Diagnosis of Amyloidosis: A Survey of Current Awareness and Clinical Challenges Among Cardiologists in Switzerland

Mircsof, Dennis

doi:10.1007/s40119-019-00160-8

Cited by 16 publications

(22 citation statements)

References 23 publications

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“…Our data are in line with the results reported by Mircsof et al among 72 Cardiologists working in Switzerland, that showed a wide variation in knowledge and in diagnostic tests used during differential diagnosis of ATTR CA, and concluded that Cardiologists in that country would benefit from information on the latest advances in ATTR 8 .…”

Section: Discussionsupporting

confidence: 93%

“…Being a relatively rarely diagnosed syndrome, the underdiagnosis may be related to the poor awareness of this disease among physicians, bringing to late or no diagnosis. However, little is known about the real knowledge of CA among physicians: a survey on physicians' attitudes in amyloidosis treatment has been recently performed 7 , while only one study assessed knowledge among cardiologists 8 . Given the late clinical manifestations of ATTRwt CA and its common presentation as heart failure, a clinical condition frequently treated by geriatricians 9 , it is pivotal for these specialists to suspect this disease when indicated.…”

Section: Introductionmentioning

confidence: 99%

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Cardiac amyloidosis awareness among residents/specialists in Geriatrics: an Italian national survey

Lelli

Leosco

Incalzi

2021

JGG

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Objective. Cardiac amyloidosis (CA) among older adults is less infrequent than usually considered, and often underdiagnosed. Geriatricians' awareness and knowledge of CA is unknown. Objectives of the study are to assess actual CA knowledge among residents/specialists in Geriatrics, and their usual clinical practice in managing suspected CA. Methods. In this Nation-wide survey carried out by the Italian Society of Gerontology and Geriatrics in April-August 2020, we administered to residents/specialists in Geriatrics a questionnaire divided in three sections: socio-demographical, procedural and knowledge. Results were stratified by qualification (resident/specialist) and by performance (best/worst performers). Results. 289 residents and 104 specialists participated. The overall proportion of correct answers was 57.9% (58.4% residents, 56.1% specialists); knowledge about clinical (89% of correct answers, 91% residents and 84% specialists, P = 0.062), ECG (72% of correct answers, no differences between groups), and echocardiographic (86% of correct answers, no differences between groups) signs of CA was good. However, only 8% participants knew how to diagnose ATTRwt CA (no differences between groups), and 19% which diuretic is indicated in CA (22% residents, 13% specialists, P = 0.069). Only 25% of the participants knew the natural history of ATTRwt (19% residents, 41% specialists, P < 0.001), and 37% was aware of the target of the ATTR treatment. Prevalence of CA was underestimated by 57% specialists and 37% residents (P = 0.001). Conclusions. Knowledge of CA among residents/specialists in Geriatrics is uncomplete. Education campaigns on this topic are desirable, to improve physicians' awareness of CA, thus reducing the number of potential misdiagnosis/delay in correct diagnoses.

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Section: Discussionsupporting

confidence: 93%

Section: Introductionmentioning

confidence: 99%

Cardiac amyloidosis awareness among residents/specialists in Geriatrics: an Italian national survey

Lelli

Leosco

Incalzi

2021

JGG

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“… 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. 19 These findings indicate a need for guidance on daily clinical scenarios to consider 99m Tc‐labelled bone radiotracer scintigraphy for a suspected diagnosis of ATTR‐CM, as well as a standardized protocol for imaging.…”

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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“…14,15 Reasons for diagnostic delay are multifactorial and include: disease rarity and phenotypic heterogeneity, lack of physician awareness, other disease mimicry, and until recently, a lack of sensitive non-invasive investigations. 16…”

Section: Treatment and The Implication Of Diagnostic Delaysmentioning

confidence: 99%

When to Suspect and How to Approach a Diagnosis of Amyloidosis

Law

Gillmore

2022

The American Journal of Medicine

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Diagnosis of Amyloidosis: A Survey of Current Awareness and Clinical Challenges Among Cardiologists in Switzerland

Cited by 16 publications

References 23 publications

Cardiac amyloidosis awareness among residents/specialists in Geriatrics: an Italian national survey

Cardiac amyloidosis awareness among residents/specialists in Geriatrics: an Italian national survey

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

When to Suspect and How to Approach a Diagnosis of Amyloidosis

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