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. 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. Tricuspid valve repair or replacement in conjunction with closure of the interatrial communication is recommended for older patients with severe symptoms despite medical therapy and those with less severe symptoms who have cardiac enlargement. Patients with symptomatic right heart failure should receive diuretics. Prophylaxis against infective endocarditis is recommended.
A systolic murmur caused by tricuspid regurgitation is usually present at the left lower sternal border.Įchocardiography is used to assess the presence and magnitude of right atrial dilation, anatomic displacement and distortion of the tricuspid valve leaflets, and the severity of tricuspid regurgitation or stenosis. The first and second heart sounds are both widely split, and a third or fourth heart sound is often present, resulting in triple or even quadruple heart sounds. On physical examination, the severity of cyanosis depends on the magnitude of right-to-left shunting. Older children with Ebstein’s anomaly often come to medical attention because of an incidental murmur, whereas adolescents and adults may be identified because of a supraventricular arrhythmia.
The clinical presentation of subjects with Ebstein’s anomaly ranges from severe right heart failure in the neonate to the absence of symptoms in the adult in whom it is discovered incidentally. On the one extreme, patients with mild apical displacement of the tricuspid valve leaflets have normal valvular function on the other extreme, those with severe leaflet displacement or abnormal anterior leaflet attachment, with resultant valvular dysfunction, have an elevated right atrial pressure and right-to-left shunting if an interatrial communication is present. The severity of the hemodynamic derangements in patients with Ebstein’s anomaly depends on the magnitude of displacement and the functional status of the tricuspid valve leaflets. Eighty percent of patients with Ebstein’s anomaly have an interatrial communication (ASD or patent foramen ovale) through which right-to-left shunting of blood can occur. The tricuspid valve is usually regurgitant, but it may be stenotic. As a result, a portion of the right ventricle is atrialized, in that it is located on the atrial side of the tricuspid valve, and the remaining functional right ventricle is small ( Figure 5).
#Ebstein anomaly free
With Ebstein’s anomaly, the tricuspid valve’s septal leaflet and often its posterior leaflet are displaced into the right ventricle, and the anterior leaflet is usually malformed and abnormally attached or adherent to the right ventricular free wall. Kellerman MD, in Conn's Current Therapy 2021, 2021 Ebstein’s Anomaly
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