ebsteins anomaly

BACKGROUND: Ebstein anomaly is a rare congenital anomaly of the tricuspid valve which presents challenges to cardiac surgeons due to the spectrum of the disease and the technical difficulty of valve repair. The natural history of the anomaly differs between patients presenting in the neonatal period to those presenting in adulthood. METHODS: A retrospective review of all patients >15 years of age with Ebstein anomaly, undergoing surgery on the tricuspid valve at 6 centers across Australia and New Zealand was performed...

Primary liver tumours in neonates with single-ventricle palliation are exceedingly rare. We present the first reported case of neonatal hepatoblastoma with severe Ebstein's anomaly following Starnes procedure. The patient's postoperative course highlights the challenges and complications in simultaneous management of these diagnoses. Transition from shunted single-ventricle physiology to bidirectional cavopulmonary connection improved end-organ function, permitting more aggressive hepatic malignancy treatment...

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A circular shunt is a poor prognostic factor associated with Ebstein's anomaly. Targeting the constriction of the ductus arteriosus (DA) in order to limit or resolve the circular shunt, has been shown to improve fetal outcomes. Prenatal non-steroidal anti-inflammatory drugs (NSAIDs) have been known to constrict the DA. Recently, prenatal NSAIDs have been used for that purpose in the treatment of circular shunt. Limited research shows that it may be an effective treatment leading to improved fetal outcomes. In this article, we did an extensive review of literature to describe this therapy's effectiveness and outcomes...

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OBJECTIVE: In this study, we investigated the clinical characteristics of patients with Ebstein anomaly and left ventricular outflow tract (LVOT) obstruction as well as possible mechanisms of obstruction as assessed by 2-dimensional echocardiography. METHODS: We queried our institutional echocardiography and surgical databases for patients with the diagnosis of Ebstein anomaly seen from 1985 through 2022. Fourteen patients had the additional diagnosis of LVOT obstruction identified from transthoracic echocardiography...

OBJECTIVE: To describe the surgical outcomes in neonates and infants who had surgery for Ebstein anomaly (EA) and tricuspid valve dysplasia (TVD). METHODS: Retrospective chart review for all patients who underwent surgery for EA or TVD during the index hospitalization after birth at our institution from January 2005 to February 2023. RESULTS: Fifteen symptomatic neonates and infants who had surgery for EA or TVD were included, 8 with EA and 7 with TVD...

BACKGROUND AND OBJECTIVE: We aimed to investigate long-term clinical and echocardiographic outcomes, including tricuspid valve durability, annular growth, and left ventricular reverse remodeling, after modified cone reconstruction in patients with Ebstein's anomaly. METHODS: This was a retrospective analysis of all pediatric patients who underwent modified cone reconstruction for Ebstein's anomaly at a single tertiary center between January 2005 and June 2021. RESULTS: A total of 14 pediatric patients underwent modified cone reconstruction for Ebstein's anomaly; the median age was 5...

The increasing prevalence of atrial fibrillation (AF) in adults with congenital heart disease raises significant questions regarding its management. The unique underlying anatomic and physiological background further adds to the difficulty in eliminating the AF burden in these patients. Herein, we provide an overview of the current knowledge on the pathophysiology and risk factors for AF in adult congenital heart disease, with a special focus on the existing challenges in AF ablation. Emerging imaging modalities and ablation techniques might have a role to play...

Accessory pathway ablation in Ebstein anomaly can be significantly more challenging than in structurally normal hearts. An alternative to the conventional approach to mapping APs is to detect points with a high-density mapping catheter based on an automated detection algorithm using open window mapping. It detects the sharpest signal at each point with high-density mapping rather than relying on the origin of the local electrogram to localize the pathway and determine a site for successful ablation. We herein report the first case in literature of a redo-accessory pathway ablation in Ebstein anomaly using this technique...

Complete trisomy 5 is a rare and lethal abnormality. Mosaic trisomy 5 presents in various phenotypes, ranging from a clinically normal fetus to fetuses presenting uterine growth restriction, congenital heart anomalies, multiple dysmorphic features and psychomotor development abnormalities. Although rare, there are cases of a normal psychomotor development regardless of the associated low fetal growth frequently associated with mosaic trisomy 5. This is the first case report to date of a live fetus with complete trisomy 5 reported in chorionic villus sampling and mosaic trisomy 5 in amniotic fluid with a concomitant Ebstein anomaly...

