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Prevalence of ATTR-CA and high-risk features to guide testing in patients referred for TAVR

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European Journal of Nuclear Medicine and Molecular Imaging Aims and scope Submit manuscript

Abstract

Purpose

Transthyretin cardiac amyloidosis (ATTR-CA) is thought to be prevalent in patients with severe aortic stenosis (AS) who are referred for transcatheter aortic valve replacement (TAVR). However, prior studies were published when TAVR was only offered to elderly, inoperable, and high-risk patients. The aim of this study was to reevaluate the prevalence of ATTR-CA in a contemporary TAVR population and identify high-risk features to guide referral for technetium-99 pyrophosphate scan (99mTc-PyP scan) screening.

Methods

Patients seen in a multidisciplinary TAVR clinic for severe AS 70 years and older were referred for a 99mTc-PyP scan to evaluate for ATTR-CA. The primary outcome was the percent with a positive scan. The discriminatory ability of high-risk features was assessed to develop a more judicious screening system.

Results

Over the study period, 380 patients underwent screening, and 20 patients (5.3%) had a positive scan, with 17 patients having confirmed ATTR-CA, 1 patient deferring confirmatory testing (combined 4.7%), 1 having light chain amyloidosis, and 1 negative on biopsy. Compared to other patient and echocardiographic measures, elevated NT-pro BNP (> 1000 ng/L) was the best discriminator on who should be referred for 99mTc-PyP scan screening, with a sensitivity of 90% and a negative predictive value of 99%.

Conclusion

The prevalence of ATTR-CA may be lower in a contemporary TAVR population due to its expanded indication for low-risk patients. NT-pro BNP is a simple test that can improve screening yield and more judiciously guide screening for ATTR-CA in this at-risk population.

Graphical abstract

Comparison of the original versus the proposed algorithm

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Data Availability

Data was made available to all investigators and IRB since the data is retrospective and belongs to Cleveland Clinic.

Abbreviations

ATTR-CA:

Transthyretin cardiac amyloidosis

AS:

Aortic stenosis

TAVR:

Transcatheter aortic valve replacement

SAVR:

Surgical aortic valve replacement

99mTc-PyP scan:

Technetium-99 pyrophosphate scan

SPECT/CT:

Single positron emission tomography/computed tomography

EF:

Ejection fraction

IVS:

Septal wall thickness

PWT:

Posterior wall thickness

LV:

Left ventricular

AVA:

Aortic valve area

AVAi:

Aortic valve area indexed to body surface area

SVI:

Stroke volume index

RAS:

Relative apical sparing

SAB:

Septal apical to basal strain ratio

CMR:

Cardiac magnetic resonance imaging

LFLG-AS:

Severe low-flow, low-gradient aortic stenosis

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Funding

The Cleveland Clinic Amyloidosis Registry is supported by Dr. Hanna’s Term Chair for Amyloid Heart Disease.

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Authors and Affiliations

  1. Heart, Vascular, and Thoracic Institute, Cleveland Clinic Foundation, Cleveland, OH, USA

    Bryan Abadie, Adel Hajj Ali, Trejeeve Martyn, Andrew Higgins, Amar Krishnaswamy, Grant Reed, Rishi Puri, James Yun, Paul Cremer, Mazen Hanna, W. H. Wilson Tang & Samir Kapadia

  2. Section of Cardiovascular Imaging, Department of Cardiovascular Medicine, Heart, Vascular, and Thoracic Institute, Cleveland Clinic, 9500 Euclid Avenue, J1-5 Main Campus, Cleveland, OH, 44195, USA

    Wael Jaber

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  1. Bryan Abadie
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Contributions

All authors contributed to the study conception and design. Material preparation and data collection were performed by Bryan Abadie MD and Adel Hajj Ali MD. Analysis was performed by Bryan Abadie MD. The first draft of the manuscript was written by Bryan Abadie MD and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

Corresponding author

Correspondence to Wael Jaber.

Ethics declarations

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Consent to participate

Consent was waived in the setting of minimal risk, as approved by the Cleveland Clinic Institutional Review Board.

Conflict of interest

Dr. Martyn receives research support from Ionis Therapeutics related to amyloidosis research, is an advisor to Recora health, and receives research support from Novartis. Dr. Hanna serves on the advisory boards of Alnylam Pharmaceuticals Inc., Eidos, Ionis Therapeutics, and Pfizer Inc. Drs. Jaber and Cremer have institutional coreLab contracts with Edwards Life Sciences, BMS, and Boston Scientific. The remaining authors have no relevant disclosures.

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Abadie, B., Ali, A.H., Martyn, T. et al. Prevalence of ATTR-CA and high-risk features to guide testing in patients referred for TAVR. Eur J Nucl Med Mol Imaging 50, 3910–3916 (2023). https://doi.org/10.1007/s00259-023-06374-2

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