keyword
MENU ▼
Read by QxMD icon Read
search

Aortopathy

keyword
https://www.readbyqxmd.com/read/27884484/ross-hybrid-arch-and-frozen-elephant-trunk-reconstruction-for-late-complications-of-bicuspid-aortic-valve-and-aortopathy
#1
Matthew Valdis, Gaetano DeRose, Linrui Guo, Michael W A Chu
Young patients with bicuspid aortic valve disease and aortopathy remain a clinical challenge, with many requiring multiple corrective operations throughout their lifetimes. Innovative surgical approaches are often required to address complex aortic pathologic conditions but leave patients at risk for reintervention, lifelong anticoagulation, and suboptimal hemodynamics. We describe an active 44-year-old female triathlete with recurrent bicuspid aortic stenosis, a small aortic root, a hypoplastic aortic arch and complex distal arch, and a descending aortic aneurysm, who underwent a single-stage reconstruction with a combined Ross procedure, hybrid arch, and frozen elephant trunk reconstruction...
December 2016: Canadian Journal of Cardiology
https://www.readbyqxmd.com/read/27882423/cardio-ankle-vascular-index-cavi-and-plasma-transforming-growth-factor-%C3%AE-1-tgf-%C3%AE-1-level-correlate-with-aortopathy-in-adults-with-repaired-tetralogy-of-fallot
#2
Yumi Shiina, Koichiro Niwa
It is challenging to evaluate aortopathy in congenital heart disease using conventional investigations such as brachial-ankle pulse wave velocity (baPWV). Therefore, we evaluated the cardio-ankle vascular index (CAVI), a dimension of the ascending aorta and plasma transforming growth factor-β1 (TGF-β1) level, in order to find novel noninvasive parameters of aortopathy in adults with repaired tetralogy of Fallot (TOF). Prospectively, we enrolled 42 consecutive adults with TOF (28 patients with repaired TOF were not on ARB, 8 patients with repaired TOF on ARB, 6 patients with no repair or only palliative repair) and 20 age-matched healthy controls...
November 24, 2016: Pediatric Cardiology
https://www.readbyqxmd.com/read/27879323/issues-and-challenges-in-diagnostic-sequencing-for-inherited-cardiac-conditions
#3
REVIEW
Roddy Walsh, Stuart A Cook
BACKGROUND: Inherited cardiac conditions are a relatively common group of Mendelian diseases associated with ill health and death, often in the young. Research into the genetic causes of these conditions has enabled confirmatory and predictive diagnostic sequencing to become an integral part of the clinical management of inherited cardiomyopathies, arrhythmias, aortopathies, and dyslipidemias. CONTENT: Currently, the principle benefit of clinical genetic testing is the cascade screening of family members of patients with a pathogenic variant, enabling targeted follow up of presymptomatic genotype-positive individuals and discharge of genotype-negative individuals to health...
November 22, 2016: Clinical Chemistry
https://www.readbyqxmd.com/read/27866031/aortopathy-in-adults-with-tetralogy-of-fallot-has-a-negative-impact-on-the-left-ventricle
#4
Yumi Shiina, Tomoaki Murakami, Naoto Kawamatsu, Koichiro Niwa
BACKGROUND: Aortic pressure wave reflection is significantly elevated in patients with congenital heart disease, even in children. Excessive aortic pressure wave reflection provokes cardiovascular events. AIM: To assess the influences of the enhanced pressure wave reflection on the left ventricle (LV) in adults with repaired tetralogy of Fallot (TOF). METHODS: Prospectively, 51 consecutive adults with repaired TOF (35.5±11.6yrs., 25 males) were enrolled and non-invasively assessed the pressure wave reflection using HEM 9000AI...
November 14, 2016: International Journal of Cardiology
https://www.readbyqxmd.com/read/27861233/aortic-volumetry-at-contrast-enhanced-magnetic-resonance-angiography-feasibility-as-a-sensitive-method-for-monitoring-bicuspid-aortic-valve-aortopathy
#5
Brian Trinh, Iram Dubin, Ozair Rahman, Marcos P Ferreira Botelho, Nicholas Naro, James C Carr, Jeremy D Collins, Alex J Barker
OBJECTIVES: Bicuspid aortic valve patients can develop thoracic aortic aneurysms and therefore require serial imaging to monitor aortic growth. This study investigates the reliability of contrast-enhanced magnetic resonance angiography (CEMRA) volumetry compared with 2-dimensional diameter measurements to identify thoracic aortic aneurysm growth. MATERIALS AND METHODS: A retrospective, institutional review board-approved, and Health Insurance Portability and Accountability Act-compliant study was conducted on 20 bicuspid aortic valve patients (45 ± 8...
