Masashi Kanemoto

New Method of Determining the Atrial Fibrillation Cycle Length During Human Atrial Fibrillation

Introduction: The aim of this study was to investigate the usefulness of the autocorrelation function (reversed fast Fourier transform analysis) in determining the atrial fibrillation cycle length (AFCL) during human atrial fibrillation (AF).

A Rare Case of Complete Stent Fracture, Coronary Arterial Transection, and Pseudoaneurysm Formation Induced by Repeated Stenting

This report describes a rare asymptomatic case of complete stent fracture, coronary arterial transection, and pseudoaneurysm formation in response to repeated stenting. The proximal and distal ends of transected coronary artery were closed, and distal bypass was performed. Coronary arterial transection can occur in patients with repeated stenting as a long-term adverse event.

Japanese patients with Fabry disease predominantly showing cardiac and neurological manifestation with novel missense mutation: R220P

BACKGROUND Fabry disease, an X-linked lysosomal sphingolipid storage disorder caused by mutation of the α-galactosidase A (GLA) gene, results in systemic organ damage. However, the age of onset of clinical manifestations and course of the disease are variable even within the same family. OBJECTIVE In this study, we evaluated the clinical phenotype and the molecular lesions associated with the GLA gene in a Japanese family with Fabry disease that predominantly showed cardiac and neurological manifestations. METHODS A genetic analysis of the GLA gene using conventional genomic sequencing was performed in all seven members of this family, including four hemizygous males and three heterozygous females. Endomyocardial biopsy was performed in two patients with severe left ventricular (LV) hypertrophy. RESULTS A novel missense mutation was identified at codon 220 in exon 5, thus resulting in an arginine to proline substitution (R220P) in all seven family members. The three adult hemizygous males had LV hypertrophy and developed neurological manifestations in their 50s. One of the adult hemizygotes developed complete atrioventricular block. On the other hand, we could not find any organ damage in a young hemizygous male or the three heterozygous females. CONCLUSION We identified a novel missense mutation in a Japanese family with Fabry disease showing cardiac and neurological manifestations. In patients with Fabry disease, advanced organ damage in the heart and brain can be life-threatening, even if renal failure is lacking.

Differentiation of the electrophysiological effects on the atrial myocardium between the pure Na channel blocker, pilsicainide, and flecainide.

AbstractThe purpose of this study was to identify the difference between the pure Na channel blocker, pilsicainide and Ic-antiarrhythmic drug, flecainide, on the atrial electrophysiological characteristics. Methods: The subjects consisted of 24 patients (48 ± 12 years-old: P-group) in whom pilsicainide was administrated intravenously (1 mg/kg/10 min) and 31 patients (47 ± 15 years-old: F-group) in whom flecainide was administrated intravenously (2 mg/kg/10 min). The atrial effective refractory period (ERP-A), intra-atrial conduction time (CT), max intra-atrial conduction delay (Max CD), repetitive atrial firing zone (RAFZ), fragmented atrial activity zone (FAZ) and intra-atrial conduction delay zone (CDZ) were measured before and after the drugs. Results: Pilsicainide and flecainide significantly prolonged the ERP-A (211 ± 27 msec to 246 ± 39 msec; p < 0.001, 217 ± 25 msec to 244 ± 33 msec; p < 0.001, respectively) and CT (121 ± 33 msec to 149 ± 43 msec; p < 0.001, 122 ± 22 msec to 153 ± 27 msec; p < 0.001, respectively) to the same degree. However, the Max CD was shortened by pilsicainide, but not by flecainide. The RAFZ, FAZ and CDZ decreased in the P-group (21 ± 25 msec to 4 ± 10 msec; p < 0.01, 24 ± 24 msec to 14 ± 18 msec; p < 0.05, 56 ± 29 msec to 43 ± 32 msec, p < 0.05, respectively), but not in the F-group. Conclusions: The effects of atrial conduction delays may differ between pilsicainide and flecainide. Further examination will be needed to explain this mechanism.