Marfan syndrome (MFS) is an inherited connective tissue disorder, noteworthy for its worldwide distribution, relatively high prevalence, and clinical variability, as well as pleiotropic manifestations involving primarily the ocular, skeletal, and cardiov
Genetic factors involve family history and the presence of certain heritable connective tissue disorders such as Ehlers-Danlos syndrome, Marfan's syndrome, neurofibromatosis, and polycystic kidney disease. Acquired factors include traumatic brain injury, sepsis, smoking, and hypertension. Management of ...
The medial layer of the aorta can also be subject to abnormalities (such as Marfan syndrome, bicuspid aortic valve, inflammatory vasculitis, atherosclerosis and infections). Despite important advances in diagnostic and therapeutic interventions, data derived from registries and population-based studies ...
The aorta is the ‘greatest artery’, through which oxygenated blood is delivered from the left ventricle to end organs with each cardiac cycle (200 million litres of blood transported in an average lifetime). The aorta can be affected by a wide spectrum of acute factors (such as cocaine us...
Background-Marfan syndrome (MFS) is caused by mutations in the fibrillin-1 gene and dysregulation of transforming growth factor-beta (TGF-beta). Recent evi... Luepker,V R. - 《Circulation》 被引量: 8发表: 2009年 In Search of Risk Factors: The Origin and Early Stages of Cardiovascular E...
[13]. The prevalence of Peter’s syndrome is beneath 1/1000000 [14]. Aniridia has an estimated prevalence between 1 : 40,000 and 1 : 100,000 [15] and the incidence of glaucoma in congenital forms ranges from 6% to 75% [16–18]. Marfan syndrome is a connective tissue ...
MARFAN-SYNDROMECANCER CENTERSEXPANSION RATEUNITED-STATESThoracoabdominal aortic aneurysms, while rare, continue to be associated with high morbidity and mortality in the modern era of vascular surgery and knowledge of this disease is essential for those in clinical practice. Given the clinically silent ...
in elderly population, highly detailed online 3D evaluation of the cavotricuspid isthmus and triangle of Koch, left ventricular dysfunction in a mouse model of Marfan syndrome, and endothelial nitric oxide synthase is essential for mobilization and ischemia-directed homing of circulating an...
Marfan syndromeANEURYSMSDISSECTIONRUPTUREDISEASESIZEtext>The international data on pathologies in the aortic arch sector are very limited, particularly because the aortic arch is rarely affected on its own; more often it is part of a larger disease (ascending aneurysm, aortic dissection, transverse ...
Thoracic aortic disease (TAD) poses substantial risks during pregnancy, particularly for women with genetic conditions such as Marfan syndrome, Loeys-Dietz syndrome, and vascular Ehlers-Danlos syndrome. This review examines the epidemiology, risk assessment, and management of TAD in pregnancy...