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https://www.readbyqxmd.com/read/28544841/transcatheter-valve-in-valve-implantation-failing-tricuspid-bioprosthesis-in-a-patient-with-ebstein%C3%A2-s-anomaly
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
https://www.readbyqxmd.com/read/28316109/airway-inflammation-and-symptoms-in-children-following-liver-and-heart-transplantation
#17
Andrew J Redmann, John C Bucuvalas, Robert E Wood, Clifford Chin, Catherine K Hart
OBJECTIVES: To describe the upper airway endoscopic findings of children with upper airway symptoms after liver transplantation (LT) or heart transplantation (HT). METHODS: Review of children undergoing airway endoscopy after LT or HT from 2011 to 2015 at a tertiary care pediatric hospital. Airway findings, biopsy results, immunosuppression, and Epstein-Barr virus (EBV) levels were recorded. RESULTS: Twenty-three of 158 LT (111) and HT (47) recipients underwent endoscopy...
March 18, 2017: Clinical Transplantation
https://www.readbyqxmd.com/read/28267539/assessment-of-left-ventricular-deformation-in-patients-with-ebstein-s-anomaly-by-cardiac-magnetic-resonance-tissue-tracking
#18
Xi Liu, Qin Zhang, Zhi-Gang Yang, Ke Shi, Hua-Yan Xu, Lin-Jun Xie, Li Jiang, Kai-Yue Diao, Ying-Kun Guo
PURPOSE: The aim of this study was to clarify the feasibility of myocardial strain using cardiovascular magnetic resonance (CMR) for the evaluation of left ventricular (LV) deformation in patients with Ebstein's anomaly (EA). MATERIALS AND METHODS: We recruited 32 patients with EA and 30 controls for CMR examination and measured LV function, dimension and tissue tracking parameters (the global and regional radial, circumferential and longitudinal peak strain), together with the right ventricle (RV) dimension...
April 2017: European Journal of Radiology
https://www.readbyqxmd.com/read/28265211/modified-cone-reconstruction-of-the-tricuspid-valve-for-ebstein-anomaly-as-performed-in-siberia
#19
Evgeny V Krivoshchekov, Jaeger P Ackerman, Olga S Yanulevich, Alexander A Sokolov, Nadezhda V Ershova, Joseph A Dearani, Frank Cetta
The cone reconstruction technique, first described by da Silva and modified by Dearani and by others, has become the repair method of choice in patients with Ebstein anomaly of the tricuspid valve. This report details the outcome of the modified cone reconstruction technique in 6 children who underwent surgical correction of Ebstein anomaly at the Tomsk Institute of Cardiology in Siberia. From 2012 through 2015, 4 boys and 2 girls (age range, 11 mo-12 yr) underwent surgery to correct Ebstein anomaly. All had presented with cyanosis, exertional dyspnea, fatigue, or new-onset atrial arrhythmia, and none had undergone previous cardiac surgery...
February 2017: Texas Heart Institute Journal
https://www.readbyqxmd.com/read/28258242/successful-cardiac-transplantation-outcomes-in-patients-with-adult-congenital-heart-disease
#20
Jonathan N Menachem, Jessica R Golbus, Maria Molina, Jeremy A Mazurek, Nicole Hornsby, Pavan Atluri, Stephanie Fuller, Edo Y Birati, Yuli Y Kim, Lee R Goldberg, Joyce W Wald
OBJECTIVES: The purpose of our study is (1) to characterise patients with congenital heart disease undergoing heart transplantation by adult cardiac surgeons in a large academic medical centre and (2) to describe successful outcomes associated with our multidisciplinary approach to the evaluation and treatment of adults with congenital heart disease (ACHD) undergoing orthotopic heart transplantation (OHT). BACKGROUND: Heart failure is the leading cause of death in patients with ACHD leading to increasing referrals for OHT...
March 3, 2017: Heart: Official Journal of the British Cardiac Society
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