The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature. Finally, two attached appendices are provided for a structured echocardiogram protocol and key information useful for comprehensive Multi-Disciplinary Team discussion.Ĭone procedure Ebstein’s anomaly Tricuspid valve. The review also assesses the effects of pregnancy on the Ebstein's circulation, and vice versa, the effects of Ebstein's on pregnancy outcomes. The surgical options available for patients with Ebstein's anomaly are detailed and analyzed, starting from the initial mono-leaflet repairs to the most recent cone repair and its modifications. For each age group, the clinical presentation, required diagnostic investigations, natural history, and management are described. This article summarizes and analyzes the literature on Ebstein's anomaly and provides a framework for the investigation, management, and follow-up of these patients, whether they present via fetal detection or late in adult life. The most symptomatic patients need intensive care support in the neonatal period. Presentation can vary from the most extreme form in fetal life, to asymptomatic diagnosis late in adult life. Associated congenital cardiac lesions tend to be found more often in younger patients and may even be the reason for presentation. Ebstein Anomaly is a defect of the tricuspid valve, which separates the right atrium from the right ventricle. Ebstein's anomaly affects the development of the tricuspid valve with widely varying morphology and, therefore, clinical presentation. We conclude that: 1) Early mortality from neonatal Ebstein's anomaly is dramatically improved in the current era 2) No identifiable risk factors reliably predict mortality in this population with complex congenital heart disease 3) The presence of associated cardiac lesions and severe tricuspid valve displacement are possible predictors of the need for surgical palliation in the newborn period.Ebstein's anomaly, first described in 1866 by Dr William Ebstein, accounts for 0.3-0.5% of congenital heart defects and represents 40% of congenital tricuspid valve abnormalities. Furthermore, in patients without associated cardiac lesions, severe tricuspid displacement was found to have a 100% negative predictive value and 75% positive predictive value for neonatal surgical intervention. Severe tricuspid displacement and associated cardiac lesions were statistically significant, independent predictors for surgery (p≤0.01, odds ratio=21.3). There was 1 early death (within the first month) giving an overall early mortality of 5%. Surgery was performed in the neonatal period in 11 patients (55%) 6 of those (55%) underwent tricuspid valve closure and shunt (with or without pulmonary valvotomy or pulmonary artery ligation), 4 (36%) underwent shunt placement alone and 1 patient (9%) was completely repaired. There were associated anatomic cardiac lesions (excluding patent ductus arteriosus) noted in 9 patients (45%) 6 with a ventricular septal defect (30%), 6 had pulmonary stenosis or atresia (30%) and 2 had a hypoplastic trabecular portion of the right ventricle (10%). The median TVD was 2.25 and the median TR was 2.5 (0=none, 1=mild, 2=moderate and 3=severe). Admission weight was 3.5☐.3 kg (range 2.6 to 5.4 kg), CT ratio was 79±5% (range 58 to 96%), initial SaO 2 was 83±4% (range 56 to 91%) and median hospital stay was 15 days (range 2 to 67 days). Except where noted, data are presented as mean☙5% confidence interval. Student t test was utilized for continuous data. Chi square and Wilcoxon Rank Sum were utilized to perform bivariate analysis of categorical data. ![]() Clinical and echocardiographic variables were assessed to determine if any could be used to predict the decision to proceed with surgical intervention. ![]() Medical and surgical records, admission chest radiographs and echocardiograms were reviewed for 20 consecutive neonates (10 male, 10 female) who ranged from 0 to 6 days old on admission. ![]() We retrospectively reviewed our institutional experience over the 10-year period between January 1988 and December 1997. Prior studies suggest that outcome in neonates may be related to the degree of tricuspid valve displacement (TVD), severity of tricuspid regurgitation (TR), cardiothoracic (CT) ratio, associated cardiac defects and oxygen saturation (SaO 2). The natural history of this disease is extremely variable, but symptomatic presentation in the neonatal period has been associated with a high mortality rate of ≈50%. Ebstein's anomaly is a rare congenital cardiac malformation characterized by tricuspid valve deformation and displacement.
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