BACKGROUND: Sickle cell anemia is a formidable problem in India, and is more prevalent in Maharashtra. Cardiovascular involvement in this condition has not been well studied. The present study therefore sought to investigate the systolic and diastolic left ventricular function of children with sickle cell anemia. METHODS AND RESULTS: This prospective controlled study comprised of 25 cases of sickle cell anemia, 25 cases of anemia (hemoglobin <11 gm/dl) with 'AA' types of hemoglobin electrophoresis and 25 non-anemic controls (hemoglobin >11 gm/dl) with normal hemoglobin electrophoresis pattern. M-mode, 2-dimensional and Doppler echcardiographic measurements of patients and controls were performed according to criteria of the American Echocardiography Society. In the study cases, age ranged from 5 years to 15 years with the mean age of 9.91 years. There were 14 males and 11 females in the study cases. Patients with sickle cell anemia had significantly larger left atrial (23.26 +/- 3.6 mm, 22.9 +/- 2.56 mm, 20.72 +/- 2.79 mm; p < 0.05), left ventricular (34.88 +/- 4.53, 33.28 +/- 3.28, 30.72 +/- 3.68: p < 0.05) and aortic root (19 +/- 2.7, 18.91 +/- 2.24, 17.56 +/- 1.44; p < 0.05) dimensions. They also had higher indexed end-diastolic left ventricular volumes (101.84 +/- 22.74 ml/m2 v. 65.05 +/- 10.81 ml/m2; p < 0.001), and higher stroke volume (29.32 +/- 11.32 ml, 27.12 +/- 7.82 ml, 22.4 +/- 6.67 ml; p < 0.05). Left ventricular mass (62.24+/- 18.44 gm, 52.53 +/- 16.23 gm, 50.2 +/- 15.68 gm; p < 0.05) was greater in sickle cell anemia patients than in controls. No statistically significant differences were detected in the Doppler finding of patients with or without anemia. No statistically significant correlation was found between echocardiographic parameters (M-mode and Doppler) and the hemoglobin in the sickle cell patients. CONCLUSIONS: Echocardiography is a useful non-invasive technique to study the changes in cardiac structure and function. In spite of left ventricular volume load and dilation in sickle cell anemic patients, left ventricular contraction was good and systolic function was normal, and there was no correlation between the echocardiographic findings and hemoglobin level.
BACKGROUND:Sickle cell anemia is a formidable problem in India, and is more prevalent in Maharashtra. Cardiovascular involvement in this condition has not been well studied. The present study therefore sought to investigate the systolic and diastolic left ventricular function of children with sickle cell anemia. METHODS AND RESULTS: This prospective controlled study comprised of 25 cases of sickle cell anemia, 25 cases of anemia (hemoglobin <11 gm/dl) with 'AA' types of hemoglobin electrophoresis and 25 non-anemic controls (hemoglobin >11 gm/dl) with normal hemoglobin electrophoresis pattern. M-mode, 2-dimensional and Doppler echcardiographic measurements of patients and controls were performed according to criteria of the American Echocardiography Society. In the study cases, age ranged from 5 years to 15 years with the mean age of 9.91 years. There were 14 males and 11 females in the study cases. Patients with sickle cell anemia had significantly larger left atrial (23.26 +/- 3.6 mm, 22.9 +/- 2.56 mm, 20.72 +/- 2.79 mm; p < 0.05), left ventricular (34.88 +/- 4.53, 33.28 +/- 3.28, 30.72 +/- 3.68: p < 0.05) and aortic root (19 +/- 2.7, 18.91 +/- 2.24, 17.56 +/- 1.44; p < 0.05) dimensions. They also had higher indexed end-diastolic left ventricular volumes (101.84 +/- 22.74 ml/m2 v. 65.05 +/- 10.81 ml/m2; p < 0.001), and higher stroke volume (29.32 +/- 11.32 ml, 27.12 +/- 7.82 ml, 22.4 +/- 6.67 ml; p < 0.05). Left ventricular mass (62.24+/- 18.44 gm, 52.53 +/- 16.23 gm, 50.2 +/- 15.68 gm; p < 0.05) was greater in sickle cell anemiapatients than in controls. No statistically significant differences were detected in the Doppler finding of patients with or without anemia. No statistically significant correlation was found between echocardiographic parameters (M-mode and Doppler) and the hemoglobin in the sickle cell patients. CONCLUSIONS: Echocardiography is a useful non-invasive technique to study the changes in cardiac structure and function. In spite of left ventricular volume load and dilation in sickle cell anemicpatients, left ventricular contraction was good and systolic function was normal, and there was no correlation between the echocardiographic findings and hemoglobin level.