Objectives: Aim of this study was to assess vascular morphology and dimension of the entire aorta and branch vessels in MFS using thoracoabdominal MR angiography (MRA).
Background: Evaluation of vascular involvement in Marfan Syndrome (MFS) is focused to the ascending aorta, which has the major risk of dissection/rupture.
Methods: From March 2006 to January 2011, 64 patients (35 ± 13 years old) with MFS underwent echocardiography and MRA of thoracic and abdominal aorta. Measurements of ascending aorta, aortic arch, descending thoracic and abdominal aorta, and aortic branch vessels were assessed. In patients with aortic dissection only non dissected vessels were considered for measurements.
Results: 64 Marfan patients were divided into 2 groups: patients in natural history (group A, 55%) and patients previously submitted to aortic root and/or ascending aorta replacement because of severe aortic dilation or dissection (group B, 45%). Dilation of the descending aorta, mostly of the aortic isthmus, occurred in 18/53 patients (8/35 group A, 10/18 group B). Abdominal aorta resulted in the normal range in group A and in 16/18 patients of group B, while iliac vessels were dilated in 7/35 patients of group A and in 10/23 patients of group B. Four patients of group B presented isolated dilation of subclavian, celiac, mesenteric, or renal arteries.
Conclusions: Vascular dilation beyond the aortic root is not uncommon in MFS. The systematic use of MRA provides a comprehensive evaluation of the entire arterial system.
Keywords: AHA/ACC; ASE; American Heart Association/American College of Cardiology; American Society of Echocardiography; Aneurysm; BSA; EDS; Ehlers–Danlos syndrome; FBN1; LDS; Loeys–Dietz syndrome; MFS; MRA; MRI; Marfan Syndrome; Marfan syndrome; SD; TTE; body surface area; fibrillin-1 gene; magnetic resonance angiography; magnetic resonance imaging; standard deviation; transthoracic echocardiography.
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