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https://www.readbyqxmd.com/read/28812464/congenital-diaphragmatic-hernia-with-liver-herniation-into-the-pericardial-sac-in-a-30-week-gestation-infant
#1
Nisha Patel, Christina Sollinger, Carl T D'Angio, Jeffrey M Vinocur, Kate G Ackerman, Philip J Katzman
Anterior diaphragmatic defects with pericardial involvement are extremely rare and diagnostically challenging entities encountered perinatally. While a majority of diaphragmatic defects occur in isolation, others are associated with multiple defects forming a complex of syndromes such as Pentalogy of Cantrell. Liver herniation into the pericardial sac poses a particular challenge and can mimic a pericardial tumor on prenatal ultrasound, yielding a different management course. The following case is an unusual presentation of a 30-week gestation female with an anterior midline diaphragmatic defect with liver herniation mimicking as a pericardial tumor, diagnosed at time of autopsy...
September 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28807863/prenatal-diagnosis-of-complex-phenotype-in-a-13-week-old-fetus-with-an-interstitial-multigene-deletion-20q13-13-q13-2-by-chromosomal-microarray
#2
Feodora Stipoljev, Danka Miric-Tesanic, Tomislav Hafner, Maja Barbalic, Monika Logara, Ruzica Lasan-Trcic, Ana Vicic, Romana Gjergja-Juraski
We report the first trimester three-dimensional ultrasonographic findings in a 13-week-old fetus with complex phenotype and a de novo 4.7 Mb multigene deletion encompassing chromosome region 20q13.13-q13.2 detected by chromosomal microarray. Fetal sonography detected radial-ray anomalies in the form of bilateral absence of thumbs and the left club hand deformity. The presence of single atrioventricular canal instead of the atrial septal defect typical for Holt-Oram syndrome pointed us to rather suspect the SALL4 related diseases...
August 11, 2017: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/28805030/a-hybrid-technique-for-ventricular-septal-defect-closure-in-young-infants
#3
EDITORIAL
Thomas Bartel, E Murat Tuzcu
Perventricular device closure is a hybrid surgical and transcatheter technique. Perventricular device closure is a safe and effective technique to close muscular VSDs even in infants. TEE guidance is the principal tool for intra-procedural guidance.
August 1, 2017: Catheterization and Cardiovascular Interventions
https://www.readbyqxmd.com/read/28805027/acute-and-midterm-results-following-perventricular-device-closure-of-muscular-ventricular-septal-defects-a-multicenter-pices-investigation
#4
Robert G Gray, Shaji C Menon, Joyce T Johnson, Aimee K Armstrong, Michael A Bingler, John P Breinholt, Damien Kenny, John Lozier, Joshua J Murphy, Shyam K Sathanandam, Nathaniel W Taggart, Sara M Trucco, Bryan H Goldstein, Brent M Gordon
OBJECTIVES: To describe acute and mid-term results of hybrid perventricular device closure of muscular ventricular septal defects (mVSDs). BACKGROUND: Perventricular device closure of mVSDs can mitigate technical limitations of percutaneous closure and need for cardiopulmonary bypass or ventriculotomy with a surgical approach. METHODS: This is a multicenter retrospective cohort study of patients undergoing hybrid perventricular mVSD device closure from 1/2004 to 1/2014...
August 1, 2017: Catheterization and Cardiovascular Interventions
https://www.readbyqxmd.com/read/28804238/percutaneous-closure-of-the-aorto-ostial-origin-of-a-coronary-artery-saphenous-bypass-graft-with-a-large-pseudoaneurysm-using-the-amplatzer-muscular-ventricular-septal-defect-occluder
#5
Nicolas W Shammas, Majid Z Chammas, Jon Robken, Dale Geiss
We report the case of a 76-year-old male patient with a history of coronary artery bypass graft surgery presented with a large pseudoaneurysm emerging from a previously occluded saphenous bypass graft (SVG). A largely contained hematoma is seen in the mediastinum on computed tomography angiography (CTA) of the chest. Flow was seen from the ascending aorta into the pseudoaneurysm through the aorto-ostial opening of the bypass graft. Closure of the aorto-ostial origin of the graft was performed using the AMPLATZER muscular ventricular septal defect (VSD) occluder (St Jude's Medical, St...
September 2017: International Journal of Angiology: Official Publication of the International College of Angiology, Inc
https://www.readbyqxmd.com/read/28803631/primary-tuberculosis-of-the-nasal-septum-the-non-ulcerated-form-presenting-as-septal-thickening
#6
Neeraj Aggarwal, Mainak Dutta, Ramanuj Sinha
No abstract text is available yet for this article.
