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This is a mono-center observational ambispective study in which patients with cardiac amyloidosis evaluated at the Fondazione Policlinico Universitario Agostino Gemelli IRCCS (Rome, Italy) will be enrolled.
The primary aim is to investigate echocardiographic findings, particularly using advanced echocardiographic techniques, such as two- and three-dimensional speckle-tracking analysis, that may be helpful in the differential diagnosis between cardiac amyloidosis and other cardiomyopathies with hypertrophic phenotype.
Secondary aims are: 1) to evaluate the reversibility of myocardial damage, assessed by echocardiography, in response to a newly available specific treatment for patients with transthyretin-related cardiac amyloidosis (tafamidis) and its correlation with the clinical response 2) to investigate potential novel echocardiographic predictors of adverse cardiovascular outcomes.
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| Measure | Description | Time Frame |
|---|---|---|
| changes in echocardiographic parameters (in patients receiving or not specific treatment) | variation in global longitudinal strain, diastolic parameters and degree of ventricular hypertrophy | 6 months |
| cardiovascular outcomes | composite endpoint of death and heart failure hospitalization | 24 months |
| Measure | Description | Time Frame |
|---|---|---|
| major cardiac arrhythmias | bradyarrhythmia, new-onset atrial fibrillation, ventricular tachycardia | 24 months |
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Inclusion Criteria:
Exclusion Criteria:
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In the retrospective phase, patients with cardiac amyloidosis evaluated at the Fondazione Policlinico Agostino Gemelli between January 2020 and November 2022 will be enrolled.
In the prospective phase, patients with the same features evaluated between December 2022 and December 2028.
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Francesca Graziani, MD | Contact | 00390630154432 | francesca.graziani@policlinicogemelli.it | |
| maria chiara meucci, MD | Contact | 00390630154432 | mariachiara.meucci@guest.policlinicogemelli.it |
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Fondazione Policlinico Gemelli IRCCS | Recruiting | Roma | RM | 00168 | Italy |
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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 |