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Aortopathy

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https://www.readbyqxmd.com/read/29917097/does-statin-therapy-impact-the-proximal-aortopathy-in-aortic-valve-disease
#1
Tatiana Sequeira Gross, Shiho Naito, Niklas Neumann, Johannes Petersen, Thomas Kuntze, Hermann Reichenspurner, Yskert von Kodolitsch, Evaldas Girdauskas
Background: Studies have demonstrated that statin therapy decreases the growth rate of abdominal aneurysms. However, the effect of statin therapy on the proximal aortic disease has not been sufficiently elucidated. Aim: We aimed to analyze the association between statin treatment and the severity of proximal aortopathy in patients with aortic valve disease. Design: Cross-sectional study. Methods: We prospectively identified 458 patients who were referred for aortic valve surgery from 2008 to 2014...
June 18, 2018: QJM: Monthly Journal of the Association of Physicians
https://www.readbyqxmd.com/read/29911003/bicuspid-aortic-valve-registry-of-the-italian-society-of-echocardiography-and-cardiovascular-imaging-registro-della-valvola-aortica-bicuspide-della-societ%C3%A3-italiana-di-ecocardiografia-e-cardiovascular-imaging-rationale-and-study-design
#2
Rodolfo Citro, Moreno Cecconi, Salvatore La Carrubba, Eduardo Bossone, Francesco Antonini-Canterin, Stefano Nistri, Fabio Chirillo, Ilaria Dentamaro, Michele Bellino, Alfredo Posteraro, Mauro Giorgi, Licia Petrella, Ines Monte, Vincenzo Manuppelli, Antonio Mantero, Scipione Carerj, Frank Benedetto, Paolo Colonna
Background: Bicuspid aortic valve (BAV) is the most common congenital heart disease, affecting 0.5%-2% of the general population. It is associated not only with notable valvular risk (aortic stenosis and/or regurgitation, endocarditis) but also with aortopathy with a wide spectrum of unpredictable clinical presentations, including aneurysmal dilation of the aortic root and/or ascending thoracic aorta, isthmic coarctation, aortic dissection, or wall rupture. Methods: The REgistro della Valvola Aortica Bicuspide della Società Italiana di ECocardiografia e CArdiovascular Imaging is a retrospective (from January 1, 2010)/prospective, multicenter, observational registry, expected to enroll 3000 patients with definitive diagnosis of BAV made by transthoracic and/or transesophageal echocardiography, computed tomography, cardiovascular magnetic resonance, or at surgery...
April 2018: Journal of Cardiovascular Echography
https://www.readbyqxmd.com/read/29909014/non-coding-rnas-in-aneurysmal-aortopathy
#3
REVIEW
Joshua M Spin, Daniel Y Li, Lars Maegdefessel, Philip S Tsao
Aortic aneurysms represent a major public health burden, and currently have no medical treatment options. The pathophysiology behind these aneurysms is complex and variable, depending on location and underlying cause, and generally involves progressive dysfunction of all elements of the aortic wall. Changes in smooth muscle behavior, endothelial signaling, extracellular matrix remodeling, and to a variable extent inflammatory signaling and cells, all contribute to the dilation of the aorta, ultimately resulting in high mortality and morbidity events including dissection and rupture...
June 14, 2018: Vascular Pharmacology
https://www.readbyqxmd.com/read/29900594/ascending-aorta-in-tetralogy-of-fallot-beyond-echocardiographic-dimensions
#4
Cristina Cruz, Teresa Pinho, Carla Sousa, Cláudia Camila Dias, José Silva Cardoso, Maria Júlia Maciel
BACKGROUND: Late after tetralogy of Fallot (TOF) repair some patients exhibit aortic dilatation and stiffness. Noninvasive assessment of aortic stiffness could contribute to understand this aortopathy and may be important in risk stratification for major aortic event. METHODS: We included prospectively 82 adults after TOF repair and 41 age- and sex-matched normal controls. Aortic diameters were measured by two-dimensional transthoracic echocardiography and the aortic z-score was estimated...
June 13, 2018: Echocardiography
https://www.readbyqxmd.com/read/29884499/bicuspid-aortic-valve-related-aortopathy-knowledge-is-good
#5
EDITORIAL
Leonard N Girardi
No abstract text is available yet for this article.