UNLABELLED: In this case report, we describe a 23-year-old male with Ebstein's anomaly who experienced out-of-hospital cardiac arrest due to commotio cordis following cliff diving. The patient previously underwent a Cone procedure and re-do reduction tricuspid valvuloplasty. Comprehensive investigations revealed no new ischemic events or structural abnormalities. He received an implantable cardioverter-defibrillator during an uneventful outpatient visit. This is the first reported case of commotio cordis in a patient with Ebstein's anomaly, suggesting a potential increased risk in individuals with congenital heart diseases...

OBJECTIVES: UK single ventricle (SV) palliation outcomes after first postnatal procedure (FPP) are well documented. However, survival determinants from fetal diagnosis to FPP are lacking. To better inform parental-fetal counselling, we examined factors favouring survival at two large UK centres. DESIGN: Retrospective multicentre cohort study. SETTING: Two UK congenital cardiac centres: Leeds and Birmingham. PATIENTS: SV fetal diagnoses from 2015 to 2021...

The tricuspid valve positioned between the right atrium and right ventricle is composed of 3 leaflets (anterior, posterior, and septal) anchored by a collagenous fibrous annulus, a saddle-shaped, oval structure, providing a firm yet dynamic structural support for the tricuspid valve. The annulus is considered to separate between the right atrium and right ventricle. Structural anomalies of the fetal tricuspid valve are rare and include Ebstein's anomaly, tricuspid atresia, partial absence, unguarded tricuspid orifice (absent leaflets) cleft, double orifice, bicuspid valve and Uhl anomaly (absence of the right ventricular myocardium with an apposing endocardium and epicardium)...

BACKGROUND: The purpose of this study was to evaluate the timing of the first cardiac surgery, the number of cardiac surgeries performed, and 30-day postoperative mortality rate for children with severe congenital heart defects (sCHDs) in their first 5 years of life. METHODS AND RESULTS: This was a population-based data linkage cohort study linking information from 9 European congenital anomaly registries to vital statistics and hospital databases. Data were extracted for 5693 children with sCHDs born from 1995 to 2004...

BACKGROUND: Due to the heterogeneity of anatomic anomalies in Ebstein's anomaly (EA), particularly in the subset of patients with atrial septal defect (ASD), hemodynamic changes, which ultimately cause left ventricular (LV) deterioration remain unclear. The current study aimed to investigate the effect of concomitant ASD on LV function using cardiovascular magnetic resonance (CMR) imaging in patients with EA. METHODS: This study included 31 EA patients with ASD, 76 EA patients without ASD, 35 patients with simple ASD and 40 healthy controls...

BACKGROUND: Ebstein's anomaly occurs when there is an apical displacement of the tricuspid valve with septal and posterior valve leaflets tethering. This condition often occurs in association with other congenital, structural, or conduction system diseases, including intracardiac shunts, valvular lesions, arrhythmias, accessory conduction pathways, and first-degree atrioventricular (AV) block. We present for the first time a case of a patient with Ebstein's anomaly who presented with second-degree Mobitz II AV block and was successfully treated with conduction system pacing (CSP) due to her young age and the likelihood of a long-term high percentage of pacing...

Ebstein anomaly (EA) is a congenital dysplasia of the tricuspid valve resulting in reduced right ventricular (RV) volume and tricuspid regurgitation. Severe EA in the neonatal period is associated with high mortality. The Starnes procedure (fenestrated RV exclusion) is reserved for EA patients with cardiogenic shock and has previously committed patients to single ventricle (SV) palliation. In this report, we present the results of a strategy to redirect patients utilizing the Da Silva Cone operation to achieve a 2 or 1...

BACKGROUND: Ebstein's anomaly (EA) is a rare and complex congenital heart anomaly, and the effect of surgical treatment is not ideal. This study aims to introduce our experience in management strategies, surgical techniques, and operative indications for patients with Ebstein's anomaly. METHODS: A retrospective study of 258 operations was performed in 253 patients by the same cardiac surgeon in The First Hospital of Tsinghua University between March 2004 and January 2020...

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