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Vyndaqel 61mg is indicated for the treatment of wild‑type or hereditary transthyretin amyloidosis in adult patients with cardiomyopathy
(ATTR-CM).¹

▼ This medicinal product is subject to additional monitoring. This will allow quick identification of new safety information. Healthcare professionals are asked to report any suspected adverse reactions. Refer to section 4.8 of the SmPC for how to report adverse reactions.

When ATTR-CM is suspected,

Diagnosis may be made noninvasively with nuclear scintigraphy and testing to rule out AL amyloidosis^1,2

View Diagnostic Flowchart

Diagnosing with nuclear scintigraphy

Nuclear scintigraphy with [^99mTc-PYP*/^99mTc-DPD/^99mTc-HMDP] provides a unique myocardial uptake pattern in amyloid and may identify ATTR deposits early in the course of disease³

Studies comparing [^99mTc-PYP/^99mTc-DPD/^99mTc-HMDP] scintigraphy with endomyocardial biopsy (EMB) found that bone radiotracers have avidity for ATTR deposits, whereas avidity for AL cardiac amyloid deposits is minimal or absent³

The recommended time between injection of [^99mTc-PYP/^99mTc-DPD/^99mTc-HMDP]* and scan is 2 or 3 hours.³ 1-hour planar-only imaging is not recommended³

SPECT imaging is required in all studies (irrespective of time between injection and scan) to ensure direct visualization of tracer uptake in the myocardium³

Learn how to interpret nuclear scintigraphy scans using the 3 steps below

Step 1: Visual interpretation³

Planar and SPECT images should be evaluated to confirm diffuse radiotracer uptake in the myocardium³

Use SPECT imaging to differentiate myocardial radiotracer uptake from residual blood pool activity, focal myocardial infarct, and overlapping bone (eg, from rib hot spots from fractures)³

If excess blood pool is noted, recommend repeat SPECT imaging at 3 hours³

If myocardial tracer uptake is visually present on SPECT, proceed to step 2, semiquantitative grading³

If no myocardial tracer uptake is present on SPECT, the visual grade is 0³

Illustrative representation of^99mTc-PYP myocardial uptake.

Step 2: Semiquantitative visual grading comparison to bone (rib) uptake at 3 hours³

Examine both planar and SPECT images for tracer uptake in the myocardium relative to ribs and grade using the following scale^3,4

Grade 0

Grade 1

Grade 2

Grade 3

Planar

SPECT

No myocardial uptake and normal bone uptake

Planar

SPECT

Myocardial uptake less than rib uptake

Planar

SPECT

Myocardial uptake equal to rib uptake

Planar

SPECT

Myocardial uptake greater than rib uptake with mild/absent bone uptake

Nuclear scintigraphy should be performed using standard protocols. The recommended time between injection of [^99mTc-PYP/^99mTc-DPD/^99mTc-HMDP]* and scanning is 2 or 3 hours³

Once AL is ruled out, Grade 2/3 myocardial uptake of radiotracer on SPECT combined with consistent echo or CMR findings is diagnostic of ATTR-CM⁵

Strongly suggestive of ATTR-CM³:
Visual scores of ≥2 on planar and SPECT images with concurrent testing to rule out AL

Not suggestive or equivocal of ATTR-CM³:
Visual scrores of <2

Step 3: H/CL uptake ratio assessment (when applicable)³

Diagnosis of ATTR-CM cannot be made solely based on heart-to-contralateral lung (H/CL) ratio. H/CL ratio is not recommended if there is absence of myocardial uptake on SPECT imaging.³

H/CL ratio is typically concordant with visual grade³

If the visual grade is 2 or 3, diagnosis is confirmed, and H/CL ratio assessment is not necessary

If discordant, or the visual grade is equivocal, H/CL ratio may be helpful to classify equivocal visual grade 1 vs 2 as positive or negative

An H/CL ratio is calculated as the fraction of heart ROI mean counts to contralateral lung ROI mean counts³

H/CL ratios of ≥1.5 at 1 hour can accurately identify ATTR cardiac amyloidosis if myocardial ^99mTc-PYP* uptake is visually confirmed on SPECT imaging and systemic AL amyloidosis is excluded

An H/CL ratio of ≥1.3 at 3 hours can identify ATTR cardiac amyloidosis

Quantification of cardiac ^99mTc-PYP* uptake using H/CL ratio⁴

Biopsy-proven ATTR cardiac amyloidosis with H/CL=2.08⁴

Illustrative representation.

