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Marfan flow

Dianna M Milewicz, Kathleen M Trybus, Dong-Chuan Guo, H Lee Sweeney, Ellen Regalado, Kristine Kamm, James T Stull
The importance of maintaining contractile function in aortic smooth muscle cells (SMCs) is evident by the fact that heterozygous mutations in the major structural proteins or kinases controlling contraction lead to the formation of aneurysms of the ascending thoracic aorta that predispose to life-threatening aortic dissections. Force generation by SMC requires ATP-dependent cyclic interactions between filaments composed of SMC-specific isoforms of α-actin (encoded by ACTA2) and myosin heavy chain (MYH11). ACTA2 and MYH11 mutations are predicted or have been shown to disrupt this cyclic interaction predispose to thoracic aortic disease...
January 2017: Arteriosclerosis, Thrombosis, and Vascular Biology
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
Chikao Teramoto, Osamu Kawaguchi, Yoshimori Araki, Masaharu Yoshikawa, Wataru Uchida, Gennta Takemura, Naoki Makino
In patients with Marfan syndrome, cardiovascular complication due to aortic dissection represents the primary cause of death. Iatrogenic acute aortic dissection during cardiac surgery is a rare, but serious adverse event. A 51-year-old woman with Marfan syndrome underwent elective aortic surgery and mitral valve reconstruction surgery for the enlarged aortic root and severe mitral regurgitation. We replaced the aortic root and ascending aorta based on reimplantation technique. During subsequent mitral valve reconstruction, we found the heart pushed up from behind...
August 2016: Kyobu Geka. the Japanese Journal of Thoracic Surgery
Ziad Mallat, Alain Tedgui, Daniel Henrion
The pathophysiology of aortic dissection is poorly understood, and its risk is resistant to medical treatment. Most studies have focused on a proposed pathogenic role of transforming growth factor-β in Marfan disease and related thoracic aortic aneurysms and aortic dissections. However, clinical testing of this concept using angiotensin II type 1 receptor antagonists to block transforming growth factor-β signaling fell short of promise. Genetic mutations that predispose to thoracic aortic aneurysms and aortic dissections affect components of the extracellular matrix and proteins involved in cellular force generation...
September 2016: Arteriosclerosis, Thrombosis, and Vascular Biology
Michael E Reznik, Aixa Damaris Espinosa-Morales, Mouhammad A Jumaa, Syed Zaidi, Andrew F Ducruet, Ashutosh P Jadhav
BACKGROUND: Recent studies have validated the use of endovascular thrombectomy in large vessel ischemic stroke provided patients are selected appropriately. However, to our knowledge, there have been no previously reported cases of endovascular thrombectomy in patients with aortic dissection. We report three such cases, two with chronic aortic dissections (including one with a history of Marfan syndrome) and another with an acute type B dissection. METHODS: Case studies and review of relevant literature...
January 2017: Journal of Neurointerventional Surgery
Hiroaki Toyama, Yusuke Takei, Kazutomo Saito, Takahisa Ota, Kenji Kurotaki, Yutaka Ejima, Takeshi Matsuura, Masatoshi Akiyama, Yoshikatsu Saiki, Masanori Yamauchi
A male patient with Marfan syndrome underwent aortic root replacement and developed left ventricular (LV) failure. Four years later, he underwent aortic arch and aortic valve replacement. Thereafter, his LV failure progressed, and cardiogenic pulmonary edema (CPE) appeared, which we treated with extracorporeal LV assist device (LVAD) placement. Three months later, the patient developed aspiration pneumonia, which caused hyperdynamic right ventricle (RV) and CPE. We treated by changing his pneumatic LVAD to a high-flow centrifugal pump...
August 2016: Journal of Anesthesia
Hung-Hsuan Wang, Hsin-Hui Chiu, Wen-Yih Isaac Tseng, Hsu-Hsia Peng
PURPOSE: To examine possible hemodynamic alterations in adolescent to adult Marfan syndrome (MFS) patients with aortic root dilatation. MATERIALS AND METHODS: Four-dimensional flow MRI was performed in 20 MFS patients and 12 age-matched normal subjects with a 3T system. The cross-sectional areas of 10 planes along the aorta were segmented for calculating the axial and circumferential wall shear stress (WSSaxial , WSScirc ), oscillatory shear index (OSIaxial , OSIcirc ), and the nonroundness (NR), presenting the asymmetry of segmental WSS...
