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In 2024, the prevalence of TTR cardiac amyloidosis is difficult to determine precisely. Indeed, the disease has long been underdiagnosed due to a lack of effective treatment and non-invasive diagnostic methods. The prevalence of ATTRwt is estimated at 155 to 191 cases per million person-years, with an incidence of 36 cases per million person-years. Both incidence and prevalence increase with age.
The most common presentation of TTR cardiac amyloidosis is heart failure with preserved ventricular ejection fraction (LVEF).
The natural history of TTR cardiac amyloidosis is progression to restrictive heart disease with all the complications described above.
The median time between diagnosis and the first cardiac symptoms was 39 months. Currently, the median survival in France is approximately 3.5 years, but survival appears to be improving, with a mortality rate of 73.5% at 30 months between 2002 and 2006, compared to 15% at 60 months between 2017 and 2022.
The number of patients diagnosed with TTR cardiac amyloidosis appears to be increasing. The overwhelming majority of available data concerns patients treated in cardiology. However, the issue of TTR cardiac amyloidosis concerns all specialists caring for patients with heart failure, particularly in internal medicine due to the high number of admissions from emergency departments, with a significant proportion of patients suffering from cardiac decompensation.
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| Measure | Description | Time Frame |
|---|---|---|
| Describe the number of cases of transthyretin (TTR) cardiac amyloidosis referred to internal medicine between 2013 and 2023. | Up to 10 years |
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Inclusion Criteria:
Exclusion Criteria:
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Sujet majeur (≥18 ans) avec un diagnostic d'amylose à TTR
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Emmanuel ANDRES, MD, PhD | Contact | 33 3 88 12 71 33 | emmanuel.andres@chru-strasbourg.fr |
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Service de Médecine Interne - CHU de Strasbourg - France | Recruiting | Strasbourg | 67091 | France |
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| ID | Term |
|---|---|
| D028227 | Amyloid Neuropathies, Familial |
| ID | Term |
|---|---|
| D020271 | Heredodegenerative Disorders, Nervous System |
| D019636 | Neurodegenerative Diseases |
| D009422 | Nervous System Diseases |
| D017772 | Amyloid Neuropathies |
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| D010523 | Peripheral Nervous System Diseases |
| D009468 | Neuromuscular Diseases |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D028226 | Amyloidosis, Familial |
| D008661 | Metabolism, Inborn Errors |
| D008659 | Metabolic Diseases |
| D009750 | Nutritional and Metabolic Diseases |
| D000686 | Amyloidosis |
| D057165 | Proteostasis Deficiencies |