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https://www.readbyqxmd.com/read/29120021/swiss-adult-congenital-heart-disease-registry-sacher-rationale-design-and-first-results
#1
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
#2
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
#3
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
https://www.readbyqxmd.com/read/29021860/atriofascicular-mahaim-with-ebstein-anomaly-a-case-report
#4
Kazushi Ueshima, Yoshihide Nakamura, Satoru Takeno, Toshiharu Miyake, Tsukasa Takemura
We report a case of a 7-year-old girl with atriofascicular Mahaim (AFM) pathway concomitant with Ebstein's anomaly. The QRS wave showed left bundle branch block pattern on electrocardiogram. Holter electrocardiogram showed prolongation of the PR interval and QRS morphological change during sinus tachycardia. An electrophysiological study demonstrated that the distal His potential appeared earlier than the proximal His potential, which suggested retrograde His conduction toward the atrioventricular node. Conduction from the Mahaim fiber to the His bundle was faster than that from the atrioventricular node towards the His bundle...
October 2017: Journal of Arrhythmia
https://www.readbyqxmd.com/read/29017675/familial-hodgkin-lymphoma
#5
Mahwish Faizan, Rahat Ul Ain Kashif, Saadia Anwar, Mansoor Safdar
Hodgkin Lymphoma (HL) is a lymphoid tumour that represents about 1% of all neoplasms occurring worldwide. HLis the most treatable of childhood malignancies. The etiology of HLis unknown. However, increase risk has been reported in males, with autoimmune diseases, poor socioeconomic status, increased family size, Ebstein Barr Virus (EBV) exposure, congenital or acquired immunodeficiency and those with a family history of HL. Familial HLis rare. The risk of developing HLis increased six times in the siblings of the affected patients...
September 2017: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/28974156/surgical-management-of-neonatal-ebstein-s-anomaly-associated-with-circular-shunt
#6
Chawki Elzein, Sujata Subramanian, Michel Ilbawi
Ebstein's anomaly is a rare congenital cardiac malformation that may present in the neonatal period with life-threatening physiologic derangement, especially when it is associated with circular shunt. Urgent surgical intervention is critical for survival because of hemodynamic compromise. We review our experience with surgical management of neonates with Ebstein's anomaly associated with circular shunt and review the literature for similar reported cases. The underlying pathophysiology and different options of treatment are discussed...
January 1, 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28966329/cardiac-resynchronization-therapy-in-patients-with-ebstein-s-anomaly
#7
Genri Numata, Eisuke Amiya, Toshiya Kojima, Katsuhito Fujiu, Masaru Hatano, Masafumi Watanabe, Issei Komuro
Ebstein's anomaly is a rare congenital heart disease characterized by apical displacement of the septal and posterior tricuspid valve leaflets. It is often complicated with left ventricular (LV) dysfunction as well as right-sided abnormalities. On the other hand, in the presence of LV dysfunction, right ventricular pacing is likely to aggravate the diseased LV function, which is termed pacemaker-induced cardiomyopathy. Thus, deteriorating effects of RV pacing on cardiac function might be enhanced and result in pacemaker-induced cardiomyopathy in patients with Ebstein's anomaly, even if they have preserved LVEF...
October 21, 2017: International Heart Journal
https://www.readbyqxmd.com/read/28958645/neonatal-ebstein-anomaly
#8
T K Susheel Kumar, Umar S Boston, Christopher J Knott-Craig
No abstract text is available yet for this article.
September 25, 2017: Seminars in Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28950308/change-in-biventricular-function-after-cone-reconstruction-of-ebstein-s-anomaly-an-echocardiographic-study
#9
E Perdreau, V Tsang, M L Hughes, M Ibrahim, S Kataria, K Janagarajan, X Iriart, S Khambadkone, J Marek
Aims: The Cone reconstruction in Ebstein's anomaly (EA) aims to reduce tricuspid valve regurgitation (TR) and reposition the valve to the anatomic annulus, but post-operative progress of ventricular function is poorly understood. This study evaluated biventricular function after Cone reconstruction using echocardiographic techniques. Methods and results: A retrospective study assessing longitudinal change was conducted from 2009 to 2014. All symptomatic patients with EA and severe TR undergoing surgery were included...
