Temporal Trends of Wild-type Attr Amyloidosis in the Transthyretin Amyloidosis Outcomes Survey

Nativi-Nicolau, José; Siu, Alfonso; Dispenzieri, Angela; Maurer, Mathew S.; Rapezzi, Claudio; Kristen, Arnt V.; García‐Pavía, Pablo; LoRusso, Samantha; Waddington-Cruz, Márcia; Lairez, Olivier; Witteles, Ronald; Chapman, Doug; Rill, Denise; Kiszko, Jan; Amass, Leslie; Grogan, Martha

doi:10.1016/j.cardfail.2020.09.239

Cited by 5 publications

(8 citation statements)

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“…All patients underwent sequencing of the TTR gene. Age ≥70 years at diagnosis was used to define the elderly cohort as (i) it was the median age of symptom onset in the Transthyretin Amyloid Outcome Survey (THAOS) registry, 17 and (ii) nowadays, the majority of individuals <70 years are not considered 'elderly' from a clinical perspective. 18…”

Section: Study Populationmentioning

confidence: 99%

Prevalence, characteristics and outcomes of older patients with hereditary versus wild‐type transthyretin amyloid cardiomyopathy

Porcari

Razvi

Masi

et al. 2023

European J of Heart Fail

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Aims Transthyretin amyloid cardiomyopathy (ATTR‐CM) is often assumed to be associated with wild‐type TTR genotype (ATTRwt) in elderly patients (aged ≥70), some of whom are not offered genetic testing. We sought to estimate the prevalence, clinical characteristics and prognostic implications of transthyretin (TTR) variants among elderly patients diagnosed with ATTR‐CM. Methods and results Data from consecutive patients over 70 years of age diagnosed with ATTR‐CM at the UK National Amyloidosis Centre between January 2010 and August 2022 were retrospectively evaluated. All patients underwent clinical evaluation, biochemical tests, echocardiography and TTR genotyping. The study outcome was all‐cause mortality. The study population consisted of 2029 patients with ATTR‐CM (median age 79 years at diagnosis, 13.5% females, 80.4% Caucasian). Variant ATTR‐CM (ATTRv‐CM) was diagnosed in 20.7% (n = 421) of the study population of whom 327 (77.7%) carried V122I, 47 (11.2%) T60A, 16 (3.8%) V30M and 31 (7.3%) other pathogenic TTR variants. During a median (range) follow‐up of 29 (12–48) months, ATTRv‐CM was associated with increased all‐cause mortality compared to ATTRwt‐CM, with the poorest survival observed in V122I‐associated ATTRv‐CM (p < 0.001). Univariable and multivariable logistic regression analyses in those with ATTR‐CM showed younger age at diagnosis (odds ratio [OR] 0.85 per year, p < 0.001), female sex (OR 2.73, p < 0.001), Afro‐Caribbean ethnicity (OR 65.5, p < 0.001), atrial fibrillation (OR 0.65, p = 0.015), ischaemic heart disease (OR 0.54, p = 0.007), peripheral polyneuropathy (OR 5.70, p < 0.001) and orthostatic hypotension (OR 6.29, p < 0.001) to be independently associated with ATTRv‐CM. Conclusion Up to 20.7% of elderly patients with ATTR‐CM have a pathogenic TTR variant. These findings support routine sequencing of the TTR gene in all patients with ATTR‐CM regardless of age.

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Section: Study Populationmentioning

confidence: 99%

Prevalence, characteristics and outcomes of older patients with hereditary versus wild‐type transthyretin amyloid cardiomyopathy

Porcari

Razvi

Masi

et al. 2023

European J of Heart Fail

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“…Since 2016, a range of recommendations on noninvasive diagnostic techniques have been published [2,15,18,19], as have suggested diagnostic 'red flags' for ATTR-CM [2,6]. Together, these have increased awareness and made diagnosis easier, although uptake outside of specialist centers remains inconsistent [20,21].…”

Section: Introductionmentioning

confidence: 99%

Estimating the health benefits of timely diagnosis and treatment of transthyretin amyloid cardiomyopathy

et al. 2021

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Aim: Delayed diagnosis of transthyretin amyloid cardiomyopathy (ATTR-CM) represents a missed opportunity for intervention. This study estimates the health benefits of timely diagnosis and treatment with tafamidis. Methods: A disease simulation model was developed to predict health outcomes under scenarios of timely and delayed diagnosis and treatment. Efficacy and quality of life (QoL) profiles were derived from the pivotal tafamidis trial and diagnostic delay durations from the literature. Results: Timely diagnosis and treatment were predicted to extend mean life expectancy by 5.46 and 7.76 years, relative to delayed diagnosis, for wild-type and hereditary ATTR-CM, respectively. Corresponding QALY gains were 4.50 and 6.22. Conclusion: Timely diagnosis and treatment with tafamidis are predicted to significantly improve survival and QoL for ATTR-CM patients.

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“…We congratulate Nativi-Nicolau et al ( 4 ) on adding these important data to the published reports on this topic, thereby showing that a major contributor to this diagnostic surge for ATTR-CA is the development of diagnostic imaging tests, specifically scintigraphy with bone-avid tracers ( 22 ). Traditionally, endomyocardial biopsy was required for every patient.…”

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confidence: 92%

“…These changes were not the result of the addition of new participating centers because the number of patients by center also increased. Thus, the findings of Nativi-Nicolau et al ( 4 ) highlight a steep increase in ATTR-CA diagnosis in the United States and worldwide, particularly for ATTRwt. Moreover, the proportion of patients in New York Heart Association functional class III or IV at the time of diagnosis diminished over time, suggesting earlier diagnosis.…”

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confidence: 96%

“…In this issue of JACC: CardioOncology, Nativi-Nicolau et al ( 4 ) present the results of their study on temporal trends in the diagnosis of ATTR-CA from the THAOS (Transthyretin Amyloidosis Outcomes Survey). In this large, global database of 1,069 ATTRwt and 525 ATTRv amyloidosis patients (95% male), Nativi-Nicolau et al ( 4 ) found an impressive increase in annual ATTRwt patient inclusion, from 2 in 2005 to >100 in 2016. By contrast, annual ATTRv patient inclusion increased from 3 in 2005 to 37 in 2011 and then plateaued.…”

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confidence: 99%

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

Dorbala¹,

Clerc²

2021

JACC: CardioOncology

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Temporal Trends of Wild-type Attr Amyloidosis in the Transthyretin Amyloidosis Outcomes Survey

Cited by 5 publications

References 0 publications

Prevalence, characteristics and outcomes of older patients with hereditary versus wild‐type transthyretin amyloid cardiomyopathy

Prevalence, characteristics and outcomes of older patients with hereditary versus wild‐type transthyretin amyloid cardiomyopathy

Estimating the health benefits of timely diagnosis and treatment of transthyretin amyloid cardiomyopathy

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

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