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.

The Prevalence of Cardiovascular Manifestations in Pediatric Sickle Cell Anemia Patients in a Large Tertiary Care Hospital in the Western Region of Saudi Arabia

“…A dilated left ventricle is one of the most common causes of increased morbidity and mortality in sickle cell pediatric patients as documented in a systematic review [ 1 ]. In our study, a dilated left ventricle was found in 16.7%; however, studies done in Qatif, Saudi Arabia, and Philadelphia, United States showed 21% and 25%, respectively [ 19 , 20 ]. In Philadelphia, the study included 172 patients with a mean age of 15 years, with the majority (72%) having severe phenotype, as opposed to our study in which the majority (65%) had mild-moderate phenotype.…”

“…Pulmonary hypertension was detected in 1.9% unlike other studies conducted in Qatif, Saudi Arabia, and Philadelphia, United States, which showed no evidence of pulmonary hypertension [19,20]. However, a study done in the United States showed that 57.7% had pulmonary hypertension of which 52 patients were enrolled with a mean age of 16.21 years [23].…”

“…This can be reasoned by the increased volume overload that is caused by chronic anemia resulting in chamber enlargement and increased wall thickness [ 9 ]. In addition, we found that increased systolic blood pressure was observed in 18.5% unlike a study conducted in Qatif, Saudi Arabia, which revealed that there was no evidence of systemic hypertension in 87 patients at a mean age of 7.8 years [ 19 ]. This could be attributed to the demographic variability reported in the population studied compared to the current study.…”