The patient exhibits multiple features suggestive of Timothy syndrome, which is a multisystem autosomal-dominant condition with findings that include prolonged QT interval, hand and foot abnormalities, dysmorphic facial features, and mental retardation. A 2:1 infranodal atrioventricular block may occasionally be seen in the setting of severely prolonged QT interval. Functional nature of atrioventricular block is demonstrated by resumption of 1:1 conduction with changes in heart rate.
Keywords: Infranodal block; Long QT syndrome; Timothy syndrome.
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