July 31, 2017: European Heart Journal Cardiovascular Imaging
https://www.readbyqxmd.com/read/28940541/ebstein-anomaly-and-sudden-childhood-death
#10
Amanda Freeman, Roger W Byard
A 13-year-old girl is reported who died suddenly and unexpectedly in her sleep from previously undiagnosed Ebstein anomaly. At autopsy, there was dilatation of the right atrium with marked dilatation of the right auricle and apical displacement of the tricuspid valve into the right ventricular cavity with atrialization of the upper portion of the right ventricle. There were also prominent dysplastic changes in both the septal and posterior leaflets of the tricuspid valve with thickening of the valve and fusion of leaflets to the wall of the ventricle...
September 20, 2017: Journal of Forensic Sciences
https://www.readbyqxmd.com/read/28928616/chronic-antepartum-maternal-hyperoxygenation-in-a-case-of-severe-fetal-ebstein-s-anomaly-with-circular-shunt-physiology
#11
Alisa Arunamata, David M Axelrod, Katherine Bianco, Sowmya Balasubramanian, Amy Quirin, Theresa A Tacy
Perinatal mortality remains high among fetuses diagnosed with Ebstein's anomaly of the tricuspid valve. The subgroup of patients with pulmonary valve regurgitation is at particularly high risk. In the setting of pulmonary valve regurgitation, early constriction of the ductus arteriosus may be a novel perinatal management strategy to reduce systemic steal resulting from circular shunt physiology. We report the use of chronic antepartum maternal oxygen therapy for constriction of the fetal ductus arteriosus and modulation of fetal pulmonary vascular resistance in a late presentation of Ebstein's anomaly with severe tricuspid valve regurgitation, reversal of flow in the ductus arteriosus, and continuous pulmonary valve regurgitation...
September 2017: Annals of Pediatric Cardiology
https://www.readbyqxmd.com/read/28919627/ebstein-s-anomaly-associated-with-atrial-septal-defect-and-wolff-parkinson-white-wpw-syndrome
#12
M N Hasan, K Ahmed, S M Ahmed, M M Rahman
Ebstein's anomaly is a rare congenital heart disorder, accounting for <1% of all cases of congenital heart disease. It is a congenital malformation of the heart that is characterized by apical displacement of the septal and posterior tricuspid valve leaflets, leading to atrialization of the right ventricle with a variable degree of malformation and displacement of the anterior leaflet. We report the case of a 25 years old female with Ebstein's anomaly which was associated with Ostium Secundum type of atrial septal defect and WPW syndrome, who presented with dyspnea, palpitations, cyanosis, clubbing and cardiomegaly...
July 2017: Mymensingh Medical Journal: MMJ
https://www.readbyqxmd.com/read/28917495/complications-associated-with-implantable-cardioverter-defibrillators-in-adults-with-congenital-heart-disease-or-left-ventricular-noncompaction-cardiomyopathy-from-the-ncdr-%C3%A2-implantable-cardioverter-defibrillator-registry
#13
MULTICENTER STUDY
Marye J Gleva, Yongfei Wang, Jeptha P Curtis, Charles I Berul, Charles B Huddleston, Jeanne E Poole
Patients with childhood heart disease are living longer and entering adulthood, and may undergo implantable cardioverter-defibrillator (ICD) implantation to reduce the risk of sudden death. We evaluated the characteristics of adult patients with congenital heart disease or left ventricular noncompaction cardiomyopathy (LVNC) in the National Cardiovascular Disease Registry ICD Registry and determined ICD-related in-hospital complications. Patients with LVNC or transposition of the great arteries, tetralogy of Fallot, Ebstein's anomaly, atrial septal defect, ventricular septal defect, or common ventricle were identified in the registry...
