Right ventricle involvement in cardiac transthyretin amyloidosis in SPECT/CT with [99mTc]Tc-DPD

نویسندگان

چکیده

Abstract Funding Acknowledgements Type of funding sources: Other. Main source(s): Pfizer Research Grant (ID#57165999). Background Transthyretin amyloid (ATTR) cardiomyopathy continues to be considered a rare disease; however, it is now more frequently diagnosed, partly due advances in imaging techniques. Application hybrid technique single-photon emission computed tomography and (SPECT/CT) provides greater anatomic resolution, enabling the assessment regional cardiac involvement. Purpose The aim this prospective study was right ventricle involvement patients with ATTR SPECT/CT [99mTc]Tc 3,3-disphono-1,2-propanodicarboxylic acid (DPD). Methods We present data based on evaluation 100 patients, including 28 (group 1) index 72 2) first-degree relatives who were studied between June 2020 January 2023. Study protocol included clinical data, free light chain blood immunoglobulins urine immunofixation, transthoracic echocardiography (TTE) global longitudinal strain (GLS) analysis, [99mTc]Tc-DPD, genetic testing by an amplicon-based next-generation TTR sequencing approach, selected cases or soft tissue biopsy. Results In group 1 there 39% diagnosed hereditary (ATTRm) 61% wild type (ATTRwt). There detected following types variants - Phe53Leu, Ala101Val, Glu112Lys, Glu109Lys, Glu122Lys, Glu82Lys. Overall, 26% pathogenic variant 7% had positive [99mTc]Tc-DPD. 2 significant predominance Phe53Leu among carriers. Importantly, [99mTc]Tc-DPD 78% presented also uptake. 80% ATTRm 76% ATTRwt left uptake (p>0.05). Conclusions Right transthyretin amyloidosis can assessed tracer as well no statistically differences rates groups Assessment may future translate into further enhancement disease improving prognosis patients.

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ژورنال

عنوان ژورنال: European Journal of Echocardiography

سال: 2023

ISSN: ['2047-2412', '2047-2404']

DOI: https://doi.org/10.1093/ehjci/jead119.420