Mechanistic insights and characterization of cardiomyopathy due to Sickle Cell Disease

✍ Scribed by Amit R Patel; Homaa Ahmad; Ankit A Desai; Thejasvi Thiruvoipati; Kristen M Turner; Lynn Weinert; Chattanong Yodwut; Peter Czobor; Nicole Artz; Sharon Trevino; Victor Mor-Avi; Roberto Machado; Joe G Garcia; Roberto Lang

• Book ID: 104499145
• Publisher: BioMed Central
• Year: 2012
• Tongue: English
• Weight: 107 KB
• Volume: 14
• Category: Article
• ISSN: 1097-6647
• DOI: 10.1186/1532-429x-14-s1-o78

✦ Synopsis

We sought to characterize the features of sickle cell cardiomyopathy and to identify causative mechanisms using comprehensive cardiac magnetic resonance, echocardiography, and arterial tonometry. We found that sickle cell cardiomyopathy is characterized by 4-chamber dilation, myocardial fibrosis, abnormal myocardial perfusion reserve, diastolic dysfunction, and only rarely myocardial iron overload. Left ventricular dilation and myocardial fibrosis are associated with increased blood transfusion requirements; where as, diastolic dysfunction is due to increased aortic stiffness.