Coronary artery dilation and left ventricular hypertrophy do not predict morbidity in children with sickle cell disease

Background: Little is known about the clinical significance of coronary artery dilation (CAD) and left ventricular hypertrophy (LVH) in patients with sickle cell disease (SCD). Procedure: In 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. Results: Multivariable 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 6.1years. 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. Conclusion: LVH and CAD are common findings in children with SCD; however, they are not associated with need for subsequent hospital or intensive care unit admission. Pediatr Blood Cancer 2015;62:115-119.