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https://www.readbyqxmd.com/read/29334594/thoracic-aortic-aneurysm-in-patients-with-loss-of-function-filamin-a-mutations-clinical-characterization-genetics-and-recommendations
#1
Ming Hui Chen, Sangita Choudhury, Mami Hirata, Siri Khalsa, Bernard Chang, Christopher A Walsh
The frequency and gender distribution of thoracic aortic aneurysm as a cardiovascular manifestation of loss-of-function (LOF) X-linked FilaminA (FLNA) mutations are not known. Furthermore, there is very limited cardiovascular morbidity or mortality data in children and adults. We analyzed cardiac data on the largest series of 114 patients with LOF FLNA mutations, both children and adults, with periventricular nodular heterotopia (PVNH), including 48 study patients and 66 literature patients, median age of 22...
February 2018: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/29334508/practical-valvular-issues-in-patients-requiring-ventricular-assist-devices
#2
Mitesh Badiwala, Terrence Yau
PURPOSE OF REVIEW: As ventricular assist device (VAD) therapy in patients with advanced heart failure continues to grow, experience with concomitant valvular diseases present either before or after VAD implantation continues to accrue. In this review, we discuss recent data and current practice as it pertains to the subject of concomitant valvular disease in patients requiring VADs. RECENT FINDINGS: Persistent aortic valve closure has been identified as a potential contributor to aortic valve 'disuse atrophy' resulting in valve degeneration...
January 13, 2018: Current Opinion in Cardiology
https://www.readbyqxmd.com/read/29332920/aorto-cutaneous-fistula-after-surgical-treatment-of-stanford-type-a-aortic-dissection
#3
Chenyu Zhao, Zhicheng Zhu, Xiaomei Zheng, Kexiang Liu
An aorto-cutaneous fistula is a rare complication that occurs after aortic surgery. Due to its rarity, postoperative complications are not normally highlighted in most standard teaching. We report here a case of aorto-cutaneous fistula after surgical treatment of a Stanford type A aortic dissection (AD) in a 67-year-old Chinese male. The patient presented with severe right heart dysfunction and a mass was found in the upper-middle of his chest, which started bleeding in the next years. On admission, preoperative aortic computed tomography angiography (CTA) showed a huge hematoma located in the anterior superior mediastinum and a shunt between the embedding cavity of the aortic root and right atrium...
January 15, 2018: International Heart Journal
https://www.readbyqxmd.com/read/29329990/transcatheter-aortic-valve-implantation-in-morphologically-complex-root-aneurysms
#4
Markus Mach, Bernhard Winkler, David Santer, Harald Pisarik, Sandra Folkmann, Marieluise Harrer, Gabriel Weiss, Franz Veit, Christoph Adlbrecht, Andreas Strouhal, Georg Delle-Karth, Martin Grabenwöger
Performing transcatheter aortic valve implantation in the presence of aneurysmatic aortopathy is widely contraindicated; however, it needs to be taken into account as bailout strategy in selected patients. Deliberate preoperative assessing measurements become the crucial key element in this context. After meticulous valve selection, retrograde access is obtained via the right subclavian artery additionally serving as backup arterial cannulation site in case of conversion. TAVI is then performed via the transapical route...
January 9, 2018: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29329825/effectiveness-and-safety-of-transcatheter-aortic-valve-implantation-in-patients-with-pure-aortic-regurgitation-and-advanced-heart-failure
#5
Gabriele Pesarini, Mattia Lunardi, Anna Piccoli, Leonardo Gottin, Daniele Prati, Valeria Ferrero, Roberto Scarsini, Aldo Milano, Alberto Forni, Giuseppe Faggian, Flavio Ribichini
Results of transcatheter aortic valve implantation (TAVI) for treatment of severe noncalcific isolated aortic regurgitation (AR) complicated by advanced heart failure or cardiogenic shock has been previously reported only in isolated case reports. Current self-expanding transcatheter aortic valves are designed to treat aortic valve stenosis, and have also been implanted in cases of severe AR due to degenerated bioprosthesis and in very few cases of native aortic valves. We report 13 consecutive inoperable patients with noncalcific, pure AR, and advanced heart failure treated with emergency percutaneous transfemoral implantation with self-expandable CoreValves at our institution between July 2012 and September 2017...