April 18, 2018: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29848614/impact-of-pathogenic-fbn1-variant-types-on-the-progression-of-aortic-disease-in-patients-with-marfan-syndrome
#6
Norifumi Takeda, Ryo Inuzuka, Sonoko Maemura, Hiroyuki Morita, Kan Nawata, Daishi Fujita, Yuki Taniguchi, Haruo Yamauchi, Hiroki Yagi, Masayoshi Kato, Hiroshi Nishimura, Yoichiro Hirata, Yuichi Ikeda, Hidetoshi Kumagai, Eisuke Amiya, Hironori Hara, Takayuki Fujiwara, Hiroshi Akazawa, Jun-Ichi Suzuki, Yasushi Imai, Ryozo Nagai, Shinichi Takamoto, Yasunobu Hirata, Minoru Ono, Issei Komuro
BACKGROUND: Marfan syndrome can cause life-threatening aortic complications. We investigated the relationship between FBN1 genotype and severe aortopathy (aortic root replacement, type A dissections, and related death). METHODS: We evaluated 248 patients with pathogenic or likely pathogenic FBN1 variants. The variants were classified as haploinsufficient type (HI, n=93) or dominant-negative type (DN, n=155) based on their location and predicted amino acid alterations, and we examined the effects of the FBN1 genotype on severe aortic events (aortic root replacement, type A dissections, and related death)...
June 2018: Circulation. Genomic and precision medicine
https://www.readbyqxmd.com/read/29793631/pregnancy-outcomes-in-women-with-heart-disease-the-carpreg-ii-study
#7
Candice K Silversides, Jasmine Grewal, Jennifer Mason, Mathew Sermer, Marla Kiess, Valerie Rychel, Rachel M Wald, Jack M Colman, Samuel C Siu
BACKGROUND: Identifying women at high risk is an important aspect of care for women with heart disease. OBJECTIVES: This study sought to: 1) examine cardiac complications during pregnancy and their temporal trends; and 2) derive a risk stratification index. METHODS: We prospectively enrolled consecutive pregnant women with heart disease and determined their cardiac outcomes during pregnancy. Temporal trends in complications were examined. A multivariate analysis was performed to identify predictors of cardiac complications and these were incorporated into a new risk index...
May 29, 2018: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/29759733/-all-inclusive-guidelines-on-bicuspid-aortic-valve-related-aortopathy
#8
EDITORIAL
Ho Jin Kim, Joon Bum Kim
No abstract text is available yet for this article.
April 21, 2018: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29684119/aortic-stiffness-in-adolescent-turner-and-marfan-syndrome-patients
#9
Michal Schäfer, Lorna P Browne, Uyen Truong, James J Jaggers, Max B Mitchell, Ladonna Malone, Gareth Morgan, Kathryn Chatfield, Daniel McLennan, Harma Turbendian, Danial Vargas, Brian Fonseca, Michael DiMaria, Anar Shah, Margaret P Ivy, Alex J Barker, Kendall S Hunter, Neil Wilson, D Dunbar Ivy, David N Campbell
OBJECTIVES: Turner syndrome (TS) and Marfan syndrome (MFS) are partially characterized by aortopathies with a risk of developing severe aortic dilation, stiffness and consequent dissection and aneurysm formation. The incidence of a bicuspid aortic valve (BAV) is also increased in TS. We investigated aortic stiffness in teenage TS and MFS patients and evaluated to what degree stiffness in TS patients is augmented by the presence of a BAV. METHODS: Fifty-seven patients with TS (n = 37) and MFS (n = 20), as well as 22 controls with similar age and size distribution underwent evaluation of thoracic aortic stiffness using phase-contrast magnetic resonance imaging...
April 19, 2018: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/29680713/bicuspid-aortic-valve-aortopathy-in-the-era-of-fake-news-a-clinician-s-perspective-on-the-truth
#10
EDITORIAL
Hector I Michelena
No abstract text is available yet for this article.
