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The Annals of Thoracic Surgery May 2020
Topics: Ebstein Anomaly; Humans
PubMed: 31982442
DOI: 10.1016/j.athoracsur.2019.12.011 -
Journal of the American College of... Jun 2023Low birth prevalence and referral bias constitute significant obstacles to elucidating the natural history of Ebstein anomaly (EA).
BACKGROUND
Low birth prevalence and referral bias constitute significant obstacles to elucidating the natural history of Ebstein anomaly (EA).
OBJECTIVES
An extensive 2-country register-based collaboration was performed to investigate the mortality in patients with EA.
METHODS
Patients born from 1970 to 2017 and diagnosed with EA were identified in Danish and Swedish nationwide medical registries. Each patient was matched by birth year and sex with 10 control subjects from the general population. Cumulative mortality and HR of mortality were computed using Kaplan-Meier failure function and Cox proportional regression model.
RESULTS
The study included 530 patients with EA and 5,300 matched control subjects with a median follow-up of 11 years. In the EA cohort, 43% (228) underwent cardiac surgery. Cumulative mortality was lower for patients diagnosed in the modern era (the year 2000 and later) than for those diagnosed in the prior era (P < 0.001). Patients with isolated lesion displayed lower cumulative mortality than patients with complex lesions did (P < 0.001). Patients with a presumed mild EA anatomy displayed a 35-year cumulative mortality of 11% (vs 4% for the matched control subjects; P < 0.001), yielding an HR for mortality of 6.0 (95% CI: 2.7-13.6), whereas patients with presumed severe EA demonstrated an HR of 36.2 (95% CI: 15.5-84.4) compared with control subjects and a cumulative mortality of 18% 35 years following diagnosis.
CONCLUSIONS
Mortality in patients with EA is high irrespective of presence of concomitant congenital cardiac malformations and time of diagnosis compared with the general population, but overall mortality has improved in the contemporary era.
Topics: Humans; Ebstein Anomaly; Retrospective Studies; Cardiac Surgical Procedures; Proportional Hazards Models; Hospital Mortality
PubMed: 37344044
DOI: 10.1016/j.jacc.2023.04.037 -
Pediatrics and Neonatology Jun 2017Ebstein's anomaly is uncommon. Genetic bases of this congenital heart defect may be related to the mutations in myosin heavy chain 7 and NKX2.5, among others.... (Review)
Review
Ebstein's anomaly is uncommon. Genetic bases of this congenital heart defect may be related to the mutations in myosin heavy chain 7 and NKX2.5, among others. Asymptomatic patients with Ebstein's anomaly can be conservatively treated and kept under close follow-up, whereas surgical operation is indicated for those patients with evidence of right heart dilation and progressively impaired ventricular systolic function. A biventricular repair consisting of the reconstruction of a competent monocuspid tricuspid valve, right ventriculorrhaphy, subtotal atrial septal defect closure, and aggressive reduction atrioplasty is suitable for most patients, and 1.5-ventricular repair (bidirectional Glenn shunt) is indicated for patients with poor right ventricular function; by contrast, heart transplantation is used in patients with severe left ventricular dysfunction.
Topics: Cardiac Surgical Procedures; Combined Modality Therapy; Ebstein Anomaly; Humans; Mutation
PubMed: 28017577
DOI: 10.1016/j.pedneo.2016.08.004 -
Cardiology Clinics Aug 2020Ebstein anomaly is a congenital malformation involving primarily the tricuspid valve, with failure of delamination from the underlying myocardium and right ventricular... (Review)
Review
Ebstein anomaly is a congenital malformation involving primarily the tricuspid valve, with failure of delamination from the underlying myocardium and right ventricular myopathy. Echocardiography is diagnostic in most patients and demonstrates apical displacement of the septal leaflet and variable tethering of leaflet tissue to the right ventricular myocardium. Operative intervention is considered for exertional symptoms, progressive right ventricular enlargement, or right ventricular dysfunction. Tricuspid valve cone repair is the preferred surgical approach. Tricuspid valve replacement and bidirectional cavopulmonary shunt also are considered in patients with advanced disease. Pregnancy generally is well tolerated. Patients with Ebstein anomaly require lifelong follow-up.
Topics: Adult; Cardiac Surgical Procedures; Ebstein Anomaly; Humans; Prognosis; Tricuspid Valve; Ventricular Dysfunction, Right
PubMed: 32622490
DOI: 10.1016/j.ccl.2020.04.004 -
Trends in Cardiovascular Medicine Aug 2018Ebstein anomaly is a congenital disorder of right ventricular myocardial development, which affects the tricuspid valve in addition to the right ventricular myocardium.... (Review)
Review
Ebstein anomaly is a congenital disorder of right ventricular myocardial development, which affects the tricuspid valve in addition to the right ventricular myocardium. Cardiac imaging by transthoracic echocardiography and cardiac magnetic resonance imaging are the key modalities used to assess timing and type of surgery. In this article, we review the current standards of echocardiographic and magnetic resonance imaging in Ebstein anomaly.