August 2016: Journal of Magnetic Resonance Imaging: JMRI
S D Singh, X Y Xu, N B Wood, J R Pepper, C Izgi, T Treasure, R H Mohiaddin
Implantation of a personalised external aortic root support (PEARS) in the Marfan aorta is a new procedure that has emerged recently, but its haemodynamic implication has not been investigated. The objective of this study was to compare the flow characteristics and hemodynamic indices in the aorta before and after insertion of PEARS, using combined cardiovascular magnetic resonance imaging (CMR) and computational fluid dynamics (CFD). Pre- and post-PEARS MR images were acquired from 3 patients and used to build patient-specific models and upstream flow conditions, which were incorporated into the CFD simulations...
January 4, 2016: Journal of Biomechanics
Marina Colombi, Chiara Dordoni, Nicola Chiarelli, Marco Ritelli
Joint hypermobility syndrome/Ehlers-Danlos syndrome hypermobility type (JHS/EDS-HT) is an evolving and protean disorder mostly recognized by generalized joint hypermobility and without a defined molecular basis. JHS/EDS-HT also presents with other connective tissue features affecting a variety of structures and organs, such as skin, eye, bone, and internal organs. However, most of these signs are present in variable combinations and severity in many other heritable connective tissue disorders. Accordingly, JHS/EDS-HT is an "exclusion" diagnosis which needs the absence of any consistent feature indicative of other partially overlapping connective tissue disorders...
March 2015: American Journal of Medical Genetics. Part C, Seminars in Medical Genetics
Martin T Yates, Gopal Soppa, Jeremy Smelt, Nick Fletcher, Jean-Pierre van Besouw, Basky Thilaganathan, Marjan Jahangiri
OBJECTIVE: Pathology of the aortic valve and ascending thoracic aorta is an uncommon but life-threatening complication of pregnancy. Cardiac surgery during pregnancy is known to carry a high risk of mortality to both the mother and fetus. We present our experience of performing aortic surgery during the patients' pregnancy. METHODS: All patients undergoing aortic surgery during pregnancy at St George's Hospital, from January 2004 until October 2013, were identified...
February 2015: Journal of Thoracic and Cardiovascular Surgery
George G S Sandor, Mohammed H Alghamdi, Leslie A Raffin, Mary T Potts, Lindsey D Williams, James E Potts, Marla Kiess, Casey van Breemen
BACKGROUND: Patients with Marfan (MFS) and Loeys-Dietz (LDS) syndromes have been shown to have abnormal aortic biophysical properties. The purpose of this study was to compare the effects of 12-months of therapy with atenolol or losartan on vascular function in young patients with MFS and LDS. METHODS: Seventeen patients with MFS or LDS were recruited and randomized to treatment with atenolol, 25-50mg, or losartan, 25mg daily. Prior to treatment and following therapy, echocardiography for left ventricular size, function and aortic root size was performed...
January 20, 2015: International Journal of Cardiology
Giorgio Querzoli, Stefania Fortini, Stefania Espa, Martina Costantini, Francesca Sorgini
Aortic root dilation and propensity to dissection are typical manifestations of the Marfan Syndrome (MS), a genetic defect leading to the degeneration of the elastic fibres. Dilation affects the structure of the flow and, in turn, altered flow may play a role in vessel dilation, generation of aneurysms, and dissection. The aim of the present work is the investigation in-vitro of the fluid dynamic modifications occurring as a consequence of the morphological changes typically induced in the aortic root by MS...
September 22, 2014: Journal of Biomechanics
Danilo Babin, Daniel Devos, Aleksandra Pižurica, Jos Westenberg, Ewout Vansteenkiste, Wilfried Philips
Aortic stiffness has proven to be an important diagnostic and prognostic factor of many cardiovascular diseases, as well as an estimate of overall cardiovascular health. Pulse wave velocity (PWV) represents a good measure of the aortic stiffness, while the aortic distensibility is used as an aortic elasticity index. Obtaining the PWV and the aortic distensibility from magnetic resonance imaging (MRI) data requires diverse segmentation tasks, namely the extraction of the aortic center line and the segmentation of aortic regions, combined with signal processing methods for the analysis of the pulse wave...
April 2014: Computerized Medical Imaging and Graphics: the Official Journal of the Computerized Medical Imaging Society
Pablo Lamata, Alex Pitcher, Sebastian Krittian, David Nordsletten, Malenka M Bissell, Thomas Cassar, Alex J Barker, Michael Markl, Stefan Neubauer, Nicolas P Smith
PURPOSE: To describe the assessment of the spatiotemporal distribution of relative aortic pressure quantifying the magnitude of its three major components. METHODS: Nine healthy volunteers and three patients with aortic disease (bicuspid aortic valve, dissection, and Marfan syndrome) underwent 4D-flow CMR. Spatiotemporal pressure maps were computed from the CMR flow fields solving the pressure Poisson equation. The individual components of pressure were separated into time-varying inertial ("transient"), spatially varying inertial ("convective"), and viscous components...