August 10, 2017: Acta Otorrinolaringológica Española
https://www.readbyqxmd.com/read/28803513/mechanisms-of-unexpected-death-and-autopsy-findings-in-friedreich-ataxia
#7
Roger W Byard, John D Gilbert
A 36-year-old woman with a clinical history of Friedreich ataxia and hypertrophic cardiomyopathy was found unexpectedly dead at her home. The heart showed asymmetric left ventricular hypertrophy, with an interventricular septal thickness of 20-25 mm (the remainder of the left ventricular wall measured 15 mm). Histologically, both ventricles had irregular areas of marked myocyte hypertrophy with associated interstitial fibrosis and focal myofibre disarray. There was neuronal loss within the dentate nucleus of the cerebellum, with vacuolation and axonal loss in the dorsal columns and spinocerebellar tracts of the upper cervical spinal cord...
January 1, 2017: Medicine, Science, and the Law
https://www.readbyqxmd.com/read/28802509/value-of-strain-imaging-and-maximal-oxygen-consumption-in-patients-with-hypertrophic-cardiomyopathy
#8
Kegan J Moneghetti, Davide Stolfo, Jeffrey W Christle, Yukari Kobayashi, Gherardo Finocchiaro, Gianfranco Sinagra, Jonathan Myers, Euan A Ashley, Francois Haddad, Matthew T Wheeler
Longitudinal strain (LS) has been shown to be predictive of outcome in hypertrophic cardiomyopathy (HC). Percent predicted peak oxygen uptake (ppVO2), among other cardiopulmonary exercise testing (CPX) metrics, is a strong predictor of prognosis in HC. However, there has been limited investigation into the combination of LS and CPX metrics. This study sought to determine how LS and parameters of exercise performance contribute to prognosis in HC. One hundred and thirty-one consecutive patients with HC who underwent CPX and stress echocardiography were included...
July 17, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28801537/closure-of-secundum-atrial-septal-defects-with-the-amplatzer-septal-occluder-a-prospective-multicenter-post-approval-study
#9
Daniel R Turner, Carl Y Owada, Charlie J Sang, Muhammad Khan, D Scott Lim
BACKGROUND: Prospective data on the medium-term safety and effectiveness of the AMPLATZER Septal Occluder in clinical practice are not available. The objective of this study was to prospectively evaluate the risk of hemodynamic compromise and obtain medium-term survival data on patients implanted with the AMPLATZER Septal Occluder for percutaneous closure of secundum atrial septal defects. METHODS AND RESULTS: Subjects were enrolled prospectively at 50 US sites and followed for 2 years...
August 2017: Circulation. Cardiovascular Interventions
https://www.readbyqxmd.com/read/28801203/echocardiographic-prediction-of-cardiac-resynchronization-therapy-response-requires-analysis-of-both-mechanical-dyssynchrony-and-right-ventricular-function-a-combined-analysis-of%C3%A2-patient-data-and-computer-simulations
#10
Wouter M van Everdingen, John Walmsley, Maarten J Cramer, Iris van Hagen, Bart W L De Boeck, Mathias Meine, Tammo Delhaas, Pieter A Doevendans, Frits W Prinzen, Joost Lumens, Geert E Leenders
BACKGROUND: Pronounced echocardiographically measured mechanical dyssynchrony is a positive predictor of response to cardiac resynchronization therapy (CRT), whereas right ventricular (RV) dysfunction is a negative predictor. The aim of this study was to investigate how RV dysfunction influences the association between mechanical dyssynchrony and left ventricular (LV) volumetric remodeling following CRT. METHODS: One hundred twenty-two CRT candidates (mean LV ejection fraction, 19 ± 6%; mean QRS width, 168 ± 21 msec) were prospectively enrolled and underwent echocardiography before and 6 months after CRT...
August 8, 2017: Journal of the American Society of Echocardiography
https://www.readbyqxmd.com/read/28797599/magnetic-resonance-spectroscopy-analysis-of-neurochemical-changes-in-the-atrophic-hippocampus-of-app-ps1-transgenic-mice
#11
Shengxiang Liang, Jia Huang, Weilin Liu, Hao Jin, Long Li, Xiufeng Zhang, Binbin Nie, Ruhui Lin, Jing Tao, Shujun Zhao, Baoci Shan, Lidian Chen
Alzheimer's disease (AD) is characterized by neuropathological changes and progressive cognitive decline, which is associated with the volume loss and neurochemical alterations. However, the specific neurochemical alterations in cerebral regions that contribute to cognitive decline still remain unknown. In the present study, we measured cerebral morphological and neurochemical alterations using structural magnetic resonance imaging (MRI) and proton magnetic resonance spectroscopy ((1)H-MRS) in an AD model of APP/PS1 transgenic mice...
