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Methodist DeBakey Cardiovascular Journal 2019Ebstein's anomaly is a malformation of the tricuspid valve with myopathy of the right ventricle (RV) that presents with variable anatomic and pathophysiologic... (Review)
Review
Ebstein's anomaly is a malformation of the tricuspid valve with myopathy of the right ventricle (RV) that presents with variable anatomic and pathophysiologic characteristics, leading to equally variable clinical scenarios. Medical management and observation is often recommended for asymptomatic patients and may be successful for many years. Tricuspid valve repair is the goal of operative intervention; repair also typically includes RV plication, right atrial reduction, and atrial septal closure or subtotal closure. Postoperative functional assessments generally demonstrate an improvement or relative stability related to degree of RV enlargement, RV dysfunction, RV fractional area change, and tricuspid valve regurgitation.
Topics: Cardiac Surgical Procedures; Cardiovascular Agents; Ebstein Anomaly; Hemodynamics; Humans; Recovery of Function; Treatment Outcome; Tricuspid Valve; Tricuspid Valve Insufficiency; Ventricular Function, Right
PubMed: 31384377
DOI: 10.14797/mdcj-15-2-138 -
Pediatric Cardiology Oct 2022Ebstein's anomaly, first described in 1866 by Dr William Ebstein, accounts for 0.3-0.5% of congenital heart defects and represents 40% of congenital tricuspid valve... (Review)
Review
Ebstein's anomaly, first described in 1866 by Dr William Ebstein, accounts for 0.3-0.5% of congenital heart defects and represents 40% of congenital tricuspid valve abnormalities. Ebstein's anomaly affects the development of the tricuspid valve with widely varying morphology and, therefore, clinical presentation. Associated congenital cardiac lesions tend to be found more often in younger patients and may even be the reason for presentation. Presentation can vary from the most extreme form in fetal life, to asymptomatic diagnosis late in adult life. The most symptomatic patients need intensive care support in the neonatal period. This article summarizes and analyzes the literature on Ebstein's anomaly and provides a framework for the investigation, management, and follow-up of these patients, whether they present via fetal detection or late in adult life. For each age group, the clinical presentation, required diagnostic investigations, natural history, and management are described. The surgical options available for patients with Ebstein's anomaly are detailed and analyzed, starting from the initial mono-leaflet repairs to the most recent cone repair and its modifications. The review also assesses the effects of pregnancy on the Ebstein's circulation, and vice versa, the effects of Ebstein's on pregnancy outcomes. Finally, two attached appendices are provided for a structured echocardiogram protocol and key information useful for comprehensive Multi-Disciplinary Team discussion.
Topics: Adult; Ebstein Anomaly; Echocardiography; Fetus; Heart Defects, Congenital; Humans; Infant, Newborn; Patient Care; Tricuspid Valve
PubMed: 35460366
DOI: 10.1007/s00246-022-02908-x -
Cardiac Electrophysiology Clinics Jun 2017Ebstein 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... (Review)
Review
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. As the population ages, the need to address atrial fibrillation management and risk stratification for sudden cardiac death becomes ever more pertinent.
Topics: Atrial Fibrillation; Catheter Ablation; Death, Sudden, Cardiac; Ebstein Anomaly; Humans
PubMed: 28457239
DOI: 10.1016/j.ccep.2017.02.007 -
Journal of the American College of... Jun 2023
Topics: Humans; Ebstein Anomaly; Risk Assessment
PubMed: 37344045
DOI: 10.1016/j.jacc.2023.05.009 -
Mayo Clinic Proceedings Apr 2023
Topics: Humans; Ebstein Anomaly
PubMed: 37019511
DOI: 10.1016/j.mayocp.2023.02.014 -
The Canadian Journal of Cardiology Jul 2022Interventions in adults with congenital heart disease (ACHD) focus on surgical and percutaneous interventions in light of rapidly evolving ACHD clinical practice. To...
