Published January 11, 2023 | Version v1
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Interobserver reproducibility and prognostic value of 99mTc-hydroxymethylene diphosphonate scintigraphy for the diagnosis of cardiac amyloidosis

Description

Background: Cardiac amyloidosis is the consequence of the accumulation of abnormal insoluble proteins, known as amyloid fibrils, in the myocardial extracellular space. There are two main subtypes of cardiac amyloidosis: AL and ATTR. A quick diagnosis is essential, especially in ATTR amyloidosis, to enable early access to new treatments, such as TAFAMIDIS, that slow progression of the disease. The bone scintigraphy is now recognized as a keystone for the diagnosis of cardiac ATTR amyloidosis in patients without monoclonal gammopathy. The aims of this study were (1) to measure the interobserver reproducibility of 99mTc-HMDP scintigraphy interpretation using the visual and semi-quantitative analyses, and (2) to assess the prognostic value of the intensity of the myocardial. Methods: The study cohort included 167 subjects who had been referred for 99mTc-HMDP bone scan to center Eugene Marquis in case of clinical suspicion of cardiac amyloidosis or typical findings on echocardiography, from January 2019 to December 2020. Myocardial uptake was assessed by visual (Perugini scale) and semi-quantitative analysis with H/CL ratio calculations, done by two independent observers. In case of discrepancy a proofreading was done by a referent in nuclear cardiology whose assessment was considered as final. Results: Fifty (30%) were diagnosed with cardiac amyloidosis (41 ATTR, 7 AL and 2 undetermined). The median follow-up was 9,8 months. The interobserver agreement was excellent for both the visual (K = 0.84) and the semi-quantitative (K = 0.89) analyses. The myocardial uptake intensity did not show any statistically significant prognostic value, neither in patients with ATTR amyloidosis (p=0.90), nor with those with AL amyloidosis (p=0.28). Conclusion: This study showed excellent inter-observer reproducibility in the interpretation of 99mTc-HMDP bone scintigraphy for the diagnostic of cardiac amyloidosis with both visual and semi-quantitative analysis, making it a reliable and reproducible test for the diagnosis of cardiac amyloidosis. The intensity of the cardiac uptake did not show any significant prognostic value. (author)

Abstract (French)

Introduction: L'amylose cardiaque est la consequence de l'accumulation de proteines insolubles anormales, appelees fibrilles amyloides, dans l'espace extracellulaire du myocarde. Il existe deux principaux sous-types d'amylose cardiaque: AL et ATTR. Un diagnostic rapide est essentiel, notamment dans l'amylose ATTR, pour permettre un acces precoce a des traitements efficaces, comme le tafamidis, qui ralentissent la progression de la maladie. La scintigraphie osseuse est aujourd'hui reconnue comme une cle de voute pour le diagnostic de l'amylose cardiaque ATTR chez les patients sans gammapathie monoclonale. Les objectifs de cette etude etaient (1) de mesurer la reproductibilite inter-observateur de la fixation myocardique dans l'interpretation de la scintigraphie au 99mTc-HMDP a l'aide d'une analyse visuelle et semi-quantitative, et (2) de determiner une valeur seuil de fixation cardiaque associee a une survie globale reduite. Methodes: La cohorte de l'etude comprenait 167 sujets qui avaient ete adresses pour realiser une scintigraphie osseuse au 99mTc-HMDP au centre Eugene Marquis en cas de suspicion clinique d'amylose cardiaque ou de constatations typiques a l'echocardiographie, de janvier 2019 a decembre 2020. La fixation myocardique a ete evaluee par analyse visuel (echelle de PERUGINI) et semi-quantitative avec calcul du ratio H/CL, realise par deux observateurs independants. En cas de divergence, une relecture etait effectuee par un referent en cardiologie nucleaire dont l'appreciation etait consideree comme definitive. Resultats: Cinquante (30%) ont recu un diagnostic d'amylose cardiaque (41 ATTR, 7 AL et 2 indetermines). Le suivi median etait de 9,8 mois. L'accord inter-observateur etait excellent pour les analyses visuelle (K = 0,84) et semi-quantitative (K = 0,89). L'intensite de fixation myocardique n'a montre aucune valeur pronostique statistiquement significative, ni chez les patients avec amylose ATTR (p = 0,90), ni chez ceux avec amylose AL (p = 0,28). Conclusion: Cette etude a montre une excellente reproductibilite inter-observateur de l'evaluation de la fixation myocardique dans l'interpretation de la scintigraphie osseuse au 99mTc-HMDP pour le diagnostic de l'amylose cardiaque. (auteur)

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Additional titles

Original title (French)
Reproductibilite inter-observateur de l'evaluation de la fixation myocardique en scintigraphie osseuse en cas de suspicion d'amylose cardiaque

Publishing Information

Imprint Pagination
38 p.
Report number
FRNC-TH--16142

Optional Information

Notes
13 refs.; Available from the INIS Liaison Officer for France, see the INIS website for current contact and E-mail addresses