Cardiovascular aspect of Beta-thalassaemia

Area of Science:

• Hematology
• Cardiology
• Genetics

Background:

• Beta-thalassaemia major is a genetic blood disorder characterized by reduced beta globin chain synthesis, leading to chronic anemia.
• Consequences include growth retardation, bone marrow expansion, extramedullary hematopoiesis, splenomegaly, increased iron absorption, infections, and hypercoagulability.
• Transfusional iron overload is a major complication, directly damaging cardiac tissue and contributing to mortality.

Purpose of the Study:

• To review the cardiac abnormalities in beta-thalassaemia major patients with iron overload.
• To discuss the mechanisms of iron-induced cardiac toxicity.
• To highlight the role of cardiac magnetic resonance T2* in early detection and management.

Main Methods:

• Literature review of cardiac complications in beta-thalassaemia major.
• Discussion of iron toxicity mechanisms and cellular damage.
• Emphasis on the diagnostic capabilities of cardiac magnetic resonance T2*.

Main Results:

• Cardiac dysfunction is the leading cause of mortality in beta-thalassaemia major.
• Common cardiac abnormalities include left ventricular dysfunction, pulmonary hypertension, valvulopathies, arrhythmias, and pericarditis.
• Iron toxicity causes oxidative stress, lipid peroxidation, organelle damage, cell death, and fibrosis.

Conclusions:

• Cardiac abnormalities are linked to comorbidities like endocrine deficiencies and inflammation.
• Cardiac magnetic resonance T2* enables early detection of cardiac iron overload and dysfunction.
• Timely iron chelation therapy can reverse cardiac damage and improve outcomes.