PCV67 Epidemiology of Transthyretin Amyloid Cardiomyopathy (ATTR-CM) in France: EPACT, a Study Based on the French Nationwide Claims Database Snds

Damy, Thibaud; Bourel, G.; Slama, M.; Algalarrondo, Vincent; Lairez, Olivier; Pelcot, F.; Durand‐Zaleski, Isabelle; Lilliu, H; Bartoli, M.; Fievez, S.; Granghaud, A.; Rudant, Jérémie; Neuville, B. de; Rault, C.; Charron, P.

doi:10.1016/j.jval.2020.08.558

Cited by 6 publications

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“…These shares are higher compared with findings in meta‐analyses 8 , 11 and studies based on confirmed cases. 23 , 24 However, recent registry studies from France 12 and Japan 14 have reported higher estimates of female share, 33.1% and 53.7%, respectively. These recent results, based on unconfirmed cases, potentially suggest that women may be underdiagnosed.…”

Section: Discussionmentioning

confidence: 99%

“…10,11 For instance, studies including autopsy for ATTR-CM diagnosis have found higher proportions of women than studies that diagnose ATTR-CM with common measures in living patients. 8 Few studies so far have used large register-based data sets to estimate the prevalence and characteristics of ATTR-CM [12][13][14] and even fewer have been based in Europe. The studies differ in methodology and estimates of prevalence from these studies range between 4 and 17 cases per 100 000.…”

Section: Introductionmentioning

confidence: 99%

“…Few studies so far have used large register‐based data sets to estimate the prevalence and characteristics of ATTR‐CM 12 , 13 , 14 and even fewer have been based in Europe. The studies differ in methodology and estimates of prevalence from these studies range between 4 and 17 cases per 100 000.…”

Section: Introductionmentioning

confidence: 99%

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Prevalence, characteristics, and mortality of patients with transthyretin amyloid cardiomyopathy in the Nordic countries

et al. 2022

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Aims Transthyretin amyloid cardiomyopathy (ATTR-CM) is a progressive condition caused by deposition of transthyretin amyloid fibrils in the heart and is associated with poor quality of life and a shortened lifespan. This study aimed to describe the prevalence, clinical characteristics, and mortality of patients with ATTR-CM, using multiple national health registers in Denmark, Finland, Norway, and Sweden. Methods and resultsTransthyretin amyloid cardiomyopathy patients were identified during 2008-2018 using a combination of diagnosis codes for amyloidosis and heart disease and were matched to patients with non-ATTR heart failure (HF). An identical study design was used in each country to facilitate comparison and aggregation of results. A total of 1930 ATTR-CM patients were identified from national health registers in the four countries. In 2018, prevalence of ATTR-CM per 100 000 inhabitants ranged from 1.4 in Denmark to 5.0 in Sweden; a steep increase over time was observed in Sweden and Norway. Median survival from diagnosis was 30 months for ATTR-CM patients and 67 months for matched HF patients. Survival was significantly lower for female than for male ATTR-CM patients (median survival: 22 and 36 months), while no significant difference was observed in the HF cohort. Conclusions This study provides the first nationwide estimates of the prevalence, clinical characteristics, and mortality of patients with ATTR-CM, using identical study design across several countries. Findings corroborate previous case series showing high mortality in ATTR-CM, two-fold higher than for other HF patients and higher in women than men, highlighting the need for more precise and early diagnosis to reduce the disease burden.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Prevalence, characteristics, and mortality of patients with transthyretin amyloid cardiomyopathy in the Nordic countries

et al. 2022

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“…Hereditary ATTR-CM is a rare disease; its prevalence is unknown, and varies between regions due to geographical clustering of TTR mutations [7]. Little information is available on the incidence of ATTR-CM; a study from France suggested an incidence rate for both subtypes combined of 1.8/100,000 in 2017 [11].…”

