The prevalence of TTR cardiac amyloidosis among patients undergoing bone scintigraphy

Bianco, Matteo; Parente, Antonella; Biolè, Carloalberto; Righetti, Camilla; Spirito, Amanda; Luciano, Alessia; Destefanis, P.; Nangeroni, G; Angusti, Tiziana; Anselmino, Matteo; Montagna, Laura

doi:10.1007/s12350-021-02575-0

Cited by 20 publications

(41 citation statements)

References 13 publications

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“…Specific clinical settings have been recognized as strongly associated with CA but further studies are needed to better characterize these populations, mainly to stratify patient risk and evaluate which patients are ideal candidates for the newly available disease-modifying therapies. Acknowledgments: We would like to thank all the nuclear medicine doctors, hematologists, neurologists, pathologists and nephrologists of the participating centres for providing their essential [8,[13][14][15][16]20,27,28,33,[36][37][38][39]41,42,[46][47][48][49][50][51][54][55][56].…”

Section: Discussionmentioning

confidence: 99%

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Re-Definition of the Epidemiology of Cardiac Amyloidosis

et al. 2022

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The epidemiology of cardiac amyloidosis (CA), traditionally considered a rare and incurable disease, has changed drastically over the last ten years, particularly due to the advances in diagnostic methods and therapeutic options in the field of transthyretin CA (ATTR-CA). On the one hand, the possibility of employing cardiac scintigraphy with bone tracers to diagnose ATTR-CA without a biopsy has unveiled the real prevalence of the disease; on the other, the emergence of effective treatments, such as tafamidis, has rendered an early and accurate diagnosis critical. Interestingly, the following subgroups of patients have been found to have a higher prevalence of CA: elderly subjects > 75 years, patients with cardiac hypertrophy hospitalized for heart failure with preserved ejection fraction, subjects operated on for bilateral carpal tunnel syndrome, patients with cardiac hypertrophy not explained by concomitant factors and individuals with aortic valve stenosis. Many studies investigating the prevalence of CA in these particular populations have contributed to rewriting the epidemiology of the disease, increasing the awareness of the medical community for a previously underappreciated condition. In this review, we summarized the latest evidence on the epidemiology of CA according to the different clinical settings typically associated with the disease.

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Section: Discussionmentioning

confidence: 99%

“…Figure 1. Prevalence of CA in different screening studies[8,[13][14][15][16]20,27,28,33,[36][37][38][39]41,42,[46][47][48][49][50][51][54][55][56].…”

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confidence: 99%

Re-Definition of the Epidemiology of Cardiac Amyloidosis

et al. 2022

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“…Notably, of those with suspected ATTR-CA (grade 2/3), 41% had a history of HF prior to scan ( 80 ). Bianco et al found a prevalence of 0.54% grade 2/3 uptake on 3,228 scans performed over 5 years using DPD or HDMP at their center ( 86 ). With higher clinical suspicion, ATTR-CA may have been suspected in some of these patients as 48% had prior HF, 34.8% had known neuropathy, and 21.7% had carpal tunnel syndrome.…”

Section: Nuclear Imagingmentioning

confidence: 99%

“…With higher clinical suspicion, ATTR-CA may have been suspected in some of these patients as 48% had prior HF, 34.8% had known neuropathy, and 21.7% had carpal tunnel syndrome. However, 21.7% were entirely asymptomatic highlighting the existence of an asymptomatic disease state during which treatment could prevent disease progression ( 86 ).…”

Section: Nuclear Imagingmentioning

confidence: 99%

Multimodality Imaging in the Evaluation and Prognostication of Cardiac Amyloidosis

Scheel

Mukherjee

Hays

et al. 2022

Front. Cardiovasc. Med.

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Cardiac amyloidosis (CA) is an infiltrative cardiomyopathy resulting from deposition of misfolded immunoglobulin light chains (AL-CA) or transthyretin (ATTR-CA) proteins in the myocardium. Survival varies between the different subtypes of amyloidosis and degree of cardiac involvement, but accurate diagnosis is essential to ensure initiation of therapeutic interventions that may slow or potentially prevent morbidity and mortality in these patients. As there are now effective treatment options for CA, identifying underlying disease pathogenesis is crucial and can be guided by multimodality imaging techniques such as echocardiography, magnetic resonance imaging, and nuclear scanning modalities. However, as use of cardiac imaging is becoming more widespread, understanding optimal applications and potential shortcomings is increasingly important. Additionally, certain imaging modalities can provide prognostic information and may affect treatment planning. In patients whom imaging remains non-diagnostic, tissue biopsy, specifically endomyocardial biopsy, continues to play an essential role and can facilitate accurate and timely diagnosis such that appropriate treatment can be started. In this review, we examine the multimodality imaging approach to the diagnosis of CA with particular emphasis on the prognostic utility and limitations of each imaging modality. We also discuss how imaging can guide the decision to pursue tissue biopsy for timely diagnosis of CA.

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“…For example, approximately one in eight patients undergoing transcatheter aortic valvular replacement [12] and about one in five patients with left ventricular hypertrophy and heart failure (HF) could be diagnosed with ATTR-CA [13]. ATTR-CA is nowadays an important clinical reality for both cardiologists [11] and radiologists [14]. In ATTR-CA, deposition of transthyretin (TTR) amyloid fibrils occurs in the extracellular space of the heart, affecting not only the ventricles but the atria, as well as resulting in impaired ventricular and atrial function [15][16][17].…”

Section: Introductionmentioning

confidence: 99%

The Prevalence of Advanced Interatrial Block and Its Relationship to Left Atrial Function in Patients with Transthyretin Cardiac Amyloidosis

Lindow

Lindqvist

2021

JCM

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Background: Advanced interatrial block (aIAB), which is associated with incident atrial fibrillation and stroke, occurs in the setting of blocked interatrial conduction. Atrial amyloid deposition could be a possible substrate for reduced interatrial conduction, but the prevalence of aIAB in patients with transthyretin cardiac amyloidosis (ATTR-CA) is unknown. We aimed to describe the prevalence of aIAB and its relationship to left atrial function in patients with ATTR-CA in comparison to patients with HF and left ventricular hypertrophy but no CA. Methods: The presence of aIAB was investigated among 75 patients (49 patients with ATTR-CA and 26 with HF but no CA). A comprehensive echocardiographic investigation was performed in all patients, including left atrial strain and strain rate measurements. Results: Among patients with ATTR-CA, 27% had aIAB and in patients with HF but no CA, this figure was 21%, (p = 0.78). The presence of aIAB was associated with a low strain rate during atrial contraction (<0.91 s−1) (OR: 5.2 (1.4–19.9)), even after adjusting for age and LAVi (OR: 4.5 (1.0–19.19)). Conclusions: Advanced interatrial block is common among patients with ATTR-CA, as well as in patients with heart failure and left ventricular hypertrophy but no CA. aIAB is associated with reduced left atrial contractile function.

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The prevalence of TTR cardiac amyloidosis among patients undergoing bone scintigraphy

Cited by 20 publications

References 13 publications

Re-Definition of the Epidemiology of Cardiac Amyloidosis

Re-Definition of the Epidemiology of Cardiac Amyloidosis

Multimodality Imaging in the Evaluation and Prognostication of Cardiac Amyloidosis

The Prevalence of Advanced Interatrial Block and Its Relationship to Left Atrial Function in Patients with Transthyretin Cardiac Amyloidosis

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