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
Cleveland Clinic.
United States
Heart
Heart 2025;
DOI: 10.1136/heartjnl-2025-325837
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.
Category
Class V. Pulmonary Hypertension Associated with Hematological, Systemic, Metabolic, Nutritional and Other Disorders
Review Articles Concerning Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease or Adult Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: No