November 15, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28867680/coexistence-of-a-double-chambered-right-ventricle-with-ebstein-like-anomaly-and-right-ventricular-apical-hypoplasia%C3%A3-usefulness-of-multimodality-imaging
#14
Kyohei Marume, Shigefumi Fukui, Keiko Shimamoto, Hideo Ohuchi, Akihiro Tsuji, Takeshi Ogo, Norifumi Nakanishi, Satoshi Yasuda
No abstract text is available yet for this article.
September 1, 2017: Circulation Journal: Official Journal of the Japanese Circulation Society
https://www.readbyqxmd.com/read/28826312/spontaneous-resolution-of-epstein-barr-virus-induced-haemophagocytic-lymphohistiocytosis-in-still-s-disease-treated-by-tocilizumab-a-case-report
#15
E Ebstein, H-K Ea
No abstract text is available yet for this article.
August 21, 2017: Scandinavian Journal of Rheumatology
https://www.readbyqxmd.com/read/28806824/surgical-treatment-of-ebstein-s-anomaly
#16
Melchior Burri, Rüdiger Lange
No abstract text is available yet for this article.
August 14, 2017: Thoracic and Cardiovascular Surgeon
https://www.readbyqxmd.com/read/28766722/fatal-diabetic-ketoacidosis-a-potential-complication-of-mdma-ecstasy-use
#17
John D Gilbert, Roger W Byard
A 19-year-old woman with insulin-dependent diabetes mellitus was found dead in bed having allegedly recently taken ecstasy and consumed alcohol. At autopsy, there were microhemorrhages in the brain with subnuclear vacuolization and Armanni-Ebstein changes in renal tubules. Biochemical analyses confirmed diabetic ketoacidosis (vitreous glucose-46.5 mmol/L; β-OH butyrate-13.86 mmol/L.). Toxicological analyses of blood showed a low level of 3,4-methylenedioxy-methamphetamine (MDMA) (0.01 mg/L), with acetone but no alcohol or other common drugs...
August 2, 2017: Journal of Forensic Sciences
https://www.readbyqxmd.com/read/28766512/radiofrequency-ablation-of-accessory-pathways-in-a-toddler-with-ebstein-s-anomaly-and-functional-single-ventricle-physiology
#18
Hasan Candaş Kafalı, İsa Özyılmaz, Serkan Ünal, Alper Güzeltaş, Yakup Ergül
No abstract text is available yet for this article.
August 2017: Anatolian Journal of Cardiology
https://www.readbyqxmd.com/read/28761400/the-two-anomalies-of-wilhelm-ebstein
#19
Magdalena Mazurak, Jacek Kusa
The year 2016 was the 180th anniversary of Wilhelm Ebstein's birth and the 150th anniversary of his description of the congenital tricuspid valve malformation that came to be known as Ebstein anomaly. We present a brief history of the life and work of Dr. Wilhelm Ebstein. Despite his distinguished career, he is seemingly forgotten in his own birthplace. We include a review of the relevant historical medical literature.
June 2017: Texas Heart Institute Journal
https://www.readbyqxmd.com/read/28749297/ebstein-anomaly-in-the-tsushima-leopard-cat-prionailurus-bengalensis-euptilurus
#20
Shunsuke Shimamura, Yukihiro Shiota, Naoko Takagi, Tatsuya Habara, Shougo Hirata, Hitoshi Komai, Satoko Nishimura, Hiroyuki Tani, Terumasa Shimada
Ebstein anomaly is a rare congenital heart disease that has been described in domestic dogs, a meerkat, a pygmy goat, and a lion. An 11-mo-old Tsushima leopard cat presented to Osaka Prefecture University Veterinary Hospital for diagnosis and treatment of right-sided congestive heart failure. Echocardiography showed a dilated right atrium and ventricle with an enlarged tricuspid valve annulus and apical displacement of the tricuspid valve leaflets. The cat was diagnosed with Ebstein anomaly. To the best of our knowledge, this is the first report of this type of congenital heart disease in a Tsushima leopard cat...
June 2017: Journal of Zoo and Wildlife Medicine: Official Publication of the American Association of Zoo Veterinarians
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