Interventions in adults with congenital heart disease (ACHD) focus on surgical and percutaneous interventions in light of rapidly evolving ACHD clinical practice. To bring rigour to our process and amplify the cumulative nature of evidence ACHD care we used the ADAPTE process; we systematically adjudicated, updated, and adapted existing guidelines by Canadian, American, and European cardiac societies from 2010 to 2020. We applied this to interventions related to right and left ventricular outflow obstruction, tetralogy of Fallot, coarctation, aortopathy associated with bicuspid aortic valve, atrioventricular canal defects, Ebstein anomaly, complete and congenitally corrected transposition, and patients with the Fontan operation. In addition to tables indexed to evidence, clinical flow diagrams are included for each lesion to facilitate a practical approach to clinical decision-making. Excluded are recommendations for pacemakers, defibrillators, and arrhythmia-directed interventions covered in separate designated documents. Similarly, where overlap occurs with other guidelines for valvular interventions, reference is made to parallel publications. There is a paucity of high-level quality of evidence in the form of randomized clinical trials to support guidelines in ACHD. We accounted for this in the wording of the strength of recommendations put forth by our national and international experts. As data grow on long-term follow-up, we expect that the evidence driving clinical practice will become increasingly granular. These recommendations are meant to be used to guide dialogue between clinicians, interventional cardiologists, surgeons, and patients making complex decisions relative to ACHD interventions.
Topics: Adult; Aortic Coarctation; Canada; Ebstein Anomaly; Fontan Procedure; Heart Defects, Congenital; Humans; United States
PubMed: 35460862
DOI: 10.1016/j.cjca.2022.03.021 -
Journal of Interventional Cardiac... Jun 2024Ebstein's anomaly of the tricuspid valve (EA) is an uncommon congenital cardiac malformation. It can present with atrioventricular tachycardia (AVRT), atrioventricular... (Review)
Review
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.
Topics: Ebstein Anomaly; Humans; Electrocardiography; Electrophysiologic Techniques, Cardiac; Catheter Ablation; Female; Male; Tachycardia, Atrioventricular Nodal Reentry
PubMed: 38289561
DOI: 10.1007/s10840-024-01744-8 -
Journal of Cardiothoracic and Vascular... Dec 2022
Topics: Humans; Ebstein Anomaly; Tricuspid Valve; Tricuspid Valve Insufficiency
PubMed: 36163155
DOI: 10.1053/j.jvca.2022.08.019 -
Pediatric Cardiology Jan 2023Ebstein anomaly is the most common form of tricuspid valve congenital anomalies. The tricuspid valve is abnormal with different degrees of displacement of the septal... (Review)
Review
Ebstein anomaly is the most common form of tricuspid valve congenital anomalies. The tricuspid valve is abnormal with different degrees of displacement of the septal leaflet and abnormal rotation of the valve towards the right ventricular outflow tract. In severe forms, it results in significant tricuspid regurgitation and requires surgical repair. There is an increased interest in understanding the anatomy of the tricuspid valve in this lesion as the surgical repair has evolved with the invention and wide adoption of the cone operation. Multimodality imaging plays an important role in diagnosis, follow-up, surgical planning and post-operative care. This review provides anatomical tips for the cardiac imagers caring for patients with Ebstein anomaly and will help provide image-based personalized medicine.
Topics: Humans; Ebstein Anomaly; Treatment Outcome; Tricuspid Valve; Tricuspid Valve Insufficiency; Cardiac Surgical Procedures
PubMed: 36151322
DOI: 10.1007/s00246-022-03011-x -
Seminars in Thoracic and Cardiovascular... 2017
Review
Topics: Cardiac Valve Annuloplasty; Cardiovascular Agents; Ebstein Anomaly; Heart Valve Prosthesis Implantation; Humans; Infant, Newborn; Oxygen Inhalation Therapy; Recovery of Function; Respiration, Artificial; Treatment Outcome; Tricuspid Valve
PubMed: 28958645
DOI: 10.1053/j.semtcvs.2017.09.006