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https://www.readbyqxmd.com/read/28079865/differences-in-mortality-characteristics-in-neonates-with-down-s-syndrome
#1
C L Cua, U Haque, S Santoro, L Nicholson, C H Backes
OBJECTIVE: Neonates with Down's syndrome (nDS) may have multiple medical issues that place them at increased risk for mortality during the newborn period. Goal of this study was to determine if there are differences in baseline characteristics, medical complications or procedures performed during hospitalization between nDS who survived versus those who died during initial hospitalization. STUDY DESIGN: Data from 2000 to 2014 were reviewed using the Pediatric Health Information Systems (PHIS) database on all DS patients admitted to the hospital <30 days postnatal life...
January 12, 2017: Journal of Perinatology: Official Journal of the California Perinatal Association
https://www.readbyqxmd.com/read/28072699/prevention-preferable-to-treatment-3-case-reports-of-patients-experiencing-right-sided-heart-failure-after-ebstein-anomaly-correction
#2
Ming Luo, Jing Lin, Zhen Qin, Lei Du
RATIONALE: Ebstein anomaly is a common congenital heart disease that may induce severe tricuspid regurgitation and dilation of the "atrialized" portion of the right ventricle. Patients who undergo surgery to correct Ebstein anomaly are at high risk of postoperative right-sided heart failure, yet little is known about what pre-, peri-, or postoperative procedures may help reduce this risk. PATIENT CONCERNS: Here, we describe 3 cases of adults with Ebstein anomaly who underwent corrective surgery and in whom right-sided heart failure occurred with severe tricuspid regurgitation detected by transesophageal echocardiography...
January 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28072626/acquired-coronary-artery-disease-in-adult-patients-with-congenital-heart-disease-a-true-or-a-false-problem
#3
Alessandro Giamberti, Mauro Lo Rito, Erika Conforti, Alessandro Varrica, Mario Carminati, Alessandro Frigiola, Lorenzo Menicanti, Massimo Chessa
BACKGROUND: The population of adults with congenital heart disease (ACHD) is increasing and aging, and a large percentage of this population is now over 65 years of age. For this reason, it is probable that acquired coronary artery disease (CAD) will become an important issue that needs to be addressed also in these patients. We retrospectively analyzed all ACHD patients who underwent surgery in our Institution with the aim to investigate the incidence of associated CAD and the results of surgical treatment...
January 7, 2017: Journal of Cardiovascular Medicine
https://www.readbyqxmd.com/read/28063755/-congenital-heart-disease-recent-technical-advances-in-three-dimensional-echocardiography
#4
Clement Karsenty, Khaled Hadeed, Philippe Acar
The recent technical advances allow the use in practice of three-dimensional echocardiography in children especially through the new high frequency matrix probe. It is difficult or even impossible to hold breathing during children' acquisition so to avoid motion full volume artifact, one beat and live 3D modes are suitable. 3D echocardiography is more accurate than 2D to assess the size, location, and relationship with surrounding structures of atrial and ventricular septal defects and thus helps in the therapeutic decision...
January 4, 2017: La Presse Médicale
https://www.readbyqxmd.com/read/28046672/su-f-t-180-evaluation-of-a-scintillating-screen-detector-for-proton-beam-qa-and-acceptance-testing
#5
A Ghebremedhin, M Taber, P Koss, G Camargo, S Ebstein, B Patyal
PURPOSE: To test the performance of a commercial scintillating screen detector for acceptance testing and Quality Assurance of a proton pencil beam scanning system. METHOD: The detector (Lexitek DRD 400) has 40cm × 40cm field, uses a thin scintillator imaged onto a 16-bit scientific CCD with ∼0.5mm resolution. A grid target and LED illuminators are provided for spatial calibration and relative gain correction. The detector mounts to the nozzle with micron precision...
June 2016: Medical Physics
https://www.readbyqxmd.com/read/28043758/-ebstein-s-like-anomaly-ventricular-double-inlet-a-rare-association
#6
Luis Muñoz Castellanos, Magdalena Kuri Nivon
INTRODUCTION AND OBJECTIVES: The association of univentricular heart with double inlet and Ebstein's "like" anomaly of the common atrioventricular valve is extremely rare. METHODS: Two hearts with this association are described with the segmental sequential system which determine the atrial situs, the types of atrioventricular and ventriculoarterial connections and associated anomalies. RESULTS: Both hearts had atrial situs solitus, and a univentricular heart with common atrioventricular valve, a foramen primum and double outlet ventricle with normal crossed great arteries...
