Cardiac manifestations of children with sickle cell disease in Qatif area

Abstract

Background: Limited information is available in literature about cardiac manifestations of children with Arab-Indian haplotype sickle cell disease (SCD). The aim of the current study was cardiovascular assessment of children with SCD. Methodology: It is a Prospective observational and cross-sectional study which was conducted at Qatif Central Hospital, Qatif, Saudi Arabia between December 2012 and June 2015. Asymptomatic children with SCD attending general pediatric and pediatric cardiology clinics were enrolled in this study. Eighty-seven patients were included in the study with mean age 7.8 (2-12) years, mean weight 23.7 (13-67) kg and 55 (63%) were male. Results: On cardiac examination, it was revealed that all patients had normal BP, normal oxygen saturation, and normal auscultation of 1st and 2nd heart sounds wherein 6% patients had tachycardia, 20 (23%) had extensive pulse pressure, and 32 (37%) had soft ejection systolic murmur, 10 (11%) had ECG evidence of left ventricular hypertrophy, and 2 (2%) had PR prolongation. Echocardiography showed that all patients had healthy chambers inflow, outflow, wall thickness, and function. No evidence of pulmonary hypertension and right atrial or ventricular enlargement was found. Among the participants 6 (7%) patients had left atrial enlargement, 18 (21%) had left ventricular enlargement, and 6 (7%) had simple congenital heart disease. Conclusion: Children with SCD originally from Qatif area had variable cardiac abnormalities. However, the prevalence of these was less frequent than that mentioned in literature with no distinct sickle cell cardiomyopathy. Although this is reassuring information to families, routine cardiac follow up is valuable.