How early can myocardial iron overload occur in beta thalassemia major?
Gaohui Yang1, Rongrong Liu1, Peng Peng2
1Department of Hematology, the First Affiliated Hospital of Guangxi Medical University, Nanning, China.
Insights
Myocardial siderosis, a complication of beta thalassemia major, affects young Chinese patients. Early cardiac T2* assessment from age 6 is crucial for detecting iron overload and preventing heart disease.
Area of Science:
- Cardiology
- Hematology
- Pediatrics
Background:
- Myocardial siderosis is a leading cause of mortality in beta thalassemia major (TM).
- This study focuses on cardiac iron overload in a young Chinese TM population.
Purpose of the Study:
- To investigate the occurrence, prevalence, and severity of cardiac iron overload.
- To assess the relationship between iron overload, cardiac function, and patient demographics in beta TM.
Main Methods:
- Analyzed T2* cardiac magnetic resonance (CMR), left ventricular ejection fraction (LVEF), and serum ferritin (SF) in 201 beta TM patients.
- Median patient age was 9 years; average blood transfusion was 13 units/year.
Main Results:
- 82.1% of patients had elevated serum ferritin (>2500 ng/ml).
- Myocardial iron overload was found in 33.8% of patients, with 12.6% having severe overload.
- Iron overload was detected in children as young as 6 years and associated with impaired LVEF and cardiac disease.
Conclusions:
- Chinese beta TM patients in this cohort are younger, less effectively chelated, and have a higher iron burden than previously reported.
- Myocardial siderosis occurs earlier than expected and is linked to reduced LVEF and cardiac issues.
- Recommend initial cardiac T2* assessment by age 6 for poorly chelated TM patients.
Background:
Myocardial siderosis is the most common cause of death in patients with beta thalassemia major(TM). This study aimed at investigating the occurrence, prevalence and severity of cardiac iron overload in a young Chinese population with beta TM.
Methods And Results:
We analyzed T2* cardiac magnetic resonance (CMR), left ventricular ejection fraction (LVEF) and serum ferritin (SF) in 201 beta TM patients. The median age was 9 years old. Patients received an average of 13 units of blood per year. The median SF level was 4536 ng/ml and 165 patients (82.1%) had SF>2500 ng/ml. Myocardial iron overload was detected in 68 patients (33.8%) and severe myocardial iron overload was detected in 26 patients (12.6%). Twenty-two patients ≤10 years old had myocardial iron overload, three of whom were only 6 years old. No myocardial iron overload was detected under the age of 6 years. Median LVEF was 64% (measured by CMR in 175 patients). Five of 6 patients with a LVEF<56% and 8 of 10 patients with cardiac disease had myocardial iron overload.
Conclusions:
The TM patients under follow-up at this regional centre in China patients are younger than other reported cohorts, more poorly-chelated, and have a high burden of iron overload. Myocardial siderosis occurred in patients younger than previously reported, and was strongly associated with impaired LVEF and cardiac disease. For such poorly-chelated TM patients, our data shows that the first assessment of cardiac T2* should be performed as early as 6 years old.
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