Tc-99m labelled bone scintigraphy in suspected cardiac amyloidosis

被引：52

作者：

Rauf, Muhammad Umaid ^{[1
]}

Hawkins, Philip N. ^{[1
]}

Cappelli, Francesco ^{[2
]}

Perfetto, Federico ^{[2
]}

Zampieri, Mattia ^{[2
]}

Argiro, Alessia ^{[2
]}

Petrie, Aviva ^{[3
]}

Law, Steven ^{[1
]}

Porcari, Aldostefano ^{[1
,4
]}

Razvi, Yousuf ^{[1
]}

Bomsztyk, Joshua ^{[1
]}

Ravichandran, Sriram ^{[1
]}

Ioannou, Adam ^{[1
]}

Patel, Rishi ^{[1
]}

Starr, Neasa ^{[1
]}

Hutt, David F. ^{[1
]}

Mahmood, Shameem ^{[1
]}

Wisniowski, Brendan ^{[1
]}

Martinez-Naharro, Ana ^{[1
]}

Venneri, Lucia ^{[1
]}

Whelan, Carol ^{[1
]}

Roczenio, Dorota ^{[1
]}

Gilbertson, Janet ^{[1
]}

Lachmann, Helen J. ^{[1
]}

Wechalekar, Ashutosh D. ^{[1
]}

Rapezzi, Claudio ^{[5
,6
]}

Serenelli, Matteo ^{[7
]}

Massa, Paolo ^{[8
,9
]}

Caponetti, Angelo Giuseppe ^{[8
,9
]}

Ponziani, Alberto ^{[8
,9
]}

Accietto, Antonella ^{[8
,9
]}

Giovannetti, Alessandro ^{[8
,9
]}

Saturi, Giulia ^{[8
,9
]}

Sguazzotti, Maurizio ^{[8
,9
]}

Gagliardi, Christian ^{[8
,10
]}

Biagini, Elena ^{[8
,10
]}

Longhi, Simone ^{[8
,10
]}

Fontana, Marianna ^{[1
]}

Gillmore, Julian D. ^{[1
]}

机构：

[1] UCL, Natl Amyloidosis Ctr, Royal Free Campus, Rowland Hill St, London NW3 2PF, England

[2] Careggi Univ Hosp, Tuscan Amyloid Referral Ctr, Florence, Italy

[3] Univ Coll London UCL, Eastman Dent Inst, London, England

[4] Univ Trieste, Ctr Diag & Treatment Cardiomyopathies, Dept Cardiovasc, Azienda Sanit Univ Giuliano Isontina ASUGI, Trieste, Italy

[5] Univ Ferrara, Cardiol Ctr, Ferrara, Italy

[6] Maria Cecilia Hosp, GVM Care & Res, Ravenna, Italy

[7] Azienda Osped Univ Ferrara, Cardiol Ctr, Ferrara, Italy

[8] IRCCS Azienda Osped Univ, Dept Cardiac Thorac & Vasc, Cardiol Unit, Bologna, Italy

[9] Univ Bologna, Dept Expt Diagnost & Specialty Med DIMES, Bologna, Italy

[10] European Reference Network Rare Complex Dis Heart, Quebec City, PQ, Canada

来源：

EUROPEAN HEART JOURNAL | 2023年 / 44卷 / 24期

关键词：

Amyloid; Amyloidosis; ATTR cardiomyopathy; eGFR; Radionuclide scintigraphy; CARDIOVASCULAR MAGNETIC-RESONANCE; SERUM; DIAGNOSIS; DISEASE;

D O I：

10.1093/eurheartj/ehad139

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Aims To perform evaluation of widely embraced bone scintigraphy-based non-biopsy diagnostic criteria (NBDC) for ATTR amyloid cardiomyopathy (ATTR-CM) in clinical practice, and to refine serum free light chain (sFLC) ratio cut-offs that reliably exclude monoclonal gammopathy (MG) in chronic kidney disease. Methods and results A multi-national retrospective study of 3354 patients with suspected or histologically proven cardiac amyloidosis (CA) referred to specialist centres from 2015 to 2021; evaluations included radionuclide bone scintigraphy, serum and urine immunofixation, sFLC assay, eGFR measurement and echocardiography. Seventy-nine percent (1636/2080) of patients with Perugini grade 2 or 3 radionuclide scans fulfilled NBDC for ATTR-CM through absence of a serum or urine monoclonal protein on immunofixation together with a sFLC ratio falling within revised cut-offs incorporating eGFR; 403 of these patients had amyloid on biopsy, all of which were ATTR type, and their survival was comparable to non-biopsied ATTR-CM patients (p = 0.10). Grade 0 radionuclide scans were present in 1091 patients, of whom 284 (26%) had CA, confirmed as AL type (AL-CA) in 276 (97%) and as ATTR-CM in only one case with an extremely rare TTR variant. Among 183 patients with grade 1 radionuclide scans, 122 had MG of whom 106 (87%) had AL-CA; 60/61 (98%) without MG had ATTR-CM. Conclusion The NBDC for ATTR-CM are highly specific [97% (95% CI 0.91-0.99)] in clinical setting, and diagnostic performance was further refined here using new cut-offs for sFLC ratio in patients with CKD. A grade 0 radionuclide scan all but excludes ATTR-CM but occurs in most patients with AL-CA. Grade 1 scans in patients with CA and no MG are strongly suggestive of early ATTR-type, but require urgent histologic corroboration.

引用

页码：2187 / 2198

页数：12

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