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https://www.readbyqxmd.com/read/28639568/intraoperative-transesophageal-echocardiography-in-the-operation-of-ebstein-s-anomaly-a-retrospective-study
#1
Xiu-Jie Tang, Min Bao, He Zhao, Lian-Yi Wang, Qing-Yu Wu
BACKGROUND: Ebstein's anomaly (EA) has various spectrums in clinical and anatomic features. This study aimed to report the experience of two-dimensional intraoperative transesophageal echocardiography (2D-ITEE) during the EA surgery and to analyze the characteristics of the tricuspid valve (TV) by comparing the data from 2D-ITEE with the results from the surgery. METHODS: 2D-ITEE data of 164 patients with EA who were operated in the First Hospital of Tsinghua University between July 2004 and April 2014 were retrospectively analyzed in this study...
July 5, 2017: Chinese Medical Journal
https://www.readbyqxmd.com/read/28633270/surgical-correction-for-a-neonate-with-ebstein-s-anomaly-associated-with-tetralogy-of-fallot
#2
Ling-Yi Wei, Jin-Chung Shih, En-Ting Wu, Shyh-Jye Chen, Yih-Sharng Chen, Shu-Chien Huang
Ebstein's anomaly in combination with tetralogy of Fallot (TOF) is rare. We report a male infant with these combined anomalies who presented with severe cyanosis, heart failure, and respiratory distress after birth. Biventricular repair was successfully performed with 1-stage correction of his ventricular septal defect (VSD), right ventricular outflow tract (RVOT) obstruction, and tricuspid regurgitation. The downward displaced tricuspid valve was also restored to the normal annulus position. The infant recovered well...
July 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28591541/lithium-use-in-pregnancy-and-the-risk-of-cardiac-malformations
#3
COMPARATIVE STUDY
Elisabetta Patorno, Krista F Huybrechts, Brian T Bateman, Jacqueline M Cohen, Rishi J Desai, Helen Mogun, Lee S Cohen, Sonia Hernandez-Diaz
BACKGROUND: There has been concern that exposure to lithium early in pregnancy may be associated with a marked increase in the risk of Ebstein's anomaly (a right ventricular outflow tract obstruction defect) in infants and overall congenital cardiac defects, but data are conflicting and limited. METHODS: We conducted a cohort study involving 1,325,563 pregnancies in women who were enrolled in Medicaid and who delivered a live-born infant between 2000 and 2010. We examined the risk of cardiac malformations among infants exposed to lithium during the first trimester as compared with unexposed infants and, in secondary analyses, with infants exposed to another commonly used mood stabilizer, lamotrigine...
June 8, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/28566198/ebstein-s-anomaly-in-adults-modified-cone-reconstruction-of-the-tricuspid-valve-is-associated-with-promising-outcomes
#4
Emre Belli, Martin Rabot, Jérôme Petit, Marielle Gouton
BACKGROUND: Ebstein's anomaly is a complex malformation. Justification of a procedure in mildly symptomatic adults is debatable: repair techniques are demanding and valve replacement is associated with poorer outcome. OBJECTIVES: We report our initial experience with the cone procedure versus medical follow-up. METHODS: Patients aged≥15years with Ebstein's anomaly were enrolled during 2007-2014. The cone procedure was performed in consecutive patients with severe tricuspid regurgitation (TR); those with less severe disease did not undergo surgery, although some underwent percutaneous catheter atrial septal defect closure...
May 26, 2017: Archives of Cardiovascular Diseases
https://www.readbyqxmd.com/read/28544841/transcatheter-valve-in-valve-implantation-failing-tricuspid-bioprosthesis-in-a-patient-with-ebstein%C3%A2-s-anomaly
#5
Pedro A Villablanca, Aman M Shah, David F Briceno, Ali N Zaidi, Mei Chau, Mario J Garcia, David Slovut, Cynthia Taub
Transcatheter valve-in-valve (VIV) implantation has been recently proposed as an alternative to surgical reoperative aortic valve replacement in patients with a failing aortic bioprosthesis. Experience with transcatheter VIV implantation at other valve positions is very limited. Herein is reported the case of an 18-year-old man with Ebstein's anomaly and severe tricuspid valve (TV) regurgitation status after bioprosthetic valve replacement, who developed new dyspnea on exertion three years after the initial valve replacement...
