Progression of conduction system disease in a paced patient with Kearns-Sayre syndrome.

The Kearns-Sayre syndrome is a rare condition characterized by external ophthalmoplegia, retinal pigmentary degeneration and progressive impairment of cardiac conduction. We report a patient with the permanent pacemaker. The spontaneous cardiac impulse formation disappeared during long-term follow-up in this patient with Kearns-Sayre Syndrome.