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Aorta bicuspide

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https://www.readbyqxmd.com/read/28815188/painless-aortic-dissection-diagnostic-dilemma-with-fatal-outcomes-what-do-we-learn
#1
Saeeda Fatima, Konika Sharma
Aortic dissection is the most catastrophic clinical condition that involves the aorta. It has a high mortality as well as high rate of misdiagnosis due to frequent unusual presentation. Typically, it presents with acute chest, back, and tearing abdominal pain. However, it can present atypically with minimal or no pain, making diagnosis difficult. Physicians should always suspect acute aortic dissection in patients with certain clinical conditions like difficult-to-control hypertension, giant cell arteritis, bicuspid aortic valve, intracranial aneurysms, simple renal cysts, family history of aortic disease, and Marfan syndrome, especially when a patient presents with ischemic symptoms involving multiple organ without an obvious cause...
July 2017: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/28805011/bicuspid-and-unicuspid-aortic-valves-different-phenotypes-of-the-same-disease-insight-from-the-gentac-registry
#2
Joseph M Krepp, Mary J Roman, Richard B Devereux, Adrienne Bruce, Siddharth K Prakash, Shaine A Morris, Dianna M Milewicz, Kathryn W Holmes, William Ravekes, Ralph V Shohet, Reed E Pyeritz, Cheryl L Maslen, Barbara L Kroner, Kim A Eagle, Liliana Preiss, Federico M Asch
BACKGROUND: Unicuspid aortic valve (UAV) is a rare disorder, often difficult to distinguish from bicuspid aortic valve (BAV). BAV and UAV share valve pathology such as the presence of a raphe, leaflet fusion, aortic stenosis, aortic regurgitation, and/or ascending aortic dilatation, but a comprehensive echocardiographic comparison of patients with UAV and BAV has not been previously performed. METHODS: We investigated UAV and BAV patients at an early stage of disease included in GenTAC, a national registry of genetically related aortic aneurysms and associated cardiac conditions...
August 14, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28773988/investigation-on-the-regional-loss-factor-and-its-anisotropy-for-aortic-aneurysms
#3
Nastaran Shahmansouri, Mohammed Alreshidan, Alexander Emmott, Kevin Lachapelle, Ismaïl El-Hamamsy, Raymond Cartier, Richard L Leask, Rosaire Mongrain
An aortic aneurysm is a lethal arterial disease that mainly occurs in the thoracic and abdominal regions of the aorta. Thoracic aortic aneurysms are prevalent in the root/ascending parts of the aorta and can lead to aortic rupture resulting in the sudden death of patients. Understanding the biomechanical and histopathological changes associated with ascending thoracic aortic aneurysms (ATAAs), this study investigates the mechanical properties of the aorta during strip-biaxial tensile cycles. The loss factor-defined as the ratio of dissipated energy to the energy absorbed during a tensile cycle-the incremental modulus, and their anisotropy indexes were compared with the media fiber compositions for aneurysmal (n = 26) and control (n = 4) human ascending aortas...
October 26, 2016: Materials
https://www.readbyqxmd.com/read/28740678/bicuspid-aortic-root-spared-during-ascending-aorta-surgery-an-update-of-long-term-results
#4
EDITORIAL
Marco Russo, Guglielmo Saitto, Paolo Nardi, Fabio Bertoldo, Carlo Bassano, Antonio Scafuri, Antonio Pellegrino, Giovanni Ruvolo
BACKGROUND: Bicuspid aortic valve (BAV) aortopathy is well known in literature even if only few data exist regarding isolated supra-coronary aneurysm with normally functioning valve and root. Aim of this study is to clarify the long-term fate of bicuspid aortic root spared at the time of ascending aorta surgery. METHODS: We identified forty-seven patients (mean age, 57±11 y; range, 35-81 y, 31 males) who were treated by means of supracoronary aortic replacement in presence of normally functioning BAV and not significantly enlarged sinues of Valsalva...
