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https://www.readbyqxmd.com/read/29326834/ablation-of-atrial-fibrillation-in-patients-with-congenital-heart-disease
#1
Marwan M Refaat, Jad Ballout, Moussa Mansour
With improved surgical techniques and medical management for patients with congenital heart diseases, more patients are living longer and well into adulthood. This improved survival comes with a price of increased morbidity, mainly secondary to increased risk of tachyarrhythmias. One of the major arrhythmias commonly encountered in this subset of cardiac patients is AF. Similar to the general population, the risk of AF increases with advancing age, and is mainly secondary to the abnormal anatomy, abnormal pressure and volume parameters in the hearts of these patients and to the increased scarring and inflammation seen in the left atrium following multiple surgical procedures...
December 2017: Arrhythmia & Electrophysiology Review
https://www.readbyqxmd.com/read/29316993/a-congenitally-corrected-variant-of-ebstein-s-anomaly
#2
Rengin Çetin Güvenç, Gökmen Akgün, Tolga S Güvenç
Ebstein's anomaly is a rare CHD that is characterised by caudal displacement of the functional tricuspid annulus and a dysfunctional tricuspid valve owing to a failure of proper leaflet coaptation. We present a balanced variant of Ebstein's anomaly, in which the overgrowth of the septal leaflet had allowed proper coaptation of the tricuspid leaflets, thus preserving the valve function.
January 10, 2018: Cardiology in the Young
https://www.readbyqxmd.com/read/29316261/arrhythmia-after-cone-repair-for-ebstein-anomaly-the-mayo-clinic-experience-in-143-young-patients
#3
Philip Wackel, Bryan Cannon, Joseph Dearani, Kristen Sessions, Kimberly Holst, Jonathan Johnson, Frank Cetta
BACKGROUND: The increased incidence of preoperative and postoperative arrhythmia in Ebstein anomaly (EA) prompted some clinicians to perform an electrophysiology study (EPS) in all patients prior to surgery for EA. The cone repair (CR) is the current surgical option of choice for most young patients with EA but the effect of the CR on arrhythmia is not well established. OBJECTIVES: To assess the burden of arrhythmia in young patients after CR and to assess the utility of selective preoperative EPS...
January 8, 2018: Congenital Heart Disease
https://www.readbyqxmd.com/read/29315367/sudden-death-in-patients-with-ebstein-anomaly
#4
Christine H Attenhofer Jost, Nicholas Y Tan, Abdalla Hassan, Emily R Vargas, David O Hodge, Joseph A Dearani, Heidi Connolly, Samuel J Asirvatham, Christopher J McLeod
Aims: Ventricular dysfunction or structural alteration of either ventricle is a well-established risk factor for sudden death (SD). Ebstein anomaly (EA) can present with both right and left heart abnormalities; however, predictors of SD have not been described. We therefore sought to characterize the incidence and risk factors of SD among a large cohort of patients with EA. Methods and results: All EA patients who underwent evaluation at a high-volume institution over a 4-decade period were retrospectively reviewed...
January 5, 2018: European Heart Journal
https://www.readbyqxmd.com/read/29305187/cardiac-arrest-and-ventricular-arrhythmia-in-adults-with-ebstein-anomaly-and-left-ventricular-non-compaction
#5
Magdalena Kumor, Magdalena Lipczyńska, Elzbieta Katarzyna Biernacka, Anna Klisiewicz, Anna Wójcik, Marek Konka, Katarzyna Kożuch, Piotr Szymański, Piotr Hoffman
BACKGROUND: Ebstein anomaly is a complex, congenital heart defect that is associated with a variety of cardiac abnormalities. Studies found a similar sarcomere gene mutation in patients with Ebstein anomaly (EA) and patients with isolated left ventricular non-compaction (LVNC). AIM: We aimed to show the prevalence of LVNC and its potential relationship with severe cardiac events (VT - ventricular tachycardia, cardiac arrest) in adult patients with EA. METHODS: We conducted a retrospective search of our institutional database from 2010 to 2014 for patients with EA and reviewed patients' medical records (age, sex, clinical presentation, electrocardiographic, echocardiographic, and CMR - cardiac magnetic resonance features)...
