Abstract

Prolonged QTc interval, a risk factor for ventricular arrhythmia, occurs in sickle cell anemia. The aim of this study was to determine the risk of prolonged QTc interval and its relationship with vaso-occlusive painful crises (VOCs) and follow-up steady-state in the same children with sickle cell anemia. This prospective cohort study enrolled 38 subjects, aged 5–17 years. History of bone pain and examination were obtained during VOC and steady-state. Assessment of QTc interval was with 12-lead electrocardiography. The QTc interval value >0.440 seconds was taken as prolonged. Median (interquartile range) of QTc interval was higher during VOC [0.447 (0.438–0.459) seconds] than during steady-state [0.435 (0.417–0.440) seconds]. Risk of prolonged QTc interval was higher during VOC (68.4%) than in steady-state (21.1%) with relative risk of 3.250 [95% confidence interval (CI) = 1.692–6.241]. Prolonged QTc interval was likely to occur [area under curve (AUC) = 0.759, p<0.001] during VOC with 68.4% sensitivity, 78.9% specificity and at cutoff point of 0.441 seconds. Prolonged QTc interval negatively correlated with packed cell volume (PCV) during VOC [rs (36) = −0.14, p = 0.387]. Binary logistics of the combined effect of PCV and gender on QTc interval showed that during VOC, males were more likely to have prolonged QTc [odds ratio (OR): 1.337 (95% CI: 0.327–5.464; p = 0.686]. Children with sickle cell anemia, particularly males, were three-times more likely to have prolonged QTc interval during VOC when QTc interval was >0.441 seconds. Routine electrocardiography may help to identify those with QTc intervals above this threshold for prompt cardiac-oriented management.

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