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 years)...

Thrombus formation in the left atrium is rare in patients in sinus rhythm. In three patients with extensive cardiac amyloidosis transthoracic echocardiography showed large atrial thrombi in or protruding into the body of the left atrium during sinus rhythm. Doppler studies showed no A wave on mitral inflow. Severe atrial and ventricular infiltration by amyloid may have resulted in mechanical at...

Diastolic mitral valve "locking," defined as sustained diastolic closure of the mitral valve after atrial systole, was investigated by simultaneous hemodynamic and echocardiographic recordings during a protocol of programmed pacing in six dogs with surgically induced atrioventricular block. Atrial extrasystoles were introduced at progressively increasing coupling intervals during programmed pro...

Vagal reflexes can be induced by stimulation of select epicardial and endocardial areas of the left atrium. The present report describes the case of a 54-year-old woman with a history of recurrent symptomatic episodes of paroxysmal atrial fibrillation. During radiofrequency application around the left superior pulmonary vein, the patient exhibited an excessive vagal response with a sinus pause ...