title: Temporal relationship of conduction system disease and ventricular dysfunction in LMNA cardiomyopathy.
abstract: BACKGROUND
  LMNA cardiomyopathy presents with electrocardiogram (ECG) abnormalities, conduction system disease (CSD), and/or arrhythmias before the onset of dilated cardiomyopathy (DCM). Knowing the time interval between the onset of CSD and its progression to DCM would help to guide clinical care.
  
  
  RESULTS
  We evaluated family members from 16 pedigrees previously identified to carry LMNA mutations for the ages of onset of ECG abnormalities, CSD, or arrhythmia and of left ventricular enlargement (LVE) and/or systolic dysfunction. Of 103 subjects, 64 carried their family LMNA mutation, and 51 (79%) had ECG abnormalities with a mean age of onset of 41.2 years (range 18-76). Ventricular dysfunction was observed in 26 with a mean age of onset of 47.6 years (range 28-82); at diagnosis 9 had systolic dysfunction but no LVE, 5 had LVE but no systolic dysfunction, and 11 had DCM. Of 16 subjects identified with ECG abnormalities who later developed ventricular dysfunction, the median ages of onset by log-rank analyses were 41 and 48 years, respectively.
  
  
  CONCLUSIONS
  ECG abnormalities preceded DCM with a median difference of 7 years. Clinical surveillance should occur at least annually in those at risk for LMNA cardiomyopathy with any ECG findings.
authors.affiliation: Cardiovascular Division, Miller School of Medicine, University of Miami, Florida, USA.
authors.forename: Chad
authors.initials: C
authors.lastname: Brodt
authors.forename: Jill D
authors.initials: JD
authors.lastname: Siegfried
authors.forename: Mark
authors.initials: M
authors.lastname: Hofmeyer
authors.forename: Jose
authors.initials: J
authors.lastname: Martel
authors.forename: Evadnie
authors.initials: E
authors.lastname: Rampersaud
authors.forename: Duanxiang
authors.initials: D
authors.lastname: Li
authors.forename: Ana
authors.initials: A
authors.lastname: Morales
authors.forename: Ray E
authors.initials: RE
authors.lastname: Hershberger
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002311:Cardiomyopathy, Dilated / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics*; D004562:Electrocardiography / Q000639:trends*; D005260:Female; D005500:Follow-Up Studies; D006329:Heart Conduction System / Q000473:pathology / Q000502:physiology*; D006801:Humans; D034904:Lamin Type A / Q000235:genetics*; D008060:Lipodystrophy / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics; D008297:Male; D008875:Middle Aged; D013997:Time Factors; D018754:Ventricular Dysfunction / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics*; D055815:Young Adult
chemical_list: C494847:LMNA protein, human; D034904:Lamin Type A