This report describes a catheter technique for ablating the His bundle and its application in nine patients with recurrent supraventricular tachycardia that was unresponsive to medical management. A tripolar electrode catheter was positioned in the region of the His bundle, and the electrode recording a large unipolar His-bundle potential was identified. In the first patient, two shocks of 25 and 50 J, respectively, were delivered by a standard cardioversion unit to the catheter electrode, resulting in an intra-His-bundle conduction defect. Subsequent delivery of 300 J resulted in complete heart block. In the next eight patients, an initial shock of 200 J was used. The His bundle was ablated by this single shock in six of these patients and by an additional shock of 300 J in one. In the remaining patient, conduction in the atrioventricular node was modified, resulting in alternating first and second-degree atrioventricular block. A stable escape rhythm was preserved in all patients. The procedure was well tolerated, without complications, and all patients have remained free of arrhythmia, without medication, for follow-up periods of two to six months.

Background-Chest pain is frequently reported in Fabry disease (FD). However, its mechanism and clinical relevance are unclear. Methods and Results-Basal troponin I level, exercise stress test, single-photon emission computed tomography imaging with 99m Tc sestamibi, coronary angiography with thrombolysis in myocardial infarction (TIMI) frame count and left ventricular angiography and endomyocardial biopsy were obtained in 13 patients with FD with angina. Ratio of external to lumen diameter of intramural arteries (E/L ratio), myocyte diameter, and extent of fibrosis were morphometrically evaluated by using tissue sections. Controls for coronary angiography and histology were 25 patients with FD without angina and 20 mitral stenosis patients with normal left ventricular function. Troponin I level was elevated in 6 of the 13 patients. Exercise stress test showed evidence of myocardial ischemia, and single-photon emission computed tomography was positive for stress-induced perfusion defects in all patients with FD with angina. Epicardial coronaries were structurally normal but showed slow flow in all and were associated with aneurisms of posterior left ventricular wall in 3 cases. Histology showed remarkable lumen narrowing of most intramural arteries (mean E/L ratioϭ3.5Ϯ1.2; PϽ0.001 versus both control groups), because of hypertrophy and proliferation of smooth muscle and endothelial cells, both engulfed by glycosphingolipids. Replacement fibrosis exceeded that of both controls (PϽ0.001). Small vessel disease correlated with coronary slow flow and extent of fibrosis, but did not with patients' age, sex, and degree of left ventricular hypertrophy.

Data are reported on three patients with the permanent form of junctional reciprocating tachycardia, in whom conduction over a slow accessory pathway was observed after His bundle ablation. Tachycardia was almost incessant and showed a retrograde P wave (P') and RP' interval longer than P'R interval in all patients; during sinus rhythm, the PR interval was normal and there was no evidence of a delta wave. An accessory pathway with a long conduction time located in the posterior pyramidal space provided the retrograde limb of the reentry circuit. After His bundle ablation, the accessory pathway was capable of conducting in both anterograde and retrograde directions with decremental properties in all patients. Postmortem documentation of the accessory pathway was achieved in one patient. Serial sections revealed an accessory atrioventricular connection composed of ordinary myocardium joining the lower rim of the coronary sinus outlet to the uppermost ventricular muscle. This anomalous atrioventricular connection pursued a sinuous, tortuous path. As a result of changing cross-sectional area, such an accessory pathway might exhibit slow conduction, thus explaining its decremental characteristics.

No abstract

There is general agreement that an orthodromic AV reentry using a concealed slow conducting accessory pathway as the retrograde limb of the circuit constitutes the underlying mechanism of the permanent form of junctional reciprocating tachycardia (PJRT). In this arrhythmia, the standard ECG typically shows a "long R-P' tachycardia" with retrograde P wave negative in the inferior leads. A careful electrophysiologic evaluation is necessary to confirm the diagnosis of PJRT. Recent reports have demonstrated that the radiofrequency current catheter technique provides a safe and highly effective therapeutic tool for patients suffering from this arrhythmia.