Evaluation of 107 Patients With Sickle Cell Anemia Through Tissue Doppler and Myocardial Performance Index

Background Sickle-cell anemia (SCA) is associated with elevated cardiac output and cardiomegaly, as demonstrated in many studies. Recently, diastolic dysfunction and pulmonary hypertension have been shown to have poor prognoses in patients with SCA. New indexes of systolic and diastolic evaluation should be used to give more information on both the adult and the pediatric populations. The objectives of this study were to determine the systolic and diastolic function of both ventricles using load-independent parameters and to evaluate cardiac findings due to transfusion or chelation therapy in patients with SCA. Methods We evaluated 107 patients with SCA, aged 3 to 18 years (mean age, 10.1 ± 4.7 years). Physical exams, electrocardiography, and Doppler echocardiography were performed in all patients. These patients were compared with 70 normal children and adolescents. Results Combined systolic and diastolic function of both ventricles was evaluated by the myocardial performance index (MPI). MPI values were higher both in the left ventricles ( P = .00) and in the right ventricles ( P = .00) of patients with SCA compared with controls. Diastolic function was evaluated by diastolic mitral valve flow and by tissue Doppler echocardiography. E-wave deceleration time was higher in patients than in controls. Patients were divided into 2 groups: group 1 consisted of 95 nontransfused patients and group 2 of 12 patients on chronic transfusion therapy. Group 1 patients demonstrated significantly larger left ventricular mass than those in group 2. Systolic and diastolic function did not differ between patients when an 8 g/dL cutoff value of hemoglobin was used. Conclusion This Doppler echocardiographic study showed that early abnormalities in systolic and diastolic function can occur at an early age in SCA, even among children and adolescents in New York Heart Association functional classes I and II.