![]() ![]() Thus it is important that these patients are regularly seen by a cardiologist with expertise in congenital heart disease. Therefore, precise knowledge of the different anatomic and hemodynamic variables, associated malformations and management options are essential. Overall, EA is a complex congenital anomaly with a broad pathologic-anatomical and clinical spectrum and no two patients are alike. The review describes management options in EA including catheter interventions, indication for operation and surgical options including tricuspid valve repair and replacement. Differential diagnosis of EA is discussed including tricuspid valve dysplasia and prolapse as well as arrhythmogenic right ventricular cardiomyopathy. The spectrum of typical ECG and conduction system changes, arrhythmias including accessory pathways and ectopic atrial tachycardias related to EA are also reviewed. Ebsteins anomaly of the tricuspid valve is a cardiac malformation characterized by downward displacement of the septal and inferior tricuspid valve (TV). What is Ebsteins Anomaly The tricuspid valve is abnormal and, instead of being between the right atrium and ventricle, it is low down in the right ventricle. Differences between Ebstein-like changes associated with congenitally corrected transposition and EA are described. Togetherness of Ebstein anomaly and giant hairy nevus in a neonate: first case in the literature. In this review, we describe current diagnostic criteria and classification, natural history, clinical features, and prognosis, typical echocardiographic features and pathologic findings, and the spectrum of associated cardiac malformations including left heart anomalies associated with EA. Özlü F, Tülüce M, Satar M, Özbarlas N, Kozanolu B, Yücel A. The tricuspid valve separates the right atrium (the chamber that receives blood from the body) from the right ventricle (the chamber that pumps blood to the lungs). Ebsteins anomaly is a rare heart defect in which parts of the tricuspid valve (which separates the right ventricle from the right atrium) are abnormal. Since its description in 1866, dramatic advances in diagnosis and therapy have been made. Ebsteins anomaly (sometimes referred to as Ebstein anomaly) is a congenital heart disease in which the tricuspid valve and the right ventricle do not develop normally. Ebsteins anomaly is a defect of the tricuspid valve. ![]() Ebstein's anomaly (EA) is a rare but fascinating congenital heart disorder accounting for <1% of all congenital heart defects.
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