Cardiovascular Complications in Hemoglobinopathies: Pathophysiology, Clinical Spectrum, and Management

Area of Science:

• Hematology
• Cardiology
• Genetics

Background:

• Sickle cell disease and thalassemia are multisystem disorders with increasing cardiovascular (CV) morbidity and mortality.
• Improved treatments have increased survival, revealing a wider spectrum of CV manifestations.

Purpose of the Study:

• To review the epidemiology, pathophysiology, and clinical manifestations of CV complications in hemoglobinopathies.
• To summarize diagnostic advancements, therapeutic strategies, and identify research priorities for improving CV outcomes.

Main Methods:

• A narrative review of studies from PubMed, Cochrane Library, and Google Scholar (2009-2025).
• Searched for original studies, systematic reviews, and guidelines on CV complications in sickle cell disease and thalassemia.

Main Results:

• CV complications include cardiomyopathy, heart failure, pulmonary hypertension, arrhythmias, and ischemia.
• Pathogenesis involves chronic anemia, iron overload, hemolysis, endothelial dysfunction, and nitric oxide deficiency.
• Disease-specific CV phenotypes necessitate tailored diagnostic and therapeutic approaches.

Conclusions:

• A multidisciplinary, proactive CV strategy is crucial for improving survival and quality of life in hemoglobinopathies.
• Addressing evidence gaps and research priorities will enhance CV care and outcomes.