January 2, 2018: Journal of Cardiology
https://www.readbyqxmd.com/read/29250225/atrial-fibrillation-in-patients-with-congenital-heart-disease
#6
REVIEW
Tabitha G Moe, Victor A Abrich, Edward K Rhee
Advances in surgical techniques have led to the survival of most patients with congenital heart disease (CHD) up to their adulthood. During their lifetime, many of them develop atrial tachyarrhythmias due to atrial dilatation and scarring from surgical procedures. More complex defects and palliative repairs are linked to a higher incidence and earlier occurrence of arrhythmias. Atrial fibrillation (AF) is common in patients who have atrial septal defects repaired after age 55 and in patients with tetralogy of Fallot repaired after age 45...
June 2017: Journal of Atrial Fibrillation
https://www.readbyqxmd.com/read/29249651/-minimally-fluoroscopic-ablation-of-an-accessory-pathway-in-a-child-with-ebstein-s-anomaly-a-case-report
#7
Rita Ibarra-Castillo, Jorge Arbaiza-Simon, Mario Viteri-Vela
No abstract text is available yet for this article.
December 14, 2017: Archivos de Cardiología de México
https://www.readbyqxmd.com/read/29237691/four-generation-family-with-ebstein-anomaly-highlights-future-challenges-in-congenital-heart-disease-genetics
#8
EDITORIAL
David S Winlaw, Sally L Dunwoodie, Edwin P Kirk
No abstract text is available yet for this article.
December 2017: Circulation. Cardiovascular Genetics
https://www.readbyqxmd.com/read/29237676/familial-ebstein-anomaly-whole-exome-sequencing-identifies-novel-phenotype-associated-with-flna
#9
Catherine L Mercer, Gaia Andreoletti, Aisling Carroll, Anthony P Salmon, I Karen Temple, Sarah Ennis
BACKGROUND: Familial Ebstein anomaly is a rare form of congenital heart disease. We report 7 individuals among 2 generations of 1 family with Ebstein anomaly. This family was first reported in 1991 by Balaji et al in which family members were also reported to have a mild skeletal phenotype. The most likely mechanism of inheritance was concluded to be autosomal dominant. We sought to identify the genetic pathogenesis in this family using a next generation sequencing approach. METHODS AND RESULTS: Whole exome sequencing was performed in 2 cousins in this family using the Agilent SureSelect Human all Exon 51 Mb version 5 capture kit...
December 2017: Circulation. Cardiovascular Genetics
https://www.readbyqxmd.com/read/29237468/left-ventricular-synchrony-torsion-and-recoil-mechanics-in-ebstein-s-anomaly-insights-from-cardiovascular-magnetic-resonance
#10
Michael Steinmetz, Simon Usenbenz, Johannes Tammo Kowallick, Olga Hösch, Wieland Staab, Torben Lange, Shelby Kutty, Joachim Lotz, Gerd Hasenfuß, Thomas Paul, Andreas Schuster
BACKGROUND: Disease progression and heart failure development in Ebstein's Anomaly (EA) of the tricuspid valve is characterized by both right and left ventricular (LV) deterioration. The mechanisms underlying LV dysfunction and their role in heart failure development are incompletely understood. We hypothesized that LV dyssynchrony and impaired torsion and recoil mechanics induced by paradoxical movement of the basal septum may play a role in heart failure development. METHODS: 31 EA patients and 31 matched controls underwent prospective cardiovascular magnetic resonance (CMR)...
December 14, 2017: Journal of Cardiovascular Magnetic Resonance
https://www.readbyqxmd.com/read/29233691/enthesopathy-in-rheumatoid-arthritis-and-spondyloarthritis-an-ultrasound-study
#11
Esther Ebstein, Baptiste Coustet, Vanina Masson-Behar, Marine Forien, Elisabeth Palazzo, Philippe Dieudé, Sébastien Ottaviani
OBJECTIVE: We aimed to compare the prevalence of enthesopathy seen on ultrasonography (US) in spondyloarthritis (SpA) and rheumatoid arthritis (RA) and compared it to healthy controls. METHODS: All included patients with RA (2010 ACR/EULAR criteria) and SpA (ASAS criteria) and healthy controls underwent clinical and US evaluation of enthesis at seven sites (quadriceps, proximal and distal patellar, Achilles and triceps tendons, plantar aponeurosis and lateral epicondyle enthesis)...
