Impact of Surgery in Ebstein’s Anomaly Using Current Surgical Criteria

Abstract

terns depend on anatomical severity, degree of right-to-left atrial shunt, and age at presentation. 4, 5 Long-term outcome is usually good for patients who survive the neonatal period and for those with no symptoms following the first years of life. 3 Therefore, many patients will develop symptoms and surgical treatment may be necessary. Current surgical criteria according to European or US guidelines include: (1) presence of symptoms or worsening functional class; (2) cyanosis (oxygen saturation <90%); (3) paradoxical embolism; (4) progressive cardiomegaly on chest X-ray; and (5) progressive RV dilation or RV systolic dysfunction. 6 Other authors recommend surgical repair in patients with more than moderate TR in the presence of symptoms (New York Heart Association [NYHA] class >II or arrhythmias) or worsening exercise capacity. 7 Optimal timing for surgical management in this patient subset remains controversial. Badiu et al found that NYHA functional class >II and cardiothoracic index >0.6 are important prognostic factors associated with long-term mortal-E bstein's anomaly (EA) accounts for <1% of all congenital heart disease. There is general consensus that EA affects not only the tricuspid valve but also the whole right ventricle (RV). This results in a particular type of right-sided cardiomyopathy. 1 The characteristic features of this anomaly are: (1) predominant septal and inferior tricuspid leaflet attachment to the underlying myocardium due to failure of delamination; (2) anterior and apical rotational displacement of the functional annulus; (3) dilation of the "atrialized" portion of the RV with variable degrees of hypertrophy and wall thinning; (4) redundancy, fenestration and tethering of the anterior leaflet; (5) dilation of the right atrioventricular junction (the true tricuspid annulus); and (6) variable degree of ventricular myocardial dysplasia and dysfunction. 1-3These anatomical and functional abnormalities cause important tricuspid regurgitation (TR) in many patients that contributes to right chamber dilation and atrial or ventricular arrhythmias. Hemodynamics and clinical pat-