndrome had decreased aortic distensibility and increased flow wave velocity in the normal-sized aorta compared with matched controls (P <.05).
synapsesocial.com/papers/6a215d42f69db56553c3e5a0 — DOI: https://doi.org/10.1148/radiology.219.2.r01ma01535
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