Ebstein's anomaly of the tricuspid valve (EA) is an uncommon congenital cardiac malformation. It can present with atrioventricular tachycardia (AVRT), atrioventricular nodal re-entrant tachycardia (AVNRT), atrial arrhythmias, and rarely with ventricular tachycardia. The 12-lead electrocardiogram (ECG) is critically important and often diagnostic even prior to an electrophysiology study (EPS). Due to its complex anatomy, it poses particular challenges for mapping and ablation, even for an experienced electrophysiologist. In this review, we aim to provide insight into the electrophysiological perspective of EA and an in-depth analysis of the various arrhythmias encountered in diverse clinical scenarios.
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http://dx.doi.org/10.1007/s10840-024-01744-8 | DOI Listing |
Case Rep Cardiol
December 2024
Department of Anesthesiology and Pain Management, Cleveland Clinic, Cleveland, Ohio, USA.
Ebstein's anomaly is a rare congenital displacement of the tricuspid valve resulting in atrialization of the right ventricle. About half of the patients with Ebstein's anomaly also have atrial septal defects, which may lead to chronic shunting and development of Eisenmenger syndrome. We describe a case of a sexagenarian male patient with a history of Ebstein's anomaly complicated with Eisenmenger syndrome undergoing robotic laparoscopic adrenalectomy who presented hemodynamic instability, hypoxemia, and likely right-to-left shunting intraoperatively, as well as the actions taken to correct it and have a successful outcome.
View Article and Find Full Text PDFJ Cardiothorac Vasc Anesth
December 2024
Department of Cardiothoracic Surgery, National Heart Center, Royal Hospital, Muscat, Oman.
A circular shunt, initially described by Shone et al. in 1962, refers to abnormal blood recirculation through complete intracardiac or intra- and extracardiac communications, bypassing the capillary beds. This pathophysiological condition is most commonly associated with complex congenital heart defects, such as Ebstein's malformation, pulmonary atresia, Gerbode defect, and so on.
View Article and Find Full Text PDFAm J Med Genet A
December 2024
Division of Medical Genetics, Kanagawa Children's Medical Center, Yokohama, Japan.
Ann Pediatr Cardiol
November 2024
Department of CVTS, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India.
Association of Ebstein's anomaly of tricuspid valve (TV) with tetralogy of Fallot (TOF) is extremely rare. Their coexistence is unique as they modify each other's physiology. We report a case of a girl child having this rare combination along with Diamond-Blackfan syndrome awaiting a bone marrow transplant.
View Article and Find Full Text PDFAnn Pediatr Cardiol
November 2024
Department of Surgery, Pediatric and Congenital Heart Surgery Unit, National Cardiovascular Center Harapan Kita, West Jakarta, Indonesia.
Background: Surgical intervention is the definitive treatment for Ebstein anomaly, offering both biventricular and nonbiventricular repair options. The objective of this study is to identify a specific cutoff value for tricuspid septal leaflet displacement, which will be a crucial factor in determining the selection of a surgical approach with lower mortality risk in biventricular repair.
Methods And Results: This is a retrospective cohort study of consecutive patients with Ebstein anomaly undergoing surgical intervention at the National Cardiovascular Center Harapan Kita from January 2010 to December 2023.
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