Amyloidotic cardiomyopathy is still a widely underdiagnosed condition that usually requires endomyocardial biopsy (EMB) for a definite diagnosis. 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid ( 99mTc-DPD) has proven highly sensitive for detecting amyloidotic cardiomyopathy due to transthyretin-related amyloid deposition. Herein we report the first description of the 99mTc-DPD scintigraphy profile in a patient with suspected amyloidotic cardiomyopathy and a final EMB- and genetically-proven diagnosis of familial apolipoprotein AI amyloidosis due to Leu174Ser variant. Abbreviations: AL: light-chain associated amyloidosis; ApoAI: apolipoprotein AI; ATTR: hereditary transthyretin-related amyloidosis; EMB: endomyocardial biopsy; HCM: hypertrophic cardiomyopathy; HR: heart retention; H/WB: heart/whole-body retention ratio; LV: left ventricular; SPECT: single-photon emission computed tomography; SSA: senile systemic amyloidosis; 99mTc-DPD: 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid; TDI: tissue Doppler imaging; TTR: transthyretin

High 99mTc-DPD myocardial uptake in a patient with apolipoprotein AI-related amyloidotic cardiomyopathy.

QUARTA, CANDIDA CRISTINA;GUIDALOTTI, PIER LUIGI;LONGHI, SIMONE;MERLINI, GIOVANNI;RAPEZZI, CLAUDIO
2013

Abstract

Amyloidotic cardiomyopathy is still a widely underdiagnosed condition that usually requires endomyocardial biopsy (EMB) for a definite diagnosis. 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid ( 99mTc-DPD) has proven highly sensitive for detecting amyloidotic cardiomyopathy due to transthyretin-related amyloid deposition. Herein we report the first description of the 99mTc-DPD scintigraphy profile in a patient with suspected amyloidotic cardiomyopathy and a final EMB- and genetically-proven diagnosis of familial apolipoprotein AI amyloidosis due to Leu174Ser variant. Abbreviations: AL: light-chain associated amyloidosis; ApoAI: apolipoprotein AI; ATTR: hereditary transthyretin-related amyloidosis; EMB: endomyocardial biopsy; HCM: hypertrophic cardiomyopathy; HR: heart retention; H/WB: heart/whole-body retention ratio; LV: left ventricular; SPECT: single-photon emission computed tomography; SSA: senile systemic amyloidosis; 99mTc-DPD: 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid; TDI: tissue Doppler imaging; TTR: transthyretin
Quarta CC;Obici L;Guidalotti PL;Pieroni M;Longhi S;Perlini S;Verga L;Merlini G;Rapezzi C
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/11585/147071
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