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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 -
Indian Journal of Thoracic and... Jan 2021Ebstein's anomaly is a rare congenital heart defect characterized by apical displacement of the tricuspid valve and reduced volume of the true functional right... (Review)
Review
Ebstein's anomaly is a rare congenital heart defect characterized by apical displacement of the tricuspid valve and reduced volume of the true functional right ventricle. In its most severe form, the disease presents as right heart failure and cyanosis in the neonate. Although many of these neonates can be managed conservatively, surgical intervention may become necessary in the sickest of them. Surgical intervention in this subgroup can be challenging and requires meticulous appreciation of the anatomy and physiology on a case to case basis. Multicenter studies are required to define a uniform algorithm and approach towards these patients.
PubMed: 33603283
DOI: 10.1007/s12055-020-00942-z -
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 -
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 -
JAMA Jun 2022
Topics: Ebstein Anomaly; Humans
PubMed: 35616918
DOI: 10.1001/jama.2022.7679 -
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 -
Journal of Thoracic Disease Mar 2020Ebstein's anomaly (EA) is a rare congenital cardiac anomaly. It is a disease at a minimum of the tricuspid valve (TV) and the right ventricular myocardium. Presentation... (Review)
Review
Ebstein's anomaly (EA) is a rare congenital cardiac anomaly. It is a disease at a minimum of the tricuspid valve (TV) and the right ventricular myocardium. Presentation varies from a severe symptomatic form during the neonatal period to an incidental detection later in life due to the wide morphological variation of the condition. The neonatal presentation can be severe and every attempt should be made at medical management ideally into infancy and early childhood. Neonates not eligible or failing medical management should be surgically managed either with a single ventricle palliative approach or a more desirable biventricular repair with a neonatal TV valvuloplasty. Some neonates initially committed to a single ventricle pathway may be converted to a biventricular repair by a delayed TV valvuloplasty. The da Silva Cone repair has become the valvuloplasty of choice especially beyond the neonatal period and can be applied to a wide morphological variation of the condition with good long-term durability. If the chance of a successful TV valvuloplasty is high, it should be offered early in childhood to prevent further cardiac dilation. Adding a Bidirectional Glenn to a valvuloplasty may help salvage marginal risk patients or marginally repairable valves. If valve replacement is the only option, a bioprosthetic valve should be used as it is less thrombogenic especially with marginal right ventricular function. Heart transplantation should be considered in patients with associated left ventricular dysfunction.
PubMed: 32274197
DOI: 10.21037/jtd.2020.01.18 -
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