Case summary reveals conduction abnormalities in a mitochondrial disorder, suggesting early intervention is crucial.
CASE SUMMARY: We describe the case of a 45-year-old man with progressive external ophthalmoplegia and pigmentary retinopathy who was referred for evaluation of a bradyarrhythmia due to exertional dyspnea and fatigue. A baseline electrocardiogram and ambulatory monitoring revealed clinically significant conduction abnormalities. TAKE-HOME MESSAGES: In Kearns-Sayre syndrome, conduction abnormalities may progress unpredictably; early permanent pacing should be strongly considered. Consider implantable cardioverter-defibrillator rather than pacemaker alone when additional high-risk features are present.
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Amaro et al. (2026) studied this question.
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