Dilation of peripheral vessels in Marfan syndrome: importance of thoracoabdominal MR angiography

EM Mariucci, L Lovato, M Rosati, LM Palena… - International journal of …, 2013 - Elsevier
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) …

[HTML][HTML] Indomethacin prevents the progression of thoracic aortic aneurysm in Marfan syndrome mice

G Guo, CE Ott, J Grünhagen, B Muñoz-García… - Aorta, 2013 - thieme-connect.com
Background: Marfan syndrome (MFS), an inherited disorder of connective tissue
characterized by abnormalities in the skeletal, ocular, and cardiovascular systems, is caused …

Long-term doxycycline is more effective than atenolol to prevent thoracic aortic aneurysm in marfan syndrome through the inhibition of matrix metalloproteinase-2 and …

AWY Chung, HHC Yang, MW Radomski… - Circulation …, 2008 - Am Heart Assoc
β-Blockers, eg, atenolol, are the cornerstone therapy for thoracic aortic aneurysm (TAA) in
patients with Marfan syndrome; however, continued aortic dilatation has been reported. We …

Circulating transforming growth factor-β in Marfan syndrome

P Matt, F Schoenhoff, J Habashi, T Holm, C Van Erp… - Circulation, 2009 - Am Heart Assoc
Background—Marfan syndrome (MFS) is caused by mutations in the fibrillin-1 gene and
dysregulation of transforming growth factor-β (TGF-β). Recent evidence suggests that …

Blood pressure‐independent inhibition of Marfan aortic root widening by the angiotensin II receptor blocker valsartan

AY Tehrani, Z White, N Milad, M Esfandiarei… - Physiological …, 2021 - Wiley Online Library
Marfan syndrome (MFS) is a genetic disorder that results in accelerated aortic root widening
and aneurysm. However, management of MFS patients with blood pressure (BP)‐lowering …

Angiotensin receptor blockers vs. beta-blocker therapy for Marfan syndrome: a systematic review and meta-analysis

Z Wang, X Deng, X Kang, A Hu - Annals of Vascular Surgery, 2022 - Elsevier
Objective Several RCTs have been conducted to assess the efficacy and safety of
angiotensin receptor blocker (ARB) and beta-blocker (BB) therapy for Marfan syndrome …

Relationship between fibrillin-1 genotype and severity of cardiovascular involvement in Marfan syndrome

R Franken, G Teixido-Tura, M Brion, A Forteza… - Heart, 2017 - heart.bmj.com
Background The effect of FBN1 mutation type on the severity of cardiovascular
manifestations in patients with Marfan syndrome (MFS) has been reported with disparity …

Current and future pharmacological treatment strategies with regard to aortic disease in Marfan syndrome

AW Hartog, R Franken, AH Zwinderman… - Expert opinion on …, 2012 - Taylor & Francis
Introduction: Marfan syndrome is a multisystemic connective tissue disorder caused mainly
by mutations in the fibrillin-1 gene. The entire cardiovascular system is affected in patients …

Vascular smooth muscle cell phenotypic changes in patients with Marfan syndrome

E Crosas-Molist, T Meirelles… - … , and vascular biology, 2015 - Am Heart Assoc
Objective—Marfan's syndrome is characterized by the formation of ascending aortic
aneurysms resulting from altered assembly of extracellular matrix microfibrils and chronic …

[HTML][HTML] Decreased rotational flow and circumferential wall shear stress as early markers of descending aorta dilation in Marfan syndrome: a 4D flow CMR study

A Guala, G Teixido-Tura, L Dux-Santoy… - Journal of …, 2019 - Elsevier
Background Diseases of the descending aorta have emerged as a clinical issue in Marfan
syndrome following improvements in proximal aorta surgical treatment and the consequent …