Ebstein Anomaly in adolescents and young adults

Firstly described by W. Ebstein in 1866, the Ebstein anomaly (EA) is composed of a wide spectrum of malformations, so that every valve taken into consideration is a unique valve. EA is rare, accounting for approximately 1-5 cases every 200,000 live births and representing less than 1 % of all CHD, but is the most frequent congenital lesion of the tricuspid valve (TV). Natural history of patients with EA is poor. Early presentation in life is associated with high percentage of mortality, while patients who survived the neonatal period have a limited life expectancy. Deaths usually occur for arrhythmic events and heart failure. From the embryological point of view, the anomaly is related to a failure of delamination of the TV leaflets which reflects with a characteristic pattern of morphological anomalies involving the TV and the right ventricle (RV). This cohort of abnormalities could be associated with rhythm disturbances and other cardiac defects. The disease may have a clinical neonatal presentation which corresponds to a severe RV and TV dysfunction or may present later in life, concurrently with an increasing degree of TV regurgitation and RV dilatation. Since the pioneer period of cardiac surgery, many techniques have been described to address the TV and RV anomalies including TV replacement which is still an option, despite reconstructive techniques being now preferred due to the improvement of the early and long-term results. The aim of the repairing techniques is to restore a normal tricuspid valve function, to preserve the right ventricular contractility, and to decrease the risk of rhythm disturbances. Results are associated with the severity of the disease, the surgeon experience, and the perioperative care of the patient.