December 30, 2016: Archivos de Cardiología de México
https://www.readbyqxmd.com/read/28043470/single-center-experience-with-the-senning-procedure-in-the-current-era
#7
Giuseppe Ferro, Raghav Murthy, Vinod A Sebastian, Kristine J Guleserian, Joseph M Forbess
The Senning procedure is an operative technique for atrial inversion in congenital heart anomalies. We sought to evaluate our contemporary outcomes employing this technique. A retrospective analysis of all patients who underwent the Senning procedure at our institution was performed. Hospital records were reviewed, and follow-up data were obtained to evaluate outcomes. Overall, a total of 19 patients underwent a Senning procedure between August 2005 and July 2014. Median age at repair was 594 days (range: 5 days to 15 years)...
July 2016: Seminars in Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28032368/clinical-presentation-and-echocardiographic-characteristics-of%C3%A2-uhl-s-anomaly
#8
Christos G Mihos, Maiteder Larrauri-Reyes, Evin Yucel, Orlando Santana
Uhl's anomaly is a rare cardiac malformation that results in partial or complete absence of the right ventricular myocardium. It most commonly presents in prenatal or newborn infants; however, it may also be found in some adults as advanced right-sided heart failure. Differential diagnoses include arrhythmogenic right ventricular dysplasia and Ebstein's anomaly. Herein, we describe the clinical presentation of Uhl's anomaly in a previously undiagnosed middle-aged adult, and review the echocardiographic criteria used to diagnose and differentiate this rare, albeit important, myocardial disorder...
December 29, 2016: Echocardiography
https://www.readbyqxmd.com/read/28017577/ebstein-s-anomaly-genetics-clinical-manifestations-and-management
#9
REVIEW
Shi-Min Yuan
Ebstein's anomaly is uncommon. Genetic bases of this congenital heart defect may be related to the mutations in myosin heavy chain 7 and NKX2.5, among others. Asymptomatic patients with Ebstein's anomaly can be conservatively treated and kept under close follow-up, whereas surgical operation is indicated for those patients with evidence of right heart dilation and progressively impaired ventricular systolic function. A biventricular repair consisting of the reconstruction of a competent monocuspid tricuspid valve, right ventriculorrhaphy, subtotal atrial septal defect closure, and aggressive reduction atrioplasty is suitable for most patients, and 1...
November 19, 2016: Pediatrics and Neonatology
https://www.readbyqxmd.com/read/28011712/genome-wide-association-study-of-parkinson-s-disease-in-east-asians
#10
Jia Nee Foo, Louis C Tan, Ishak D Irwan, Wing-Lok Au, Hui Qi Low, Kumar-M Prakash, Azlina Ahmad-Annuar, Jinxin Bei, Anne Yy Chan, Chiung Mei Chen, Yi-Chun Chen, Sun Ju Chung, Hao Deng, Shen-Yang Lim, Vincent Mok, Hao Pang, Zhong Pei, Rong Peng, Hui-Fang Shang, Kyuyoung Song, Ai Huey Tan, Yih-Ru Wu, Tin Aung, Ching-Yu Cheng, Fook Tim Chew, Soo-Hong Chew, Siow-Ann Chong, Richard P Ebstein, Jimmy Lee, Seang-Mei Saw, Adeline Seow, Mythily Subramaniam, E-Shyong Tai, Eranga N Vithana, Tien-Yin Wong, Khai Koon Heng, Wee-Yang Meah, Chiea Chuen Khor, Hong Liu, Furen Zhang, Jianjun Liu, Eng-King Tan
Genome-wide association studies (GWAS) on Parkinson's disease (PD) have mostly been done in Europeans and Japanese. No study has been done in Han Chinese, which make up nearly a fifth of the world population. We conducted the first Han Chinese GWAS analyzing a total of 22,729 subjects (5,125 PD cases and 17,604 controls) from Singapore, Hong Kong, Malaysia, Korea, mainland China and Taiwan. We performed imputation, merging and logistic regression analyses of 2,402,394 SNPs passing quality control filters, adjusted for the first three principal components...
