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Advances in Experimental Medicine and... 2024Ebstein anomaly (EA) is a rare, congenital cardiac defect of the tricuspid valve with a birth prevalence between 0.5 and 1 in 20,000 [1]. It is characterized by... (Review)
Review
Ebstein anomaly (EA) is a rare, congenital cardiac defect of the tricuspid valve with a birth prevalence between 0.5 and 1 in 20,000 [1]. It is characterized by displacement of the tricuspid valve toward the apex of the right ventricle (RV) and "atrialization" of the RV (Fig. 57.1) [2]. EA accounts for about 0.5% of all congenital heart diseases (CHD) [2]. Depending on severity of the defect and due to heterogeneity of the disease, patient's presentation varies from severe heart failure symptoms and arrhythmia in neonatal life to asymptomatic adults.
Topics: Ebstein Anomaly; Humans; Tricuspid Valve; Genetic Predisposition to Disease; Mutation
PubMed: 38884759
DOI: 10.1007/978-3-031-44087-8_57 -
Journal of the American College of... Sep 2023There are marked variations in the incidence of sudden cardiac death (SCD) and in the substrates for ventricular arrhythmias (VAs) across the gamut of congenital heart... (Review)
Review
There are marked variations in the incidence of sudden cardiac death (SCD) and in the substrates for ventricular arrhythmias (VAs) across the gamut of congenital heart defects. In this 2-part review, patients with higher-risk forms of congenital heart disease (CHD) were conceptually categorized into those with discrete anatomic isthmuses for macro-reentrant ventricular tachycardia (VT) (Group A) and those with more diffuse or less well-defined substrates (Group B) that include patchy or extensive myocardial fibrosis. The latter category encompasses CHD lesions such as Ebstein anomaly, transposition of the great arteries with a systemic right ventricle (RV), and congenital aortic stenosis. For Group B patients, polymorphic VT and ventricular fibrillation account for a higher proportion of VA. The prognostic value of programmed ventricular stimulation is less well established, and catheter ablation plays a less prominent role. As cardiomyopathies evolve over time, pathophysiological mechanisms for VA among Groups A and B become increasingly blurred.
Topics: Humans; Adult; Transposition of Great Vessels; Heart Defects, Congenital; Arrhythmias, Cardiac; Ebstein Anomaly; Tachycardia, Ventricular
PubMed: 37673513
DOI: 10.1016/j.jacc.2023.06.036 -
The Thoracic and Cardiovascular Surgeon Dec 2017Surgical repair of the tricuspid valve is a milestone in the medical history of patients with Ebstein’s anomaly. The timely alleviation of the insufficiency has an... (Review)
Review
Surgical repair of the tricuspid valve is a milestone in the medical history of patients with Ebstein’s anomaly. The timely alleviation of the insufficiency has an important impact on the prognosis. In this review, we describe features of the disease relevant to surgical correction and the evolution of surgical techniques over six decades. We compare the results of different repair and replacement techniques. Additionally, we discuss concomitant antiarrhythmic surgery and bailout strategies for postoperative right ventricular failure. Finally, we review the surgical options in symptomatic neonates with Ebstein’s disease.
Topics: Cardiac Surgical Procedures; Ebstein Anomaly; Humans; Tricuspid Valve
PubMed: 28806824
DOI: 10.1055/s-0037-1604469 -
Current Cardiology Reports Oct 2020Ebstein's anomaly (EA) is a rare, but complex form of congenital heart disease consisting of a right ventricular myopathy and morphologic tricuspid valve disease leading... (Review)
Review
PURPOSE OF REVIEW
Ebstein's anomaly (EA) is a rare, but complex form of congenital heart disease consisting of a right ventricular myopathy and morphologic tricuspid valve disease leading to a high incidence of right ventricular dysfunction and arrhythmias. This review offers an updated overview of the current understanding and management of patients with EA with a focus on the adult population.
RECENT FINDINGS
Increased understanding of anatomic accessory atrioventricular pathways in EA has resulted in an improvement in ablation techniques and long-term freedom of atrial arrhythmia recurrence. Despite an improvement in understanding and recognition of EA, significant disease heterogeneity and complex treatment options continue to challenge providers, with the best outcomes achieved at expert congenital heart disease centers.
