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Journal of the American College of... Aug 2023
Topics: Humans; Ebstein Anomaly; Tricuspid Valve; Cardiac Surgical Procedures; Tricuspid Valve Insufficiency
PubMed: 37532421
DOI: 10.1016/j.jacc.2023.05.041 -
Giornale Italiano Di Cardiologia (2006) Mar 2015Ebstein's anomaly is a rare congenital heart disorder secondary to a malformation of the tricuspid valve and right ventricle. The heterogeneous spectrum of presentation... (Review)
Review
Ebstein's anomaly is a rare congenital heart disorder secondary to a malformation of the tricuspid valve and right ventricle. The heterogeneous spectrum of presentation of its structural anomalies and associated cardiomyopathy accounts for a widely varied clinical and hemodynamic manifestation of the pathology and its impact on timing of diagnosis and prognosis. Neonatal Ebstein's anomaly is characterized by reduced survival, while an average risk of mortality per year of 1% to 1.4% has been reported in infancy and adulthood. Medical management and a careful clinical and echocardiographic follow-up are advisable for all asymptomatic patients and those with minimal abnormalities. Surgical correction is recommended in presence of progressive dilation of the right atrium and right ventricle, development of right ventricular dysfunction, occurrence of supraventricular or ventricular arrhythmias or episodes of paradoxical embolization, reduced exercise capacity, or significant desaturation. Prosthetic valve replacement or repair of the tricuspid valve are both common strategies in the correction of tricuspid regurgitation. During the last three decades, important contributions in the development of repair techniques were driven from the experience of Danielson, Carpentier and da Silva, with satisfactory results in terms of safety and durability at long-term follow-up.
Topics: Adult; Ebstein Anomaly; Echocardiography; Heart Ventricles; Humans; Infant; Infant, Newborn; Prognosis; Survival; Tricuspid Valve
PubMed: 25837461
DOI: 10.1714/1820.19828 -
Heart Failure Reviews Jul 2020Ebstein anomaly comprises approximately 1% of all congenital heart diseases. It occurs when the tricuspid valve fails to properly delaminate from the right ventricle,... (Review)
Review
Ebstein anomaly comprises approximately 1% of all congenital heart diseases. It occurs when the tricuspid valve fails to properly delaminate from the right ventricle, resulting in a clinical spectrum of abnormal tricuspid valve morphology and right ventricular dysfunction. Due to the anatomy of the tricuspid valve and right ventricle, as well as associated right- and left-sided pathology, patients are at risk for both right and left ventricular failure and the associated symptoms of each. Ebstein patients are also at risk for atrial arrhythmias, due to the atrial enlargement intrinsic to the anatomy, as well as the presence of potential accessory pathways. Arrhythmias are generally poorly tolerated, particularly in the setting of ventricular dysfunction. Cyanosis may also be present in Ebstein patients, due to the common occurrence of atrial communications, which can exacerbate other symptoms of heart failure. Treatment of heart failure can be through pharmacologic and procedural interventions, depending on the underlying cause of heart failure. While early heart failure symptoms may be treated with medical management, most Ebstein patients will require surgery. Various surgical and catheter-based interventions targeting the tricuspid valve and the atrialized right ventricular tissue have been developed to help treat the underlying cause of the heart failure. The optimal timing of transcatheter and surgical intervention in the Ebstein patient to prevent or treat heart failure needs further study.
Topics: Adult; Cardiac Surgical Procedures; Disease Management; Ebstein Anomaly; Heart Failure; Humans
PubMed: 32472521
DOI: 10.1007/s10741-020-09930-2 -
Advances in Experimental Medicine and... 2024Ebstein's anomaly is a congenital malformation of the tricuspid valve characterized by abnormal attachment of the valve leaflets, resulting in varying degrees of valve... (Review)
Review
Ebstein's anomaly is a congenital malformation of the tricuspid valve characterized by abnormal attachment of the valve leaflets, resulting in varying degrees of valve dysfunction. The anatomic hallmarks of this entity are the downward displacement of the attachment of the septal and posterior leaflets of the tricuspid valve. Additional intracardiac malformations are common. From an embryological point of view, the cavity of the future right atrium does not have a direct orifice connected to the developing right ventricle. This chapter provides an overview of current insight into how this connection is formed and how malformations of the tricuspid valve arise from dysregulation of molecular and morphological events involved in this process. Furthermore, mouse models that show features of Ebstein's anomaly and the naturally occurring model of canine tricuspid valve malformation are described and compared to the human model. Although Ebstein's anomaly remains one of the least understood cardiac malformations to date, the studies summarized here provide, in aggregate, evidence for monogenic and oligogenic factors driving pathogenesis.
