Electrophysiological and histopathological characteristics of progressive atrioventricular block accompanied by familial dilated cardiomyopathy caused by a novel mutation of lamin A/C gene.

Unlabelled: Conduction defect caused by lamin A/C gene mutation.

Introduction: Mutations of lamin A/C gene (LMNA) cause dilated cardiomyopathy (DCM) with atrioventricular (AV) conduction defect, although the electrophysiological and histological profiles are not fully understood.

Methods And Results: We analyzed a large Japanese family (21 affected and 203 unaffected members) of DCM with AV block.

Clinical relationship of myocardial sympathetic nervous activity to cardiovascular functions in chronic heart failure: assessment by myocardial scintigraphy with 123I-metaiodobenzylguanidine.

The aim of this study was to clarify the relationship between cardiac sympathetic nervous activity (SNA) assessed by radioiodinated metaiodobenzylguanidine (123I-MIBG), an analogue of norepinephrine and cardiovascular functions in patients with chronic heart failure (CHF). Subjects were 17 patients with CHF. A dose of 111 MBq of 123I-MIBG was administered intravenously, and 5-minute anterior planar images were obtained 15 minutes (early image) and 3 hours (delayed image) after the injection.