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https://www.readbyqxmd.com/read/29778849/influence-of-aortic-dilation-on-the-regional-aortic-stiffness-of-bicuspid-aortic-valve-assessed-by-4-dimensional-flow-cardiac-magnetic-resonance-comparison-with-marfan-syndrome-and-degenerative-aortic-aneurysm
#1
Andrea Guala, Jose Rodriguez-Palomares, Lydia Dux-Santoy, Gisela Teixido-Tura, Giuliana Maldonado, Laura Galian, Marina Huguet, Filipa Valente, Laura Gutiérrez, Teresa González-Alujas, Kevin M Johnson, Oliver Wieben, Augusto Sao Avilés, David Garcia-Dorado, Arturo Evangelista
OBJECTIVES: This study sought to ascertain whether patients with a bicuspid aortic valve (BAV) have an intrinsic alteration in regional aortic stiffness compared with patients with tricuspid aortic valve (TAV) and Marfan syndrome (MFS) patients with similar aortic sizes, as well as to assess the influence of ascending aorta (AAo) dilation on regional stiffness parameters in BAV patients. BACKGROUND: Imaging biomarkers as predictors of BAV, MFS, and degenerative AAo aneurysms in TAV patients (DA-TAV) are lacking...
May 11, 2018: JACC. Cardiovascular Imaging
https://www.readbyqxmd.com/read/29682122/surgical-treatment-of-a-left-axillary-arterial-aneurysm-with-marfan-syndrome-by-combining-two-skin-incisions
#2
Hiroshi Sato, Syuichi Naraoka
An axillary arterial aneurysm is a rare disorder, with few surgical cases reported to date. Here we report the case of a left axillary arterial aneurysm associated with Marfan syndrome. A 44-year-old female with Marfan syndrome presented with numbness of the left upper limb and a pulsatile mass on the left chest. A computed tomography scan revealed a highly enlarged and tortuous left axillary arterial aneurysm. We performed a graft replacement and resection of the aneurysm with two skin incisions. After the surgery, the blood flow to the patient's left upper limb was confirmed, and the neurological symptoms improved significantly...
March 25, 2018: Annals of Vascular Diseases
https://www.readbyqxmd.com/read/29286269/landmark-lecture-perloff-lecture-tribute-to-professor-joseph-kayle-perloff-and-lessons-learned-from-him-aortopathy-in-adults-with-chd
#3
Koichiro Niwa
Marfan syndrome, bicuspid aortic valve, and/or coarctation of the aorta are associated with medial abnormalities of the ascending aortic or para-coarctation aorta. Medial abnormalities in the ascending aorta are prevalent in other type of patients with a variety of CHDs such as single ventricle, persistent truncus arteriosus, transposition of the great arteries, hypoplastic left heart syndrome, and tetralogy of Fallot, encompassing a wide age range and may predispose to dilatation, aneurysm, and rapture necessitating aortic valve and root surgery...
December 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28780662/bicuspid-aortic-valvulopathy-and-associated-aortopathy-a-review-of-contemporary-studies-relevant-to-clinical-decision-making
#4
REVIEW
Michael H Kwon, Thoralf M Sundt
The bicuspid aortic valve (BAV) phenotype is becoming increasingly recognized as a complex and heterogeneous clinical entity, with some but not all patients developing accelerated degrees of both aortic insufficiency (AI) and aortic stenosis (AS) in comparison to patients with tricuspid aortic valves (TAV). In addition, there remains a well-established association between the BAV phenotype and aortic enlargement independent of valve function as well as progression among some to ascending aortic aneurysm and the attendant concern over risk of aortic dissection...
September 2017: Current Treatment Options in Cardiovascular Medicine
https://www.readbyqxmd.com/read/28761511/genetic-associations-of-intracranial-aneurysm-formation-and-sub-arachnoid-hemorrhage
#5
REVIEW
Christian B Theodotou, Brian M Snelling, Samir Sur, Diogo C Haussen, Eric C Peterson, Mohamed Samy Elhammady
Risk factors for cerebral aneurysms typically include age, hypertension, smoking, and alcohol usage. However, the possible connection of aneurysms with genetic conditions such as Marfan's syndrome, polycystic kidney disease, and neurofibromatosis raises the question of possible genetic risk factors for aneurysm, and additionally, genetic risk factors for rupture. We conducted a literature review using the PubMed database for studies regarding genetic correlation with cerebral aneurysm formation as well as rupture from December 2008 to Jun 2015...
July 2017: Asian Journal of Neurosurgery
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/28616536/nt-probnp-and-diastolic-left-ventricular-function-in-patients-with-marfan-syndrome
#7
Petra Gehle, Peter N Robinson, Frank Heinzel, Frank Edelmann, Mustafa Yigitbasi, Felix Berger, Volkmar Falk, Burkert Pieske, Ernst Wellnhofer
AIMS: Subclinical diastolic dysfuntion in patients with preclinical heart failure with preserved ejection fraction (HFpEF) has been demonstrated in patients with Marfan syndrome (MFS). We investigated the relationship between diastolic dysfunction and NT-proBNP levels in patients with MFS. METHODS AND RESULTS: NT-proBNP, C-reactive protein (CRP) and diastolic function were assessed in 217 patients with MFS (31 ± 16 y, 110 f. and in 339 patients referred for suspected MFS in whom the diagnosis was ruled out according to the Ghent nosology (30 ± 15 y, 154 f)...