March 29, 2018: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29671812/enlightening-the-association-between-bicuspid-aortic-valve-and-aortopathy
#11
REVIEW
Froso Sophocleous, Elena Giulia Milano, Giulia Pontecorboli, Pierpaolo Chivasso, Massimo Caputo, Cha Rajakaruna, Chiara Bucciarelli-Ducci, Costanza Emanueli, Giovanni Biglino
Bicuspid aortic valve (BAV) patients have an increased incidence of developing aortic dilation. Despite its importance, the pathogenesis of aortopathy in BAV is still largely undetermined. Nowadays, intense focus falls both on BAV morphology and progression of valvular dysfunction and on the development of aortic dilation. However, less is known about the relationship between aortic valve morphology and aortic dilation. A better understanding of the molecular pathways involved in the homeostasis of the aortic wall, including the extracellular matrix, the plasticity of the vascular smooth cells, TGFβ signaling, and epigenetic dysregulation, is key to enlighten the mechanisms underpinning BAV-aortopathy development and progression...
April 19, 2018: Journal of Cardiovascular Development and Disease
https://www.readbyqxmd.com/read/29666014/assessing-wall-stresses-in-bicuspid-aortic-valve-associated-aortopathy-forecasting-the-perfect-storm
#12
EDITORIAL
Alex J Barker, Michael Markl, Paul W M Fedak
No abstract text is available yet for this article.
March 31, 2018: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29656818/the-aortic-root-does-not-dilate-over-time-after-replacement-of-the-aortic-valve-and-ascending-aorta-in-patients-with-bicuspid-or-tricuspid-aortic-valves
#13
Sonya K Hui, Chun-Po Steve Fan, Shakira Christie, Christopher M Feindel, Tirone E David, Maral Ouzounian
OBJECTIVE: Whether the aortopathy associated with bicuspid aortic valve (BAV) disease occurs secondary to genetic or hemodynamic factors remains controversial. In this article we describe the natural history of the aortic root in patients with bicuspid versus tricuspid aortic valves (TAVs) after replacement of the aortic valve and ascending aorta. METHODS: From 1990 to 2010, 406 patients (269 BAV, 137 TAV) underwent aortic valve and ascending aorta replacement at a single institution...
March 13, 2018: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29625509/adult-congenital-heart-disease-with-pregnancy
#14
REVIEW
Koichiro Niwa
The number of women with congenital heart disease (CHD) at risk of pregnancy is growing because over 90% of them are grown-up into adulthood. The outcome of pregnancy and delivery is favorable in most of them provided that functional class and systemic ventricular function are good. Women with CHD such as pulmonary hypertension (Eisenmenger syndrome), severe left ventricular outflow stenosis, cyanotic CHD, aortopathy, Fontan procedure and systemic right ventricle (complete transposition of the great arteries [TGA] after atrial switch, congenitally corrected TGA) carry a high-risk...
April 2018: Korean Circulation Journal
https://www.readbyqxmd.com/read/29625025/ltbp3-pathogenic-variants-predispose-individuals-to-thoracic-aortic-aneurysms-and-dissections
#15
Dong-Chuan Guo, Ellen S Regalado, Amelie Pinard, Jiyuan Chen, Kwanghyuk Lee, Christina Rigelsky, Lior Zilberberg, Ellen M Hostetler, Micheala Aldred, Stephanie E Wallace, Siddharth K Prakash, Suzanne M Leal, Michael J Bamshad, Deborah A Nickerson, Marvin Natowicz, Daniel B Rifkin, Dianna M Milewicz
The major diseases affecting the thoracic aorta are aneurysms and acute dissections, and pathogenic variants in 11 genes are confirmed to lead to heritable thoracic aortic disease. However, many families in which multiple members have thoracic aortic disease do not have alterations in the known aortopathy genes. Genes highly expressed in the aorta were assessed for rare variants in exome sequencing data from such families, and compound rare heterozygous variants (p.Pro45Argfs∗ 25 and p.Glu750∗ ) in LTBP3 were identified in affected members of one family...
April 5, 2018: American Journal of Human Genetics
https://www.readbyqxmd.com/read/29506732/common-presentation-of-rare-diseases-aortic-aneurysms-valves
#16
Eloisa Arbustini, Valentina Favalli, Alessandro Di Toro, Lorenzo Giuliani, Giuseppe Limongelli
The concept "common presentation of rare diseases" implies that rare diseases are masked by common phenotypic manifestations. This concept applies to both aneurysmal and valvular diseases that can be syndromic and non-syndromic. Syndromic disorders include genetic connective tissue diseases and chromosomal disorders that are diagnosed independently from the aneurysm or valve disease. Non-syndromic diseases, on the other hand, are defined by the presence of aneurysm or valve disease or both. The reasons for suspecting these rare diseases include young age, the absence of risk factors, a positive family history for aortic or valvular disease/event, and extra-cardiovascular traits for syndromes...
