Results 41 to 50 of about 2,210,543 (259)

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

Journal of Cardiovascular Magnetic Resonance, 2019
BackgroundDiseases of the descending aorta have emerged as a clinical issue in Marfan syndrome following improvements in proximal aorta surgical treatment and the consequent increase in life expectancy.
A. Guala, G. Teixidó-Turà, L. Dux-Santoy, C. Granato, A. Ruiz-Muñoz, F. Valente, L. Galian-Gay, L. Gutiérrez, T. González‐Alujas, K. M. Johnson, O. Wieben, A. S. Avilés, Arturo Evangelista, J. Rodríguez-Palomares   +13 more
semanticscholar   +1 more source

Management of Cardiovascular Health Issues in Turner Syndrome: Expert Insights and Expanded Recommendations From the 2024 Guideline Development Team

American Journal of Medical Genetics Part C: Seminars in Medical Genetics, EarlyView.
ABSTRACT Turner syndrome (TS) is frequently complicated by congenital heart disease (CHD). While left‐sided lesions such as bicuspid aortic valve (BAV) and coarctation of the aorta are the most common structural heart lesions in TS, other anomalies, such as aortic arch malformations, hypoplastic left heart syndrome (HLHS), persistent left superior vena
Katya de Groote, Sheetal R. Patel, Kristian Havmand Mortensen, Isabel Witvrouwen, Anthonie Duijnhouwer, Nicole M. Brown, Jasmine Grewal, Kathryn C. Chatfield, Aaron T. Dorfman, Siddharth K. Prakash   +9 more
wiley   +1 more source

Health-related quality of life in children and adolescents with Marfan syndrome or related disorders: a controlled cross-sectional study

Orphanet Journal of Rare Diseases
Background This cross-sectional controlled study aims to assess health-related quality of life (HRQoL) of children and adolescents with a molecular diagnosis of Marfan syndrome (MFS) or related disorders and to evaluate the factors associated with HRQoL ...
Thomas Edouard, Marie-Christine Picot, Fernanda Bajanca, Helena Huguet, Aitor Guitarte, Maud Langeois, Bertrand Chesneau, Philippe Khau Van Kien, Eric Garrigue, Yves Dulac, Pascal Amedro   +10 more
doaj   +1 more source

SOX6 enhances vascular smooth muscle cell phenotypic switching and elevates blood pressure by activating autophagy

Animal Models and Experimental Medicine, EarlyView.
Sox6 expression is elevated in hypertensive conditions and promotes vascular smooth muscle cell phenotypic switching through autophagy activation. Using gain‐ and loss‐of‐function approaches both in vitro and in vivo, we demonstrate that Sox6 regulates blood pressure and vascular remodeling, highlighting its potential as a novel therapeutic target for ...
Qianhui Ling, Xilan Dong, Liyan Mao, Chengjun Huang, Linjing Cong, Haizeng Zhang, Jun Cai, Zhenzhen Chen   +7 more
wiley   +1 more source

Maternal and fetal outcomes in pregnancies complicated by Marfan syndrome

Heart, 2019
Objectives Information to guide counselling and management for pregnancy in women with Marfan syndrome (MFS) is limited. We therefore conducted a UK multicentre study. Methods Retrospective observational study of women with MFS delivering between January
M. Cauldwell, P. Steer, S. Curtis, Aarthi R. Mohan, S. Dockree, L. Mackillop, H. Parry, J. Oliver, Monique Sterrenberg, S. Wallace, G. Malin, G. Partridge, L. Freeman, A. Bolger, F. Siddiqui, Dirk G. Wilson, M. Simpson, N. Walker, K. Hodson, K. Thomas, F. Bredaki, Rebecca Mercaldi, F. Walker, Mark R. Johnson   +23 more
semanticscholar   +1 more source

Revascularization of a Saphenous Vein Graft Ostial Stenosis in a Patient With Marfan Syndrome and a Previous Bentall Procedure Who was Resuscitated From Ventricular Fibrillation

Catheterization and Cardiovascular Interventions, EarlyView.
ABSTRACT A 24‐year‐old male with Marfan syndrome was admitted following an episode of ventricular fibrillation (VF) during exercise. He had previously undergone multiple aortic surgical procedures for symptomatic aortic regurgitation and an ascending aortic false aneurysm.
Shota Tokuno, Toshiharu Takeuchi, Naoki Nakagawa   +2 more
wiley   +1 more source

Neonatal Marfan Syndrome [PDF]

American Journal of Perinatology, 2019
Abstract Objective The Marfan syndrome (MFS) is an autosomal dominant disorder of connective tissue resulting from pathogenic variants of the fibrillin-1 gene (FBN1) with skeletal, cardiac, and ocular involvement. Study Design We report on a full-term male neonate, who showed at birth characteristics and dysmorphisms suggestive of ...
Tognato E., Perona A., Aronica A., Bertola A., Cimminelli L., De Vecchi S., Eshraghy M. R., Loperfido B., Vivenza C., Manzoni P.   +9 more
openaire   +4 more sources

The effects of acute and elective cardiac surgery on the anxiety traits of patients with Marfan syndrome

BMC Psychiatry, 2017
Background Marfan syndrome is a genetic disease, presenting with dysfunction of connective tissues leading to lesions in the cardiovascular and skeletal muscle system.
Kálmán Benke, Bence Ágg, Miklós Pólos, Alex Ali Sayour, Tamás Radovits, Elektra Bartha, Péter Nagy, Balázs Rákóczi, Ákos Koller, Viola Szokolai, Julianna Hedberg, Béla Merkely, Zsolt B. Nagy, Zoltán Szabolcs   +13 more
doaj   +1 more source

Study of Risk Factors and Image Findings of Isolated Abdominal Incidentally Detected Aortic Dissection

Journal of Clinical Ultrasound, Volume 53, Issue 3, Page 429-435, March/April 2025.
We detect the risk factors and image characteristics of incidentally detected IAAD. Independent risk factors of IAAD included fatty liver and smoking. In the ultrasound findings of IAAD, an intimal flap was frequently observed. In the CT scan, displacement of intimal calcifications was frequently observed. For suspected IAAD, the abdominal aorta should
Hiromasa Tsubouchi, Hidenori Onishi, Koji Maeno, Masaya Nakagaichi, Ikue Tsukushi, Youichi Kitano, Yoshitaka Makino, Hiroyuki Hayashi, Hidekazu Terasawa, Hiroko Kabuto, Toshihiro Misawa, Taku Sanada, Azusa Hisada, Kazuo Notsumata, Kazuhiro Okafuji, Osamu Yamamura   +15 more
wiley   +1 more source

Marfan's syndrome: an overview [PDF]

Sao Paulo Medical Journal, 2010
Marfan's syndrome is an autosomal dominant condition with an estimated prevalence of one in 10,000 to 20,000 individuals. This rare hereditary connective tissue disorder affects many parts of the body. The diagnosis of Marfan's syndrome is established in accordance with a review of the diagnostic criteria, known as the Ghent nosology, through a ...
Yuan, Shi-Min, Jing, Hua
openaire   +6 more sources