title: Ventricular Hypertrophy on Electrocardiogram Correlates with Obstructive Lesion Severity in Williams Syndrome.
abstract: OBJECTIVE
  Williams syndrome (WS) is a congenital, multisystem developmental disorder affecting 1 in 8000 live births. Cardiovascular abnormalities are present in 80% of WS patients. The present study sought to characterize fully the electrocardiographic findings in WS and correlate findings with anatomic pathology.
  
  
  METHODS
  A retrospective review was performed of the electrocardiograms (ECGs) of patients with WS evaluated at the Children's Hospital of Philadelphia from January 1, 1980 through December 31, 2007. When available, the five most recent ECGs in each patient were evaluated. Ventricular hypertrophy was diagnosed based on previously reported voltage criteria in normal populations.
  
  
  RESULTS
  There were 187 patients with 499 ECGs for evaluation. Median age at study ECG was 8.0 years (range 0.1-58.9); median number of ECGs per patient was 2.7 (range 1-5). Heart rate, PR interval, and QRS interval were normal for age. Right ventricular hypertrophy (RVH) was present on 44% (219/499) of ECGs. Left ventricular hypertrophy (LVH) was present on 30% (150/499) of ECGs. Fifty-seven percent (106/187) of subjects had ≥1 ECG demonstrating RVH, and 39% (72/187) had ≥1 ECG demonstrating LVH. The severity of right- and left-sided obstructive lesions correlated with the ECG presence of RVH (P < .0001, odds ratio 21.8) and LVH (P < .001, odds ratio 14.5-61), respectively.
  
  
  CONCLUSIONS
  Electrocardiographic intervals in patients with WS follow expected trends seen in normal patients. Voltage criteria for RVH and LVH are commonly met on ECGs of patients with WS. The presence of ventricular hypertrophy on ECG in WS correlates with lesion severity.
authors: Department of Pediatrics, Arkansas Children's Hospital, Little Rock, Ark.
authors: Venusa
authors: V
authors: Phomakay
authors: Department of Pediatrics, Arkansas Children's Hospital, Little Rock, Ark.|Department of Pediatrics, University of Arkansas for Medical Sciences, Little Rock, Ark.
authors: Jeffrey M
authors: JM
authors: Gossett
authors: Department of Pediatrics, Children's Hospital of Philadelphia, Philadelphia, Pa, USA.|Department of Pediatrics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pa, USA.
authors: Paige B
authors: PB
authors: Kaplan
authors: Department of Pediatrics, Arkansas Children's Hospital, Little Rock, Ark.|Department of Pediatrics, University of Arkansas for Medical Sciences, Little Rock, Ark.
authors: Christopher J
authors: CJ
authors: Swearingen
authors: Department of Pediatrics, Arkansas Children's Hospital, Little Rock, Ark.|Department of Pediatrics, University of Arkansas for Medical Sciences, Little Rock, Ark.
authors: R Thomas
authors: RT
authors: Collins
mesh_terms: D000200:Action Potentials; D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D004562:Electrocardiography; D005260:Female; D006329:Heart Conduction System / Q000503:physiopathology*; D006339:Heart Rate; D006776:Hospitals, Pediatric; D006801:Humans; D017379:Hypertrophy, Left Ventricular / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology; D017380:Hypertrophy, Right Ventricular / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology; D007223:Infant; D008297:Male; D008875:Middle Aged; D016017:Odds Ratio; D015143:Philadelphia; D011237:Predictive Value of Tests; D012189:Retrospective Studies; D012307:Risk Factors; D012720:Severity of Illness Index; D014694:Ventricular Outflow Obstruction / Q000175:diagnosis / Q000209:etiology*; D018980:Williams Syndrome / Q000150:complications* / Q000175:diagnosis; D055815:Young Adult
keywords: Electrocardiogram; Ventricular Hypertrophy; Williams Syndrome