Akademik Veri Yönetim Sistemi
European Journal of Haematology, 2026 (SCI-Expanded, Scopus)
Sickle cell disease and thalassemia syndromes are increasingly recognized as chronic multisystem disorders, with cardiovascular (CV) sequelae representing a significant proportion of long-term morbidity and mortality. Survival has improved considerably due to better transfusion practice, the administration of iron chelation, and disease-modifying treatments; a broad and expanding phenotypic spectrum of cardiac and vascular manifestations is being recognized. These include cardiomyopathy, heart failure, pulmonary hypertension, arrhythmias, microvascular ischemia, and functional valvular disease, often resulting insidiously and progressing over time. CV pathogenesis of hemoglobinopathies is complex and heterogeneous. Chronic anemia induces a long-lasting high-output circulatory state, while iron overload related to transfusion produces myocardial siderosis, oxidative damage, and electrical instability in particular in transfusion-dependent thalassemia. Moreover, hemolysis-mediated endothelial dysfunction, deficiency of nitric oxide, inflammation, and microvascular pathology play a central role in sickle cell disease, resulting in diastolic dysfunction, pulmonary vascular remodeling, and heart failure with preserved ejection fraction. These pathomechanisms create disease-specific CV phenotypes that have important diagnostic and therapeutic implications. This narrative review was performed by searching the PubMed, Cochrane Library, and Google Scholar databases for original studies, systematic reviews, and guidelines for the period of 2009–2025. This review summarizes available data on the epidemiology and pathophysiological mechanisms contributing to CV complications of hemoglobinopathies and clinical manifestations of CV compromise. The review summarizes developments in diagnosis and screening methods such as echocardiography, cardiac magnetic resonance imaging, biomarkers, and functional measures, disease-modifying drugs, and CV lovastatin by phenotype. We finally highlight major evidence gaps and research priorities needed to improve CV outcomes. This multidisciplinary approach is essential for transforming mechanistic insight into improved survival and quality of life for individuals with hemoglobinopathies through a systematic, interdisciplinary, and proactive CV strategy.