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Wolff–Parkinson–White Syndrome

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Clinical and Surgical Aspects of Congenital Heart Diseases

Abstract

Wolff–Parkinson–White (WPW) is a rare syndrome in which an accessory conduction pathway causes a form of ventricular pre-excitation. To date, the genetics and the origin of the syndrome have not been completely elucidated, despite the emergence of specific relevant mutations and the identification of its autosomal dominant pattern of inheritance in the familial type of WPW cases. The abnormal accessory electrical circuit is called the “Bundle of Kent” and causes two types of pre-excitation (A and B). The presence of two atrioventricular communications allows the development of re-entry circuits that circumvent the atrioventricular node, resulting in symptomatic supraventricular tachycardia episodes and even sudden cardiac death. The clinical appearance of the syndrome is similar to that of several tachyarrhythmias; however, specific ECG patterns are encountered in the WPW syndrome, including the characteristic delta wave, a widened QRS complex (duration > 120 ms), a shortened PR segment (duration < 120 ms), and a T wave opposite to the delta wave. The early diagnosis of the syndrome seems to be crucial since the syndrome can lead to sudden lethal arrhythmias even in asymptomatic patients. However, the reduced number of adults suffering from the WPW syndrome makes its recognition and clinical management even more challenging. Fortunately, the radiofrequency-based transcatheter ablation is considered nowadays as the gold standard treatment for carefully selected patients with recurrent arrhythmias. It treats the syndrome by ablating the accessory conduction pathway and thereby blocking the arrhythmogenic substrate in great success and low complication rates, while specific antiarrhythmic drug treatment is currently reserved for urgent manifestations of the syndrome.

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Kelepouri, A., Kamzolas, O., Papazoglou, A.S., Moysidis, D.V., Tsagkaris, C. (2023). Wolff–Parkinson–White Syndrome. In: Tagarakis, G., Gheni Sarfan, A., Hashim, H.T., Varney, J. (eds) Clinical and Surgical Aspects of Congenital Heart Diseases . Springer, Cham. https://doi.org/10.1007/978-3-031-23062-2_28

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