April 15, 2018: International Journal of Cardiology
https://www.readbyqxmd.com/read/29462317/evaluation-of-microribonucleic-acids-as-potential-biomarkers-in-the-bicuspid-aortic-valve-associated-aortopathy%C3%A2
#17
Evaldas Girdauskas, Johannes Petersen, Niklas Neumann, Tatiana Groß, Shiho Naito, Mathias Hillebrand, Hermann Reichenspurner, Stefan Blankenberg, Tanja Zeller
OBJECTIVES: We aimed to evaluate the impact of microRNAs to predict the bicuspid aortic valve (BAV)-associated aortopathy. METHODS: Sixty-three BAV patients (mean age 47 ± 11 years, 92% men) with root dilatation, who underwent aortic valve ± proximal aortic surgery (mean post-AVR follow-up 10.3 ± 6.9 years) were included. The BAV aortopathy entities were categorized in the 'less dilated' (aortic root <50 mm) and 'severely dilated' (aortic root ≥50 mm) aorta...
February 14, 2018: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/29433109/pathogenesis-of-aortic-wall-complications-in-marfan-syndrome
#18
Nimrat Grewal, Adriana C Gittenberger-de Groot
BACKGROUND: Patients with Marfan (MFS) syndrome and patients with a bicuspid aortic valve (BAV) are more prone to develop aortic dilation and dissection compared to persons with a tricuspid aortic valve (TAV). To elucidate potential common as well as distinct pathways of clinical relevance, we compared the histopathological substrates of aortic pathology. PATIENT AND METHODS: Ascending aortic wall specimen were divided in five groups: BAV (n=36) and TAV (n=23) without and with dilation and non-dilated MFS (n=8)...
March 2018: Cardiovascular Pathology: the Official Journal of the Society for Cardiovascular Pathology
https://www.readbyqxmd.com/read/29426841/altered-dna-methylation-indicates-an-oscillatory-flow-mediated-epithelial-to-mesenchymal-transition-signature-in-ascending-aorta-of-patients-with-bicuspid-aortic-valve
#19
Hanna M Björck, Lei Du, Silvia Pulignani, Valentina Paloschi, Karin Lundströmer, Alexandra S Kostina, Cecilia Österholm, Anna Malashicheva, Anna Kostareva, Arturo Evangelista, Gisela Teixidó-Tura, Shohreh Maleki, Anders Franco-Cereceda, Per Eriksson
Disturbed flow has been suggested to contribute to aneurysm susceptibility in bicuspid aortic valve (BAV) patients. Lately, flow has emerged as an important modulator of DNA methylation. Hear we combined global methylation analysis with in vitro studies of flow-sensitive methylation to identify biological processes associated with BAV-aortopathy and the potential contribution of flow. Biopsies from non-dilated and dilated ascending aortas were collected from BAV (n = 21) and tricuspid aortic valve (TAV) patients (n = 23)...
February 9, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29362194/coronary-button-pseudoaneurysms-after-aortic-root-replacement-in-a-child-with-loeys-deitz-syndrome
#20
Charles D Fraser, Xun Zhou, Rui Han Liu, Cecillia Lui, Duke E Cameron, Marshall L Jacobs, Luca A Vricella, Narutoshi Hibino
Loeys-Deitz syndrome (LDS) is a connective tissue disorder characterized by aggressive aortopathy with a proclivity for aortic aneurysmal rupture and dissection. Prophylactic surgical intervention is often indicated to ameliorate risk of aneurysm rupture. Aortic root replacement involves excision of the coronary arteries from the aortic root with a button of surrounding aortic tissue and subsequent anastomosis of these buttons to the synthetic aortic graft. We report the case of a 16-year-old girl with LDS who developed pseudoaneurysms at the sites of previous coronary button implantation...
February 2018: Annals of Thoracic Surgery
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