Open window mapping for redo accessory pathway ablation in Ebstein anomaly.

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.

Mosaic trisomy 5 in amniotic fluid in a live fetus with Ebstein anomaly and complete trisomy 5 in chorionic villus sampling.

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.

Cliff diving leading to commotio cordis in a patient with Ebstein's 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.

Fetal single ventricle journey to first postnatal procedure: a multicentre UK cohort study.

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.

Prenatal sonographic findings of prominent fetal tricuspid annulus: A case report.

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).

Timing of Cardiac Surgical Interventions and Postoperative Mortality in Children With Severe Congenital Heart Defects Across Europe: Data From the EUROlinkCAT Study.

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.

Effect of concomitant atrial septal defect on left ventricular function in adult patients with unrepaired Ebstein's anomaly: a cardiovascular magnetic resonance imaging study.

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.

Distal His bundle pacing in a patient with surgically corrected complex Ebstein anomaly and symptomatic second-degree atrioventricular block: a case report.

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.

Using DaSilva Cone Operation to Establish 1.5 or 2 Ventricle Circulation After Initial Single Ventricle Palliation with Starnes Procedure.

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.

Strategy and technique for surgical treatment of Ebstein's anomaly.

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.

An incidental finding of a right atrial myxoma with undiagnosed Ebstein anomaly: a case report.

Background: Ebstein anomaly (EA) is a rare congenital abnormality of the tricuspid valve which can lead to progressive right heart dilatation and arrhythmias. While often seen in conjunction with other congenital cardiac lesions, such as atrial septal defects, it is not normally associated with atrial myxomas.

Case Summary: We present a case report of an incidental finding of a right atrial myxoma in the context of undiagnosed EA, in a 16-year-old male who presented with appendicitis.

Cardiac Catheterization in Adults with Ebstein Anomaly: Pathophysiologic Insights Regarding Surgical Repair and Prognosis.

The underlying invasive hemodynamics and physiology in Ebstein anomaly (EA) are poorly understood. Moreover, the hemodynamic impact of tricuspid valve intervention in EA has not been well studied. Retrospective cohort of 52 adults with repaired and 36 with unrepaired EA undergoing right heart catheterization at Mayo Clinic, MN between 1993 and 2021.

Impact of Neonatal Intervention on Left Ventricular Performance in Ebstein's Anomaly and Tricuspid Valve Dysplasia.

Background: Severe neonatal Ebstein's anomaly (EA) and tricuspid valve dysplasia (TVD) are associated with high perinatal morbidity and mortality. The authors recently demonstrated left ventricular (LV) dysfunction and dyssynchrony to be prevalent in affected newborns and to contribute to poor outcomes. The aim of this study was to investigate the impact of patent ductus arteriosus (PDA) closure, spontaneous or surgical ligation, or right ventricular exclusion (Starnes procedure) on LV performance in neonatal EA and TVD.

Rare loss-of-function variants in matrisome genes are enriched in Ebstein's anomaly.

Ebstein's anomaly, a rare congenital heart disease, is distinguished by the failure of embryological delamination of the tricuspid valve leaflets from the underlying primitive right ventricle myocardium. Gaining insight into the genetic basis of Ebstein's anomaly allows a more precise definition of its pathogenesis. In this study, two distinct cohorts from the Chinese Han population were included: a case-control cohort consisting of 82 unrelated cases and 125 controls without cardiac phenotypes and a trio cohort comprising 36 parent-offspring trios.

The Role of Speckle-Tracking Echocardiography in Predicting Mortality and Morbidity in Patients With Congenital Heart Disease: A Systematic Review and Meta-analysis.

Background: Speckle-tracking echocardiography (STE) is now routinely included in cardiac evaluations, but its role in predicting mortality and morbidity in congenital heart disease (CHD) is not well described. We conducted a systematic review to evaluate the prognostic value of STE in patients with CHD.

Methods: The EMBASE, Medline, Web of Science, Scopus, and Cochrane Central Register of Controlled Trials (CENTRAL) databases were searched from inception to January 2023 for terms related to all CHD, STE, and prognosis.

The appearance of new accessory pathway after the Cone procedure.

Introduction: Acquired Wolff-Parkinson-White (WPW) syndrome can occur after congenital heart disease (CHD) surgery.

Methods And Results: A 27-year-old male with Ebstein's anomaly and manifest WPW syndrome received catheter ablation twice. The first electrophysiology study (EPS) induced orthodromic atrioventricular reentrant tachycardia and successfully eliminated the posteroseptal accessory pathway (AP).

Intraoperative Transesophageal Echocardiography Evaluation of Tricuspid Valve Re-repair for Ebstein's Anomaly.

Postoperative stenosis or regurgitation of the tricuspid valve is common and affects the prognosis after repair surgery of Ebstein's anomaly. However, it is unclear how intraoperative echocardiography influences the postoperative course. We report a longitudinal echocardiography course including intraoperative transesophageal echocardiography in a cone reconstruction procedure for Ebstein's anomaly in a 17-year-old woman.

Significantly elevated hepatic extracellular volume in adult patients with a Fontan circulation and its correlation with impaired functional capacity.

Fontan-associated liver disease is a major concern in patients who have undergone the procedure. Regular imaging of the liver is currently recommended for Fontan patients, but not other congenital heart diseases. The extracellular volume (ECV) of the liver obtained during CMR scanning and studies can show the high liver ECV in Fontan patients.