BACKGROUND AND PURPOSE Emery-Dreifuss muscular dystrophy (EDMD) is a rare inherited disorder associated with cardiac involvement. We investigated the spectrum and relevance of the cardiac manifestations of EDMD, focusing on bradyarrhythmias and tachyarrhythmias (including atrial fibrillation/flutter), embolic stroke, and heart failure. METHODS AND RESULTS Eighteen patients (age 42.8+/-19.6 ye...

An asymptomatic patient with cardiomegaly caused by isolated right atrial standstill is reported. The right atrium showed no evidence of contraction on pressure records or angiographically, while the left atrium functioned normally.

Persistent atrial standstill occurred in a father and his youngest son with familial Ebstein's anomaly. In both cases routine electrocardiograms showed no atrial electrical activity and cross sectional echocardiograms showed inferior displacement of the septal tricuspid leaflet and tethering of the tricuspid leaflets to the right ventricle. The father had a cerebral embolism and died of a myoca...

INTRODUCTION Atrial standstill is a rare condition, characterized by absence of atrial electrical and mechanical activity evident in surface electrocardiography echocardiography, or fluoroscopy, which is associated with unresponsiveness of atria to maximal output electrical stimulation. This condition can be present with thromboembolic complication, low cardiac output, and sometimes palpitation...

We introduce a 32-year-old man who was evaluated for a dizziness and headache of unknown origin for at least two months and was referred to our center after ECG findings. He was finally diagnosed as a case of idiopathic, familial, diffuse, persistent atrial standstill, which is a rare arrhythmogenic condition characterized by the absence of electrical and mechanical activity in the atria. He su...

A 59-year-old woman with partial atrial standstill was studied using magnetocardiograms (MCGs), which revealed through QRS-T subtraction and time-frequency analysis that there was a high-frequency (6 Hz) magnetic source at the low atrial septum. MCGs are useful for noninvasively evaluating the clinical course of patients with atrial fibrillation.