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Comparative Study
. 2013 Mar 1;6(2):195-201.
doi: 10.1161/CIRCIMAGING.112.000132. Epub 2013 Feb 11.

(99m)Tc-pyrophosphate scintigraphy for differentiating light-chain cardiac amyloidosis from the transthyretin-related familial and senile cardiac amyloidoses

Sabahat Bokhari  1 , Adam Castaño, Ted Pozniakoff, Susan Deslisle, Farhana Latif, Mathew S Maurer
Affiliations
Comparative Study

(99m)Tc-pyrophosphate scintigraphy for differentiating light-chain cardiac amyloidosis from the transthyretin-related familial and senile cardiac amyloidoses

Sabahat Bokhari et al. Circ Cardiovasc Imaging. .

Abstract

Background: Differentiating immunoglobulin light-chain (AL) from transthyretin-related cardiac amyloidoses (ATTR) is imperative given implications for prognosis, therapy, and genetic counseling. We validated the discriminatory ability of (99m)Tc-pyrophosphate ((99m)Tc-PYP) scintigraphy in AL versus ATTR.

Methods and results: Forty-five subjects (12 AL, 16 ATTR wild type, and 17 ATTR mutants) underwent (99m)Tc-PYP planar and single-photon positive emission computed tomography cardiac imaging. Scans were performed by experienced nuclear cardiologists blinded to the subjects' cohort assignment. Cardiac retention was assessed with both a semiquantitative visual score (range, 0; no uptake to 3, diffuse uptake) and by quantitative analysis by drawing a region of interest over the heart corrected for contralateral counts and calculating a heart-to-contralateral ratio. Subjects with ATTR cardiac amyloid had a significantly higher semiquantitative cardiac visual score than the AL cohort (2.9±0.06 versus 0.8±0.27; P<0.0001) as well as a higher quantitative score (1.80±0.04 versus 1.21±0.04; P<0.0001). Using a heart-to-contralateral ratio >1.5 consistent with intensely diffuse myocardial tracer retention had a 97% sensitivity and 100% specificity with area under the curve 0.992, P<0.0001 for identifying ATTR cardiac amyloidosis.

Conclusions: (99m)Tc-PYP cardiac imaging distinguishes AL from ATTR cardiac amyloidosis and may be a simple, widely available method for identifying subjects with ATTR cardiac amyloidosis, which should be studied in a larger prospective manner.

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Figures

Figure 1
Figure 1. (A–B). Semi-quantitative method of calculating the distribution of 99mTc-PYP uptake
Raw images of a representative negative (A) and positive subject (B) are shown 1 hour after radiotracer infusion. ROI circles are depicted in red and the contralateral comparison circle is depicted in blue. ROI = region of interest; C/L = contralateral; Cts = counts; Std Dev = standard deviation.
Figure 2
Figure 2. Mean heart to contralateral ratio according to amyloid subtype
Comparison of 99mTc-PYP mean H/CL ratio between patients with AL, ATTRwt, and ATTRm cardiac amyloidosis. AL and transthyretin-related amyloidoses are differentiated by mean H/CL ratio of 1.5. The outlier with H/CL 1.3 is an ATTRm patient with the unusual Thr59Lys mutation. AL = amyloid light-chain; ATTRwt = wild-type transthyretin amyloidosis; ATTRm = mutant transthyretin amyloidosis.
See this image and copyright information in PMC

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References

    1. Dharmarajan K, Maurer MS. Transthyretin cardiac amyloidoses in older north americans. J Am Geriatr Soc. 2012;60:765–774. - PMC - PubMed
    1. Rapezzi C, Merlini G, Quarta CC, Riva L, Longhi S, Leone O, Salvi F, Ciliberti P, Pastorelli F, Biagini E, Coccolo F, Cooke RM, Bacchi-Reggiani L, Sangiorgi D, Ferlini A, Cavo M, Zamagni E, Fonte ML, Palladini G, Salinaro F, Musca F, Obici L, Branzi A, Perlini S. Systemic cardiac amyloidoses: Disease profiles and clinical courses of the 3 main types. Circulation. 2009;120:1203–1212. - PubMed
    1. Connors LH, Lim A, Prokaeva T, Roskens VA, Costello CE. Tabulation of human transthyretin (ttr) variants, 2003. Amyloid: the international journal of experimental and clinical investigation: the official journal of the International Society of Amyloidosis. 2003;10:160–184. - PubMed
    1. Jacobson DR, Gorevic PD, Buxbaum JN. A homozygous transthyretin variant associated with senile systemic amyloidosis: Evidence for a late-onset disease of genetic etiology. American journal of human genetics. 1990;47:127–136. - PMC - PubMed
    1. Jacobson DR, Pastore RD, Yaghoubian R, Kane I, Gallo G, Buck FS, Buxbaum JN. Variant-sequence transthyretin (isoleucine 122) in late-onset cardiac amyloidosis in black americans. The New England journal of medicine. 1997;336:466–473. - PubMed

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