Changes in the perceived epidemiology of amyloidosis: 20 year-experience from a Tertiary Referral Centre in Tuscany

Zampieri, Mattia; Nardi, Giulia; Monaco, Guido Del; Allinovi, Marco; Gabriele, Martina; Zocchi, Chiara; Casagrande, Silvia; Fumagalli, Carlo; Mario, Carlo Di; Olivotto, Iacopo; Perfetto, Federico; Cappelli, Francesco

doi:10.1016/j.ijcard.2021.04.023

Cited by 22 publications

(17 citation statements)

References 19 publications

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“…Kyle et al reported an incidence rate of AL amyloidosis from 1990 through 2015 of 1.2 per 100,000 person years in the Olmsted County, with rates similar across the decades of 1990–1999, 2000–2009, and 2010–2015 at 1.1, 0.9, and 1.6 per 100,000 person years, respectively, with no suggestion of an increase [ 63 ]. Similar findings were reported in two Italian studies, enrolling about 600 patients each, both showing a rapid and marked increase in the number of new diagnoses of ATTR-CA over the last decade, with only 20–30% of new diagnoses being AL-CA, and stable during the years [ 64 , 65 ]. All the studies show that there is a slight male predominance of AL-CA, and the disease generally presents from the fifth to seventh decade, although it may occur at all ages from the fourth decade onward [ 60 ].…”

Section: Challenges In Diagnosis and Treatment In Casupporting

confidence: 85%

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: Challenges In Diagnosis and Treatment In Casupporting

confidence: 85%

Re-Definition of the Epidemiology of Cardiac Amyloidosis

et al. 2022

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“…A potential limitation of this study may be a sampling bias reflecting patient recruitment from selected centers with expertise in ATTR-CA, mostly in the United States. However, these results mirror similar recent observations of a diagnostic surge in other cohorts across the world ( 5 , 6 , 7 , 8 ). The newly increased awareness of ATTR-CA has spurred physicians to search for it actively, with a resulting wave of new diagnoses.…”

supporting

confidence: 89%

Progress in Cardiac Imaging Uncovers the Epidemiology of Wild-Type Transthyretin Amyloid Cardiomyopathy

Dorbala¹,

Clerc²

2021

JACC: CardioOncology

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“… 13 In addition, the use of bone‐avid radiotracer cardiac imaging allowed physicians to increasingly recognize that ATTR‐CM is an underdiagnosed cause of heart failure in several populations; specifically, ATTR‐CM has been identified in a significant subgroup of patients with heart failure with preserved ejection fraction (HFpEF), aortic stenosis, or hypertrophic cardiomyopathy. 14 , 15 , 16 , 17 Nevertheless, the utility of 99m Tc‐labelled bone radiotracer scintigraphy as a diagnostic modality and the importance of early ATTR‐CM diagnosis have not yet been internalized as common practice by general cardiologists. 18 Moreover, the procedures for 99m Tc‐labelled bone radiotracer scintigraphy are highly variable among institutions.…”

Section: Introductionmentioning

confidence: 99%

^99mTechnetium‐pyrophosphate scintigraphy: a practical guide for early diagnosis of transthyretin amyloid cardiomyopathy

et al. 2021

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Transthyretin amyloid cardiomyopathy (ATTR‐CM) is caused by the cardiac deposition of insoluble amyloid fibrils formed by misfolded transthyretin proteins and is associated with various cardiac symptoms, such as progressive heart failure, conduction disturbance, and arrhythmia. The implementation of 99mtechnetium (99mTc)‐labelled bone radiotracer scintigraphy for diagnosing ATTR‐CM has enabled accurate diagnosis of the disease with high sensitivity and specificity and positioned this diagnostic modality as an integral part of disease diagnostic algorithms. In 2020, 99mTc‐pyrophosphate scintigraphy received exceptional approval for Japanese national health insurance reimbursement as a diagnostic method of ATTR‐CM. Nevertheless, the utility of 99mTc‐labelled bone radiotracer scintigraphy and the importance of an early diagnosis of suspected ATTR‐CM using this technique have yet to be internalized as common practice by general cardiologists, and guidance on daily clinical scenarios to consider this technique for a diagnosis of suspected ATTR‐CM is warranted. In this review, we discuss the utility of 99mTc‐labelled bone radiotracer scintigraphy for the early diagnosis of ATTR‐CM based on published literature and the outcomes of an advisory board meeting. This review also discusses clinical scenarios that could support early diagnosis of suspected ATTR‐CM as well as common pitfalls, correct implementation, and future perspectives of 99mTc‐labelled bone radiotracer scintigraphy in daily clinical practice. The clinical scenarios to consider 99mTc‐labelled bone radiotracer scintigraphy in daily practice may include, but are not limited to, patients with a family history of the hereditary type of disease; elderly patients (aged ≥60 years) with unexplained cardiac findings (e.g. cardiac hypertrophy associated with abnormalities on an electrocardiogram, heart failure with preserved ejection fraction associated with unexplained left ventricular hypertrophy, and heart failure with reduced ejection fraction associated with atrial fibrillation and left ventricular hypertrophy); and patients with cardiac hypertrophy associated with diastolic dysfunction, right ventricular/interatrial septum/valve thickness, left ventricular sparkling, or apical sparing. Cardiac hypertrophy and persistent elevation in cardiac troponin in elderly patients are also suggestive of ATTR‐CM. 99mTc‐labelled bone radiotracer scintigraphy is also recommended in patients with characteristic cardiac magnetic resonance findings (e.g. diffuse subendocardial late gadolinium enhancement patterns, native T1 increase, and increase in extracellular volume) or patients with cardiac hypertrophy and bilateral carpal tunnel syndrome.

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Changes in the perceived epidemiology of amyloidosis: 20 year-experience from a Tertiary Referral Centre in Tuscany

Cited by 22 publications

References 19 publications

Re-Definition of the Epidemiology of Cardiac Amyloidosis

Re-Definition of the Epidemiology of Cardiac Amyloidosis

Progress in Cardiac Imaging Uncovers the Epidemiology of Wild-Type Transthyretin Amyloid Cardiomyopathy

^99mTechnetium‐pyrophosphate scintigraphy: a practical guide for early diagnosis of transthyretin amyloid cardiomyopathy

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