December 22, 2016: Human Molecular Genetics
https://www.readbyqxmd.com/read/27989087/the-double-orifice-valve-technique-to-treat-tricuspid-valve-incompetence
#11
Roland Hetzer, Mariano Javier, Eva Maria Delmo Walter
BACKGROUND: A straightforward tricuspid valve (TV) repair technique was used to treat either moderate or severe functional (normal valve with dilated annulus) or for primary/organic (Ebstein's anomaly, leaflet retraction/tethering and chordal malposition/tethering, with annular dilatation) TV incompetence, and its long-term outcome assessed. METHODS: A double-orifice valve technique was employed in 91 patients (mean age 52.6 ± 23.2 years; median age 56 years; range: 0...
January 2016: Journal of Heart Valve Disease
https://www.readbyqxmd.com/read/27987225/arrhythmias-in-adults-with-congenital-heart-disease-what-are-risk-factors-for-specific-arrhythmias
#12
Rohit S Loomba, Matthew W Buelow, Saurabh Aggarwal, Rohit R Arora, Joshua Kovach, Salil Ginde
INTRODUCTION: An increasing number of patients with congenital heart disease are now surviving into adulthood. This has also led to the emergence of complications from the underlying congenital heart disease, related surgical interventions, and associated combordities. While the prevalence of particular arrhythmias with specific congenital heart disease has been previously described, a detailed analysis of all lesions and a large number of comorbidities has not been previously published...
December 16, 2016: Pacing and Clinical Electrophysiology: PACE
https://www.readbyqxmd.com/read/27982446/armanni-ebstein-lesions-in-terminal-hyperglycemia
#13
Chong Zhou, Andrea J Yool, Roger W Byard
Armanni-Ebstein lesions (AEL) occur in deaths related to uncontrolled diabetes mellitus. To investigate the relationship between AEL and terminal hyperglycemia, we retrospectively reviewed 71 cases with vitreous glucose levels ≥11.1 mmol/L; 27 (38%) cases had AEL (vitreous glucose 14.0-77.3 mmol/L); and 44 cases (62%) did not (vitreous glucose 11.1-91.9 mmol/L). There was no significant difference (p = 0.271) in vitreous glucose levels between the cases with AEL (mean 39.2, SD 16.7 mmol/L) and those without (mean 34...
December 16, 2016: Journal of Forensic Sciences
https://www.readbyqxmd.com/read/27910083/radiofrequency-catheter-ablation-of-accessory-pathways-in-patients-with-ebstein-s-anomaly-at-8-years-of-follow-up
#14
Michał Orczykowski, Paweł Derejko, Robert Bodalski, Piotr Urbanek, Joanna Zakrzewska-Koperska, Radosław Sierpiński, Katarzyna Kalin, Andrzej Hasiec, Grzegorz Warmiński, Maria Miszczak-Knecht, Katarzyna Bieganowska, Rafał Baranowski, Maria Bilińska, Elżbieta Biernacka, Piotr Hoffman, Łukasz Szumowski
BACKGROUND: Data regarding long-term follow-up of radiofrequency catheter ablation (RFCA) of accessory pathways (APs) in patients with Ebstein's anomaly (EA) are limited. The procedures are challenging due to multiple or wide APs. METHODS: Analysis was performed on clinical and periprocedural data of patients with EA referred to the centre in order to perform catheter ablation of accessory pathway (AP). The group consisted of 22 patients (female 40.9%, mean age 33...
December 2, 2016: Cardiology Journal
https://www.readbyqxmd.com/read/27818476/congenitally-corrected-transposition-of-the-great-arteries-and-situs-inversus-in-an-octogenarian-with-systemic-right-ventricular-failure
#15
Mikio Shiba, Shigefumi Fukui, Hideo Ohuchi, Jin Ueda, Akihiro Tsuji, Yoshiaki Morita, Aya Miyazaki, Takeshi Ogo, Satoshi Yasuda, Isao Shiraishi, Norifumi Nakanishi
Systemic right ventricular (RV) failure in patients with congenitally corrected transposition of the great arteries (ccTGA), a major cause of mortality in the long-term follow-up, is usually induced by concomitant severe morphologically tricuspid regurgitation (TR) with/without Ebstein's anomaly or progressive conduction tissue disturbances. However, whether or not myocardial fibrosis is a common cause of systemic RV failure in patients with ccTGA remains unclear. Here, we describe an 82-year-old man who had been diagnosed previously as having uncomplicated ccTGA and situs inversus and recently developed systemic RV failure, which was neither associated with severe TR nor advanced conduction tissue abnormalities...
