Elio Haroun, Ankit Agrawal, Aro Daniela Arockiam, Joseph El Dahdah, Joseph Kassab, Michael Nakhla, Michel Chedid El Helou, Harsha Sanaka, Ziad Zalaquett, Simrat Kaur, Tiffany Dong, Rabi Hanna, Brian Griffin, Tom Kai Ming Wang
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Cardiovascular disease in Sickle cell: mechanisms, diagnostics and therapeutic advances.
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.
期刊介绍:
Heart is an international peer reviewed journal that keeps cardiologists up to date with important research advances in cardiovascular disease. New scientific developments are highlighted in editorials and put in context with concise review articles. There is one free Editor’s Choice article in each issue, with open access options available to authors for all articles. Education in Heart articles provide a comprehensive, continuously updated, cardiology curriculum.