November 17, 2016: Investigative Radiology
https://www.readbyqxmd.com/read/27855987/simulations-of-morphotype-dependent-hemodynamics-in-non-dilated-bicuspid-aortic-valve-aortas
#6
Kai Cao, Samantha K Atkins, Andrew McNally, Janet Liu, Philippe Sucosky
Bicuspid aortic valves (BAVs) generate flow abnormalities that may promote aortopathy. While positive helix fraction (PHF) index, flow angle (θ), flow displacement (d) and wall shear stress (WSS) exhibit abnormalities in dilated BAV aortas, it is unclear whether those anomalies stem from the abnormal valve anatomy or the dilated aorta. Therefore, the objective of this study was to quantify the early impact of different BAV morphotypes on aorta hemodynamics prior to dilation. Fluid-structure interaction models were designed to quantify standard peak-systolic flow metrics and temporal WSS characteristics in a realistic non-dilated aorta connected to functional tricuspid aortic valve (TAV) and type-I BAVs...
November 11, 2016: Journal of Biomechanics
https://www.readbyqxmd.com/read/27852591/ventricular-vascular-coupling-in-marfan-and-non-marfan-aortopathies
#7
Farina Loeper, Jantine Oosterhof, Mark van den Dorpel, Denise van der Linde, Yaxin Lu, Elizabeth Robertson, Brett Hambly, Richmond Jeremy
BACKGROUND: Marfan syndrome (MFS) and familial non-syndromal thoracic aortic aneurysm and dissection (ns-TAAD) are genetic aortopathies causing aortic dilatation with increased aortic stiffness. Left ventricular (LV) contractility and ventricular-vascular coupling index (VVI) were compared between MFS and ns-TAAD and determinants of VVI were investigated. METHODS AND RESULTS: Patients with MFS (M 57, F 47) and ns-TAAD (M 72, F 39) were studied by echocardiography and compared with controls (M 77, F 71)...
November 16, 2016: Journal of the American Heart Association
https://www.readbyqxmd.com/read/27816335/developmental-changes-in-aortic-mechanical-properties-in-normal-fetuses-and-fetuses-with-cardiovascular-disease
#8
Mio Taketazu, Masaya Sugimoto, Hirofumi Saiki, Hirotaka Ishido, Satoshi Masutani, Hideaki Senzaki
BACKGROUND: We hypothesized that fetal aortic mechanical properties assessed by aortic diameter (AoD) and flow show maturational changes during the gestational period, and that these properties are different in fetuses with congenital heart diseases and fetuses with normal development. METHODS: Phasic changes in ascending AoD along with Doppler flow profile were measured in 84 consecutive normal fetuses (gestational age, 18-36 weeks) and in 30 consecutive fetuses with cardiovascular diseases (gestational age, 22-39 weeks)...
September 28, 2016: Pediatrics and Neonatology
https://www.readbyqxmd.com/read/27782106/a-missense-tgfb2-variant-p-arg320cys-causes-a-paradoxical-and-striking-increase-in-aortic-tgfb1-2-expression
#9
Raya Al Maskari, Yasmin, S Cleary, Nikki Figg, Sarju Mehta, Doris Rassl, Ian Wilkinson, Kevin M O'Shaughnessy
Loeys-Dietz syndrome (LDS) is an autosomal dominant connective tissue disorder with a range of cardiovascular, skeletal, craniofacial and cutaneous manifestations. LDS type 4 is caused by mutations in TGFβ ligand 2 (TGFB2) and based on the family pedigrees described to date, appears to have a milder clinical phenotype, often presenting with isolated aortic disease. We sought to investigate its molecular basis in a new pedigree. We identified a missense variant p.(Arg320Cys) (NM_003238.3) in a highly evolutionary conserved region of TGFB2 in a new LDS type 4 pedigree with multiple cases of aortic aneurysms and dissections...
