Prevalence of wild type ATTR assessed as myocardial uptake in bone scan in the elderly population

Salem, Laroussi Mohamed; Mateo, Juan José Santos; Sánchez-Serna, Juan; Hernández-Vicente, Álvaro; Reyes-Marle, Rafael; Sanchez, Manuel Villa; Claver-Valderas, María Antonia; González-Vioque, Emiliano; Moral, Francisco J. Haro-del; García‐Pavía, Pablo; Pascual-Figal, Domingo A.

doi:10.1016/j.ijcard.2018.06.006

Cited by 81 publications

(47 citation statements)

References 19 publications

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“…Based on nuclear scintigraphy, cardiac amyloidosis is overwhelmingly due to transthyretin deposits. ATTR amyloidosis was prevalent in 13% of patients hospitalized for HF with preserved ejection fraction and LV wall thickness >12 mm (2); 16% in patients undergoing transcatheter aortic valve replacement for severe aortic stenosis (7); in 5% in patients with presumed hypertrophic cardiomyopathy (8) and in 1–3% of subjects > 75 years of age (9). Could ATTR be present in the hearts of older adult patients with AD?…”

Section: If Amyloidosis Is Present In the Hearts Of Patients With Admentioning

confidence: 99%

Amyloidosis of the Brain and Heart

Schaich

Maurer

Nadkarni

2019

JACC: Heart Failure

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Section: If Amyloidosis Is Present In the Hearts Of Patients With Admentioning

confidence: 99%

Amyloidosis of the Brain and Heart

Schaich

Maurer

Nadkarni

2019

JACC: Heart Failure

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“…The commonest hATTR‐CM is that associated with the V122I (p.V142I) TTR variant (V122I‐hATTR‐CM), carried by 3.9% of individuals of African descent 1 . The prevalence of ATTR‐CM is not known, but high‐grade cardiac uptake on 99m technetium‐labelled 3,3‐diphosphono‐1,2‐propanodicarboxylic acid scintigraphy was reported in 3.9% of men over 75 years of age in a recent Spanish study 2 . Advances in imaging techniques 3–5 and development of validated non‐biopsy diagnostic criteria for ATTR‐CM 6,7 have led to an exponential rise in diagnoses of ATTR‐CM throughout the world 8 …”

Section: Introductionmentioning

confidence: 99%

Disease progression in cardiac transthyretin amyloidosis is indicated by serial calculation of National Amyloidosis Centre transthyretin amyloidosis stage

Law¹,

Petrie

Chacko³

et al. 2020

ESC Heart Failure

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Aims Cardiac transthyretin amyloidosis (ATTR-CM) is a progressive and fatal condition. Prognosis can be determined at diagnosis according to the National Amyloidosis Centre (NAC) transthyretin amyloidosis (ATTR) stage. We sought to examine how NAC ATTR stage changes during follow-up and whether it maintains its prognostic value throughout the disease course. Methods and results We performed a retrospective study of 945 patients with wild-type ATTR-CM (wtATTR-CM) or hereditary ATTR-CM associated with the V122I variant (V122I-hATTR-CM) who were diagnosed and serially evaluated at the UK NAC. Patients who commenced any disease-modifying therapy for amyloidosis were censored at the time of doing so. Landmark Kaplan-Meier survival analyses were performed at diagnosis (n = 945) and at 6 ± 1 (n = 432), 12 ± 3 (n = 562), and 24 ± 3 (n = 316) months and stratified by recalculated NAC ATTR stage at the relevant time point. Cox regression analyses were performed to assess the prognostic significance during follow-up of an increase in NAC ATTR stage from Stage I at diagnosis. Mortality in ATTR-CM was predicted by NAC ATTR stage at each time point [Stage II vs. I, hazard ratios (HRs) 1.95-2.67; P < 0.001; Stage III vs. II, HRs 1.64-2.25; P < 0.001-0.013]. An increase from NAC ATTR Stage I, which occurred in 21%, 32%, and 44% of evaluable patients at 6, 12, and 24 months of follow-up respectively, was highly predictive of ongoing mortality at each time point (HRs 2.58-3.22; P < 0.001) and in each genotypic subgroup (HRs 1.86-4.38; P < 0.05). Increase in NAC ATTR stage occurred earlier in V122I-hATTR-CM than in wtATTR-CM (43% vs. 27% at 12 months of follow-up; P = 0.003). Conclusions National Amyloidosis Centre ATTR stage predicts ongoing survival throughout the disease natural history in ATTR-CM, and an increase from NAC ATTR Stage I at diagnosis to a higher NAC ATTR stage predicts mortality throughout follow-up. Serial calculation of NAC ATTR stage suggests a more aggressive phenotype in V122I-hATTR-CM than in wtATTR-CM.

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“…14 Furthermore, ≈1% to 3% of persons older than 75 showed myocardial retention of the diphosphono-1,2-propanodicarboxylic acid, which is indicative of TTR cardiac amyloidosis. 15,16 Delays in diagnosis of ATTR-CM amyloidosis commonly occur because of physician-and disease-related reasons, including fragmented knowledge among different specialists and subspecialists, shortage of centers and specialists dedicated to disease management, erroneous belief that it is an incurable disease, perceived rarity of the condition, intrinsic phenotypic and genotypic heterogeneity, and, in some cases, the necessity of target organ tissue histological diagnosis. 12,17,18 The Amyloidosis Research Consortium recently led the development of a comprehensive set of consensus recommendations for the suspicion and diagnosis of ATTR amyloidosis.…”

mentioning

confidence: 99%

Expert Consensus Recommendations for the Suspicion and Diagnosis of Transthyretin Cardiac Amyloidosis

Maurer

Bokhari

Damy

et al. 2019

Circ: Heart Failure

392

457

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Cardiomyopathy is a manifestation of transthyretin amyloidosis (ATTR), which is an underrecognized systemic disease whereby the transthyretin protein misfolds to form fibrils that deposit in various tissues and organs. ATTR amyloidosis is debilitating and associated with poor life expectancy, especially in those with cardiac dysfunction, but a variety of treatment options have recently become available. Considered a rare disease, ATTR amyloidosis may be more prevalent than thought, particularly in older persons. Diagnosis is often delayed because of a lack of disease awareness and the heterogeneity of symptoms at presentation. Given the recent availability of effective treatments, early recognition and diagnosis are especially critical because treatment is likely more effective earlier in the disease course. The Amyloidosis Research Consortium recently convened a group of experts in ATTR amyloidosis who, through an iterative process, agreed on best practices for suspicion, diagnosis, and characterization of disease. This review describes these consensus recommendations for ATTR associated with cardiomyopathy as a resource to aid cardiologists and others in the recognition and diagnosis of ATTR associated with cardiomyopathy. Included in this review is an overview of red flag signs and symptoms and a recommended diagnostic approach, including testing for monoclonal protein, scintigraphy, or biopsy and, if ATTR associated with cardiomyopathy is identified, TTR genotyping.

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Prevalence of wild type ATTR assessed as myocardial uptake in bone scan in the elderly population

Cited by 81 publications

References 19 publications

Amyloidosis of the Brain and Heart

Amyloidosis of the Brain and Heart

Disease progression in cardiac transthyretin amyloidosis is indicated by serial calculation of National Amyloidosis Centre transthyretin amyloidosis stage

Expert Consensus Recommendations for the Suspicion and Diagnosis of Transthyretin Cardiac Amyloidosis

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