Abstract
Abstract Background Although wild-type transthyretin amyloid cardiomyopathy (ATTRwt-CM) was previously considered a rare disease, recent diagnostic imaging modalities have revealed that it is considerably underdiagnosed among elderly patients with heart failure. The severity of CM is thought to be related to the extent of amyloid deposition in heart. 99mTc-labeled pyrophosphate (99mTc-PYP) scintigraphy, cardiovascular magnetic resonance (CMR), global longitudinal strain (GLS) provide diagnostic and prognostic information in ATTRwt-CM. However, the relevance of these imaging modalities and their association with cardiac amyloid load has not been fully evaluated. Purpose The aim of study was to elucidate the associations between pathological amyloid load and cardiac retention evaluated by 99mTc-PYP scintigraphy, CMR, GLS in patients with ATTRwt-CM. Method Cardiac amyloid load was calculated as (amyloid deposition area/ total myocardium area)×100 using endomyocardial biopsy specimen. Cardiac retention was quantified by heart to contralateral (H/CL) ratio by 99mTc-PYP scintigraphy. Native T1 and extracellular volume (ECV) were obtained by CMR. GLS was analyzed using the 2D echo at the time of diagnosis. Result The mean cardiac amyloid load was 23.0±15.2% (n=57) and correlation with H/CL ratio (1.94±0.36 n=57), native T1 (1426.7±52.5 n=57), ECV (57.9±12.9 n=54), GLS (−9.1±2.4 n=57) were positive (r=0.375 p=0.004, r=0.496 r=0.304 p<0.001, r=0.304 p=0.025, r=0.473 p<0.001). Conclusion Increased cardiac amyloid load correlated with an increased 99mTc-PYP positivity, native T1, ECV, and an impaired GLS. These results suggest that imaging parameters may reflect histological and functional changes due to amyloid deposition in the myocardium. Funding Acknowledgement Type of funding sources: None.
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