December 11, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/29327146/mid-term-outcome-of-100-consecutive-ross-procedures-excellent-survival-but-yet-to-be-a-cure
#6
Corina Zimmermann, Christine Attenhofer Jost, René Prêtre, Christoph Mueller, Matthias Greutmann, Burkhardt Seifert, Emanuela Valsangiacomo Büchel, Oliver Kretschmar, Hitendu Hasmukhlal Dave, Roland Weber
The Ross procedure offers excellent short-term outcome but the long-term durability is under debate. Reinterventions and follow-up of 100 consecutive patients undergoing Ross Procedure at our centre (1993-2011) were analysed. Follow-up was available for 96 patients (97%) with a median duration of 5.3 (0.1-17.1) years. Median age of the patient cohort was 15.2 (0.04-58.4) years with 76 males. 93% had underlying congenital aortic stenosis. Root replacement technique was applied in all. The most common valved conduits used for reconstruction of the right ventricular outflow tract were homografts (66 patients) and bovine jugular vein (ContegraR) graft (31 patients)...
January 12, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29325103/twenty-year-experience-with-stentless-biological-aortic-valve-and-root-replacement-informing-patients-of-risks-and-benefits
#7
Adriaan W Schneider, Hein Putter, Mark G Hazekamp, Eduard R Holman, Eline F Bruggemans, Michel I M Versteegh, Martin J Schalij, Rens R B Varkevisser, Robert J M Klautz, Jerry Braun
OBJECTIVES: The aim of this study was to provide predictive data on the performance of the Freestyle stentless bioprosthesis that can be used to support and improve the shared decision-making process of prosthetic valve choice for aortic valve replacement. METHODS: Between 1993 and 2014, 604 patients received the Freestyle stentless bioprosthesis (143 subcoronary, 58 root inclusion and 403 full-root replacement). Perioperative data were collected retrospectively, and follow-up data were collected prospectively from 2015...
January 6, 2018: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/29324511/evolutionary-improvements-in-the-jarvik-2000-left-ventricular-assist-device
#8
Craig H Selzman, Antigone Koliopoulou, Jason P Glotzbach, Stephen H McKellar
Mechanical circulatory support devices experience a wide range of operating conditions during patient use. Since its first implant in June 2000, the Jarvik 2000 left ventricular assist device has witnessed systematic stepwise modifications to reduce the risk of serious adverse events and improve patient outcomes. Over time, clinical experience revealed a number of low-incidence failure modes that presented opportunities for improvement. Design changes have included, but are not limited to, a Y cable to permit battery changes without pump stoppage, increased pull strength of external cables from 35 to 200 lbs, an intermittent low-speed controller to improve aortic root washout, sintered titanium microsphere surface on the pump housing to prevent apical thrombus, and novel cone bearings to reduce thrombus formation...
January 10, 2018: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
https://www.readbyqxmd.com/read/29323665/arterial-tortuosity-syndrome-40-new-families-and-literature-review
#9
Aude Beyens, Juliette Albuisson, Annekatrien Boel, Mazen Al-Essa, Waheed Al-Manea, Damien Bonnet, Ozlem Bostan, Odile Boute, Tiffany Busa, Nathalie Canham, Ergun Cil, Paul J Coucke, Margot A Cousin, Majed Dasouki, Julie De Backer, Anne De Paepe, Sofie De Schepper, Deepthi De Silva, Koenraad Devriendt, Inge De Wandele, David R Deyle, Harry Dietz, Sophie Dupuis-Girod, Eudice Fontenot, Björn Fischer-Zirnsak, Alper Gezdirici, Jamal Ghoumid, Fabienne Giuliano, Neus Baena Diéz, Mohammed Z Haider, Joshua S Hardin, Xavier Jeunemaitre, Eric W Klee, Uwe Kornak, Manuel F Landecho, Anne Legrand, Bart Loeys, Stanislas Lyonnet, Helen Michael, Pamela Moceri, Shehla Mohammed, Laura Muiño-Mosquera, Sheela Nampoothiri, Karin Pichler, Katrina Prescott, Anna Rajeb, Maria Ramos-Arroyo, Massimiliano Rossi, Mustafa Salih, Mohammed Z Seidahmed, Elise Schaefer, Elisabeth Steichen-Gersdorf, Sehime Temel, Fahrettin Uysal, Marine Vanhomwegen, Lut Van Laer, Lionel Van Maldergem, David Warner, Andy Willaert, Tom R Collins, Andrea Taylor, Elaine C Davis, Yuri Zarate, Bert Callewaert
PurposeWe delineate the clinical spectrum and describe the histology in arterial tortuosity syndrome (ATS), a rare connective tissue disorder characterized by tortuosity of the large and medium-sized arteries, caused by mutations in SLC2A10.MethodsWe retrospectively characterized 40 novel ATS families (50 patients) and reviewed the 52 previously reported patients. We performed histology and electron microscopy (EM) on skin and vascular biopsies and evaluated TGF-β signaling with immunohistochemistry for pSMAD2 and CTGF...
