Abstract
A 20-year-old man with Loeys-Dietz syndrome with familial TGFBR2 mutation and Wolff-Parkinson-White syndrome presented with palpitations. His transthoracic echocardiogram revealed normal left ventricular systolic function and an enlarged aortic root based on his age and size. The baseline electrocardiogram during rapid atrial pacing revealed preexcitation with a pattern suggestive of a left posterolateral accessory pathway (AP) (Figure 1A). There was no evidence of decremental AP conduction. A wide complex tachycardia was induced during programmed stimulation. Intracardiac electrograms showed variable His bundle depolarization (black arrows) (Figure 1B). What is the mechanism of the tachycardia and its varying His bundle activation?
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