September 2016: IJC Heart & Vasculature
https://www.readbyqxmd.com/read/28392101/surgical-and-endovascular-treatments-of-extracranial-carotid-artery-aneurysms-report-of-six-cases
#8
Shusuke Yamamoto, Naoki Akioka, Daina Kashiwazaki, Masaki Koh, Naoya Kuwayama, Satoshi Kuroda
BACKGROUND: Although the natural course of extracranial carotid artery aneurysms (ECAAs) is still unknown, they may cause stroke or cranial nerve dysfunction unless they are treated. In this report, we reviewed the clinical results of 6 patients who underwent endovascular and surgical treatments for ECAAs. METHODS: A total of 6 patients underwent endovascular and surgical treatments for ECAAs for 9 years. The primary causes of ECAAs included Marfan syndrome (1 patient), infection (1 patient), trauma (2 patients), and unknown (2 patients)...
July 2017: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/28327193/longitudinal-evaluation-of-aortic-hemodynamics-in-marfan-syndrome-new-insights-from-a-4d-flow-cardiovascular-magnetic-resonance-multi-year-follow-up-study
#9
Julia Geiger, Daniel Hirtler, Kristina Gottfried, Ozair Rahman, Emilie Bollache, Alex J Barker, Michael Markl, Brigitte Stiller
BACKGROUND: The aim of this 4D flow cardiovascular magnetic resonance (CMR) follow-up study was to investigate longitudinal changes in aortic hemodynamics in adolescent patients with Marfan syndrome (MFS). METHODS: 4D flow CMR for the assessment of in-vivo 3D blood flow with full coverage of the thoracic aorta was performed twice (baseline scan t1/follow-up scan t2) in 19 adolescent MFS patients (age at t1: 12.7 ± 3.6 years, t2: 16.2 ± 4.3 years) with a mean follow-up duration of 3...
March 22, 2017: Journal of Cardiovascular Magnetic Resonance
https://www.readbyqxmd.com/read/28317480/novel-insights-into-complex-cardiovascular-pathologies-using-4d-flow-analysis-by-cardiovascular-magnetic-resonance-imaging
#10
Adam James Lewandowski, Betty Raman, Rajarshi Banerjee, Matteo Milanesi
BACKGROUND: Blood flow assessment is essential to fully understand cardiovascular function in disease pathologies and for identification of individuals at long-term risk of cardiovascular disease development. Qualitative and quantitative assessments of blood flow by imaging modalities have been limited, and much of the accurate quantification has relied on invasive measures. METHODS: This review discusses how four-dimensional velocity cardiovascular magnetic resonance (4D flow CMR) offers increasing potential for the non-invasive assessment of blood flow in the heart and major blood vessels such as the aorta...
2017: Current Pharmaceutical Design
https://www.readbyqxmd.com/read/28302143/altered-aortic-3d-hemodynamics-and-geometry-in-pediatric-marfan-syndrome-patients
#11
Roel L F van der Palen, Alex J Barker, Emilie Bollache, Julio Garcia, Michael J Rose, Pim van Ooij, Luciana T Young, Arno A W Roest, Michael Markl, Joshua D Robinson, Cynthia K Rigsby
BACKGROUND: Blood flow dynamics make it possible to better understand the development of aortopathy and cardiovascular events in patients with Marfan syndrome (MFS). Aortic 3D blood flow characteristics were investigated in relation to aortic geometry in children and adolescents with MFS. METHODS: Twenty-five MFS patients (age 15.6 ± 4.0 years; 11 females) and 21 healthy controls (age 16.0 ± 2.6 years; 12 females) underwent magnetic resonance angiography and 4D flow CMR for assessment of thoracic aortic size and 3D blood flow velocities...
March 17, 2017: Journal of Cardiovascular Magnetic Resonance
https://www.readbyqxmd.com/read/27879251/altered-smooth-muscle-cell-force-generation-as-a-driver-of-thoracic-aortic-aneurysms-and-dissections
#12
REVIEW
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
https://www.readbyqxmd.com/read/27816335/developmental-changes-in-aortic-mechanical-properties-in-normal-fetuses-and-fetuses-with-cardiovascular-disease
#13
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)...
June 2017: Pediatrics and Neonatology
https://www.readbyqxmd.com/read/27476568/-intra-operative-acute-aortic-dissection-during-aortic-root-reimplantation-and-mitral-valve-reconstruction-surgery-in-a-patient-with-marfan-syndrome-report-of-a-case
#14
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
https://www.readbyqxmd.com/read/27444198/role-of-microvascular-tone-and-extracellular-matrix-contraction-in-the-regulation-of-interstitial-fluid-implications-for-aortic-dissection
#15
REVIEW
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
https://www.readbyqxmd.com/read/27199384/endovascular-thrombectomy-in-the-setting-of-aortic-dissection
#16
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
https://www.readbyqxmd.com/read/27001080/management-of-three-cardiogenic-pulmonary-edemas-occurring-in-a-patient-scheduled-for-left-ventricular-assist-device-implantation-indicators-for-determining-left-ventricular-assist-device-pump-speed
#17
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
https://www.readbyqxmd.com/read/26854646/does-altered-aortic-flow-in-marfan-syndrome-relate-to-aortic-root-dilatation
#18
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
https://www.readbyqxmd.com/read/26654673/aortic-flow-patterns-before-and-after-personalised-external-aortic-root-support-implantation-in-marfan-patients
#19
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
https://www.readbyqxmd.com/read/25821090/differential-diagnosis-and-diagnostic-flow-chart-of-joint-hypermobility-syndrome-ehlers-danlos-syndrome-hypermobility-type-compared-to-other-heritable-connective-tissue-disorders
#20
REVIEW
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
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