Topics: Adult; Arrhythmias, Cardiac; Ebstein Anomaly; Heart Defects, Congenital; Humans; Tricuspid Valve; Tricuspid Valve Insufficiency
PubMed: 33037480
DOI: 10.1007/s11886-020-01412-z -
The Journal of Maternal-fetal &... Sep 2022To present the crucial role of echocardiographic examination in perinatal care and analyze influence of prenatal treatment for neonatal outcome. Furthermore, the attempt...
OBJECTIVES
To present the crucial role of echocardiographic examination in perinatal care and analyze influence of prenatal treatment for neonatal outcome. Furthermore, the attempt to answer the question if there was any relationship between the occurrence of fetal Ebstein's anomaly and environmental risk factors in polish population.
METHODS
Forty-five prenatal diagnoses of Ebstein's anomaly were compiled over the 21-year period (1998‒2018) form our single unit. The analysis included the assessment of maternal parameters (age, past obstetric history, and place of residence) and fetal parameters (sex, gestational age, anatomy, the fetal cardiovascular condition assessed by the CVPS, associated extracardiac anomalies or malformations, prenatal treatment, delivery and follow-up).
RESULTS
The average age of gravida was 29.5 years (± 5.2 years) and gravidae <35 years of age accounted for 80% . There were 43 singleton pregnancies and 2 cases of multiple pregnancy. Ebstein's anomaly was mostly (averagely) diagnosed at 28th week of gestation. Forty-three fetuses had normal karyotypes and two had trisomy 21. Cardiomegaly was present in 91% (41) of fetuses. The average heart area to chest area ratio was 0.56 (± 0.12). In 21 cases, there was only fetal monitoring - echocardiographic examinations and postnatal mortality was 44.4%. In 5 cases, transplacental digoxin treatment was administered and mortality was: 40%. In another 5 cases, only steroid therapy was applied and postnatal mortality was 100%. Steroids and transplacental digoxin treatment were administered in 11 cases and mortality was 63.6%. In 3 last cases transplacental digoxin treatment, steroids and maternal hyperoxygenation therapy were given and mortality was 0%. Cesarean section rate was 49%. Moreover, due to Ebstein's anomaly regional peak of occurrence benzopyrene was deliberated as environmental risk factor.
CONCLUSIONS
Fetal Ebstein's anomaly occurred in our population in healthy young women, expecting their first child and malformation was not related to fetus gender, nor to maternal health condition. Our data can be a new signal for the development of novel treatment strategies in therapy in fetuses with Ebstein's anomaly.
Topics: Adult; Cesarean Section; Digoxin; Ebstein Anomaly; Female; Fetal Diseases; Humans; Infant, Newborn; Pregnancy; Retrospective Studies
PubMed: 32933366
DOI: 10.1080/14767058.2020.1818207 -
International Journal of Cardiology Apr 2018
Topics: Ebstein Anomaly; Humans
PubMed: 29402524
DOI: 10.1016/j.ijcard.2018.01.027 -
Advances in Experimental Medicine and... 2024Ebstein anomaly is a rare congenital heart defect, accounting for less than 1% of cardiac malformations and occurring in approximately 1 out of 210,000 live births. It... (Review)
Review
Ebstein anomaly is a rare congenital heart defect, accounting for less than 1% of cardiac malformations and occurring in approximately 1 out of 210,000 live births. It is characterized by an abnormality of the tricuspid valve, where the valve is positioned lower than normal in the right ventricle. Although primarily a tricuspid valve defect, the right ventricle itself is often structurally abnormal and weakened (myopathic).
Topics: Ebstein Anomaly; Humans; Tricuspid Valve; Heart Ventricles
PubMed: 38884758
DOI: 10.1007/978-3-031-44087-8_56 -
Echocardiography (Mount Kisco, N.Y.) Jan 2015Ebstein's anomaly is a complex congenital lesion which primarily involves the tricuspid valve. The tricuspid leaflets are tethered to varying degrees to the right... (Review)
Review
Ebstein's anomaly is a complex congenital lesion which primarily involves the tricuspid valve. The tricuspid leaflets are tethered to varying degrees to the right ventricular free wall and the ventricular septum often resulting in significant tricuspid regurgitation and a small functioning right ventricular chamber. Although the septal leaflet originates normally at the right atrioventricular junction, the proximal portion is often completely tethered to the ventricular septum resulting in a misconception and erroneous statements in many publications that its attachment is apically displaced. Although two-dimensional echocardiography represents the primary modality for the diagnosis of this anomaly, three-dimensional echocardiography provides incremental value in characterizing the extent and severity of tethering of individual tricuspid valve leaflets. This information is useful in surgical decision making whether to repair or replace the tricuspid valve.