December 9, 2017: Joint, Bone, Spine: Revue du Rhumatisme
https://www.readbyqxmd.com/read/29216221/copy-number-variants-in-ebstein-anomaly
#12
Andreas Giannakou, Robert J Sicko, Wei Zhang, Paul Romitti, Marilyn L Browne, Michele Caggana, Lawrence C Brody, Laura Jelliffe-Pawlowski, Gary M Shaw, Denise M Kay, James L Mills
BACKGROUND: Ebstein anomaly (EA) is a rare congenital defect characterized by apical displacement of the septal tricuspid leaflets and atrialization of the right ventricle. The etiology of EA is unclear; however, recurrence in families and the association of EA with genetic syndromes and copy number variants (CNVs) suggest a genetic component. OBJECTIVE: We performed a population-based study to search for recurrent and novel CNVs in a previously unreported set of EA cases...
2017: PloS One
https://www.readbyqxmd.com/read/29209541/a-rare-manifestation-of-asymptomatic-ebstein-s-anomaly-with-tricuspid-valve-endocarditis
#13
Carmel Moazez, Vicken Zeitjian, Christian Breburda, Ranjini Roy
Ebstein's anomaly is a rare congenital heart disease that presents with apical displacement of the septal and posterior leaflets of the tricuspid valve. It has a wide spectrum of clinical presentations and has been shown to manifest itself any time from birth to adulthood. Our patient is a 43-year-old male with a history of intravenous heroin abuse who presented to the emergency department with worsening shortness of breath and lower extremity edema. He denied any prior cardiac history. A transthoracic echo showed normal left ventricular function, but a large 2...
2017: Case Reports in Cardiology
https://www.readbyqxmd.com/read/29183028/radiofrequency-ablation-of-atrial-fibrillation-in-patients-with-ebstein-s-anomaly-a-two-case-report
#14
Konstantinos E Iliodromitis, Marc Bonsels, Rolf Borchard, Anja Dorszewski
Ebstein's anomaly (EA) is a rare congenital heart disease characterized by "atrialization" of the right ventricle, due to apical displacement of the tricuspid leaflets into the right ventricle. Patients with EA may develop all kinds of supraventricular arrhythmias requiring radiofrequency ablation. Atrial fibrillation (Afib) is a common arrhythmia in EA patients, and results in debilitating symptoms that often require surgical treatment. This is a follow-up report of 2 patients with EA undergoing radiofrequency ablation for Afib...
November 29, 2017: Cardiology
https://www.readbyqxmd.com/read/29174783/improving-results-of-surgery-for-ebstein-anomaly-where-are-we-after-235-cone-repairs
#15
Kimberly A Holst, Joseph A Dearani, Sameh Said, Roxann B Pike, Heidi M Connolly, Bryan C Cannon, Kristen L Sessions, Megan M O'Byrne, Patrick W O'Leary
BACKGROUND: Ebstein anomaly has heterogeneous anatomy and numerous operative techniques are described. Cone repair provides a near anatomic tricuspid valve repair. The purpose of this study was to examine our experience with cone repair. METHODS: Cone repair was performed in 235 consecutive patients with Ebstein anomaly, 134 children (57%) and 101 adults (43%), from June 2007 to October 2015. Median age was 15.6 years (range, 6 months to 73 years). Cone repair was the first operation in 192 patients (82%), the second in 41 (17%), and the third in 2 (1%)...
January 2018: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29165381/factors-associated-with-the-need-for-and-the-impact-of-extracorporeal-membrane-oxygenation-in-children-with-congenital-heart-disease-during-admissions-for-cardiac-surgery
#16
Salvatore Aiello, Rohit S Loomba
INTRODUCTION: This study aimed to determine factors associated with the need for extracorporeal membrane oxygenation (ECMO) in children with congenital heart disease (CHD) during admission for cardiac surgery (CS). A secondary aim was to determine how ECMO impacted length, cost, and mortality of the admission. METHODS: Data from the Kids' Inpatient Database (KIDS) were utilized. Admissions with CHD under 18 years of age with cardiac surgery were included. Need for ECMO in these admissions was then identified...
