Evaluation of 107 patients with sickle cell anemia through tissue Doppler and myocardial performance index
Monica C Caldas1, Zilda A Meira, Marcia M Barbosa
1Ecocenter, Belo Horizonte, Brazil. mmccaldas@terra.com.br
Insights
Sickle-cell anemia (SCA) causes early heart abnormalities in children, affecting both systolic and diastolic function. Early detection using Doppler echocardiography is crucial for managing cardiac health in pediatric SCA patients.
Area of Science:
- Pediatric Cardiology
- Hematology
- Cardiovascular Research
Background:
- Sickle-cell anemia (SCA) is linked to increased cardiac output and cardiomegaly.
- Diastolic dysfunction and pulmonary hypertension are poor prognostic indicators in SCA.
- Pediatric SCA patients require advanced cardiac evaluation beyond standard metrics.
Purpose of the Study:
- Assess systolic and diastolic ventricular function in pediatric SCA using load-independent parameters.
- Evaluate cardiac changes related to transfusion or chelation therapy in SCA.
- Identify early cardiac abnormalities in children and adolescents with SCA.
Main Methods:
- Evaluated 107 pediatric patients (3-18 years) with SCA.
- Utilized physical exams, electrocardiography, and Doppler echocardiography.
- Compared SCA patients with 70 healthy controls.
Main Results:
- Myocardial Performance Index (MPI) was significantly higher in both ventricles of SCA patients versus controls.
- E-wave deceleration time was prolonged in SCA patients, indicating diastolic dysfunction.
- Nontransfused SCA patients had larger left ventricular mass than those on chronic transfusion therapy.
Conclusions:
- Early systolic and diastolic cardiac dysfunction is detectable in pediatric SCA patients.
- Abnormalities occur even in early functional classes (I and II).
- Doppler echocardiography provides critical insights into cardiac health in young SCA patients.
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.
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