Catheter Ablation of Reentrant Left Ventricular Tachycardia Associated with Fabry disease: A Case Report

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)

Identification of Unusual Reentry Circuit Sites of Nonischemic Ventricular Outflow Tract Tachycardia

“…Because of the small number of cases, we could not statistically analyze the relationship between the type of reentry circuit sites and termination rate, but ablation of isthmus sites (exit, central‐proximal) had a higher rate of termination than nonisthmus sites (inner loop, outer loop). Our results are in agreement with studies that have reported a higher rate of VT termination in exit or central‐proximal segments (30–40%) than in inner loop or outer loop (10%) segments 11,20 . There are differences in opinion, regarding whether inner and outer loop sites should be ablated because of poor termination rate.…”

“…Patients 4 and 5 had normal coronary angiography and diagnosed DCM by findings of left ventricular dilatation and sever reduction of left ventricular ejection fraction using echocardiography and myocardial biopsy. Patient 6 was already diagnosed as having Fabry disease based on his family history, the enzyme activity, and the results of kidney biopsy and genetic evaluation 11 . All 6 patients had inadequate suppression of their VT despite being on oral antiarrhythmics during the study period (amiodarone 1, mexiletine 3, aprindine 2).…”

Radiofrequency ablation of ventricular tachycardia in Anderson–Fabry disease: a case series

“…In another study, Nakano et al . 10 report on a 51-year-old man with an LVEF of 68%, presenting with SMVT. CMRI demonstrated high T2 signal in the basal epicardium and antero-septal endocardium.…”