No abstract

A 51-year-old man, who was diagnosed with Fabry disease resulting from a kidney biopsy for proteinuria and renal failure in 2002, was admitted to our hospital for sustained ventricular tachycardia (VT). In the electrophysiological study, VT (cycle length: 310 ms) was successfully induced by right ventricle programmed stimulation and the twelve-lead electrocardiogram showed a right bundle branch block configuration with right axis deviation. The mechanism of the VT was considered to be reentry by entrainment phenomenon. An electro-anatomical mapping system identified a low voltage area located close to the left ventricular anterior-apical wall. During VT an isolated pre-potential was recorded 42 ms prior to the QRS onset near the border zone which was located between the low and normal voltage areas. At this mapping site entrainment with fusion and a post-pacing interval that matched the VT cycle length were observed. A radiofrequency energy delivery at this site terminated the VT after 35 seconds. The entrainment mapping could be useful for identifying a critical reentry circuit path. This case is the first description of reentrant VT originating from the thickened left ventricle wall in a patient with Fabry disease. (J Arrhythmia 2010; 26: 209-215)