Atrial standstill in a case of Kugelberg-Welander syndrome with cardiac involvement: an electrophysiologic study

A patient with Kugelberg-Welander syndrome associated with junctional rhythm and restrictive cardiomyopathy is presented. An electrophysiologic study was performed. Persistent atrial standstill was demonstrated by detailed right atrium and coronary sinus mapping and failure of capture of both right atrium and coronary sinus pacing. A prolonged junctional recovery time, normal HV interval and normal pacing threshold of the right ventricle were also noted. The patient was successfully treated with cardiac pacing and diuretics.