June 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28740468/evolution-of-precision-medicine-and-surgical-strategies-for-bicuspid-aortic-valve-associated-aortopathy
#5
Ali Fatehi Hassanabad, Alex J Barker, David Guzzardi, Michael Markl, Chris Malaisrie, Patrick M McCarthy, Paul W M Fedak
Bicuspid aortic valve (BAV) is a common congenital cardiac malformation affecting 1-2% of people. BAV results from fusion of two adjacent aortic valve cusps, and is associated with dilatation of the aorta, known as bicuspid valve associated aortopathy. Bicuspid valve aortopathy is progressive and associated with catastrophic clinical events, such as aortic dissection and rupture. Therefore, frequent monitoring and early intervention with prophylactic surgical resection of the proximal aorta is often recommended...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/28730163/brain-strokes-related-to-aortic-aneurysma-the-analysis-of-three-cases
#6
Żanna Pastuszak, Adam Stępień, Joanna Kordowska, Agnieszka Rolewska, Dariusz Galbarczyk
Brain stroke connected with aortic blood flow disturbances is a rare disease and its incidence is difficult to assume. Nevertheless, 10-50% of patients with aortic dissection may not experience any pain. In case of 18-30% patients with aortic dissection neurological signs are first disease presentation and among them ischemic stroke is the most common. The most popular aortic dissection classification is with use of Stanford system. Type A involves the ascending aorta and type B is occurring distal to the subclavian artery...
2017: Open Medicine (Warsaw, Poland)
https://www.readbyqxmd.com/read/28694784/4d-flow-analysis-of-bav-related-fluid-dynamic-alterations-evidences-of-wall-shear-stress-alterations-in-absence-of-clinically-relevant-aortic-anatomical-remodeling
#7
Filippo Piatti, Francesco Sturla, Malenka M Bissell, Selene Pirola, Massimo Lombardi, Igor Nesteruk, Alessandro Della Corte, Alberto C L Redaelli, Emiliano Votta
Bicuspid aortic valve (BAV) is the most common congenital cardiac disease and is a foremost risk factor for aortopathies. Despite the genetic basis of BAV and of the associated aortopathies, BAV-related alterations in aortic fluid-dynamics, and particularly in wall shear stresses (WSSs), likely play a role in the progression of aortopathy, and may contribute to its pathogenesis. To test whether WSS may trigger aortopathy, in this study we used 4D Flow sequences of phase-contrast cardiac magnetic resonance imaging (CMR) to quantitatively compare the in vivo fluid dynamics in the thoracic aorta of two groups of subjects: (i) five prospectively enrolled young patients with normo-functional BAV and with no aortic dilation and (ii) ten age-matched healthy volunteers...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/28683903/genetic-disorders-of-the-thoracic-aorta-and-indications-for-surgery
#8
REVIEW
Jack C J Sun
Genetic disorders of the aorta are rare but can lead to life-threatening thoracic aortic aneurysms. Although the genetic causes of many of these connective tissue diseases are well defined, others such as familial thoracic aortic aneurysm and bicuspid aortic valve aortopathy are not. The natural history of genetic thoracic aortic aneurysms is not well understood or predictable, and surgical guidelines for treatment remain imprecise. Future research should strive to provide in-depth and detailed genetic profiling to drive clinical management, including medical and surgical therapies...
August 2017: Cardiology Clinics
https://www.readbyqxmd.com/read/28671863/rethinking-the-paradigm-modern-approach-to-proximal-aortic-reconstruction-demonstrates-excellent-outcomes
#9
Jonathan M Hemli, Edward R R DeLaney, Kush R Dholakia, Dror Perk, Nirav C Patel, S Jacob Scheinerman, Derek R Brinster
BACKGROUND: Techniques for aortic surgery continue to evolve. A real-world snapshot of patients undergoing elective surgery for aneurysm in the modern era is helpful to assist in deciding the appropriate timing for intervention. We herein describe our experience with 100 consecutive patients who underwent primary elective surgery for aneurysm of the proximal thoracic aorta over a two-year period at a single institution. METHODS: The majority of our patients were male, mean age 61...
June 20, 2017: Heart Surgery Forum
https://www.readbyqxmd.com/read/28666329/increased-blood-levels-of-transforming-growth-factor-%C3%AE-in-patients-with-aortic-dilatation
#10
Carmen Rueda-Martínez, Oscar Lamas, Fernando Carrasco-Chinchilla, Juan Robledo-Carmona, Carlos Porras, Gemma Sánchez-Espín, Manuel Jiménez Navarro, Borja Fernández
OBJECTIVES: Recent studies have shown that patients with syndromic thoracic aortic aneurysm, particularly patients with bicuspid aortic valve, have increased blood levels of transforming growth factor β1 (TGF-β1), indicating this molecule as a prognostic biomarker. However, it is not known whether TGF-β1 is also elevated in the blood of patients with tricuspid aortic valve and aortic dilatation. METHODS: We analysed the plasma levels of TGF-β1 in 52 patients with tricuspid or bicuspid aortic valve and with normal or dilated ascending aorta who underwent cardiac surgery in our hospital...