October 26, 2016: European Journal of Human Genetics: EJHG
https://www.readbyqxmd.com/read/27779199/mesenchymal-state-of-intimal-cells-may-explain-higher-propensity-to-ascending-aortic-aneurysm-in-bicuspid-aortic-valves
#10
Shohreh Maleki, Sanela Kjellqvist, Valentina Paloschi, Joelle Magné, Rui Miguel Mamede Branca, Lei Du, Kjell Hultenby, Johan Petrini, Jonas Fuxe, Janne Lehtiö, Anders Franco-Cereceda, Per Eriksson, Hanna M Björck
Individuals with a bicuspid aortic valve (BAV) are at significantly higher risk of developing aortic complications than individuals with tricuspid aortic valves (TAV) and defective signaling during the embryonic development and/or life time exposure to abnormal hemodynamic have been proposed as underlying factors. However, an explanation for the molecular mechanisms of aortopathy in BAV has not yet been provided. We combined proteomics, RNA analyses, immunohistochemistry, and electron microscopy to identify molecular differences in samples of non-dilated ascending aortas from BAV (N = 62) and TAV (N = 54) patients...
October 25, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27770001/aortic-cross-sectional-area-height-ratio-and-outcomes-in-patients-with-a-trileaflet-aortic-valve-and-a-dilated-aorta
#11
Ahmad Masri, Vidyasagar Kalahasti, Lars G Svensson, Eric E Roselli, Douglas Johnston, Donald Hammer, Paul Schoenhagen, Brian P Griffin, Milind Y Desai
BACKGROUND: -In patients with a dilated proximal ascending aorta and trileaflet aortic valve (TAV), we sought to assess a) factors independently associated with increased long-term mortality and b) the incremental prognostic utility of indexing aortic root to patient height. METHODS: -We studied consecutive patients with a dilated aortic root (≥4 cm) that underwent echocardiography and gated contrast-enhanced thoracic aortic computed tomography or magnetic resonance angiography between 2003-2007...
October 21, 2016: Circulation
https://www.readbyqxmd.com/read/27765875/hemiarch-the-real-operation-for-ascending-aortic-aneurysm
#12
Ramesh Singh, Katsuhiro Yamanaka, T Brett Reece
The extent and technique of resection for ascending aortopathies remains debated. The 2 main camps are divided into those who believe in the hemiarch resection utilizing hypothermic circulatory arrest and those who feel that the same goal can be accomplished with just an ascending aortic resection with an aortic cross clamp. While this debate continues to happen within groups and in some cases even in the same hospital, it certainly has not happened in the literature. There are no studies directly comparing the safety and efficacy of these 2 ideologies...
October 19, 2016: Seminars in Cardiothoracic and Vascular Anesthesia
https://www.readbyqxmd.com/read/27751339/bovine-aortic-arch-with-supravalvular-aortic-stenosis
#13
Mohammed Idhrees, Vijay Thomas Cherian, Sabarinath Menon, Thomas Mathew, Baiju S Dharan, K Jayakumar
A 5-year-old boy was diagnosed to have supravalvular aortic stenosis (SVAS). On evaluation of CT angiogram, there was associated bovine aortic arch (BAA). Association of BAA with SVAS has not been previously reported in literature, and to best of our knowledge, this is the first case report of SVAS with BAA. Recent studies show BAA as a marker for aortopathy. SVAS is also an arteriopathy. In light of this, SVAS can also possibly be a manifestation of aortopathy associated with BAA.
September 2016: Indian Heart Journal
https://www.readbyqxmd.com/read/27727313/endogenous-reference-genes-for-gene-expression-studies-on-bicuspid-aortic-valve-associated-aortopathy-in-humans
#14
Oliver J Harrison, Narain Moorjani, Christopher Torrens, Sunil K Ohri, Felino R Cagampang
Bicuspid aortic valve (BAV) disease is the most common congenital cardiac abnormality and predisposes patients to life-threatening aortic complications including aortic aneurysm. Quantitative real-time reverse transcription PCR (qRT-PCR) is one of the most commonly used methods to investigate underlying molecular mechanisms involved in aortopathy. The accuracy of the gene expression data is dependent on normalization by appropriate housekeeping (HK) genes, whose expression should remain constant regardless of aortic valve morphology, aortic diameter and other factors associated with aortopathy...
2016: PloS One
https://www.readbyqxmd.com/read/27666780/granular-media-calcinosis-in-the-aortic-walls-of-patients-with-bicuspid-and-tricuspid-aortic-valves
#15
Josephina Haunschild, Isabel N Schellinger, Sandy von Salisch, Farhad Bakhtiary, Martin Misfeld, Friedrich Wilhelm Mohr, Uwe Raaz, Christian D Etz
BACKGROUND: Bicuspid aortic valve (BAV), the most frequent congenital cardiac abnormality, is associated with a higher risk for ascending aortic aneurysms and aorta-related complications (ie, dissection and rupture). The aim of this study was to quantify granular media calcinosis (GMC) in the ascending aortic wall of patients with BAV. METHODS: We analyzed samples of the ascending aorta from patients with BAV (n = 54) and patients with tricuspid aortic valve (TAV) (n = 33) who underwent aortic repair, regarding medial thickness and diameter expansion...
