Significance of Pain in Children with Sickle Cell Anaemia and Ischaemia-induced Cardiac Injury

Aims: To determine the relationships between ischaemic cardiac injury (ICI) evidenced by ischaemic electrocardiogram (ECG) with raised cardiac troponin T (cTnT), pain intensity and frequency in children with sickle cell anaemia (SCA).

Study Design: Case-control.

Place and Duration of Study: Department of Paediatrics, University of Calabar Teaching Hospital over a 6-month period.

Methodology: Children with SCA aged 4 – 17 years with vaso-occlusive painful crises (VOC) were enrolled. Cases were those with ICI while controls were those without ICI. VOC was diagnosed by history and examination with Faces Pain Scale – Revised. Electrocardiography and cTnT estimation were done. Cut-off level (97.5th percentile) of cTnT was obtained from age and sex-matched healthy children with haemoglobin genotype-AA. Serum cTnT analysis was by electrochemiluminescence immunoassay. Ischaemic ECG assessment was according to World Heart Federation criteria.

Results: Fifty-six children with SCA participated among who 27(48.2%) were cases and 29(51.8%) controls. Ischaemic ECG (71.4%) and elevated cTnT (57.2%) were significantly related (P=.01). All cases had severe pain (P=.02) and accounted for >50% of those with chest pain (P=.25). Controls had more < 3 pain episodes per annum than cases while frequent VOC (≥3 pain episodes per annum) occurred more in the cases though the differences were not statistically significant.

Conclusion: Severe pain with frequent VOC is associated with ICI even in the absence of chest pain in children with SCA. Regular electrocardiography and cTnT measurement will identify at-risk children for adequate management.