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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 -
Arquivos Brasileiros de Cardiologia Nov 2011
Topics: Cardiac Surgical Procedures; Ebstein Anomaly; Heart Ventricles; Humans; Infant, Newborn; Tricuspid Valve
PubMed: 22189609
DOI: 10.1590/s0066-782x2011001400001 -
Cardiovascular Diagnosis and Therapy Dec 2021Ebstein's anomaly is a rare congenital heart disease with malformation of the tricuspid valve and myopathy of the right ventricle. The septal and inferior leaflets... (Review)
Review
Ebstein's anomaly is a rare congenital heart disease with malformation of the tricuspid valve and myopathy of the right ventricle. The septal and inferior leaflets adhere to the endocardium due to failure of delamination. This leads to apical displacement of their hinge points with a shift of the functional tricuspid valve annulus towards the right ventricular outflow tract with a possibly restrictive orifice. Frequently, a coaptation gap yields tricuspid valve regurgitation and over time the "atrialized" portion of the right ventricle may dilate. The highly variable anatomy determines the clinical presentation ranging from asymptomatic to very severe with need for early operation. Echocardiography and magnetic resonance imaging are the most important diagnostic modalities to assess the tricuspid valve as well as ventricular morphology and function. While medical management of asymptomatic patients can be effective for many years, surgical intervention is indicated before development of significant right ventricular dilatation or dysfunction. Onset of symptoms and arrhythmias are further indications for surgery. Modified cone reconstruction of the tricuspid valve is the state-of-the-art approach yielding the best results for most patients. Alternative procedures for select cases include tricuspid valve replacement and bidirectional cavopulmonary shunt depending on patient age and other individual characteristics. Long-term survival after surgery is favorable but rehospitalization and reoperation remain significant issues. Further studies are warranted to identify the optimal surgical strategy and timing before adverse right ventricular remodeling occurs. It is this article's objective to provide a comprehensive review of current literature and an overview on the management of Ebstein's Anomaly. It focuses on imaging, cardiac surgery, and outcome. Additionally, a brief insight into arrhythmias and their management is given. The "future perspectives" summarize open questions and fields of future research.
PubMed: 35070800
DOI: 10.21037/cdt-20-771 -
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 -
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 -
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 -
British Medical Journal May 1978
Topics: Ebstein Anomaly; Humans
PubMed: 647249
DOI: No ID Found