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[Echocardiographic assessment of patients with sickle cell anemia]
R Blandón1, I M Leandro, M Altafulla
1Servicio de Hematología, Hospital del Niño, Panamá.
Insights
Sickle cell trait (ASHg.) patients show higher cardiomyopathy rates than previously thought. Early detection and treatment of cardiac issues in sickle cell disease (SSHg.) and trait are crucial for better outcomes.
Area of Science:
- Cardiology
- Hematology
- Genetics
Background:
- Sickle cell disease (SSHg.) and sickle cell trait (ASHg.) are inherited blood disorders with potential cardiac complications.
- Previous investigations reported varying incidences of cardiomyopathy in these conditions.
Purpose of the Study:
- To evaluate the incidence and electrocardiographic (ECG) and echocardiographic (echo) characteristics of cardiomyopathy in patients with sickle cell disease (SSHg.) and sickle cell trait (ASHg.).
- To assess the effectiveness of current treatments for cardiac failure and hemosiderosis in these patient groups.
Main Methods:
- Retrospective analysis of 164 patients: 60 with SSHg., 97 with ASHg., and 17 controls.
- Electrocardiography (ECG) and M-Mode/2-D echocardiography (echo) were used for cardiac assessment.
- Patients with cardiac failure received cardiotonics, diuretics, and ACE inhibitors; antioxidant therapy (alpha-tocopherol, ubiquinones) was used for hemosiderosis.
Main Results:
- The incidence of cardiomyopathy in ASHg. was higher than previously reported.
- Significant ECG changes included arrhythmias, conduction blocks, and ischemic modifications.
- Echo revealed similar end-diastolic volumes in AS and SS patients with diagnosed cardiomyopathy.
- Cardiac failure patients showed frequent compensation with standard treatment.
- Antioxidant therapy effectively managed hemosiderosis.
Conclusions:
- Sickle cell trait (ASHg.) presents a greater risk for cardiomyopathy than previously recognized.
- Comprehensive cardiac evaluation, including ECG and echo, is essential for patients with SSHg. and ASHg.
- Effective management strategies exist for cardiac complications and hemosiderosis in these populations.
Abstract:
One hundred and sixty four (164) patients were evaluated. Sixty (60) with Sickle cell disease (SSHg.) and ninety seven (97) with Trait (ASHg.); seventeen (17) were normal control group. The study confirmed that the incidence of cardiomyopathy in Trait (ASHg.) is greater than reported by other clinical investigations. Cardiac arrhythmia, atrial fibrillation, premature ventricular contractions, bundle branch blocks, and T and ST modifications with sub epicardial isquemia were most significant electrocardiographics changes. The possibility of myocardial infarction in SS patients with low or normal hemoglobin is significant. M-Mode and 2-D echo, demonstrated similar end diastolic volumes in AS and SS patients in which cardiomyopathy were diagnosticated. Patients with cardiac failure, treated with cardiotonics, diuretics and ACE were compensated most frequently. To prevent hemosiderosis, antioxydant (alfatocoferol and Ubiquinones) were used with satisfactory response.