January 11, 2018: Genetics in Medicine: Official Journal of the American College of Medical Genetics
https://www.readbyqxmd.com/read/29322433/biological-aortic-valve-replacement-advantages-and-optimal-indications-of-stentless-compared-to-stented-valve-substitutes-a-review
#10
REVIEW
Reza Tavakoli, Pichoy Danial, Ahmed Hamid Oudjana, Peiman Jamshidi, Max Gassmann, Pascal Leprince, Guillaume Lebreton
Controversy still surrounds the optimal biological valve substitute for aortic valve replacement. In light of the current literature, we review advantages and optimal indications of stentless compared to stented aortic bio-prostheses. Recent meta-analyses, prospective randomized controlled trials and retrospective studies comparing the most frequently used stentless and stented aortic bio-prostheses were analyzed. In the present review, the types and implantation techniques of the bio-prosthesis that are seldom taken into account by most studies and reviews were integrated in the interpretation of the relevant reports...
January 10, 2018: General Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29321389/aortic-root-calcification-score-as-an-independent-factor-for-predicting-major-adverse-cardiac-events-in-familial-hypercholesterolemia
#11
Hirofumi Okada, Hayato Tada, Kenshi Hayashi, Hiroki Kawashima, Tadanori Takata, Kenji Sakata, Atsushi Nohara, Hiroshi Mabuchi, Masakazu Yamagishi, Masa-Aki Kawashiri
AIM: The aims of this study were: 1) to determine whether the accumulation of aortic root calcification (ARC) assessed using coronary computed tomography angiography (CCTA) can predict future cardiovascular events, and 2) to estimate the onset and progression of ARC in patients with familial hypercholesterolemia (FH). METHODS: One hundred thirteen consecutive Japanese patients with heterozygous FH (male=54, mean age=52.1±15.6 years, mean LDL-C=299.0±94.6 mg/dL), without known coronary artery disease, who underwent 64-detector row CCTA were retrospectively evaluated...
January 10, 2018: Journal of Atherosclerosis and Thrombosis
https://www.readbyqxmd.com/read/29321129/novel-reversible-model-of-atherosclerosis-and-regression-using-oligonucleotide-regulation-of-the-ldl-receptor
#12
Debapriya Basu, Yunying Hu, Lesley-Ann Huggins, Adam E Mullick, Mark J Graham, Tomasz A Wietecha, Shelley L Barnhart, Allison Mogul, Katharina Pfeiffer, Andreas Zirlik, Edward A Fisher, Karin E Bornfeldt, Florian Willecke, Ira J Goldberg
Rationale: Animal models have been used to explore factors that regulate atherosclerosis. More recently, they have been used to study the factors that promote loss of macrophages and reduction in lesion size after lowering of plasma cholesterol levels. However, current animal models of atherosclerosis regression require challenging surgeries, time-consuming breeding strategies, and/or methods that block liver lipoprotein secretion. Objective: We sought to develop a more direct and time-effective method to create and then reverse hypercholesterolemia as well as atherosclerosis via transient knockdown of the hepatic LDL receptor (LDLR) followed by its rapid restoration...
January 10, 2018: Circulation Research
https://www.readbyqxmd.com/read/29317993/transcatheter-aortic-valve-implantation-operators-get-involved-in-imaging
#13
Miriam Brinkert, Stefan Toggweiler
Pre-procedural planning is the key element of transcatheter aortic valve implantation (TAVI). Multislice computed tomography of the chest, abdomen and pelvis with the ability to perform a 3-dimensional reconstruction has become the cornerstone of pre-procedural planning. We would like to encourage TAVI operators (interventional cardiologist and surgeons) to get involved in imaging. All TAVI operators should know how to assess the annulus, the annular root, and the iliofemoral access. We strongly believe that this will improve outcomes of this evolving procedure...