Topics: Ebstein Anomaly; Echocardiography; Echocardiography, Three-Dimensional; Female; Follow-Up Studies; Heart Valve Prosthesis Implantation; Humans; Male; Postoperative Complications; Risk Assessment; Treatment Outcome; Tricuspid Valve
PubMed: 24888693
DOI: 10.1111/echo.12486 -
Circulation. Arrhythmia and... Jul 2022Optimal management of cardiac implantable electronic devices (CIEDs) in patients with Ebstein anomaly during tricuspid valve (TV) surgery is unknown. Thus, we aimed to...
BACKGROUND
Optimal management of cardiac implantable electronic devices (CIEDs) in patients with Ebstein anomaly during tricuspid valve (TV) surgery is unknown. Thus, we aimed to characterize CIED management/outcomes in patients with Ebstein anomaly undergoing TV surgery.
METHODS
Patients at the Mayo Clinic from 1987 to 2020 with Ebstein anomaly and CIED procedure were reviewed for procedural details, complications, echocardiogram, and lead parameters. Five-year cumulative incidence of CIED complications were estimated using the Kaplan-Meier method.
RESULTS
Ninety-three patients were included; 51 were female, and mean age was 40.7±17.5 years. A new CIED was implanted in 45 patients at the time of TV surgery with the majority receiving an epicardial (n=37) CIED. Among 34 patients who had preexisting CIED (11 epicardial, 23 transvenous) at time of TV surgery, 20 had a transvenous right ventricular lead managed by externalizing the lead to the TV (n=15) or extracting the transvenous lead with epicardial lead implantation (n=5). Fourteen patients underwent CIED implantation (4 epicardial, 10 transvenous) without concurrent surgery. Placement of lead across the TV was avoided in 85% of patients. The 5-year cumulative incidence of CIED complications was 24% with no significant difference between epicardial and transvenous CIEDs (26% versus 23%, =0.96). Performance of lead parameters was similar in epicardial and transvenous leads during median (interquartile range) follow-up of 44.5 (61.1) months.
CONCLUSIONS
In patients with Ebstein anomaly undergoing TV surgery, the use of epicardial leads and externalization of transvenous leads to the TV can avoid lead placement across the valve leaflets. Lead performance and CIED complications was similar between epicardial and transvenous CIEDs.
Topics: Adult; Defibrillators, Implantable; Ebstein Anomaly; Electronics; Female; Heart; Humans; Male; Middle Aged; Pacemaker, Artificial; Retrospective Studies; Treatment Outcome; Young Adult
PubMed: 35763435
DOI: 10.1161/CIRCEP.121.010744 -
Journal of Pediatric Nursing 2015Ebstein anomaly is a rare congenital heart defect. Many nurses have probably never encountered this anomaly, with very few able to accurately depict the pathological... (Review)
Review
Ebstein anomaly is a rare congenital heart defect. Many nurses have probably never encountered this anomaly, with very few able to accurately depict the pathological anatomy of the condition. As technology further develops, providers are better equipped to recognize and manage Ebstein anomaly. There are important considerations for nurses when caring for an individual with Ebstein anomaly. The aim of this article is to give an overview of the condition exploring the pathophysiology, how patients typically present, and how to effectively care for a patient with Ebstein anomaly regarding medical and surgical courses of treatment. It is important for nurses to have a resource to reference on Ebstein anomaly, and the majority of current literature is solely based for medical providers. Furthermore, Ebstein patients may be seen on a variety of units in the hospital beyond cardiology (i.e., pregnant patient with a diagnosis of Ebstein anomaly).
Topics: Chronic Disease; Clinical Competence; Ebstein Anomaly; Female; Heart Defects, Congenital; Humans; Infant, Newborn; Long-Term Care; Male; Monitoring, Physiologic; Nurse's Role; Pediatric Nursing; Risk Assessment
PubMed: 26395651
DOI: 10.1016/j.pedn.2015.08.003