If clinical suspicion for cardiac amyloidosis remains high despite a negative or inconclusive scintigraphy scan, consider EMB³

Endomyocardial biopsy: an invasive approach to diagnose ATTR-CM⁶

Congo red positive staining of myocardial tissue on light microscopy and apple-green birefringence on polarized light microscopy images¹

Congo red positive staining for amyloid myocardial biopsy
To determine amyloid type, immunohistochemistry (IHC) tests and/or mass spectrometry should be performed.^₁

Illustrative representation.

Apple-green birefringence myocardial biopsy
Risk of complications and the need for specialized centers and expertise may contribute to a diagnostic delay.^1,6

Illustrative representation.

Genetic testing can confirm ATTR-CM type

Used to determine if the disease is hereditary due to a mutation in the TTR gene or if it is wild type⁷

Genetic counseling and gene sequencing are recommended following confirmation of hATTR-CM⁷

*Please consult individual labeling for risks.

[^99mTc-DPD=^99mtechnetium-labeled 3,3-diphosphono-1,2-propanodicarboxylic acid]; [^99mTc-HMDP=^99mtechnetium-hydroxymethylene diphosphonate];[^99mTc-PYP=^99mtechnetium-pyrophosphate]; AL=immunoglobulin light chain amyloidosis; ATTR=transthyretin amyloidosis; CMR=cardiac magnetic resonance; ROI=region of interest; SPECT=single photon emission computed tomography.

Nuclear scintigraphy, endomyocardial biopsy and genetic testing can support an early ATTR-CM diagnosis

Explore More

Pivotal and 5-year data for ATTR-CM

View the Body of Evidence

ATTR-CM MOD

Learn the Mechanism of Disease

NEXT: ATTR-CM diagnostic flowchart

References:

Gillmore JD, Maurer MS, Falk RH, et al. Nonbiopsy diagnosis of cardiac transthyretin amyloidosis. Circulation. 2016;133(24):2404-2412.doi:10.1161/CIRCULATIONAHA.116.021612

Bokhari S, Castano A, Pozniakoff T, Deslisle S, Latif F, Maurer MS.^99mTc-pyrophosphate scintigraphy for differentiating light-chain cardiac amyloidosis from the transthyretin-related familial and senile cardiac amyloidosis. Circ Cardiovasc Imaging. 2013;6(2):195-201. doi:10.1161/CIRCIMAGING.112.000132

Dorbala S, Ando Y, Bokhari S, et al. ASNC/AHA/ASE/EANM/HFSA/ISA/SCMR/SNMMI expert consensus recommendations for multimodality imaging in cardiac amyloidosis: part 1 of 2—evidence base and standardized methods of imaging. J Nucl Cardiol. 2019;26(6):2065-2123. doi:10.1007/s12350-019-01760-6. Addendum: J Nucl Cardiol. Published on line July 1, 2021. doi:10.1007/s12350-020-02455-z

American Society of Nuclear Cardiology (ASNC). ASNC practice points: ^99mTechnetium-pyrophosphate imaging for transthyretin cardiac amyloidosis. Available at: https://www.asnc.org/files/19110%20ASNC%20Amyloid%20Practice%20Points%20WEB(2).pdf. © 2019 American Society of Nuclear Cardiology.

Dorbala S, Ando Y, Bokhari S, et al. ASNC/AHA/ASE/EANM/HFSA/ISA/SCMR/SNMMI expert consensus recommendations for multimodality imaging in cardiac amyloidosis: part 2 of 2—diagnostic criteria and appropriate utilization. J Nucl Cardiol. 2020;27(2):659-673. doi:10.1007/s12350-019-01761-5

Narotsky DL, Castano A, Weinsaft JW, Bokhari S, Maurer MS. Wild-type transthyretin cardiac amyloidosis: novel insights from advanced imaging. Can J Cardiol. 2016;32(9):1166.e1-1166.e10. doi:10.1016!J.cjca.2016.05.008

Maurer MS, Elliott P, Comenzo R, Semigran M, Rapezzi C. Addressing common questions encountered in the diagnosis and management of cardiac amyloidosis. Circulation. 2017;135(14):1357-1377. doi:10.1161/CIRCULATIONAHA.116.024438

VYNDAQEL Prescribing Information

About ATTR-CM

Legal Category: S1A.Further information available upon request

PP-VYN-IRL-0177. December 2022

Adverse events should be reported. Report an adverse event to your national reporting system (HPRA Pharmacovigilance)

Adverse events should also be reported to Pfizer Medical Information on 1800 633 363

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