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ebsteins anomaly

Andrés Ricardo Pérez-Riera, Raimundo Barbosa-Barros, Rodrigo Daminello-Raimundo, Luiz Carlos de Abreu, Kjell Nikus
Ebstein's anomaly is a congenital heart disease where the most important anatomic feature is the inferior displacement of the tricuspid valve leaflets. Vectorcardiographic features are mainly forgotten and electrocardiographic features may be unrecognized by cardiologists handling adult patients.
August 14, 2018: Annals of Noninvasive Electrocardiology
Muzaffer Kahyaoğlu, Çetin Geçmen, Özkan Candan, İbrahim Akın İzgi
No abstract text is available yet for this article.
August 2018: Anatolian Journal of Cardiology
Christos Tourmousoglou, Harilaos Bogossian, Vlasis Ninios, Elias Ninios
Ebstein's anomaly is a rare congenital cardiac disease characterized by apical displacement of the tricuspid valve with decreased right ventricular function. Left ventricular noncompaction is a cardiomyopathy characterized by the presence of numerous and prominent trabeculations together with deep intertrabecular recesses in a portion of the ventricular wall, principally at the lateral and apical level. We describe the very rare case of a patient living for 78 years with a benign form of Ebstein's anomaly and left ventricular noncompaction cardiomyopathy with slightly reduced systolic function...
August 6, 2018: Asian Cardiovascular & Thoracic Annals
Fatemat Hassan, Akash Patel, Vadiyala Mohan Reddy, Hythem Nawaytou
No abstract text is available yet for this article.
February 2018: CASE: Cardiovascular Imaging Case Reports
Rebekka Lytzen, Niels Vejlstrup, Jesper Bjerre, Olav Bjørn Petersen, Stine Leenskjold, James Keith Dodd, Finn Stener Jørgensen, Lars Søndergaard
Importance: The occurrence of major congenital heart disease (CHD) is affected by several variables. Determining the development of the true incidence is critical to the establishment of proper treatment of these patients. Objective: To evaluate time trends in incidence, detection rate, and termination of pregnancy (TOP) rate of major CHD in fetuses in Denmark and assess the influence of the introduction of general prenatal screening in 2004. Design, Setting, and Participants: Nationwide, population-based, retrospective observational study in Denmark from 1996 to 2013 that included a consecutive sample of 14 688 live-born children and terminated fetuses diagnosed as having CHD...
July 18, 2018: JAMA Cardiology
Meriem Mostefa-Kara, Lucile Houyel, Damien Bonnet
BACKGROUND: A ventricular septal defect (VSD) is an integral part of most congenital heart defects (CHD). To determine the prevalence of VSD in various types of CHD and the distribution of their anatomic types. METHODS: We reviewed 1178 heart specimens with CHD from the anatomic collection of the French Reference Centre for Complex Congenital Heart Defects. During the morphologic study a special attention was paid to the localisation of the VSD viewed from the right ventricular side...
July 18, 2018: Orphanet Journal of Rare Diseases
Nalini Sharma, Thiek Jion Lalnunnem, Megha Nandwani, Singh Ahanthem Santa, Baingen Warjari Synrang
Background: Ebstein anomaly is an uncommon, complex congenital malformation of the heart with prevalence of 0.3-0.5%. It occurs in 1% of congenital heart disease cases. It is characterized by dysplastic abnormalities of tricuspid valve which involves both basal and free attachments of the tricuspid valve leaflets, with downward displacement and elongation of the septal and anterior cusp which resulting in tricuspid regurgitation, the proximal part of the right ventricle is "atrialised", becoming thin walled and poorly contractile, along with an enlarged right atrium...
April 2018: Journal of Reproduction & Infertility
Horacio Márquez-González, Mario H Vargas, Lucelli Yáñez-Gutiérrez, Eduardo Almeida-Gutiérrez, Juan Garduño-Espinosa
Objective: Magnetic resonance imaging (MRI) and cardiac catheterization are diagnostic tools for right ventricle dysfunction (RVD), but those are expensive and often unavailable techniques. Thus, our objective was to identify clinical and/or echocardiographic variables capable of predicting a catheterization-based diagnosis of RVD. Design: This was cross-sectional, diagnostic test accuracy study, considering the catheterization-based diagnosis of RVD as the gold standard. Patients: Pediatric patients with non-repaired CHD with overload pressure were evaluated...
