title: Cardiac screening investigations in adult-onset progressive external ophthalmoplegia patients.
abstract: BACKGROUND
  Patients with mitochondrial myopathies may develop cardiac complications such as cardiomyopathy and/or cardiac conduction defects. To identify these potentially life-threatening and treatable conditions, it is common practice to screen patients intermittently with electrocardiography and echocardiography. The optimal time interval for such screening investigations is unknown. We developed this study to review our screening results in adult-onset patients with progressive external ophthalmoplegia (PEO).
  
  
  METHODS
  This study was a retrospective review of PEO patients with 5 years or more of cardiac screening investigations who did not have any cardiac symptoms.
  
  
  RESULTS
  Fifteen patients were included, and cardiomyopathy was identified on screening echocardiogram in 1 patient. Four patients had other abnormalities identified, which were unrelated to their mitochondrial myopathy.
  
  
  CONCLUSIONS
  Only 1 patient in 15 developed cardiac complications related to mitochondrial disease during 5 years of follow-up. We suggest that a screening interval of 3-5 years is probably appropriate for adult-onset PEO patients who do not have cardiac symptoms.
authors.affiliation: Division of Neurology, University of British Columbia, Vancouver, British Columbia, Canada.
authors.forename: Gerald
authors.initials: G
authors.lastname: Pfeffer
authors.forename: Michelle M
authors.initials: MM
authors.lastname: Mezei
mesh_terms: D000328:Adult; D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D009202:Cardiomyopathies / Q000175:diagnosis* / Q000209:etiology* / Q000235:genetics; D004562:Electrocardiography / Q000379:methods*; D005260:Female; D006801:Humans; D008297:Male; D008403:Mass Screening / Q000379:methods*; D008875:Middle Aged; D017246:Ophthalmoplegia, Chronic Progressive External / Q000150:complications* / Q000235:genetics; D012189:Retrospective Studies