• Mark C Johnson, Micean J Johnikin, Joshua C Euteneuer, Michael R DeBaun, and Charles Hildebolt.
• Division of Pediatric Cardiology, Department of Pediatrics, Washington University School of Medicine/St. Louis Children's Hospital, St. Louis, Missouri.
• Pediatr Blood Cancer. 2015 Jan 1; 62 (1): 115-9.

BackgroundLittle is known about the clinical significance of coronary artery dilation (CAD) and left ventricular hypertrophy (LVH) in patients with sickle cell disease (SCD).ProcedureIn a retrospective cohort, we studied the prevalence of CAD and LVH in 101 children with SCD in comparison to 93 healthy African-American patients without SCD. Hospital days, number of admissions, and intensive care unit admission after the echocardiogram were assessed as measures of morbidity.ResultsMultivariable analysis of echocardiographic measures of LVH and CAD did not predict subsequent intensive care unit admission, hospital days/year or number of hospital admissions/year during a median follow-up time of 6.1 years. LVH as measured by left ventricular mass index was present in 46% of children with SCD and was inversely related to age (P = 0.0004). Height-indexed dimensions in children with SCD demonstrated that the prevalence of dilation was 49% for the left main coronary artery (LMCA), 29% for the left anterior descending (LAD), and 6% for the right coronary artery (RCA). LMCA dilation was related to relative wall thickness (P = 0.006), inversely to age (P < 0.0006) and weakly to disease severity as determined by hemoglobin (P = 0.03). CAD and LVH were not related to a clinical history of vaso-occlusive pain episode, acute chest syndrome, or cerebrovascular accident.ConclusionLVH and CAD are common findings in children with SCD; however, they are not associated with need for subsequent hospital or intensive care unit admission.© 2014 Wiley Periodicals, Inc.

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