Left ventricular function and cardiac valvar annular dimensions among children with sickle cell anemia compared to those with hemoglobin AA type in Enugu, Nigeria.

Background: Enumerating the relationship between cardiac structures, function and chamber sizes in children with sickle cell anemia would help in delineating some cardiovascular abnormalities which will aid the Pediatric cardiologist and the cardiac surgeons in a number of decision-making situations. Objectives: The objectives of this study are to assess the dimension of cardiac structures and left ventricular function in children with sickle cell anemia in steady state and controls using echocardiography. Methods: A cross-sectional prospective study that assessed cardiac structures and left ventricular function among fifty-one children with sickle cell anemia (HBSS) and compared with fifty children with HB AA type serving as controls. Results: A significant high proportion of children with sickle cell anemia had abnormal Valvar dimension and left ventricular function above two standard deviations (2-SD) from the mean of the standard population compared to the control group, showing a statistically significant difference (χ2 = 10.42, p= 0.001).All the mean annular valves diameter, left ventricular internal dimension in systole and diastole, inter-sinus distance diameter and sinu-tubular junction diameter are higher in children with sickle cell anemia than controls and this is statistically significant. (p<0.005). Conclusion: This result shows that children with sickle cell anemia have increased valvar size diameter compared with those with normal hemoglobin type. A significantly higher proportion of respondents in type SS group had abnormal left ventricular systolic and diastolic dysfunction when compared with those in type AA group.