Section: Introductionmentioning

confidence: 99%

Impact of Delayed Diagnosis and Misdiagnosis for Patients with Transthyretin Amyloid Cardiomyopathy (ATTR-CM): A Targeted Literature Review

et al. 2021

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Introduction Transthyretin amyloid cardiomyopathy (ATTR-CM) is a progressive, fatal and under-recognized disease. This targeted literature review assessed the extent and consequences of diagnostic delay and misdiagnosis in ATTR-CM. Methods The Embase database was searched together with proceedings of eight cardiology conferences to identify publications or abstracts on ATTR-CM. Outcomes of interest were time from symptom onset to diagnosis, rates of delayed diagnosis and misdiagnosis, and costs, healthcare resource use or clinical outcomes whilst undiagnosed/misdiagnosed. Results Twenty-three articles were included. Weighted means of reported mean and median diagnostic delays were 6.1 and 3.4 years for wild-type (ATTRwt-CM) and 5.7 and 2.6 years for hereditary (ATTRv-CM). Misdiagnosis occurred in 34–57% of patients when reported. Evaluation and misdiagnosis by multiple healthcare providers before receiving an ATTR-CM diagnosis was common, and there was evidence that patients undergo unnecessary or inappropriate evaluations or treatments while misdiagnosed. Diagnostic “red flags” were reported to be underused. Data on the consequences of delay for patients and health systems were sparse, but given the progressive nature of ATTR-CM, delay is likely to have adverse consequences. Conclusion ATTR-CM patients commonly experience diagnostic delay and misdiagnosis. Efforts are required to provide timely diagnosis so that patients can benefit from earlier access to new disease-modifying therapies. Supplementary Information The online version contains supplementary material available at 10.1007/s40119-021-00219-5.

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“…9 Therefore, early diagnosis of ATTR-CM is essential to ensure that patients can benefit from disease-modifying treatment in order to maximize quality of life and reduce the need for healthcare interactions. 9,10 Although the epidemiology of ATTR-CM is beginning to emerge in published research using register data, 8,[11][12][13] treatment patterns for ATTR-CM patients and their healthcare resource use are not well documented. There is a large body of work on the humanistic and economic burden of heart failure from around the world, [14][15][16] but only few studies on the healthcare resource use of ATTR-CM.…”

Section: Introductionmentioning

confidence: 99%

Healthcare resource use of patients with transthyretin amyloid cardiomyopathy

et al. 2022

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Aims Transthyretin amyloid cardiomyopathy (ATTR-CM) is the cardiac manifestation of transthyretin amyloidosis (ATTR).The aim of this study was to estimate healthcare resource use for ATTR-CM patients compared with heart failure (HF) patients, in Denmark, Finland, Norway, and Sweden. Methods and results Data from nationwide healthcare registers in the four countries were used. ATTR-CM patients were defined as individuals diagnosed with amyloidosis and cardiomyopathy or HF between 2008 and 2018. Patients in the ATTR-CM cohort were matched to patients with HF but without ATTR-CM diagnosis. Resource use included number of visits to specialty outpatient and inpatient hospital care. A total of 1831 ATTR-CM and 1831 HF patients were included in the analysis. The mean number of hospital-based healthcare contacts increased in both the ATTR-CM and HF cohort during 3 years pre-diagnosis and was consistently higher for the ATTR-CM cohort compared with the HF cohort, with 6.1 [

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PCV67 Epidemiology of Transthyretin Amyloid Cardiomyopathy (ATTR-CM) in France: EPACT, a Study Based on the French Nationwide Claims Database Snds

Cited by 6 publications

References 0 publications

Prevalence, characteristics, and mortality of patients with transthyretin amyloid cardiomyopathy in the Nordic countries

Prevalence, characteristics, and mortality of patients with transthyretin amyloid cardiomyopathy in the Nordic countries

Impact of Delayed Diagnosis and Misdiagnosis for Patients with Transthyretin Amyloid Cardiomyopathy (ATTR-CM): A Targeted Literature Review

Healthcare resource use of patients with transthyretin amyloid cardiomyopathy

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