Coronary Artery Dilation in Sickle Cell Disease

ObjectiveTo evaluate the prevalence of coronary artery dilation in children with sickle cell disease (SCD).Study designThis 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.ResultsSeventeen 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.ConclusionCAE is common in SCD and is associated with left ventricular hypertrophy and inflammation.