Biophysical properties of the aorta in patients with Marfan syndrome and related connective tissue disorders: evaluation with MRI and computational fluid dynamics modeling
نویسندگان
چکیده
Background Patients with Marfan Syndrome and related disorders have abnormal wall properties throughout the thoracic aorta including low distensibility in areas that eventually dilate. CFD modeling based on MRI data can be used to quantify distensibility and indices such as velocity and wall shear stress (WSS) throughout the cardiac cycle and for the entire thoracic aorta. We sought to use cardiac MRI and CFD modeling to evaluate the distensibility and indices of WSS in the aorta of patients with Marfan Syndrome and related disorders compared to ageand gender-matched controls.
منابع مشابه
Informative STR Markers for Marfan Syndrome in Birjand, Iran
Objective(s)Marfan syndrome (MFS) is a severe connective tissue disorder withan autosomal dominant inheritance pattern. Early diagnosis is critical in MFS. Because of the large size of fibrillin-1 gene (FBN1), the uniqueness of mutations, and the absence of genotype-to-phenotype correlations linkage analysis can be very helpful for early diagnosis of MFS. In this study, eight polymorphic marker...
متن کاملMarfan Syndrome, Dissecting Aneurysm of the Aorta, and Pregnancy.
A patient with the Marfan syndrome died suddenly from aortic rupture and dissection in the early puerperium of her second pregnancy. Although the association of the Marfan syndrome and pregnancy is extremely rare, the case reported here being only the fifth on record, the concurrence of dissecting aneurysm or aortic dissection with pregnancy is more frequent. Furthermore it is accepted that aor...
متن کاملOpen repair in chronic type B dissection with connective tissue disorders.
Aneurysms associated with inherited connective tissue disorders (CTD) constitute a specific but important issue in thoracoabdominal aortic aneurysm (TAAA) surgery. In this respect, Marfan syndrome and Ehlers-Danlos syndrome (EDS) type IV represent the most significant disorders causing aneurysmal dilatation of the thoracic aorta. Marfan syndrome typically causes aortic root dilatation, aortic v...
متن کاملComputational fluid dynamics study and GA modeling approach of the bend angle effect on thermal-hydraulic characteristics in zigzag channels
In the study, the thermal-hydraulic performance of the zigzag channels with circular cross-section was analyzed by Computational Fluid Dynamics (CFD). The standard K-Ꜫ turbulent scalable wall functions were used for modeling. The wall temperature was assumed constant 353 K and water was used as the working fluid. The zigzag serpentine channels with bend angles of 5 - 45° were studied for turbul...
متن کاملConnective tissue disorders with spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension: a prospective study.
OBJECTIVE Intracranial hypotension attributable to a spontaneous spinal cerebrospinal fluid (CSF) leak is an increasingly recognized cause of postural headaches. The cause of these leaks is poorly understood, but it is likely multifactorial and may involve a primary connective tissue disorder. We undertook a study to estimate the contribution of systemic connective tissue disorders to the devel...
متن کامل