Mechanistic insights and characterization of cardiomyopathy due to Sickle Cell Disease
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Mechanistic insights and characterization of cardiomyopathy due to Sickle Cell Disease Amit R Patel1*, Homaa Ahmad1, Ankit A Desai2, Thejasvi Thiruvoipati1, Kristen M Turner1,4, Lynn Weinert1, Chattanong Yodwut3,1, Peter Czobor1, Nicole Artz4, Sharon Trevino2, Victor Mor-Avi1, Roberto Machado2, Joe G Garcia2, Roberto Lang1 From 15th Annual SCMR Scientific Sessions Orlando, FL, USA. 2-5 February 2012 Summary 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.
generated from first pass perfusion images as the stress to rest ratio of mid-ventricular myocardium upslope (normalized by the left ventricular cavity upslope). LGE was considered present if the signal intensity (SI) was >5 standard deviations above normal remote myocardium and if seen in 2 consecutive slices or 2 imaging planes. Myocardial and hepatic T2* times were calculated using the formula ΔTE/ ln(SI TE2/SI TE1). Presence of diastolic dysfunction (DD) was determined using American Society of Echocardiography criteria. Aortic augmentation index was determined using standard tonometry methods.
Background Cardiovascular disease is an important cause of morbidity and mortality in adults with sickle cell disease (SCD). We sought to characterize the features of SCD cardiomyopathy and to identify causative mechanisms.
Results Compared to controls, patients with SCD had severe dilation of the left ventricle (124±27 vs 79±12 ml/m2), right ventricle (127±28 vs 83±14 ml/m2), left atrium (65 ±16 vs 41±9 ml/m2), and right atrium (78±17 vs 56±17 ml/m2), p
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