October 2014: Magnetic Resonance in Medicine: Official Journal of the Society of Magnetic Resonance in Medicine
N Mangner, G Schuler
In 2012 the new and collaborative "Guidelines on the management of valvular heart disease (version 2012)" were published by the European Society of Cardiology (ESC) and the European Association for Cardio-Thoracic Surgery (EACTS). These guidelines emphasize that decision-making in patients with valvular heart disease should ideally be carried out by a"heart team" with particular expertise in valvular heart disease. In aortic regurgitation pathologies of the aortic root are frequent and in patients with Marfan syndrome, surgery is indicated when the maximal ascending aortic diameter is ≥50 mm, while the threshold for intervention should be lower in patients with risk factors for progression...
December 2013: Herz
Jake Sloane, Abdul Aziz, Khalid Makhdoomi
INTRODUCTION: A 32 year old man presented to our vascular clinic with a lump over the left eyebrow. It had become larger in size and was cosmetically unacceptable. PRESENTATION OF CASE: Clinical examination of the lump demonstrated a pulsatile swelling consistent with an aneurysm of the superficial temporal artery (STA). Doppler ultrasound demonstrated arterial flow within the lump that could be controlled with pressure over the proximal branch of the vessel. The STA aneurysm was excised under local anaesthesia by ligation of the feeding and draining branches...
2013: International Journal of Surgery Case Reports
Jennifer K Wagner
Whether the integration of genetic/omic technologies in sports contexts will facilitate player success, promote player safety, or spur genetic discrimination depends largely upon the game rules established by those currently designing genomic sports medicine programs. The integration has already begun, but there is not yet a playbook for best practices. Thus far discussions have focused largely on whether the integration would occur and how to prevent the integration from occurring, rather than how it could occur in such a way that maximizes benefits, minimizes risks, and avoids the exacerbation of racial disparities...
2013: PeerJ
Munenori Takata, Eisuke Amiya, Masafumi Watanabe, Kazuko Omori, Yasushi Imai, Daishi Fujita, Hiroshi Nishimura, Masayoshi Kato, Tetsuro Morota, Kan Nawata, Atsuko Ozeki, Aya Watanabe, Shuichi Kawarasaki, Yumiko Hosoya, Tomoko Nakao, Koji Maemura, Ryozo Nagai, Yasunobu Hirata, Issei Komuro
Marfan syndrome is an inherited disorder characterized by genetic abnormality of microfibrillar connective tissue proteins. Endothelial dysfunction is thought to cause aortic dilation in subjects with a bicuspid aortic valve; however, the role of endothelial dysfunction and endothelial damaging factors has not been elucidated in Marfan syndrome. Flow-mediated dilation, a noninvasive measurement of endothelial function, was evaluated in 39 patients with Marfan syndrome. Aortic diameter was measured at the aortic annulus, aortic root at the sinus of Valsalva, sinotubular junction and ascending aorta by echocardiography, and adjusted for body surface area (BSA)...
July 2014: Heart and Vessels
Atsushi Kitagawa, Roy K Greenberg, Matthew J Eagleton, Tara M Mastracci, Eric E Roselli
OBJECTIVE: The treatment of patients with arch and thoracoabdominal aortic aneurysms (TAAAs) and chronic dissections is challenging. We report the results of fenestrated and branched endovascular aortic repair (FEVAR) of such aneurysms. METHODS: A single-center prospective FEVAR trial enrolled 356 patients (2006 to 2011), of whom 30 had chronic dissections with arch aneurysm or TAAAs, or both. Patients were divided into group A, 15 patients (mean age, 58 years) with extensive dissections extending from the arch through the visceral segment, and group B, 15 patients (mean age, 74 years old) with focal dissections and no extension into the thoracic aorta...
September 2013: Journal of Vascular Surgery
Thomas A Hope, John-Peder Escobar Kvitting, Michael D Hope, D Craig Miller, Michael Markl, Robert J Herfkens
BACKGROUND: Over the past two decades elective valve-sparing aortic root replacement (V-SARR) has become more common in the treatment of patients with aortic root and ascending aortic aneurysms. Currently there are little data available to predict complications in the post-operative population. The study goal was to determine if altered flow patterns in the thoracic aorta, as measured by MRI, are associated with complications after V-SARR. METHODS: Time-resolved three-dimensional phase-contrast MRI (4D flow) was used to image 12 patients with Marfan syndrome after V-SARR...
November 2013: Magnetic Resonance Imaging
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