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ArticleClinical research in cardiology : official journal of the German Cardiac Society2026

Cardiac screening of amyloid TTR pathogenic variant carriers: complementary value of echocardiographic global longitudinal strain imaging and bone scintigraphy.

Sandra Sanders-van Wijk, Sebastiaan H C Klaassen, Jerremy Weerts, Anouk Achten, Marish I F J Oerlemans, Hendrea S A Tingen, Michelle Michels, Paul A van der Zwaag, Riemer H J A Slart, Mathijs van Gemert and 4 more

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Article in Clinical research in cardiology : official journal of the German Cardiac Society, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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1 · What the graph read from it

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3 · Its place in the literature

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4 · The record

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5 · Who and what money

Authors and funding

14 authors.

Sandra Sanders-van Wijk *Department of Cardiology, Zuyderland Medical Center, Heerlen, the Netherlands.
Sebastiaan H C Klaassen *Department of Cardiology, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.
Jerremy WeertsDepartment of Cardiology, Maastricht University Medical Center, CARIM, PO Box 5800, 6202 AZ, Maastricht, the Netherlands.
Anouk AchtenDepartment of Cardiology, Maastricht University Medical Center, CARIM, PO Box 5800, 6202 AZ, Maastricht, the Netherlands.
Marish I F J OerlemansDepartment of Cardiology, University Medical Center Utrecht, Utrecht, the Netherlands.
Hendrea S A TingenMedical Imaging Centre, Department of Nuclear Medicine and Molecular Imaging, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.
Michelle MichelsDepartment of Cardiology, Thorax Center, Erasmus University Medical Center, Erasmus University Rotterdam, Rotterdam, the Netherlands.
Paul A van der ZwaagDepartment of Genetics, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
Riemer H J A SlartMedical Imaging Centre, Department of Nuclear Medicine and Molecular Imaging, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.
Mathijs van GemertDepartment of Cardiology, Maastricht University Medical Center, CARIM, PO Box 5800, 6202 AZ, Maastricht, the Netherlands.
Dirk J van VeldhuisenDepartment of Cardiology, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.
Hans L A NienhuisDepartment of Internal Medicine, University Medical Center Groningen, Groningen, the Netherlands.
Christian Knackstedt *Department of Cardiology, Maastricht University Medical Center, CARIM, PO Box 5800, 6202 AZ, Maastricht, the Netherlands. c.knackstedt@mumc.nl.ORCID http://orcid.org/0000-0002-5457-3010
Maarten P van den Berg *Department of Cardiology, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundCardiac involvement is the major determinant of mortality in variant TTR amyloidosis (ATTRv). Treatment options have recently become available, which make early diagnosis important. Several imaging modalities for screening are proposed including bone scintigraphy, conventional, and strain echocardiography but data on their relative value is limited in this population.

objectivesTo investigate the value of left ventricular global longitudinal strain (LV-GLS) for screening for cardiac involvement in a population of transthyretin (TTR) pathogenic variant carriers.

methodsFifty-three TTR pathogenic variant carriers from two Dutch academic hospitals underwent transthoracic echocardiography (TTE) and bone scintigraphy as part of routine out-patient care to assess cardiac involvement. LV-GLS was measured by two blinded reviewers. Abnormalities in LV-GLS (cut-off - 21.5%) and other TTE parameters were evaluated in bone scintigraphy positive (≥ grade 1) and negative cases.

resultsCarriers with a positive bone scintigraphy (n = 20/53) were 16 years older than those with a negative scan (n = 33/53). In 17 out of 20 (85%) of patients with a positive bone scintigraphy, LV-GLS was abnormal. When bone scintigraphy was negative, LV-GLS was abnormal in 11 cases (38%). Although most of these patients had no obvious alternative explanation for the reduced LV-GLS, the clinical significance of this finding remains uncertain. LV-GLS as a single echo parameter was equally sensitive to detect an abnormal bone scintigraphy versus all other TTE parameters together.

conclusionsLV-GLS and bone scintigraphy provided complementary information in TTR pathogenic variant carriers. Discordant findings between both modalities warrant further evaluation, as abnormal LV-GLS may reflect early ATTRv-related changes but is not disease-specific. These findings are exploratory and hypothesis-generating and warrant confirmation in prospective studies incorporating additional reference standards.LV-GLS was the most sensitive single echo parameter, making it useful for repeated assessment in this population.

Indexed as

AmyloidosisBone scintigraphyEchocardiographyGlobal longitudinal strainScreeningSpeckle tracking

Identifiers

PMID42606605

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