![]() Multicenter studies are needed to establish the best modalities for surgical management. We describe our approach, surgical technique, and postoperative results here. We are heavily biased toward a biventricular repair. Single-ventricle palliation has been advocated by some groups. Most techniques are based on plication of the redundant atrialized right ventricle, tricuspid annuloplasty, use of the sail-like anterior leaflet in construction of a competent monocuspid valve, closure of atrial septal defect, and reduction of right atrium. Techniques of surgical repair have evolved over a period of time. Surgical intervention is needed when patients fail to respond to standard medical resuscitative measures. Those who respond to medical measures should be watched over a period of time. Medical management depends on the degree of hemodynamic stability and centers around supplemental oxygen and prostaglandin infusion. Echocardiogram usually provides sufficient anatomic and hemodynamic information. The pathologic hallmark is the downward and apical displacement of the tricuspid valve into the right ventricle. The presentation depends on the severity of the disorder and can range from severe cyanosis and heart failure in the newborn to mild exercise intolerance in the adult. Knott-Craig MD, in Critical Heart Disease in Infants and Children (Third Edition), 2019 AbstractĮbstein anomaly is a rare congenital disorder of the tricuspid valve and right ventricular myocardium. 3 In the general population, the rate of preexcitation occurs in only 0.25% of persons. 3įrom 6% to 36% of patients with Ebstein's anomaly have an accessory pathway leading to ventricular preexcitation (Wolff-Parkinson-White syndrome). Because the left ventricular systolic pressure is always larger than the right atrial pressure, the muscular portion of the atrioventricular septum moves in a paradoxical manner away from the center of the left ventricle during ventricular systole.Īn interatrial communication (which generally appears as a large patent foramen ovale) is present in 80% to 94% of patients with Ebstein's anomaly. 2īecause of apical displacement of the tricuspid valve, the inlet portion of the RV becomes atrialized. The magnitude of such displacement is small (8 mm/m 2) in severe cases, the atrioventricular septum may extend almost to the RV apex. Normally, tricuspid annulus is located more apically than the mitral annuls. In contrast, the anterior leaflet is frequently large, sail-like, and often fenestrated. The septal and posterior leaflets often are very short and adherent to the surrounding ventricular wall. ![]() ![]() In persons with Ebstein's anomaly, proper delamination fails to occur. 1 Associated anomalies include interatrial communication and Wolff-Parkinson-White syndrome.Įmbryologically, tricuspid leaflets develop from the ventricular tissue by the process of delamination. Muhamed Saric MD, PhD, FASE, Itzhak Kronzon MD, FASE, in Dynamic Echocardiography, 2011 DefinitionĮbstein's anomaly, which is named after the German physician Wilhelm Ebstein, who described it first in 1864, is a triad of tricuspid leaflet dysplasia, apical displacement of the tricuspid annulus, and the attendant abnormalities of the right atrium and the right ventricle (RV). In a multivariate model, younger age at diagnosis, male gender, cardiothoracic ratio greater than or equal to 0.65, and the severity of tricuspid valve leaflet displacement on echocardiography were independent predictors of cardiac mortality. Univariate predictors of cardiac-related death included cardiothoracic ratio greater than or equal to 0.65, increasing severity of tricuspid valve displacement on echocardiography, NYHA class III or IV, cyanosis, severe tricuspid regurgitation, and younger age at diagnosis. 6 The estimated cumulative overall survival was 89%, 76%, 53%, and 41% at 1, 10, 15, and 20 years of follow-up, respectively. In a natural history study of 72 unoperated patients with Ebstein anomaly, the mean age at diagnosis was 23.9 ± 10.4 years and arrhythmias were the most common clinical presentation (51%). However, late survival of unoperated adult patients with Ebstein anomaly is less than expected. The oldest patient at the Mayo Clinic to undergo primary operation was 79 years of age. Dearani, in Diagnosis and Management of Adult Congenital Heart Disease (Second Edition), 2011 Late OutcomeĮbstein anomaly may remain undetected until late childhood or adulthood. ![]()
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