Topics: Ebstein Anomaly; Animals; Disease Models, Animal; Humans; Dogs; Mice; Tricuspid Valve
PubMed: 38884760
DOI: 10.1007/978-3-031-44087-8_58 -
Heart (British Cardiac Society) Feb 2023
Topics: Humans; Ebstein Anomaly; Atrial Fibrillation; Heart Septal Defects, Atrial
PubMed: 36379695
DOI: 10.1136/heartjnl-2022-321876 -
Seminars in Thoracic and Cardiovascular... 2022
Topics: Ebstein Anomaly; Humans; Treatment Outcome
PubMed: 34000429
DOI: 10.1053/j.semtcvs.2021.04.028 -
Journal of Obstetrics and Gynaecology :... May 2022Pregnancy complicated with uncorrected Ebstein's anomaly is uncommon and may pose a serious threat to maternal and foetal life in the clinical setting of altered... (Review)
Review
Pregnancy complicated with uncorrected Ebstein's anomaly is uncommon and may pose a serious threat to maternal and foetal life in the clinical setting of altered hemodynamics of pregnancy. Data of eight pregnancies in four women with Ebstein's anomaly who delivered in a tertiary care institute was analysed. Among the four women, one had associated atrial septal defect, one had pulmonary hypertension and three had right bundle branch block. There were two miscarriages and six successful pregnancies resulting in live births. Three of the pregnancies were delivered by caesarean section. There was one pregnancy complicated by severe preeclampsia, no preterm births or maternal cardiac complications. There was one neonate with congenital ostium secundum atrial septal defect. All patients were managed by a multidisciplinary team involving Obstetrician, Cardiologist, Anaesthesiologist and Neonatologist.IMPACT STATEMENT Ebstein's anomaly is a rare congenital anomaly with apical displacement of the septal tricuspid leaflet in association with leaflet dysplasia. It may cause varied presentation in pregnancy depending on the severity of the lesion. Ebstein's anomaly may become symptomatic for the first-time during pregnancy. Patients with NYHA class II symptoms and no cyanosis generally tolerate pregnancy well. Miscarriages and intrauterine growth restriction may occur in the presence of this condition. Vaginal delivery is advised and caesarean is done only for obstetric indications. The management of pregnancy with uncorrected Ebstein's anomaly is highly challenging especially in a low resource setting and requires tertiary centre care. Multidisciplinary team involvement can help to improve the outcomes in such pregnancies.
Topics: Abortion, Spontaneous; Cesarean Section; Cyanosis; Ebstein Anomaly; Female; Humans; Infant, Newborn; Pregnancy; Tertiary Care Centers
PubMed: 34404328
DOI: 10.1080/01443615.2021.1932777 -
The Journal of Thoracic and... Mar 2021To evaluate late-term tricuspid valve competence and biventricular function following cone reconstruction for Ebstein anomaly, and to explore biventricular remodeling.
OBJECTIVES
To evaluate late-term tricuspid valve competence and biventricular function following cone reconstruction for Ebstein anomaly, and to explore biventricular remodeling.
METHODS
Consecutive adult and pediatric patients who underwent cone reconstruction from 2009 to 2019 were reviewed for inclusion in this retrospective cardiac magnetic resonance imaging study. Tricuspid valve competence was assessed with tricuspid regurgitation fraction. Biventricular systolic function was assessed by ejection fraction, cardiac index, indexed stroke volume, and indexed aortic and pulmonary artery beat volume. Biventricular remodeling was assessed by planimetered areas (right atrium, functional right ventricle, left heart), and indexed end-diastolic and end-systolic ventricular volumes. Paired t tests or Wilcoxon signed-rank tests were used for analyses.
RESULTS
Of 58 included patients, 50 underwent cardiac magnetic resonance imaging. Twelve patients had both preoperative and late postoperative cardiac magnetic resonance imaging with a median follow-up of 5.11 years (interquartile range, 3.12-6.07 years). Focusing on these, tricuspid regurgitation fraction decreased (from 69% to 10%; P = .014), right ventricle ejection fraction remained stable, and antegrade pulmonary artery beat volume increased (from 26.7 to 41.6 mL/beat/m; P = .037). The left ventricle stroke volume (from 30.4 to 44.1 mL/m; P = .015) and antegrade aortic beat volume (from 28.5 to 41.1 mL/beat/m; P = .014) also increased, and the left ventricle stroke volume improved progressively with time since surgery (P = .048). Whereas the right atrium area decreased (P = .004), the functional right ventricle and left heart area increased (cm, P = .021 and P = .004). Right ventricle volumes showed a tendency to normalize and left ventricle indexed end-diastolic volume increased (from 50 to 69 mL/m; P = .03) over time.
CONCLUSIONS
Cone valve integrity was sustained. Biventricular function improved progressively during follow-up, and there are positive signs of biventricular remodeling late after cone reconstruction.
Topics: Adolescent; Cardiac Surgical Procedures; Child; Child, Preschool; Databases, Factual; Ebstein Anomaly; Female; Humans; London; Magnetic Resonance Imaging; Male; Recovery of Function; Retrospective Studies; Time Factors; Treatment Outcome; Tricuspid Valve; Ventricular Function, Left; Ventricular Function, Right; Ventricular Remodeling; Young Adult
PubMed: 33293067
DOI: 10.1016/j.jtcvs.2020.10.124 -
JACC. Clinical Electrophysiology Sep 2021
Review
Topics: Arrhythmias, Cardiac; Ebstein Anomaly; Humans; Morphogenesis; Tricuspid Valve
PubMed: 34454887
DOI: 10.1016/j.jacep.2021.05.008 -
Internal Medicine (Tokyo, Japan) Dec 2022Ebstein's anomaly is an uncommon congenital disorder affecting the tricuspid valve. We herein report a 38-year-old woman who experienced consciousness and sensory...
Ebstein's anomaly is an uncommon congenital disorder affecting the tricuspid valve. We herein report a 38-year-old woman who experienced consciousness and sensory disturbance during treatment for heart failure caused by Ebstein's anomaly. Urgent magnetic resonance imaging and cerebral angiography demonstrated acute cerebral infarction and internal carotid artery obstruction with the development of collateral arteries. We diagnosed her with multiple cerebral infarctions due to moyamoya disease. Ebstein's anomaly concomitant with moyamoya disease is extremely rare. However, we should consider the possibility of this rare but important concurrence when treating patients with heart failure due to Ebstein's anomaly to avoid excessive diuresis and vasodilation and irreversible brain injury.
Topics: Female; Humans; Adult; Ebstein Anomaly; Moyamoya Disease; Tricuspid Valve; Heart Failure
PubMed: 35569986
DOI: 10.2169/internalmedicine.9516-22