2018: Frontiers in Pediatrics
Serkan Topaloglu, Ozcan Ozeke, Serkan Cay, Firat Ozcan, Serhat Koca, Dursun Aras
Ebstein's anomaly is often accompanied by either Wolff-Parkinson-White syndrome or atriofascicular Mahaim. These bypass tracts give rise to antidromic atrioventricular (AV) re-entrant tachycardias, in which the bypass tract serves as the anterograde limb of the circuit and the AV node as the retrograde limb of the reentrant circuit. Since the antidromic AV reentrant tachycardia over a Mahaim fibre has a typically left bundle braunch block (LBBB) morphology, it is easy to make a misdiagnosis of supraventricular tachycardia with functional LBBB or even of ventricular tachycardia particularly in the presence of negative concordance...
July 2018: Journal of Electrocardiology
Inga Voges, Mouaz H Al-Mallah, Giancarlo Scognamiglio, Giovanni Di Salvo
The right ventricle plays a major role in congenital heart disease. This article describes the right ventricular mechanics in some selected congenital heart diseases affecting the right ventricle in different ways: tetralogy of Fallot, Ebstein anomaly, and the systemic right ventricle.
July 2018: Heart Failure Clinics
Marta-Catalina Miranda-Fernández, Silvia Ramírez-Oyaga, Carlos M Restrepo, Victor-Manuel Huertas-Quiñones, Magally Barrera-Castañeda, Rossi Quero, Camilo-José Hernández-Toro, Claudia Tamar Silva, Paul Laissue, Rodrigo Cabrera
Ebstein anomaly (EA) is a rare congenital heart defect (CHD) with a poorly characterized genetic etiology. However, some EA patients carry deletions in 1p36, all of which have been reported to carry distal deletions and share loss of the PRDM16 gene, which is currently considered the most likely candidate for EA development in this region. Here, we report a patient with an 11.96-Mb proximal 1p36 deletion, without loss of PRDM16 , who presented with EA and a proximal deletion phenotype. This finding suggests that PRDM16 loss is not required for the development of EA in 1p36 deletions and that the loss of an additional proximal locus in 1p36 is also likely associated with EA...
May 2018: Molecular Syndromology
Ibrahim Aliyu, Zainab Fumilayo Ibrahim
Cor-triatriatum is uncommon and cor-triatriatum dexter is rarer, and patients may remain asymptomatic in isolated cases especially if the partitioning is mild and nonobstructing. There may be other cardiac defects associated with it which are mostly right-sided cardiac defects such as atrial septal defect, Ebstein anomaly, and pulmonary stenosis. However, left-sided heart anomaly such as aortic regurgitation has been associated with it, but its association with transposition of the great vessels has not been documented before now...
April 2018: Journal of Cardiovascular Echography
X F Yu, L S Qiu, J F Liu, X Liu, H F Hong
Objective: To explore the surgical strategy for Ebstein anomaly in children. Methods: From January 2003 to December 2015, a total of 141 cases of Ebstein anomaly were treated at Department of Pediatric Cardiothoracic Surgery, Shanghai Children's Medical Center, School of Medicine, Shanghai Jiaotong University. There were 65 male and 76 female patients, with age of (6.9±1.6) years (ranging from 10 months to 15 years), weight of (19.6±4.7) kg (ranging from 6.5 to 59.0 kg). All patients were diagnosed by 2 dimensional Doppler echocardiography and the septal leaflet and posterior leaflet displaced downward from 1...
June 1, 2018: Zhonghua Wai Ke za Zhi [Chinese Journal of Surgery]
X Y Zhang, Q Y Wu, B Dong, H Y Li, M K Zhang, Y Q Jin
Objective: To evaluate the early and long-term outcomes cardiac surgery of patients with Ebstein anomaly. Methods: The clinic data of 237 patients with Ebstein anomaly received surgical procedures from March 2004 to December 2017 at Department of Cardiac Surgery, First Hospital of Tsinghua University was analyzed retrospectively. There were 105 male and 132 female patients with age of (19.4±16.7) years (ranging from 3 months to 64 years). The surgical procedures include anatomical repair in 188 patients, one and a half ventricle repair in 37 patients, tricuspid valve repair in 4 patients, tricuspid valve replacement in 10 patients, and Fontan procedure in 3 patients (total cavopulmonary connection in 2 patients; Glenn procedure in 1 patient)...
