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. 2023 Apr;16(4):464-477.
doi: 10.1016/j.jcmg.2022.07.008. Epub 2022 Sep 14.

Multi-Imaging Characterization of Cardiac Phenotype in Different Types of Amyloidosis

Adam Ioannou  1 , Rishi K Patel  1 , Yousuf Razvi  1 , Aldostefano Porcari  1 , Daniel Knight  1 , Ana Martinez-Naharro  1 , Tushar Kotecha  1 , Lucia Venneri  1 , Liza Chacko  1 , James Brown  1 , Charlotte Manisty  2 , James Moon  2 , Brendan Wisniowski  1 , Helen Lachmann  1 , Ashutosh Wechelakar  1 , Carol Whelan  1 , Peter Kellman  3 , Philip N Hawkins  1 , Julian D Gillmore  1 , Marianna Fontana  4
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Free article

Multi-Imaging Characterization of Cardiac Phenotype in Different Types of Amyloidosis

Adam Ioannou et al. JACC Cardiovasc Imaging. 2023 Apr.
Free article

Abstract

Background: Bone scintigraphy is extremely valuable when assessing patients with suspected cardiac amyloidosis (CA), but the clinical significance and associated phenotype of different degrees of cardiac uptake across different types is yet to be defined.

Objectives: This study sought to define the phenotypes of patients with varying degrees of cardiac uptake on bone scintigraphy, across multiple types of systemic amyloidosis, using extensive characterization comprising biomarkers as well as echocardiographic and cardiac magnetic resonance (CMR) imaging.

Methods: A total of 296 patients (117 with immunoglobulin light-chain amyloidosis [AL], 165 with transthyretin amyloidosis [ATTR], 7 with apolipoprotein AI amyloidosis [AApoAI], and 7 with apolipoprotein AIV amyloidosis [AApoAIV]) underwent deep characterization of their cardiac phenotype.

Results: AL patients with grade 0 myocardial radiotracer uptake spanned the spectrum of CMR findings from no CA to characteristic CA, whereas AL patients with grades 1 to 3 always produced characteristic CMR features. In ATTR, the CA burden strongly correlated with myocardial tracer uptake, except in Ser77Tyr. AApoAI presented with grade 0 or 1 and disproportionate right-sided involvement. AApoAIV always presented with grade 0 and characteristic CA. AL grade 1 patients (n = 48; 100%) had characteristic CA, whereas only ATTR grade 1 patients with Ser77Tyr had characteristic CA on CMR (n = 5; 11.4%). After exclusion of Ser77Tyr, AApoAI, and AApoAIV, CMR showing characteristic CA or an extracellular volume of >0.40 in patients with grade 0 to 1 cardiac uptake had a sensitivity and specificity of 100% for AL.

Conclusions: There is a wide variation in cardiac phenotype between different amyloidosis types across different degrees of cardiac uptake. The combination of CMR and bone scintigraphy can help to define the diagnostic differentials and the clinical phenotype in each individual patient.

Keywords: bone scintigraphy; cardiac amyloidosis; cardiac magnetic resonance.

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Conflict of interest statement

Funding Support and Author Disclosures Dr Fontana is supported by a British Heart Foundation Intermediate Clinical Research Fellowship (FS/18/21/33447). All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.

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