Permanent paralysis of the atrium in a patient with facioscapulohumeral muscular dystrophy

A 32 year old man with the facioscapulohumeral (Landouzy-Dejerine) type of muscular dystrophy was evaluated because of a slow pulse rate. Hemodynamic studies were performed at rest and during ventricular pacing. The patient's atria were found to be electrically and mechanically silent. This unusual disorder of permanent atrial paralysis (standstill) is reviewed. There seems to be more than a casual relation between permanent atrial paralysis and this form of muscular dystrophy.