Left Ventricular Diastolic Dysfunction in β-Thalassemia Major with Heart Failure

Area of Science:

• Cardiology
• Hematology
• Medical Imaging

Background:

• Beta-thalassemia major (β-TM) is a severe inherited blood disorder.
• Heart failure (HF) is a significant complication in adult β-TM patients.
• Cardiac iron overload is a known risk factor for HF in β-TM.

Purpose of the Study:

• To investigate the clinical, electrocardiographic, echocardiographic, and cardiac magnetic resonance (CMR) characteristics of adult β-TM patients with HF.
• To identify the predominant patterns of cardiac dysfunction and iron overload in this population.

Main Methods:

• Retrospective study of 16 adult β-TM patients with HF.
• Data collection included clinical assessment, electrocardiography, echocardiography, Doppler, and T2* CMR.
• Analysis focused on left and right ventricular function and T2* CMR values for iron overload assessment.

Main Results:

• Most patients (15/16) had HF with preserved systolic function of the left ventricle (HFpEF); only one had HF with reduced systolic function (HFrEF).
• Right ventricular systolic dysfunction was prevalent (13/16 patients).
• Only 30% of patients had T2* CMR values indicating intermediate risk of left ventricular dysfunction due to iron overload; 70% had normal values. Typical electrocardiographic abnormalities were observed in 69% of patients.

Conclusions:

• In adult β-TM patients with HF, HFpEF and right ventricular systolic dysfunction are the predominant forms.
• Cardiac iron overload, assessed by T2* CMR, was not a major issue in this cohort.
• Electrocardiographic abnormalities are common in this patient group.