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This is a non-interventional, prospective, retrospective, non-comparative, multi-center study.
In order not to interfere with patient management, the study is observational. Thus, no follow-up visit is imposed. The data collection will be limited to the data related to the management of the patients included throughout their follow-up.
This study is intended for all patients with a confirmed or suspected diagnosis of cardiac amyloidosis. Three cohorts will be identified: the HEAR (Healthcare European Amyloidosis Registry)-Retrospective Cohort, the HEAR(Healthcare European Amyloidosis Registry)-Retrospective-Prospective Cohort and the HEAR (Healthcare European Amyloidosis Registry)-Prospective Cohort.
Amyloidosis is a rare disease characterized by infiltration and continuous accumulation of insoluble fibrillar proteins in the extracellular matrix in various organs including kidney, nerve, liver, heart and skeletal muscle. Its prevalence is estimated at 0.5-1.3/100,000. The main forms are:
There is a strong need to help physicians better characterize the clinical and biological presentations of the disease and to improve diagnostic tools and standardize therapeutic management.
All data collected for the study are key, routine data for the condition, readily available in the patients' medical records. It is also possible to use additional and specific computerized tools to collect these data, within the participating expert centers.
Data will be recorded in an electronic observation book.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| HEAR(Healthcare European Amyloidosis Registry)-Retrospective Cohort | Retrospective collection of deceased patients data with inclusion criteria | ||
| HEAR(Healthcare European Amyloidosis Registry)-Retrospective-Prospective Cohort | Retrospective and prospective collection of patient data and real-life follow-up of patients from the date of inclusion Living patients who met the inclusion criteria | ||
| HEAR(Healthcare European Amyloidosis Registry)-Prospective Cohort | Prospective data collection and real-life follow-up of patients from the date of inclusion These patients are either newly followed in the centre with the inclusion criteria |
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| Measure | Description | Time Frame |
|---|---|---|
| The clinical, biological and imaging characteristics of patients with cardiac amyloidosis | Describe the clinical, biological and imaging characteristics of patients with cardiac amyloidosis. | 6 years |
| Measure | Description | Time Frame |
|---|---|---|
| he prevalence and incidence of the different types of amyloidosis and their evolution. | To estimate the prevalence and incidence of the different types of amyloidosis and their evolution. | 6 years |
| The therapeutic management (cardiological and specific), their beneficial and secondary effects. |
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INCLUSION CRITERIA
Patients must meet all of the following inclusion criteria to be included in the study:
Prospective Cohort:
Patients referred or who have been referred to the participating centre for suspected amyloidosis.
Patient who signed the patient information "Prospective Cohort" note
Retro-prospective Cohort:
Patient already followed in the center with a confirmed diagnosis of amyloidosis Patient who signed the "retro-prospective cohort" patient information note
Retrospective cohort:
Deceased patients followed in the center with a confirmed diagnosis of amyloidosis
Study participants will not be compensated for their participation
EXCLUSION CRITERIA The patient has expressed his/her refusal to participate Participation in another study, even an interventional one, is not a criterion for non-inclusion.
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This study is intended for all patients with a confirmed or suspected diagnosis of cardiac amyloidosis.
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Mounira Kharoubi | Contact | +33650029257 | mounira.kharoubi@gmail.com | |
| Rébecca Gene | Contact | +33628274249 | rebecca.gene@gmail.com |
| Name | Affiliation | Role |
|---|---|---|
| Thibaud Pr Damy | Henri Mondor University Hospital | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Hôpital Henri Mondor | Recruiting | Créteil | 94000 | 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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Describe the therapeutic management (cardiological and specific), their beneficial and secondary effects. |
| 6 years |
| 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 |