Xiao Li1, Jian Li2, Lu Lin1, Kaini Shen2, Zhuang Tian3, Jian Sun4, Congli Zhang2, Jing An5, Zhengyu Jin1, Rozemarijn Vliegenthart6, Joseph B Selvanayagam7, Yining Wang1. 1. Department of Radiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China. 2. Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China. 3. Department of Cardiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China. 4. Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China. 5. Siemens Shenzhen Magnetic Resonance Ltd., Siemens MRI Center, Hi-Tech Industrial Park, Shenzhen, China. 6. Department of Radiology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands. 7. Department of Cardiovascular Medicine, Flinders University, Flinders Medical Centre, Adelaide, SA, Australia.
Abstract
BACKGROUND: Previous cardiac magnetic resonance (CMR) studies have shown that both late gadolinium enhancement (LGE) and left ventricular (LV) strain have prognostic value in amyloid light-chain (AL) amyloidosis, but the right ventricular (RV) strain has not yet been studied. We aim to determine the incremental prognostic value of LV and RV LGE and strain in AL amyloidosis. METHODS: This prospective study recruited 87 patients (age, 56.9±9.1 years; M/F, 56/31) and 20 healthy subjects (age, 52.7±8.1 years; M/F, 11/9) who underwent CMR. The LV LGE was classified into no, patchy and global groups. The RV LGE was classified into negative and positive groups. Myocardial deformation was measured using a dedicated software. Follow-up was performed for all-cause mortality using Cox proportional hazards regression and Kaplan-Meier curves. RESULTS: During a median follow-up of 21 months, 34 deaths occurred. Presence of LV LGE [HR 2.44, 95% confidence interval (CI), 1.10-5.45, P=0.029] and global longitudinal strain (GLS) (HR 1.13 per 1% absolute decrease, 95% CI, 1.02-1.25, P=0.025) were independent LV predictors. RV LGE (HR 4.07, 95% CI, 1.09-15.24, P=0.037) and GLS (HR 1.10 per 1% absolute decrease, 95% CI, 1.00-1.21, P=0.047) were independent RV predictors. Complementary to LV LGE, LV GLS impairment or RV LGE further reduced survival (both log rank P<0.001). CONCLUSIONS: This study confirms the incremental prognostic value of LV GLS and RV LGE in AL amyloidosis, which refines the conventional risk evaluation based on LV LGE. GLS based on non-contrast-enhanced CMR are promising new predictors. 2020 Cardiovascular Diagnosis and Therapy. All rights reserved.
BACKGROUND: Previous cardiac magnetic resonance (CMR) studies have shown that both late gadolinium enhancement (LGE) and left ventricular (LV) strain have prognostic value in amyloid light-chain (AL) amyloidosis, but the right ventricular (RV) strain has not yet been studied. We aim to determine the incremental prognostic value of LV and RV LGE and strain in AL amyloidosis. METHODS: This prospective study recruited 87 patients (age, 56.9±9.1 years; M/F, 56/31) and 20 healthy subjects (age, 52.7±8.1 years; M/F, 11/9) who underwent CMR. The LV LGE was classified into no, patchy and global groups. The RV LGE was classified into negative and positive groups. Myocardial deformation was measured using a dedicated software. Follow-up was performed for all-cause mortality using Cox proportional hazards regression and Kaplan-Meier curves. RESULTS: During a median follow-up of 21 months, 34 deaths occurred. Presence of LV LGE [HR 2.44, 95% confidence interval (CI), 1.10-5.45, P=0.029] and global longitudinal strain (GLS) (HR 1.13 per 1% absolute decrease, 95% CI, 1.02-1.25, P=0.025) were independent LV predictors. RV LGE (HR 4.07, 95% CI, 1.09-15.24, P=0.037) and GLS (HR 1.10 per 1% absolute decrease, 95% CI, 1.00-1.21, P=0.047) were independent RV predictors. Complementary to LV LGE, LV GLS impairment or RV LGE further reduced survival (both log rank P<0.001). CONCLUSIONS: This study confirms the incremental prognostic value of LV GLS and RV LGE in AL amyloidosis, which refines the conventional risk evaluation based on LV LGE. GLS based on non-contrast-enhanced CMR are promising new predictors. 2020 Cardiovascular Diagnosis and Therapy. All rights reserved.
Entities:
Keywords:
Amyloidosis; cardiomyopathies; magnetic resonance imaging
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