November 22, 2017: Children
https://www.readbyqxmd.com/read/29153244/outcomes-of-transcatheter-tricuspid-valve-in-valve-implantation-in-patients-with-ebstein-anomaly
#17
Nathaniel W Taggart, Allison K Cabalka, Andreas Eicken, Jamil A Aboulhosn, John D R Thomson, Brian Whisenant, Martin L Bocks, Stephan Schubert, Thomas K Jones, Jeremy D Asnes, Thomas E Fagan, Jeffery Meadows, Mark Hoyer, Mary H Martin, Frank F Ing, Daniel R Turner, Azeem Latib, Aphrodite Tzifa, Stephan Windecker, Bryan H Goldstein, Jeffrey W Delaney, James A Kuo, Susan Foerster, Matthew Gillespie, Gianfranco Butera, Shabana Shahanavaz, Eric Horlick, Younes Boudjemline, Daniel Dvir, Doff B McElhinney
We sought to describe the acute results and short- to medium-term durability of transcatheter tricuspid valve-in-valve (TVIV) implantation within surgical bioprostheses among patients with Ebstein anomaly (EA). Cases were identified from a voluntary, multicenter, international registry of 29 institutions that perform TVIV. Demographic, clinical, procedural, and follow-up data were analyzed. Eighty-one patients with EA underwent TVIV from 2008 to 2016. Thirty-four patients (42%) were New York Heart Association (NYHA) class 3/4 at time of TVIV...
January 15, 2018: American Journal of Cardiology
https://www.readbyqxmd.com/read/29120021/swiss-adult-congenital-heart-disease-registry-sacher-rationale-design-and-first-results
#18
Daniel Tobler, Markus Schwerzmann, Judith Bouchardy, Reto Engel, Dominik Stambach, Christine Attenhofer Jost, Kerstin Wustmann, Fabienne Schwitz, Tobias Rutz, Harald Gabriel, Hans Peter Kuen, Christoph Auf der Maur, Angela Oxenius, Theresa Seeliger, Bruno Santos Lopes, Francesca Bonassin, Matthias Greutmann, On Behalf Of Sacher
BACKGROUND: In 2013, a prospective registry for adults with congenital heart disease (CHD) was established in Switzerland, providing detailed data on disease characteristics and outcomes: Swiss Adult Congenital HEart disease Registry (SACHER). Its aim is to improve the knowledge base of outcomes in adults with CHD. The registry design and baseline patient characteristics are reported. METHODS: All patients with structural congenital heart defects or hereditary aortopathies, followed-up at dedicated adult CHD clinics, are asked to participate in SACHER...
November 9, 2017: Swiss Medical Weekly
https://www.readbyqxmd.com/read/29049811/effect-of-cone-reconstruction-on-right-ventricular-function-in-patients-with-ebstein-s-anomaly-a-meta-analysis
#19
Dongxu Li, Yasutaka Hirata, Xu Zhou, Akihiro Masuzawa, Minoru Ono, Qi An
OBJECTIVES: Cone reconstruction (CR) is a novel technique for surgically treating the tricuspid valve and right ventricle (RV) in patients with Ebstein's anomaly. However, precise changes in the RV function after CR remain unclear. This study aimed to evaluate the RV size and New York Heart Association (NYHA) functional class by conducting a meta-analysis of reported data. METHODS: The MEDLINE, EMBASE, Cochrane Library and China National Knowledge Infrastructure databases were searched for relevant studies...
September 23, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/29024827/left-ventricular-non-compaction-with-ebstein-anomaly-attributed-to-a-tpm1-mutation
#20
Aleksandra Nijak, Maaike Alaerts, Cuno Kuiperi, Anniek Corveleyn, Bert Suys, Bernard Paelinck, Johan Saenen, Emeline Van Craenenbroeck, Lut Van Laer, Bart Loeys, Aline Verstraeten
Left ventricular non-compaction (cardiomyopathy) (LVN(C)) is a rare hereditary cardiac condition, resulting from abnormal embryonic myocardial development. While it mostly occurs as an isolated condition, association with other cardiovascular manifestations such as Ebstein anomaly (EA) has been reported. This congenital heart defect is characterized by downward displacement of the tricuspid valve and leads to diminished ventricular size and function. In an autosomal dominant LVN(C) family consisting of five affected individuals, of which two also presented with EA and three with mitral valve insufficiency, we pursued the genetic disease cause using whole exome sequencing (WES)...
October 9, 2017: European Journal of Medical Genetics
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