Topics: Ebstein Anomaly; Echocardiography, Doppler, Color; Echocardiography, Transesophageal; Heart Ventricles; Humans; Magnetic Resonance Imaging; Predictive Value of Tests; Prognosis; Tomography, X-Ray Computed; Tricuspid Valve; Ventricular Function, Right
PubMed: 29409687
DOI: 10.1016/j.tcm.2018.01.002 -
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 -
Heart (British Cardiac Society) Jan 2024Although survival has significantly improved in the last four decades, the diagnosis of Ebstein's anomaly is still associated with a 20-fold increased risk of mortality,... (Review)
Review
Although survival has significantly improved in the last four decades, the diagnosis of Ebstein's anomaly is still associated with a 20-fold increased risk of mortality, which generally drops after neonatal period and increases subtly thereafter. With increasing age of presentation, appropriate timing of intervention is challenged by a wide spectrum of disease and paucity of data on patient-tailored interventional strategies. The present review sought to shed light on the wide grey zone of post-neonatal Ebstein's manifestations, highlighting current gaps and achievements in knowledge for adequate risk assessment and appropriate therapeutic strategy.A 'wait-and-see' approach has been adopted in many circumstances, though its efficacy is now questioned by the awareness that Ebstein's anomaly is not a benign disease, even when asymptomatic. Moreover, older age at intervention showed a negative impact on post-surgical outcome.In order to tackle the extreme heterogeneity of Ebstein's anomaly, this review displays the multimodality imaging assessment necessary for a proper anatomical classification and the multidisciplinary approach needed for a comprehensive risk stratification and monitoring strategy. Currently available predictors of clinical outcome are summarised for both operated and unoperated patients, with the aim of supporting the decisional process on the choice of appropriate therapy and optimal timing for intervention.
Topics: Infant, Newborn; Child; Adult; Humans; Ebstein Anomaly; Risk Assessment; Multimodal Imaging
PubMed: 37487694
DOI: 10.1136/heartjnl-2023-322420 -
Neonatal Network : NN 2014Cardiac congenital abnormalities are a leading cause in neonatal mortality occurring in up to 1 in 200 of live births. Ebstein anomaly, also known as Kassamali anomaly,... (Review)
Review
Cardiac congenital abnormalities are a leading cause in neonatal mortality occurring in up to 1 in 200 of live births. Ebstein anomaly, also known as Kassamali anomaly, accounts for 1 percent of all congenital cardiac anomalies. This congenital abnormality involves malformation of the tricuspid valve and of the right ventricle. In this review, the causes of the anomaly are outlined and the pathophysiology is discussed, with a focus on the symptoms, management, and treatments available to date.
Topics: Ebstein Anomaly; Education, Nursing, Continuing; Humans; Infant, Newborn; Prognosis
PubMed: 25161135
DOI: 10.1891/0730-0832.33.5.268 -
Annals of Noninvasive Electrocardiology... May 2019Ebstein's anomaly is a congenital heart disease where the most important anatomic feature is the inferior displacement of the tricuspid valve leaflets.... (Review)
Review
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.
Topics: Cardiac Electrophysiology; Ebstein Anomaly; Echocardiography; Electrocardiography; Female; Heart Defects, Congenital; Humans; Infant, Newborn; Male; Multimodal Imaging; Sensitivity and Specificity; Tricuspid Valve; Vectorcardiography
PubMed: 30106198
DOI: 10.1111/anec.12590 -
Seminars in Cardiothoracic and Vascular... Mar 2016Ebstein's anomaly is a complex and heterogeneous form of congenital heart disease characterized by malformation and apical displacement of the tricuspid valve leaflets.... (Review)
Review
Ebstein's anomaly is a complex and heterogeneous form of congenital heart disease characterized by malformation and apical displacement of the tricuspid valve leaflets. Patients may present at any time from the neonatal period to adulthood with symptoms ranging from cardiac failure and cyanosis to paroxysmal arrhythmias. Depending on the timing of presentation, various surgical options are available for the management of symptomatic patients. This review will discuss the perioperative and anesthetic management of patients with Ebstein's anomaly with reference to the more common surgical approaches.
Topics: Anesthesia; Cardiac Surgical Procedures; Child; Child, Preschool; Ebstein Anomaly; Humans; Infant; Infant, Newborn; Perioperative Care
PubMed: 26472205
DOI: 10.1177/1089253215605390