Background: Beta thalassemia major (BTM), an inherited hemoglobinopathy, is characterized by absent or severely reduced β-globin chain synthesis, resulting in transfusion-dependent anemia that begins early in life. Cardiac complications, primarily iron-overload cardiomyopathy and congestive heart failure, remain the most common cause of mortality in these patients despite several decades of advances in supportive care.
Objective: To investigate the cardiac function in Iraqi children with BTM using ECG and Echocardiography, to determine whether abnormal findings of these tests are related to inadequate chelation therapy (assessed by urine drug levels) and body iron overload (estimated by serum ferritin), and to assess the correlation between CXR parameters versus echocardiographic measurements.
Methods: A cross-sectional study of 49 patients with beta-thalassemia major (BTM) disorder followed up at the outpatient clinic of AL-Zahraa Teaching Hospital, Al-Najaf, Iraq, from June to November 2025, comprising 29 males and 20 females aged between 10 months and 17 years.) ECG, transthoracic echocardiography, and CXR were performed in all the patients. We defined chelation adequacy as deferoxamine use≥ 4 days/week (optimal) or <4 days/week (suboptimal). Statistical analysis: Chi-square and Student's t-tests were applied using SPSS v20 (Significance level P < 0.05).
Results: Among the 42 chelated patients, ECG abnormalities were paradoxically more frequent in those receiving optimal chelation (62.5%) than in those on suboptimal regimens (30.8%; P = 0.04). Cardiomegaly on CXR followed a similar pattern (25% vs. 7.7%, P > 0.05), as did pericardial thickening on echocardiography (50% vs. 42.3%). In contrast, serum ferritin level showed a clear dose-response relationship with cardiac burden: patients with ferritin ≥1000 ng/mL had significantly higher rates of cardiomegaly (29.4% vs. 3.1%, P < 0.05) and ECG abnormalities (70.6% vs. 18.8%, P < 0.001) than those with lower ferritin. Six patients (12%) had clinically overt congestive heart failure.
Conclusions: Cardiac involvement is substantial in this Iraqi BTM cohort, and serum ferritin is a strong correlate of cardiac abnormalities. The paradoxically higher prevalence of ECG changes and cardiomegaly among optimally chelated patients most likely reflects confounding by indication — cardiac abnormalities prompting intensification of an already-interrupted regimen — rather than any direct effect of treatment. These findings underscore the need for early, uninterrupted chelation and regular cardiac surveillance in this population.