title: Coronary artery dilation in sickle cell disease.
abstract: OBJECTIVE
  To evaluate the prevalence of coronary artery dilation in children with sickle cell disease (SCD).
  
  
  METHODS
  This is a retrospective analysis performed in patients, between 10 and 19 years old, with SCD who underwent a routine transthoracic echocardiographic evaluation over a 20-month period. The left main, left anterior descending, and proximal right coronary artery diameters, as well as clinical and laboratory variables and other echocardiographic results were collected. Echocardiographic measurements were converted to z scores by using information from a large control population of normal children. Coronary artery ectasia (CAE) was defined as a coronary artery diameter z score ≥ 2. The patients with CAE were compared with those without CAE by using univariate and multivariate analyses.
  
  
  RESULTS
  Seventeen of 96 patients with SCD (17.7%) had CAE. There were no differences in sex, age, height, weight, body surface area, or genotype between those with and those without CAE. Patients with CAE had larger left ventricular end-diastolic dimension, shortening fraction, septal thickness, posterior wall thickness, mass, mass-to-volume ratio, and white blood cell count. Multivariate analysis revealed that the mass-to-volume ratio and elevated white blood cell count were associated with CAE.
  
  
  CONCLUSIONS
  CAE is common in SCD and is associated with left ventricular hypertrophy and inflammation.
authors: Department of Pediatrics, Children's Hospital at Montefiore, Bronx, NY, USA. gnichols@montefiore.org
authors: George T
authors: GT
authors: Nicholson
authors: Daphne T
authors: DT
authors: Hsu
authors: Steven D
authors: SD
authors: Colan
authors: Deepa
authors: D
authors: Manwani
authors: William B
authors: WB
authors: Burton
authors: Darlene
authors: D
authors: Fountain
authors: Leo
authors: L
authors: Lopez
mesh_terms: D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000150:complications*; D002648:Child; D003331:Coronary Vessels / Q000473:pathology*; D003971:Diastole; D004108:Dilatation, Pathologic / Q000000981:diagnostic imaging; D004452:Echocardiography; D005260:Female; D006346:Heart Septum / Q000473:pathology; D006801:Humans; D017379:Hypertrophy, Left Ventricular / Q000473:pathology; D007249:Inflammation / Q000473:pathology; D007958:Leukocyte Count; D008297:Male; D015999:Multivariate Analysis; D012189:Retrospective Studies; D013318:Stroke Volume; D055815:Young Adult