January 2017: Journal of Heart Valve Disease
https://www.readbyqxmd.com/read/28528887/the-cone-technique-for-ebstein-repair-first-cases
#6
Juan-Miguel Gil-Jaurena, María-Teresa González-López, Ana Pita-Fernández, Ramón Pérez-Caballero, Raquel Prieto, Constancio Medrano
No abstract text is available yet for this article.
May 18, 2017: Revista Española de Cardiología
https://www.readbyqxmd.com/read/28491781/radiofrequency-catheter-ablation-for-drug-refractory-paroxysmal-atrial-fibrillation-in-a-patient-with-ebstein-s-anomaly
#7
Yong-Giun Kim, Shin-Jae Kim, Gi-Byoung Nam
No abstract text is available yet for this article.
January 2017: HeartRhythm Case Reports
https://www.readbyqxmd.com/read/28481744/congenital-giant-right-atrial-aneurysm-echocardiographic-diagnosis-and-surgical-management
#8
Yao Wang, Aijun Liu, Weihua Ye
Congenital right atrial aneurysm is a rare abnormality and may easily be confused with various anomalies, such as pericardial effusion, pericardial cysts, tumors, and Ebstein's anomaly. Patients with right atrial aneurysm may be asymptomatic; but some patients may develop life-threatening complications, such as arrhythmias, congestive heart failure, or pulmonary embolism. Therefore, it is essential for correct diagnosis and appropriate patient management. We report a case of giant right atrial aneurysm in a 7-year-old boy who presented with progressive protrusion of chest...
April 28, 2017: Heart Surgery Forum
https://www.readbyqxmd.com/read/28469692/complex-ebstein-s-anomaly-in-an-86-year-old-iranian-man-a-case-report
#9
Bahieh Moradi, Farideh Roshanali
Ebstein's anomaly is defined as the significant apical displacement of the tricuspid valve causing tricuspid regurgitation. Although a variety of concomitant lesions have been previously described, we herein introduce an unusual presentation. Our patient was an 86-year-old man with a primary presentation of typical chest pain in the setting of recently diagnosed coronary artery disease with concomitant Ebstein's anomaly. We found mild-to-moderate tricuspid regurgitation, bicuspid aortic valve, persistent left superior vena cava, and patent foramen ovale...
January 2017: Journal of Tehran Heart Center
https://www.readbyqxmd.com/read/28457239/ebstein-anomaly
#10
REVIEW
Elizabeth D Sherwin, Dominic J Abrams
Ebstein anomaly is a rare form of congenital heart disease with a uniquely high prevalence of arrhythmias. The most prevalent arrhythmia mechanisms are intrinsic to the underlying embryologic defects and may manifest at any stage. Current electrophysiological and surgical strategies are well equipped to address these arrhythmia mechanisms, yet despite available technology and a robust understanding of the mechanisms, these cases remain challenging. Surgical techniques that render arrhythmia substrates unreachable mandate comprehensive presurgical electrophysiological assessment and potential ablation...
June 2017: Cardiac Electrophysiology Clinics
https://www.readbyqxmd.com/read/28452857/a-case-of-hemophagocytic-lymphohistiocytosis-and-kawasaki-disease-concurrent-or-overlapping-diagnoses
#11
Allison S Bechtel, Michael Joyce
Hemophagocytic lymphohistiocytosis (HLH) is not always suspected at the time of presentation. It is often mistaken for other diagnoses; complicated by the fact that it is often associated with an inciting event that has significant overlap. Kawasaki disease, along with other disorders, such as Ebstein Barr Virus infection, are conditions that may lead HLH. Our patient had a presentation that was consistent with Kawasaki disease on initial presentation, however subsequently met the diagnostic criteria of HLH...
April 27, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28450668/impact-of-surgery-in-ebstein-s-anomaly-using-current-surgical-criteria
#12
Nydia Ávila-Vanzzini, Juan Francisco Fritche-Salazar, Héctor Herrera-Bello, Nilda Espinola-Zavaleta, Jorge Kuri Alfaro, Hugo Rodriguez-Zanella, Mirna Verónica Álvarez Bran, Jorge Cossio Aranda, Manuel B A Gaxiola Macias, Pedro Curi-Curi
BACKGROUND: In Ebstein's anomaly (EA) current surgical criteria may not translate into better long-term survival. The aim of this study was therefore to determine if surgical treatment for EA increases survival, and to analyze factors associated with mortality.Methods and Results:A retrospective study was carried out involving 103 patients with surgical indication using current criteria, comparing operated (n=49; 47.5%) and non-operated patients (n=54; 52.4%); the severity of disease was similar in all cases...