August 8, 2017: Behavioural Brain Research
https://www.readbyqxmd.com/read/28797424/retrograde-chronic-total-occlusion-percutaneous-coronary-intervention-through-ipsilateral-collateral-channels-a-multicenter-registry
#12
Lorenzo Azzalini, Pierfrancesco Agostoni, Susanna Benincasa, Paul Knaapen, Stefan P Schumacher, Joseph Dens, Joren Maeremans, Adriaan O Kraaijeveld, Leo Timmers, Michael Behnes, Ibrahim Akin, Aurel Toma, Franz-Josef Neumann, Antonio Colombo, Mauro Carlino, Kambis Mashayekhi
OBJECTIVES: The aim of this study was to describe the procedural aspects and outcomes of retrograde chronic total occlusion (CTO) percutaneous coronary intervention (PCI) through ipsilateral collateral channels (ILCs). BACKGROUND: Retrograde CTO PCI via ILCs is rarely performed, usually when no other retrograde options exist, and available evidence derives mostly from case reports. METHODS: A large retrospective multinational registry was compiled, including all consecutive patients undergoing retrograde CTO PCI through ILCs at 6 centers between September 2011 and October 2016...
August 14, 2017: JACC. Cardiovascular Interventions
https://www.readbyqxmd.com/read/28796462/malformations-surveillance-comparison-between-findings-at-birth-and-age-1-year
#13
Emma G Thomas, Cathleen Higgins, Marie-Noel Westgate, Angela E Lin, Marlene Anderka, Lewis B Holmes
BACKGROUND: Malformations surveillance programs among newborn infants are used to determine the prevalence of congenital anomalies. A comparison in the same group of infants between the malformations detected at birth and those detected at 1 year of age will identify errors in the surveillance process and, also, the abnormalities more likely not to be detected at birth, but later in the first year of life. METHODS: The malformations identified at birth by Brigham and Women's Hospital (BWH) in the years 2000 and 2005 have been compared with the abnormalities detected in the same infants up to age 1 year by the Massachusetts Birth Defects Monitoring Program...
August 10, 2017: Birth defects research
https://www.readbyqxmd.com/read/28795038/indentation-in-the-right-ventricle-by-an-incomplete-pericardium-on-3-dimensional-reconstructed-computed-tomography
#14
Hak Ju Kim, Jae Gun Kwak
We report the case of a 17-year-old girl who presented with an indentation in the right ventricle caused by an incomplete pericardium on preoperative 3-dimensional reconstructed computed tomography. She was to undergo surgery for a partial atrioventricular septal defect and secundum atrial septal defect. Preoperative electrocardiography revealed occasional premature ventricular beats. We found the absence of the left side of the pericardium intraoperatively, and this absence caused strangulation of the diaphragmatic surface of the right ventricle...
August 2017: Korean Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28794137/discrepant-measurements-of-maximal-left-ventricular-wall-thickness-between-cardiac-magnetic-resonance-imaging-and-echocardiography-in-patients-with-hypertrophic-cardiomyopathy
#15
Waseem Hindieh, Adaya Weissler-Snir, Helene Hammer, Arnon Adler, Harry Rakowski, Raymond H Chan
BACKGROUND: We sought to compare maximal left ventricular (LV) wall thickness (WT) measurements as obtained by routine clinical practice between echocardiography and cardiac magnetic resonance (CMR) and document causes of discrepancy. METHODS AND RESULTS: One-hundred and ninety-five patients with hypertrophic cardiomyopathy (median age, 52.8±15.1 years) who underwent echocardiography and CMR imaging within 6 months (median, 41 days; interquartile range, 16-97 days) were included...
