Cardiovascular disease in Sickle cell: mechanisms, diagnostics and therapeutic advances.

Abstract

Cardiovascular complications are increasingly recognised as a major driver of morbidity and early mortality in patients with sickle cell disease (SCD), yet they remain underdiagnosed and underappreciated. This contemporary review synthesises current knowledge across a spectrum of cardiovascular manifestations-including myocardial dysfunction, pulmonary hypertension, cardiac iron overload, arrhythmias, myocardial infarction, stroke and sudden death-with emphasis on their unique pathophysiological mechanisms in SCD. We highlight emerging diagnostic tools such as cardiac magnetic resonance with T2* mapping and extracellular volume sequences, speckle-tracking echocardiography and invasive exercise testing, which can revealing a distinct phenotype combining restrictive cardiomyopathy and high-output heart failure. Practical algorithms for risk stratification and disease monitoring are presented alongside evidence-based and SCD-specific management approaches, including the role of hydroxyurea, transfusions, anticoagulation and gene therapy. By integrating multimodality imaging, updated guideline recommendations and recent clinical insights, this review provides a comprehensive resource to support early recognition, personalised therapy and improved cardiovascular outcomes in SCD.