June 1, 2018: Zhonghua Wai Ke za Zhi [Chinese Journal of Surgery]
Yakup Ergul, Serhat Koca, Celal Akdeniz, Volkan Tuzcu
In Ebstein's anomaly (EA), tachycardia substrates are complex, and accessory pathway (AP) ablations are often challenging. This study demonstrates the utility of the EnSite Velocity system (St. Jude Medical, St Paul, MN) in the catheter ablation of supraventricular tachycardia in children with EA. Twenty patients [Female/Male = 8/12, median age 11.5 years (2.6-18)] with EA who underwent catheter ablation guided by the EnSite Velocity system between December 2011 and December 2016 were retrospectively evaluated...
June 7, 2018: Pediatric Cardiology
Rebecca S Beroukhim, Linyuan Jing, David M Harrild, Brandon K Fornwalt, Abba Mejia-Spiegeler, Jonathan Rhodes, Sitaram Emani, Andrew J Powell
BACKGROUND: In addition to tricuspid regurgitation (TR) and right ventricular (RV) enlargement, patients with Ebstein anomaly are at risk for left ventricular (LV) dysfunction and dyssynchrony. We studied the impact of the cone tricuspid valve reconstruction operation on LV size, function, and dyssynchrony. METHODS: All Ebstein anomaly patients who had both pre- and postoperative cardiovascular magnetic resonance (CMR) studies were retrospectively identified. From cine images, RV and LV volumes and ejection fractions (EF) were calculated, and LV circumferential and longitudinal strain were measured by feature tracking...
May 21, 2018: Journal of Cardiovascular Magnetic Resonance
Kimberly A Holst, Joseph A Dearani, Sameh M Said, Ryan R Davies, Christian Pizarro, Christopher Knott-Craig, T K Susheel Kumar, Vaughn Starnes, S Ram Kumar, Sara K Pasquali, Dylan P Thibault, James M Meza, Kevin D Hill, Karen Chiswell, Jeffrey P Jacobs, Marshall L Jacobs
BACKGROUND: Ebstein anomaly (EA) encompasses a broad spectrum of morphology and clinical presentation. Those who are symptomatic early in infancy are generally at highest risk, but there are limited data regarding multi-centric practice patterns and outcomes. We analyzed multi-institutional data concerning operations and outcomes in neonates and infants with EA. METHODS: Index operations reported in the STS Congenital Heart Surgery Database (2010-2016) were potentially eligible for inclusion...
May 16, 2018: Annals of Thoracic Surgery
Miki Kanoh, Kei Inai, Tokuko Shinohara, Eriko Shimada, Mikiko Shimizu, Hirofumi Tomimatsu, Masaki Ogawa, Toshio Nakanishi
INTRODUCTION: We examined the perinatal outcomes and right ventricular function before pregnancy, during pregnancy, and after delivery in women with Ebstein's anomaly. MATERIAL AND METHODS: We retrospectively investigated the clinical course and mode of delivery and monitored hemodynamic parameters throughout pregnancy in 17 women with Ebstein's anomaly who delivered at our institution during the period of 1995-2015. RESULTS: Eight women, including nine pregnancies, underwent elective cesarean section, and nine women, including 14 pregnancies, underwent vaginal delivery...
May 16, 2018: Acta Obstetricia et Gynecologica Scandinavica
Safwat Aly, John Bokowski, Karim Diab, Brie Ann Muller
Ebstein anomaly of mitral valve (MV) is an extremely rare congenital heart disease. In the current report, we present a case of Ebstein of MV that was diagnosed prenatally. Fetal echocardiogram showed that the posterior leaflet of MV was tethered to the lateral wall of left ventricle (LV) with downward displacement into LV cavity. Postnatal transthoracic and transesophageal echocardiograms confirmed the diagnosis with apical displacement of the level of coaptation MV into the LV cavity. To the best of our knowledge, fetal diagnosis of Ebstein anomaly of MV has not yet been reported in the medical literature...
May 14, 2018: Pediatric Cardiology
Gabriel Cismaru, Lucian Muresan, Radu Rosu, Mihai Puiu, Gabriel Gusetu, Rodica Toganel, Dana Pop, Dumitru Zdrenghea
We present the case of a 17-year-old girl with Ebstein anomaly and repeated episodes of reentrant tachycardia due to a right posterior accessory pathway. Catheter ablation was performed using intracardiac echocardiography. A ViewFlex Xtra probe was inserted and showed an anormal tricuspid valve with elongated anterior leaflet and low insertion of the septal leaflet towards the apex. The anatomical annulus was identified by the course of the right coronary artery. RF application on the posterior annulus stopped the reentrant arrhythmia...
May 2, 2018: Medical Ultrasonography
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