April 26, 2017: Circulation Journal: Official Journal of the Japanese Circulation Society
https://www.readbyqxmd.com/read/28434546/surgical-strategy-toward-biventricular-repair-for-severe-ebstein-anomaly-in-neonates-and-infancy
#13
Shu-Chien Huang, En-Ting Wu, Shyh-Jye Chen, Chi-Hsiang Huang, Jin-Chung Shih, Hen-Wen Chou, Chung-I Chang, Ing-Sh Chiu, Yih-Sharng Chen
BACKGROUND: Neonates with severe forms of Ebstein anomaly present a surgical challenge, and the Starnes operation as single ventricle palliation is highly advocated. Cone reconstruction for tricuspid valvuloplasty (TVP) has become a widely accepted technique, although very few cases of TVP have been reported in neonates. This report describes a surgical strategy for neonatal Ebstein anomaly, with an aim toward biventricular repair. METHODS: Since 2007, 7 neonates or young infants with severe Ebstein anomalies have received TVP at the National Taiwan University Hospital, Taipei, Taiwan...
April 20, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28426384/a-2-year-old-boy-with-circulatory-failure-owing-to-streptococcal-toxic-shock-syndrome-case-report
#14
Werner Keenswijk, Johan Vande Walle
A 2-year-old boy presented with severe hypotension and acute kidney injury after a prodrome of non-bloody diarrhoea and fever in the preceding 3 days. He had a mild Ebstein cardiac anomaly but otherwise a normal past history and growth. On examination, he looked ill, his temperature was 37.5 °C, circulation was poor, and there were several purpuric lesions on the face, hands and scrotum. Haemoglobin was 7.8 g/dL (11-14), total white cell count 27 × 10(9)/L, platelets 62 × 10(9)/L, blood urea nitrogen 20...
April 20, 2017: Paediatrics and International Child Health
https://www.readbyqxmd.com/read/28410946/right-atrial-dysfunction-in-the-fetus-with-severely-regurgitant-tricuspid-valve-disease-a-potential-source-of-cardiovascular-compromise
#15
Lisa W Howley, Nee Scze Khoo, Anita J Moon-Grady, Sonali S Patel, Fayeza Alrais, Wayne Tworetzky, Timothy Colen, Paul Brooks, Jean Trines, Tiina Ojala, Lisa K Hornberger
BACKGROUND: In severe right heart obstruction (RHO), redistribution of cardiac output to the left ventricle (LV) is well tolerated by the fetal circulation. Although the same should be true of severely regurgitant tricuspid valve disease (rTVD) with reduced or no output from the right ventricle, affected fetuses more frequently develop hydrops or suffer intrauterine demise. We hypothesized that right atrium (RA) function is altered in rTVD but not in RHO, which could contribute to differences in outcomes...
April 11, 2017: Journal of the American Society of Echocardiography
https://www.readbyqxmd.com/read/28405996/ebstein-s-anomaly-with-paradoxical-embolism-to-lower-limb
#16
Gouranga Santra, Shinjan Patra
Ebstein's anomaly (EA) is a rare congenital heart disease. Paradoxical embolism (PDE) may occur in EA due to associated atrial septal defect (ASD). An 18 year old male with EA presented with left lower limb ischemia due to PDE to the limb. No features of cerebral embolism was present. PDE is a potential complication of EA. High level of awareness is needed for early diagnosis of PDE in EA to save a limb in case of embolism to the extremity.
