Phase 4 paroxysmal AV block in a patient with scleroderma

A 72-year-old man with limited cutaneous systemic scleroderma was hospitalized for two episodes of witnessed syncope. The baseline 12-lead electrocardiogram was normal but on telemetry there were numerous episodes of paroxysmal AV block with asystolic periods of up to 7.5 s duration. Analysis of the rhythm strips revealed phase 4 intra-His bundle block characterized by critical P-P intervals that triggered the AV block, and a narrow range of junctional escape to subsequent P wave intervals that were required to release the AV block. A dual chamber pacemaker was implanted.