Ebstein's anomaly, atrial paralysis and atrio-ventricular block: an uncommon association

We report here the case of a 22-year-old female patient with an incomplete Ebstein's anomaly, complete heart block and atrial standstill. Atrial paralysis associated with Ebstein's anomaly is the most important feature, since there is a report of familial Ebstein's anomaly associated with atrial standstill but isolated cases have not been described. The patient presented with atypical chest pain and a symptomatic bradycardia of 37 beats per minute. A VVIR pacemaker was implanted. She has subsequently been symptom free.