June 28, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/28659818/managing-thoracic-aortic-aneurysm-in-patients-with-bicuspid-aortic-valve-based-on-aortic-root-involvement
#11
REVIEW
Elizabeth Norton, Bo Yang
Bicuspid aortic valve (BAV) can be both sporadic and hereditary, is phenotypically variable, and genetically heterogeneous. The clinical presentation of BAV is diverse and commonly associated with a high prevalence of valvular dysfunction producing altered hemodynamics and aortic abnormalities (e.g., aneurysm and dissection). The thoracic aortic aneurysm (TAA) in BAV frequently involves the proximal aorta, including the aortic root, ascending aorta, and aortic arch, but spares the aorta distal to the aortic arch...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/28655506/in-silico-shear-and-intramural-stresses-are-linked-to-aortic-valve-morphology-in-dilated-ascending-aorta
#12
S Pasta, G Gentile, G M Raffa, D Bellavia, G Chiarello, R Liotta, A Luca, C Scardulla, M Pilato
OBJECTIVE/BACKGROUND: The development of ascending aortic dilatation in patients with bicuspid aortic valve (BAV) is highly variable, and this makes surgical decision strategies particularly challenging. The purpose of this study was to identify new predictors, other than the well established aortic size, that may help to stratify the risk of aortic dilatation in BAV patients. METHODS: Using fluid-structure interaction analysis, both haemodynamic and structural parameters exerted on the ascending aortic wall of patients with either BAV (n = 21) or tricuspid aortic valve (TAV; n = 13) with comparable age and aortic diameter (42...
June 24, 2017: European Journal of Vascular and Endovascular Surgery
https://www.readbyqxmd.com/read/28651938/elevated-oxidative-stress-in-the-aortic-media-of-patients-with-bicuspid-aortic-valve
#13
Marie Billaud, Julie A Phillippi, Mary P Kotlarczyk, Jennifer C Hill, Bradley W Ellis, Claudette M St Croix, Nadiezhda Cantu-Medéllin, Eric E Kelley, Thomas G Gleason
OBJECTIVE: Congenital bicuspid aortic valve (BAV) is distinctly associated with the development of ascending aortopathy in adulthood, portending risk of both ascending aortic aneurysm and dissection. Our previous work implicated deficiency in oxidative stress response as a mediator of the BAV-associated aortopathy. We hypothesize that reactive oxygen species generation invokes elevated local oxidative tissue damage in ascending aorta of patients with BAV. METHODS: Ascending aortic specimens were obtained from patients undergoing elective aortic replacement and/or aortic valve replacement and during heart transplant operations...
May 25, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28648025/-comparison-of-aortic-valve-dysfunction-and-ascending-aorta-dimension-between-patients-with-different-bicuspid-aortic-valve-morphology
#14
X S Ren, Y T Yu, K Liu, Z H Hou, Y Gao, W H Yin, B Lyu
Objective: To compare the characteristics of aortic valve dysfunction and ascending aorta dimension in patients with different bicuspid aortic valve (BAV) morphology. Methods: A total of 197 patients who underwent aortic valve replacement between April 2014 and March 2015 and were diagnosed with BAV by pathology were included, and their clinical data were retrospectively analyzed. Patients were divided into raphe(+) group(109 cases) and raphe(-) group(88 cases) according to the presence or absence of raphe, and L-R group(fusion of left and right cusp, 125 cases) and L/R-N group(fusion of left or right and noncoronary cusp, 72 cases) according to fusion type of the cusps...
June 24, 2017: Zhonghua Xin Xue Guan Bing za Zhi
https://www.readbyqxmd.com/read/28629660/characterization-and-risk-factors-for-aortic-dilatation-in-pediatric-patients-with-bicuspid-aortic-valve
#15
Anna Sabaté-Rotés, Laura Sabidó Sanchez, Ferran Gran Ipiña, Dimpna Albert Brotons, Raúl F Abella, Ferran Rosés Noguer
INTRODUCTION AND OBJECTIVES: Dilatation of the ascending aorta associated with bicuspid aortic valve is a major cause of morbidity and mortality in adults. The main objective was to recognize the aortic involvement in children, its characteristics and risk factors. METHODS: Aortic measures of all pediatric patients with bicuspid aortic valve followed in a tertiary pediatric hospital between 1997 and 2015 were retrospectively taken. Patients with syndromes associated with aortic dilatation were excluded (n=17)...
