C33. Complex ebstein anomaly in adult patient: a case report

Abstract

Abstract Background Ebstein’s anomaly is a rare form of congenital heart disease. In adult often present with arrhythmias, progressive cyanosis, and right heart failure. Management is complex and individualized. Case Summary A 24-year-old woman presented to the emergency room with heart failure presentation, palpitations and fainting. ECG examination shows abberant SVT with pulse, hemodinamically was stable with peripheral saturation 94% without oxygen supplementation. The patient then carried out cardioversion and got anti-arrhythmia amiodarone. Resting ECG obtained sinus rhythm right axis deviation with incomplete LBBB (splintered QRS). Echocardiography examination revealed displacement of the tricuspid valve, a large atrialized component of the right ventricle, the anterior leaflet of the tricuspid valve moves freely with bidirectional shunt ASD and moderate pericardial effusion, confirmed for Ebstein's anomaly type B . Diuresis therapy was given and anti-arrhythmic therapy was continued. Echocardiographic evaluation showed reduced pericardial effusion to a minimum. Patients were scheduled for electrophysiological studies and angiographic evaluation and total corrections were planned. Discussion Ebstein anomaly is a characterized by abnormally formed and apically displaced leaflets of Tricuspid caused by is failure of proper delamination of the tricuspid valve, primarily of the septal and anterior leaflets from ventricular myocardium. The cardinal symptoms in Ebstein’s anomaly are cyanosis, right-sided heart failure, arrhythmias, and sudden cardiac death. Surgery is indicated in adults with symptoms of heart failure. Medical therapy is generally used only for temporary stabilization : diuretic therapy for volume overload, control of heart rhythm abnormalities, and standard medical therapy for left ventricular systolic dysfunction.