August 2017: Circulation. Cardiovascular Imaging
https://www.readbyqxmd.com/read/28794135/pulmonary-vasodilator-therapy-is-associated-with-greater-survival-in-eisenmenger-syndrome
#16
Clare Arnott, Geoff Strange, Andrew Bullock, Adrienne C Kirby, Clare O'Donnell, Dorothy J Radford, Leeanne E Grigg, David S Celermajer
OBJECTIVE: Eisenmenger syndrome (ES) is a severe form of pulmonary hypertension in adults with congenital heart disease (CHD) and has a poor prognosis. We aimed to understand factors associated with survival in ES and particularly to assess the potential benefits of advanced pulmonary vasodilator therapy (AT). METHODS: From January 2004, when AT became generally available for patients with ES, we followed 253 ES adults from 12 adult congenital heart disease centres across Australia and New Zealand...
August 9, 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28793143/intraoperative-diagnosis-of-anderson-fabry-disease-in-patients-with-obstructive-hypertrophic-cardiomyopathy-undergoing-surgical-myectomy
#17
Franco Cecchi, Maria Iascone, Niccolò Maurizi, Laura Pezzoli, Irene Binaco, Elena Biagini, Maria Laura Fibbi, Iacopo Olivotto, Federico Pieruzzi, Ana Fruntelata, Lucian Dorobantu, Claudio Rapezzi, Paolo Ferrazzi
Importance: Diagnostic screening for Anderson-Fabry cardiomyopathy (AFC) is performed in the presence of specific clinical red flags in patients with hypertrophic cardiomyopathy (HCM) older than 25 years. However, left ventricular outflow tract obstruction (LVOTO) has been traditionally considered an exclusion criteria for AFC. Objective: To examine a series of patients diagnosed with HCM and severe basal LVOTO undergoing myectomy in whom the diagnosis of AFC was suspected by the cardiac surgeon intraoperatively and confirmed by histological and genetic examinations...
August 9, 2017: JAMA Cardiology
https://www.readbyqxmd.com/read/28793032/protein-from-hevea-brasiliensis-hev-b-13-latex-attenuates-systemic-inflammatory-response-and-lung-lesions-in-rats-with-sepsis
#18
L A Araújo, P R Melo-Reis, F Mrue, C M Gomes, M A P Oliveira, H M Silva, M M Alves, N J Silva-Júnior
Sepsis induces a severe systemic inflammatory response that may result in multiple organ dysfunction and death. Studies using a protein derived from natural Hevea brasiliensis (rubber tree) latex, denominated Hev b 13, have demonstrated important anti-inflammatory effects, but no data have been published regarding its effects on sepsis. The aim of this study was to investigate the effects of Hev b 13 on the inflammatory response and lung lesions of septal rats. Male Wistar rats were submitted to cecal ligation and puncture (CLP), randomized into groups and treated with subcutaneously administered doses of 0...
July 27, 2017: Brazilian Journal of Biology, Revista Brasleira de Biologia
https://www.readbyqxmd.com/read/28792086/functional-redundancy-of-division-specific-penicillin-binding-proteins-in-bacillus-subtilis
#19
Jad Sassine, Meizhu Xu, Karzan R Sidiq, Robyn Emmins, Jeff Errington, Richard A Daniel
Bacterial cell division involves the dynamic assembly of a diverse set of proteins that coordinate the invagination of the cell membrane and synthesis of cell wall material to create the new cell poles of the separated daughter cells. Pencillin-binding protein PBP 2B is a key cell division protein in Bacillus subtilis proposed to have a specific catalytic role in septal wall synthesis. Unexpectedly, we find that a catalytically inactive mutant of PBP 2B supports cell division, but in this background the normally dispensable PBP 3 becomes essential...
August 9, 2017: Molecular Microbiology
https://www.readbyqxmd.com/read/28791849/echocardiographic-assessment-and-n-terminal-pro-brain-natriuretic-peptide-in-hypertensives-with-metabolic-syndrome
#20
Paweł Krzesiński, Beata Uziebło-Życzkowska, Grzegorz Gielerak, Adam Stańczyk, Katarzyna Piotrowicz, Wiesław Piechota, Paweł Smurzyński, Andrzej Skrobowski
BACKGROUND: N-terminal pro-brain natriuretic peptide (NT-proBNP) release is associated with left ventricular expansion and pressure overload. Elevation of serum levels of natriuretic peptides is observed in patients with impaired as well as preserved left ventricular systolic function. High NT-proBNP has been shown to be related not only to preload but also to increased afterload, especially blood pressure and arterial stiffness. OBJECTIVES: The aim of the study was to evaluate the association of NT-proBNP and echocardiographic parameters in hypertensives with metabolic syndrome...
March 2017: Advances in Clinical and Experimental Medicine: Official Organ Wroclaw Medical University
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