Role of myocardial deformation imaging in transfusion-dependent thalassemia—Correlation with severity of myocardial siderosis. (March 2023)

• Record Type: Journal Article
• Authors: Rao, Sruti; Hasan, Babar S.; Hoodbhoy, Zahra; Habib, Ibrahim; Mohsin, Shazia; Tomredle, Rebekah; Ashiqali, Salima; Tipoo, Fateh A.; Cartoski, Mark J.; Bijnens, Bart; Chowdhury, Devyani
• Is Part Of: Progress in pediatric cardiology. Volume 68(2023)
• Publication Date: 2023-03
• Subjects: AHA American Heart Association -- AKUH Aga Khan University Hospital -- ch chamber -- CMRI cardiac magnetic resonance imaging -- DT mitral deceleration time -- EF ejection fraction -- EPFR early peak filling rate -- FT-CMRI feature-tracking cardiac magnetic resonance imaging -- GLS global longitudinal strain -- IVCT isovolumic contraction time -- IVRT isovolumic relaxation time -- LMIC low- and middle-income countries -- LV left ventricle/left ventricular -- LVEF left ventricular ejection fraction -- MRI magnetic resonance imaging -- NYHA New York Heart Association -- SSFP steady-state free precession -- STE speckle tracking echocardiography -- TAPSE tricuspid annular plane systolic excursion -- T2* CMRI T2* analysis on cardiac magnetic resonance imaging -- TDT transfusion-dependent thalassemia; Transfusion dependent thalassemia -- Heart failure -- T2* CMRI -- Strain imaging -- FT CMR -- Chelation; Pediatric cardiology -- Periodicals; Cardiovascular Diseases -- Periodicals; Infant; Child; Cardiologie pédiatrique -- Périodiques; 618.9212005
• DOI: 10.1016/j.ppedcard.2022.101607
• Languages: English
• ISSNs: 1058-9813

Abstract

Abstract: Objectives: To study clinical and echocardiographic parameters in Transfusion Dependent Thalassemia (TDT) patients, correlating them with T2*CMRI. To identify features of Speckle Tracking Echocardiography (STE) in those with severe TDT to prioritize patients in need of early T2*CMRI and aggressive chelation. Background: Hemosiderosis from life-long transfusions in TDT is amplified in Low- and Middle-Income Countries (LMIC) when access to resources may be scarce, delaying timely chelation. T2* imaging is the gold standard to non-invasively estimate myocardial iron and guide therapy. In resource constraint areas, we propose use of STE to prioritize those with severe disease to obtain early T2* CMRI. Methods: TDT patients (N = 25) who underwent clinical, echocardiographic, and CMRI evaluation were included, with recruitment of age matched controls. Echocardiographic indices of ventricular function and strain were correlated with T2*. Severe disease was defined as T2* < 10 ms. Differences among patients with severe disease and controls were analyzed in addition to differences between those with normal and abnormal T2*. Results: There was moderate (R −0.60, p = 0.002) inverse correlation between T2* and peak systolic global longitudinal strain (GLS) and regional basal strain (R −0.62, p < 0.001) indicating these parameters were lower in patients with severe disease when compared with controls and non-severe TDT group. GLS and most indices of regional strain were lower in patients with severe disease (T2* < 10 ms) when compared with the control group (p < 0.05). The Apex- base ratio reached significance (p = 0.02) in patients with abnormal T2*(<20 ms) when compared with normal T2*(<20 ms) in TDT positive patients. Conclusions: Early decrease in global and regional strain can serve as a useful tool to identify patients with severe TDT before reduction in ejection fraction. This study makes a case for utilizing STE as an adjunct or initial screen to T2* CMRI in LMIC to prioritize patients in need of T2*CMRI to facilitate aggressive iron chelation protocols. Highlights: Hemosiderosis from life-long transfusions in Transfusion Dependent Thalassemia (TDT) is amplified in Low- and Middle-Income Countries (LMIC) when access to resources maybe scarce, delaying timely chelation. T2* imaging is the gold standard to non-invasively estimate myocardial iron and guide therapy. However, a dedicated CMRI machine with T2* capabilities is limited in LMIC areas in comparison to the global reach of echocardiography Our study found a decrease in global and regional strain despite normal ejection fraction in patients with severe TDT The Apex-base strain ratio was significant in patients with abnormal T2* vs normal T2* in TDT patients In resource constraint areas, we propose use of Speckle Tracking Echocardiography (STE) to prioritize those with severe disease to obtain early T2* CMRI.