December 26, 2017: World Journal of Cardiology
https://www.readbyqxmd.com/read/29317805/compression-of-the-right-coronary-artery-by-an-aortic-pseudoaneurysm-after-infective-endocarditis-an-unusual-case-of-myocardial-ischemia
#14
Juan Lacalzada-Almeida, Alejandro De la Rosa-Hernández, María Manuela Izquierdo-Gómez, Javier García-Niebla, Iván Hernández-Betancor, Juan Alfonso Bonilla-Arjona, Antonio Barragán-Acea, Ignacio Laynez-Cerdeña
A 61-year-old male with a prosthetic St Jude aortic valve size 24 presented with heart failure symptoms and minimal-effort angina. Eleven months earlier, the patient had undergone cardiac surgery because of an aortic root dilatation and bicuspid aortic valve with severe regurgitation secondary to infectious endocarditis by Coxiela burnetii and coronary artery disease in the left circumflex coronary artery. Then, a prosthesis valve and a saphenous bypass graft to the left circumflex coronary artery were placed...
2018: Clinical Interventions in Aging
https://www.readbyqxmd.com/read/29317531/melanocortin-overexpression-limits-diet-induced-inflammation-and-atherosclerosis-in-ldlr-mice
#15
Salla Nuutinen, Liisa Ailanen, Eriika Savontaus, Petteri Rinne
Atherosclerosis is a chronic inflammatory disease of the arteries. The disease is initiated by endothelial dysfunction that allows the transport of leukocytes and low-density lipoprotein into the vessel wall forming atherosclerotic plaques. The melanocortin system is an endogenous peptide system that regulates, for example, energy homeostasis and cardiovascular function. Melanocortin treatment with endogenous or synthetic melanocortin peptides reduces body weight, protects the endothelium and alleviates vascular inflammation, but the long-term effects of melanocortin system activation on atheroprogression remain largely unknown...
January 9, 2018: Journal of Endocrinology
https://www.readbyqxmd.com/read/29313911/spinal-cord-injury-arising-in-anaesthesia-practice
#16
REVIEW
D W Hewson, N M Bedforth, J G Hardman
Spinal cord injury arising during anaesthetic practice is a rare event, but one that carries a significant burden in terms of morbidity and mortality. In this article, we will review the pathophysiology of spinal cord injury. We will then discuss injuries relating to patient position, spinal cord hypoperfusion and neuraxial techniques. The most serious causes of spinal cord injury - vertebral canal haematoma, spinal epidural abscess, meningitis and adhesive arachnoiditis - will be discussed in turn. For each condition, we draw attention to practical, evidence-based measures clinicians can undertake to reduce their incidence, or mitigate their severity...
January 2018: Anaesthesia
https://www.readbyqxmd.com/read/29313246/correction-to-valve-sparing-aortic-root-surgery-con-remodeling
#17
Takashi Kunihara
csvv.
January 8, 2018: General Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29312774/marfan-syndrome-with-pneumothorax-case-report-and-review-of-literatures
#18
Weiming Hao, Yong Fang, Hao Lai, Yaxing Shen, Hao Wang, Miao Lin, Lijie Tan
Marfan syndrome is a multisystem connective tissue disorder of autosomal dominant inheritance, which typically involves manifestations of the cardiovascular, skeletal, and ocular systems. Pulmonary involvement occurs less frequently. We report a case of a 28-year-old woman suffering from hours of sudden onset, progressive shortness of breath and right-sided chest pain. On physical examination, she presented decreased breath sound on the right side of chest, together with severe scoliosis and marfanoid habitus...
December 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/29307552/aortic-root-dilation-do-patients-with-marfan-syndrome-fare-worse-than-those-with-marfanoid-features
#19
Meghana R K Helder, Hartzell V Schaff, Thomas A Foley, Nandan S Anavekar, David R Deyle, Alberto Pochettino, Heidi M Connolly
OBJECTIVE: To discover whether patients with aortic root dilation and leptosomic features but without a diagnosis of Marfan syndrome (MFS) fare similarly to patients with MFS. METHODS: Of 124 patients with aortic root dilation identified from August 1, 1994, through October 31, 2012, 66 had MFS and 58 had leptosomic features but did not meet the Ghent criteria. Genetic testing was performed in 35% of patients (n=43). We compared z scores and aortic root diameters for patients who presented with aortic root dilation with and without an MFS diagnosis and with and without aortic root repair...
January 4, 2018: Mayo Clinic Proceedings
https://www.readbyqxmd.com/read/29307550/aortic-root-dilation-in-patients-with-leptosomic%C3%A2-features
#20
EDITORIAL
Frank V Brozovich
No abstract text is available yet for this article.
January 4, 2018: Mayo Clinic Proceedings
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