December 2016: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/28388435/de-novo-disruption-of-the-proteasome-regulatory-subunit-psmd12-causes-a-syndromic-neurodevelopmental-disorder
#17
Sébastien Küry, Thomas Besnard, Frédéric Ebstein, Tahir N Khan, Tomasz Gambin, Jessica Douglas, Carlos A Bacino, William J Craigen, Stephan J Sanders, Andrea Lehmann, Xénia Latypova, Kamal Khan, Mathilde Pacault, Stephanie Sacharow, Kimberly Glaser, Eric Bieth, Laurence Perrin-Sabourin, Marie-Line Jacquemont, Megan T Cho, Elizabeth Roeder, Anne-Sophie Denommé-Pichon, Kristin G Monaghan, Bo Yuan, Fan Xia, Sylvain Simon, Dominique Bonneau, Philippe Parent, Brigitte Gilbert-Dussardier, Sylvie Odent, Annick Toutain, Laurent Pasquier, Deborah Barbouth, Chad A Shaw, Ankita Patel, Janice L Smith, Weimin Bi, Sébastien Schmitt, Wallid Deb, Mathilde Nizon, Sandra Mercier, Marie Vincent, Caroline Rooryck, Valérie Malan, Ignacio Briceño, Alberto Gómez, Kimberly M Nugent, James B Gibson, Benjamin Cogné, James R Lupski, Holly A F Stessman, Evan E Eichler, Kyle Retterer, Yaping Yang, Richard Redon, Nicholas Katsanis, Jill A Rosenfeld, Peter-Michael Kloetzel, Christelle Golzio, Stéphane Bézieau, Paweł Stankiewicz, Bertrand Isidor
No abstract text is available yet for this article.
April 6, 2017: American Journal of Human Genetics
https://www.readbyqxmd.com/read/28361074/neonatal-ebstein-repair-where-are-we-now
#18
EDITORIAL
Philip L Wackel, Joseph A Dearani, Frank Cetta
No abstract text is available yet for this article.
March 2017: Annals of Translational Medicine
https://www.readbyqxmd.com/read/28343629/biallelic-variants-in-otud6b-cause-an-intellectual-disability-syndrome-associated-with-seizures-and-dysmorphic-features
#19
Teresa Santiago-Sim, Lindsay C Burrage, Frédéric Ebstein, Mari J Tokita, Marcus Miller, Weimin Bi, Alicia A Braxton, Jill A Rosenfeld, Maher Shahrour, Andrea Lehmann, Benjamin Cogné, Sébastien Küry, Thomas Besnard, Bertrand Isidor, Stéphane Bézieau, Isabelle Hazart, Honey Nagakura, LaDonna L Immken, Rebecca O Littlejohn, Elizabeth Roeder, Bulent Kara, Katia Hardies, Sarah Weckhuysen, Patrick May, Johannes R Lemke, Orly Elpeleg, Bassam Abu-Libdeh, Kiely N James, Jennifer L Silhavy, Mahmoud Y Issa, Maha S Zaki, Joseph G Gleeson, John R Seavitt, Mary E Dickinson, M Cecilia Ljungberg, Sara Wells, Sara J Johnson, Lydia Teboul, Christine M Eng, Yaping Yang, Peter-Michael Kloetzel, Jason D Heaney, Magdalena A Walkiewicz
Ubiquitination is a posttranslational modification that regulates many cellular processes including protein degradation, intracellular trafficking, cell signaling, and protein-protein interactions. Deubiquitinating enzymes (DUBs), which reverse the process of ubiquitination, are important regulators of the ubiquitin system. OTUD6B encodes a member of the ovarian tumor domain (OTU)-containing subfamily of deubiquitinating enzymes. Herein, we report biallelic pathogenic variants in OTUD6B in 12 individuals from 6 independent families with an intellectual disability syndrome associated with seizures and dysmorphic features...
April 6, 2017: American Journal of Human Genetics
https://www.readbyqxmd.com/read/28340054/outcomes-of-atrial-arrhythmia-radiofrequency-catheter-ablation-in-patients-with-ebstein-s-anomaly
#20
Abdallah Hassan, Nicholas Y Tan, Htin Aung, Heidi M Connolly, David O Hodge, Emily R Vargas, Bryan C Cannon, Douglas L Packer, Samuel J Asirvatham, Christopher J McLeod
Aims: Atrial arrhythmias are common in patients with Ebstein's anomaly (EA) despite cardiac surgical repair and concomitant Maze procedures. We aimed to evaluate the outcome of radiofrequency catheter ablation in this group of patients. Methods and results: All patients with EA and atrial arrhythmias who underwent catheter ablation for atrial arrhythmias between 1/1999 and 1/2016 were included. Atrial arrhythmia recurrence was identified as the primary outcome; secondary outcomes included repeat ablation, need for antiarrhythmic medications after ablation, and death...
February 27, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
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