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Browsing by Author "Joe G N Garcia"

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    Evaluation of myocardial deformation in patients with sickle cell disease and preserved ejection fraction using three-dimensional speckle tracking echocardiography
    (2012-09-01) Homaa Ahmad; Etienne Gayat; Chattanong Yodwut; M. Cristina Abduch; Amit R. Patel; Lynn Weinert; Ankit Desai; Wendy Tsang; Joe G N Garcia; Roberto M. Lang; Victor Mor-Avi; University of Chicago; Hopital Saint-Louis; Instituto do Coracao do Hospital das Clinicas; Mahidol University; University of Illinois at Chicago
    Background: Sickle cell disease (SCD) is a hemoglobinopathy that affects one in 500 African Americans. Although it is well established that patients with SCD have left ventricular (LV) diastolic dysfunction, it is not clear whether they have subtle LV systolic dysfunction despite preserved ejection fraction (EF). We used three-dimensional speckle tracking echocardiography (3DSTE) to assess changes in both systolic and diastolic LV function in SCD. Methods: Transthoracic real time 3D images were obtained (Philips iE33) in 56 subjects, including 28 stable outpatients with SCD (age 33 ± 7 years) and 28 normal controls (age 35 ± 9 years). 3DSTE was performed using prototype software (4DLV Analysis, TomTec) to obtain LV volume and deformation time curves, from which indices of systolic and diastolic LV function were calculated. Results: In SCD patients, 3DSTE-derived LV filling parameters were significantly different from normal controls, reflecting an increase in both rapid and atrial filling volumes and prolonged active relaxation, depicted by a decrease in filling volume fractions at fixed times and an increase in rapid filling duration. Global LV systolic function was not only preserved but increased compared to controls, as reflected by significantly increased global longitudinal strain. Importantly, twist angle and torsion as well as radial and circumferential components of 3D strain were similar in both groups. Conclusions: 3DSTE was able to confirm diastolic dysfunction, as expected in some patients with SCD. However, 3DSTE strain analysis did not reveal any changes in LV systolic function. These findings provide novel insight into the pathophysiology of the cardiovascular complications of SCD. © 2012, Wiley Periodicals, Inc.
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    Mechanistic insights and characterization of sickle cell disease-associated cardiomyopathy
    (2014-01-01) Ankit A. Desai; Amit R. Patel; Homaa Ahmad; John V. Groth; Thejasvi Thiruvoipati; Kristen Turner; Chattanong Yodwut; Peter Czobor; Nicole Artz; Roberto F. MacHado; Joe G N Garcia; Roberto M. Lang; University of Illinois at Chicago; University of Chicago Medical Center; Ochsner Medical Center - New Orleans; Loyola University Medical Center; Mahidol University; Unity Point Health; University of Arizona
    Background-Cardiovascular disease is an important cause of morbidity and mortality in sickle cell disease (SCD). We sought to characterize sickle cell cardiomyopathy using multimodality noninvasive cardiovascular testing and identify potential causative mechanisms. Methods and Results-Stable adults with SCD (n=38) and healthy controls (n=13) prospectively underwent same day multiparametric cardiovascular magnetic resonance (cine, T2* iron, vasodilator frst pass myocardial perfusion, and late gadolinium enhancement imaging), transthoracic echocardiography, and applanation tonometry. Compared with 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/m 2; P<0.01 for all). Patients with SCD also had a 21% lower myocardial perfusion reserve index than control subjects (1.47±0.34 vs 1.87±0.37; P=0.034). A signifcant subset of patients with SCD (25%) had evidence of late gadolinium enhancement, whereas only 1 patient had evidence of myocardial iron overload. Diastolic dysfunction was present in 26% of patients with SCD compared with 8% in controls. Estimated flling pressures (E/e′, 9.3±2.7 vs 7.3±2.0; P=0.0288) were higher in patients with SCD. Left ventricular dilation and the presence of late gadolinium enhancement were inversely correlated to hepatic T2* times (ie, hepatic iron overload because of frequent blood transfusions; P<0.05 for both), whereas diastolic dysfunction and increased flling pressures were correlated to aortic stiffness (augmentation pressure and index, P<0.05 for all). Conclusions-Sickle cell cardiomyopathy is characterized by 4-chamber dilation and in some patients myocardial fbrosis, abnormal perfusion reserve, diastolic dysfunction, and only rarely myocardial iron overload. Left ventricular dilation and myocardial fbrosis are associated with increased blood transfusion requirements, whereas left ventricular diastolic dysfunction is predominantly correlated with increased aortic stiffness. © 2014 American Heart Association, Inc.

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