September 22, 2016: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/27634926/fibrillin-2-is-upregulated-in-the-ascending-aorta-of-patients-with-bicuspid-aortic-valve
#16
Carmen Rueda-Martínez, Oscar Lamas, María José Mataró, Juan Robledo-Carmona, Gemma Sánchez-Espín, Inmaculada Moreno-Santos, Fernando Carrasco-Chinchilla, Pastora Gallego, Miguel Such-Martínez, Eduardo de Teresa, Manuel Jiménez-Navarro, Borja Fernández
OBJECTIVES: Bicuspid aortic valve (BAV) is the most prevalent congenital cardiac malformation, frequently associated with aortic dilatation (AD). The molecular mechanisms involved in AD and its aetiological link with BAV formation are poorly understood. Altered fibrillin-1 (FBN1) and metalloprotease-2, -9 (MMP2,9) protein activities have been suggested to be involved in BAV aortopathy. In addition, FBN2 participates in embryonic valve formation, but its possible involvement in BAV-associated AD has never been explored...
September 15, 2016: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/27611364/genetic-testing-of-248-chinese-aortopathy-patients-using-a-panel-assay
#17
Hang Yang, Mingyao Luo, Yuanyuan Fu, Yandong Cao, Kunlun Yin, Wenke Li, Chunjie Meng, Yanyun Ma, Jing Zhang, Yuxin Fan, Chang Shu, Qian Chang, Zhou Zhou
Inherited aortopathy, which is characterized by a high risk of fatal aortic aneurysms/dissections, can occur secondarily to several syndromes. To identify genetic mutations and help make a precise diagnosis, we designed a gene panel containing 15 genes responsible for inherited aortopathy and tested 248 probands with aortic disease or Marfan syndrome. The results showed that 92 individuals (37.1%) tested positive for a (likely) pathogenic mutation, most of which were FBN1 mutations. We found that patients with a FBN1 truncating or splicing mutation were more prone to developing severe aortic disease or valvular disease...
2016: Scientific Reports
https://www.readbyqxmd.com/read/27583373/genetics-of-bicuspid-aortic-valve-aortopathy
#18
Maria G Andreassi, Alessandro Della Corte
PURPOSE OF REVIEW: The incidence of aortic dilation and acute complications (rupture and dissection) is higher in patients with a bicuspid aortic valve (BAV), the most frequent congenital heart defect.The present review focuses on the current knowledge in the genetics of BAV, emphasizing the clinical implications for early detection and personalized care. RECENT FINDINGS: BAV is a highly heritable trait, but the genetic causes remain largely elusive. NOTCH1 is the only proven candidate gene to be associated with both familial and sporadic BAV...
November 2016: Current Opinion in Cardiology
https://www.readbyqxmd.com/read/27581676/infectious-aortitis
#19
F Daniel Ramirez, Bruce M Jamison, Benjamin Hibbert
Aortitis is broadly divided into infectious and non-infectious etiologies, each with distinct treatment implications. We present the case of a patient who sustained a type A aortic dissection during urgent coronary angiography for acute coronary syndrome. Clinical findings and events during the procedure raised suspicion for an underlying vascular disorder; however, the diagnosis of staphylococcal aortitis was not made until pathological examination of resected tissue. Clues to the diagnosis of infectious aortitis noted throughout the patient's clinical course are detailed as are potential consequences of diagnostic delays and treatment decisions, underscoring the difficulties in recognizing and managing the condition...
September 28, 2016: International Heart Journal
https://www.readbyqxmd.com/read/27550231/bicuspid-aortic-valve-a-review-with-recommendations-for-genetic-counseling
#20
REVIEW
Samantha L Freeze, Benjamin J Landis, Stephanie M Ware, Benjamin M Helm
Bicuspid aortic valve (BAV) is the most common congenital heart defect and falls in the spectrum of left-sided heart defects, also known as left ventricular outflow tract obstructive (LVOTO) defects. BAV is often identified in otherwise healthy, asymptomatic individuals, but it is associated with serious long term health risks including progressive aortic valve disease (stenosis or regurgitation) and thoracic aortic aneurysm and dissection. BAV and other LVOTO defects have high heritability. Although recommendations for cardiac screening of BAV in at-risk relatives exist, there are no standard guidelines for providing genetic counseling to patients and families with BAV...
December 2016: Journal of Genetic Counseling
keyword
keyword
116116
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"