Evolution of the treatment approaches to ebstein's anomaly: a state-of-the-art literatre review

Ebstein's anomaly is a complex congenital anomaly with a broad anatomic spectrum from mild forms in asymptomatic patients to severe pulmonary atresia. Both, right and left heart chambers, are affected. This causes a variety of clinical manifestations and determines the use of different surgical approaches to treat patients with Ebstein's anomaly. Considering 70-years’ experience of treating this group of patients, there is no any universal approach. Conservative treatment of patients with Ebstein's anomaly has shown its poor effectiveness. There is no any other option than surgical treatment, and the establishment of biventricular circulation through surgical repair is considered as a method of choice. The comparative analysis of different surgical approaches that are currently used in the routine clinical practice allows concluding that the most promising method is the cone reconstruction of the tricuspid valve. It can be successfully used in patients of any age, even in newborns. Preoperative management and timely diagnosis of heart rhythm disturbances, including Wolff-Parkinson-White syndrome, are of great importance. The need for either interruption of multiple accessory conduction before surgical repair or concomitant treatment should be considered.

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