title: Electrocardiogram in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy patients without any clinical evidence of coronary artery disease: a case-control study.
abstract: OBJECTIVE
  Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited systemic arteriopathy caused by highly stereotyped mutations in NOTCH3. The clinical expression of CADASIL is confined to the central nervous system with characteristic recurrent subcortical infarcts and vascular dementia. However, cases have been reported with associated circulatory small vessel abnormalities in the retina or the myocardium and with myocardial infarction. Classical cardiovascular risk factors may influence such circulatory abnormalities. Thus, we conducted a case control study to determine the frequency of electrical abnormalities on a 12-lead resting ECG in CADASIL patients without classical atherosclerotic risk factors.
  
  
  METHODS
  Twenty-three CADASIL patients (mean age+/-SD; 55.1+/-11 years) free of any classical cardiovascular risk factors except for hypercholesterolemia were recruited from 1 neurology department and compared with 23 sex- and age-matched healthy controls (mean age+/-SD; 54.7+/-9.5 years). A resting supine 12-lead ECG was recorded at inclusion and analyzed later by 2 reviewers. Signs of myocardial infarction or ischemia, conduction, and rhythm disturbances were looked for.
  
  
  RESULTS
  We found no ECG sign evoking myocardial infarction or myocardial ischemia. CADASIL patients had, compared with healthy controls, a significantly higher heart rate and a significantly lower Sokolow index, but these values remained in the normal ranges.
  
  
  CONCLUSIONS
  In this case-control study, we found no ECG evidence for myocardial infarction or ischemia, conduction disturbances, or arrhythmias in CADASIL patients compared with healthy controls.
authors.affiliation: Department of Neurology, AP-HP, Lariboisière Hospital, Université Diderot, Paris, France.
authors.forename: Rodica
authors.initials: R
authors.lastname: Cumurciuc
authors.forename: Patrick
authors.initials: P
authors.lastname: Henry
authors.forename: Claire
authors.initials: C
authors.lastname: Gobron
authors.forename: Eric
authors.initials: E
authors.lastname: Vicaut
authors.forename: Marie-Germaine
authors.initials: MG
authors.lastname: Bousser
authors.forename: Hugues
authors.initials: H
authors.lastname: Chabriat
authors.forename: Katayoun
authors.initials: K
authors.lastname: Vahedi
mesh_terms: D000328:Adult; D000818:Animals; D001145:Arrhythmias, Cardiac / Q000150:complications / Q000175:diagnosis; D046589:CADASIL / Q000150:complications*; D016022:Case-Control Studies; D004562:Electrocardiography; D005260:Female; D006329:Heart Conduction System / Q000503:physiopathology; D006331:Heart Diseases / Q000150:complications* / Q000175:diagnosis* / Q000503:physiopathology; D006339:Heart Rate; D006801:Humans; D006937:Hypercholesterolemia / Q000150:complications; D008297:Male; D008499:Medical Records; D008875:Middle Aged; D009203:Myocardial Infarction / Q000150:complications / Q000175:diagnosis; D017202:Myocardial Ischemia / Q000150:complications / Q000175:diagnosis; D012307:Risk Factors