June 16, 2017: Medicina Clínica
https://www.readbyqxmd.com/read/28623496/association-between-flow-skewness-and-aortic-dilatation-in-patients-with-aortic-stenosis
#16
Hojin Ha, Hyun Jung Koo, June Goo Lee, Guk Bae Kim, Jihoon Kweon, Sang Joon Lee, Joon Won Kang, Tae Hwan Lim, Dae Hee Kim, Jong Min Song, Duk Hyun Kang, Jae Kwan Song, Young Hak Kim, Namkug Kim, Dong Hyun Yang
We investigated association between hemodynamic characteristics and aortic dilatation in patients with severe aortic stenosis (AS). Eighty patients with severe AS (mean age, 67.2 ± 12.5 years) who underwent multi-detector computed tomography and phase-contrast magnetic resonance imaging at the ascending aorta were retrospectively analyzed. Patients with an ascending aorta diameter >4 cm had a significantly higher forward flow rate at systole (28.5 ± 6.0 vs. 36.2 ± 8.6 L min, P < 0...
June 16, 2017: International Journal of Cardiovascular Imaging
https://www.readbyqxmd.com/read/28620320/abnormal-haemodynamic-flow-patterns-in-bicuspid-pulmonary-valve-disease
#17
Malenka M Bissell, Margaret Loudon, Stefan Neubauer, Saul G Myerson
Abnormal flow patterns in the aortas of those with bicuspid aortic valves (BAVs) are increasingly recognized as important in the pathogenesis of aortic dilatation but pulmonary flow patterns in bicuspid pulmonary valves have not been studied. Bicuspid pulmonary valve disease is rare and a small numbers of case reports describe concomitant pulmonary artery dilation similar to the dilation of the ascending aorta, which is often seen in BAVs disease. We examined three cases of bicuspid pulmonary valve disease, 10 healthy volunteers and 10 patients with BAV disease but a tricuspid pulmonary valve...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/28611119/turbulent-kinetic-energy-assessed-by-multipoint-4-dimensional-flow-magnetic-resonance-imaging-provides-additional-information-relative-to-echocardiography-for-the-determination-of-aortic-stenosis-severity
#18
COMPARATIVE STUDY
Christian Binter, Alexander Gotschy, Simon H Sündermann, Michelle Frank, Felix C Tanner, Thomas F Lüscher, Robert Manka, Sebastian Kozerke
BACKGROUND: Turbulent kinetic energy (TKE), assessed by 4-dimensional (4D) flow magnetic resonance imaging, is a measure of energy loss in disturbed flow as it occurs, for instance, in aortic stenosis (AS). This work investigates the additional information provided by quantifying TKE for the assessment of AS severity in comparison to clinical echocardiographic measures. METHODS AND RESULTS: Fifty-one patients with AS (67±15 years, 20 female) and 10 healthy age-matched controls (69±5 years, 5 female) were prospectively enrolled to undergo multipoint 4D flow magnetic resonance imaging...
June 2017: Circulation. Cardiovascular Imaging
https://www.readbyqxmd.com/read/28599977/fate-of-remnant-sinuses-of-valsalva-in-patients-with-bicuspid-and-trileaflet-valves-undergoing-aortic-valve-ascending-aorta-and-aortic-arch-replacement
#19
Rita Karianna Milewski, Andreas Habertheuer, Joseph E Bavaria, Mary Siki, Wilson Y Szeto, Eric Krause, Varun Korutla, Nimesh D Desai, Prashanth Vallabhajosyula
OBJECTIVE: In patients presenting with aortic valvulopathy with concomitant ascending aortic aneurysm, surgical management of the sinus of Valsalva segment remains undefined, especially for moderately dilated aortic roots. In patients with this pathology undergoing aortic valve replacement with supracoronary ascending aorta replacement, we assessed the fate of the remnant preserved sinus of Valsalva segment stratified by aortic valve morphology and pathology. METHODS: From 2002 to 2015, 428 patients underwent elective aortic valve replacement with supracoronary ascending aorta replacement...
August 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28596303/prevalence-and-predictors-of-intracranial-aneurysms-in-patients-with-bicuspid-aortic-valve
#20
Alexander C Egbe, Ratnasari Padang, Robert D Brown, Arooj R Khan, Sushil A Luis, John Huston, Emmanuel Akintoye, Heidi M Connolly
OBJECTIVE: To determine the prevalence and outcomes of intracranial aneurysm (IA) in patients with bicuspid aortic valve (BAV) METHODS: Retrospective review of patients with BAV who underwent brain MR angiography at the Mayo Clinic from 1994 to 2013. RESULTS: There were 678 patients included in this study-mean age 57±13 years, men 480 (71%), mean follow-up 10±3 years (5913 patient-years). Coarctation of aorta (COA) was present in 154 (23%) patients.There were 59 IAs identified in 52 of 678 patients (7...
June 8, 2017: Heart: Official Journal of the British Cardiac Society
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