November 4, 2016: International Heart Journal
https://www.readbyqxmd.com/read/27793395/assessment-of-progressive-pathophysiology-after-early-prenatal-diagnosis-of-the-ebstein-anomaly-or-tricuspid-valve-dysplasia
#16
Elif Seda Selamet Tierney, Doff B McElhinney, Lindsay R Freud, Wayne Tworetzky, Bettina F Cuneo, Maria C Escobar-Diaz, Catherine Ikemba, Brian T Kalish, Rukmini Komarlu, Stéphanie M Levasseur, Michael D Puchalski, Gary M Satou, Norman H Silverman, Anita J Moon-Grady
In fetuses with Ebstein anomaly or tricuspid valve dysplasia (EA/TVD), poor hemodynamic status is associated with worse neonatal outcome. It is not known whether EA/TVD fetuses with more favorable physiology earlier in gestation progress to more severe disease in the third trimester. We evaluated if echocardiographic indexes in EA/TVD fetuses presenting <24 weeks of gestation are reliable indicators of physiologic status later in pregnancy. This multicenter, retrospective study included 51 fetuses presenting at <24 weeks of gestation with EA/TVD and serial fetal echocardiograms ≥4 weeks apart...
January 1, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/27788187/genetic-variants-in-isolated-ebstein-anomaly-implicated-in-myocardial-development-pathways
#17
Robert J Sicko, Marilyn L Browne, Shannon L Rigler, Charlotte M Druschel, Gang Liu, Ruzong Fan, Paul A Romitti, Michele Caggana, Denise M Kay, Lawrence C Brody, James L Mills
Ebstein anomaly (EA) is a rare heart defect in which the tricuspid valve is malformed and displaced. The tricuspid valve abnormalities can lead to backflow of blood from the right ventricle to the right atrium, preventing proper circulation of blood to the lungs. Although the etiology of EA is largely unresolved, increased prevalence of EA in those with a family history of congenital heart disease suggests EA has a genetic component. Copy number variants (CNVs) are a major source of genetic variation and have been implicated in a range of congenital heart defect phenotypes...
2016: PloS One
https://www.readbyqxmd.com/read/27787885/an-isolated-perfused-rat-kidney-model-for-the-evaluation-of-the-effect-of-glucose-on-renal-tubular-epithelial-morphology
#18
Chong Zhou, Andrea J Yool, Roger W Byard
An isolated perfused kidney model was used to evaluate the effect of hyperglycemia on renal tubular epithelial cell morphology. Ten Sprague-Dawley rat kidneys were perfused with Krebs-Henseleit buffer containing 70 mmol/L of glucose (five for 1 h and five for 2 h). Two control groups consisted of 10 kidneys perfused with Krebs-Henseleit buffer without hyperglycemia (five for 1 h and five for 2 h), and 10 nonperfused contralateral kidneys placed in the same environment for the same duration. The hyperglycemia group had significantly increased renal tubular vacuolization (p < 0...
January 2017: Journal of Forensic Sciences
https://www.readbyqxmd.com/read/27787883/hyperosmolarity-induces-armanni-ebstein-like-renal-tubular-epithelial-swelling-and-cytoplasmic-vacuolization
#19
Chong Zhou, Robert Vink, Roger W Byard
Armanni-Ebstein lesions have been considered pathognomonic for diabetes mellitus and appear as markedly swollen renal tubular epithelial cells with cytoplasmic clearing and glycogen accumulation. However, the extent to which hyperosmolarity contributes to the Armanni-Ebstein phenotype is unclear. Ten sheep were injected intravenously with 20% mannitol at 11 mOsm/kg, and subsequent histological evaluation of the kidneys showed variable degrees of osmotic nephrosis and cytoplasmic clearing of renal tubular epithelial cells similar to that seen with Armanni-Ebstein lesions...
January 2017: Journal of Forensic Sciences
https://www.readbyqxmd.com/read/27777295/single-ventricle-outcomes-after-neonatal-palliation-of-severe-ebstein-anomaly-with-modified-starnes-procedure
#20
S Ram Kumar, Grace Kung, Nathan Noh, Novel Castillo, Brian Fagan, Winfield J Wells, Vaughn A Starnes
BACKGROUND: We have previously shown that neonates in profound cardiogenic shock caused by a severe Ebstein anomaly can be successfully salvaged with fenestrated right ventricular exclusion and systemic to pulmonary shunt (modified Starnes procedure). The long-term outcome of single-ventricle management in these patients is not known. METHODS: We retrospectively reviewed the records of patients who underwent neonatal Starnes procedure between 1989 and 2015. Patient demographics, clinical variables, and outcome data were collected...
October 25, 2016: Circulation
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