Cardiac Remodeling Patterns in Pediatric and Adolescent Patients with Sickle Cell Disease and Their Association with the Genotype and Clinical Severity of the Disease: A Systematic Review

Abstract

Background: Sickle cell disease (SCD) is the most common inherited blood disorder globally, affecting approximately 300,000 newborns annually. Cardiac remodeling, resulting from chronic anemia, vascular obstruction, and endothelial dysfunction, substantially contributes to morbidity and mortality in patients with SCD. Therefore, characterizing these patterns is essential to clinical management and outcome improvement in pediatric patients. However, a comprehensive synthesis of cardiac remodeling patterns in pediatric and adolescent patients with SCD and their associations with genotype and clinical severity is lacking. Methods: We conducted a systematic review in accordance with the PRISMA 2020 guidelines and searched five databases for studies published from January 1978 to December 2024.