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ebsteins anomaly

Jeremy P Moore, Kevin M Shannon, Roberto G Gallotti, Christopher J McLeod, Anca Chiriac, Edward P Walsh, Narayanswami Sreeram, Akash R Patel, Natasja M De Groot, Johannes von Alvensleben, Seshadri Balaji, David S Frankel, Christina Y Miyake, James C Perry, Kalyanam Shivkumar
OBJECTIVES: The purpose of this study was to determine the ventricular arrhythmia (VA) substrates in patients with unoperated and post-surgical Ebstein's Anomaly (EA). BACKGROUND: EA is associated with variable atrialization of the right ventricle and a propensity for VA and sudden death. There are scant data on catheter ablation for VA in this population. METHODS: This was a retrospective study involving 11 congenital heart disease centers...
October 2018: JACC. Clinical Electrophysiology
Edward P Walsh
Ebstein's anomaly of the tricuspid valve is a relatively rare form of congenital heart disease that has long been a challenge to electrophysiologists and cardiac surgeons. In addition to the hemodynamic burden of the actual valve defect, Ebstein's patients must also contend with an extraordinarily high incidence of tachyarrhythmias, most of which can be attributed to accessory atrioventricular pathways (APs) located along the posterior and septal border of the tricuspid valve where the valve leaflets are most abnormal...
October 2018: JACC. Clinical Electrophysiology
Haleema Akhter Hashmi, Hafeeza Khatoon, Syed Ijlal Ahmed
Ebstein anomaly is a rare malformation of the tricuspid valve of the heart. The malformed tricuspid valve may be incompetent, stenotic, or rarely, imperforate. Pregnant patients with Ebstein anomaly become a challenge for the physician in terms of management. We report a case in which patient delivered successfully with Ebstein anomaly. The only complication was breathlessness. Authors concluded that women with Ebstein anomaly can have successful outcomes of pregnancy with close monitoring.
September 2018: JPMA. the Journal of the Pakistan Medical Association
Ann-Sofie Alderweireldt, Daniel De Wolf
We report the use of an Amplatzer Vascular plug for percutaneous closure of a paravalvular leak after Cone repair in a 5-year-old boy with Ebstein's anomaly. A paravalvular leak of the tricuspid valve developed gradually after Cone repair-surgery. The combination of fluoroscopy, transesophageal and transthoracic imaging during general anesthesia was necessary for correct and safe positioning of the device by percutaneous approach. The results were promising.
October 2, 2018: Catheterization and Cardiovascular Interventions
Dan Yang, Xiao Li, Jia-Yu Sun, Wei Cheng, Andreas Greiser, Tian-Jing Zhang, Hong Liu, Ke Wan, Yong Luo, Qi An, Yiu-Cho Chung, Yuchi Han, Yu-Cheng Chen
BACKGROUND: Myocardial fibrosis is a common pathophysiological process that is related to ventricular remodeling in congenital heart disease. However, the presence, characteristics, and clinical significance of myocardial fibrosis in Ebstein's anomaly have not been fully investigated. This study aimed to evaluate myocardial fibrosis using cardiovascular magnetic resonance (CMR) late gadolinium enhancement (LGE) and T1 mapping techniques, and to explore the significance of myocardial fibrosis in adolescent and adult patients with Ebstein's anomaly...
September 27, 2018: Journal of Cardiovascular Magnetic Resonance
Bibhuti B Das, Maryanne Chrisant, Gerald Lavandosky, Megan Zakrzewski, Robert Winchester, Immanuel Turner, Steven Bibevski, Frank Scholl
No abstract text is available yet for this article.
September 11, 2018: Journal of Pediatrics
Akash Kasatwar, Rajiv Borle, Nitin Bhola, Rajanikanth K, G S V Prasad, Anendd Jadhav
Background: Cleft lip and palate is one of the most common congenital craniofacial deformities seen in children. Various congenital anomalies are reported in the literature to be associated with cleft lip and palate. Cardiac anomalies are one of the most common congenital disorders associated in cleft lip and palate patientsIt includes Cyanotic and acyanotic cardiac diseases likel fallot's tetralogy, transposition of greater vessels, atresia of tricuspid, total anomalous pulmonary venous return (TAPVR), truncus arteriosus, ebstein's anomaly, hypoplastic left heart syndrome and pulmonary atresia, patent ductus arteriosus, ventricular septal defect, atrial septal defect, pulmonary stenosis, aortic stenosis and coarctation of aorta...
September 2018: Journal of Oral Biology and Craniofacial Research
Masahiko Nishioka, Tai Fuchigami, Toru Akashige, Nobuhiro Nagata
A combination of double outlet right ventricle(DORV) with Ebstein's anomaly and aortic coarctation (CoA) is vary rare. We report a successful staged surgical repair for DORV with Ebstein's anomaly associated with CoA. The patient had subaortic perimembronous ventricular septal defect (10×7.5 mm) and small atrial septal defect( 3 mm). Two great arteries were side by side position. The posterior leaflet of the tricuspid valve was 10 mm displaced to the right ventricle and tricuspid valve regurgitation was severe...
August 2018: Kyobu Geka. the Japanese Journal of Thoracic Surgery
Amber Malhotra, Vishal Agrawal, Kartik Patel, Mausam Shah, Kamal Sharma, Pranav Sharma, Sumbul Siddiqui, Nilesh Oswal, Himani Pandya
OBJECTIVE: Ebstein's anomaly remains a relatively ignored disease. Lying in the 'No Man's land' between congenital and valve surgeons, it largely remains inadequately studied. We report our short-term results of treating it as a 'one and a half ventricle heart' and propose that the true tricuspid annulus (TTA) 'Z' score be used as an objective criterion for estimation of 'functional' right ventricle (RV). METHODS: 22 consecutive patients undergoing surgery for Ebstein's anomaly were studied...
July 2018: Brazilian Journal of Cardiovascular Surgery
Victor Waldmann, Paul Khairy
No abstract text is available yet for this article.
July 2018: Journal of Thoracic Disease
Sang Hyun Lee, Hyung Oh Choi, Ki Won Hwang
No abstract text is available yet for this article.
August 2018: HeartRhythm Case Reports
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...
September 1, 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
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