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Severe aortic stenosis is defined with a mean transvalvular pressure gradient (MTPG) > 40mmHg and a calculated aortic valve area of < 1cm2. However, a considerable proportion of patients do have a MTPG < 40mmHg due to a reduced stroke volume (stroke volume indexed to body surface area ≤ 35ml/m2) despite a normal left ventricular ejection fraction (LVEF > 50%). This entity is termed paradoxical low flow low gradient aortic stenosis (PLFLG AS) and is associated with a worse prognosis.
ATTR amyloidosis is a disease of the elderly and might coexist in patients with severe aortic stenosis. Case reports and small observational studies suggest that senile ATTR amyloidosis could be frequent but underdiagnosed in patients with aortic stenosis. There is significant overlap between PLFLG AS and cardiac amyloidosis with regard to symptoms, increasing prevalence with age, concentric hypertrophy, impaired diastolic filling of the left ventricle (LV), as well as longitudinal LV dysfunction despite preserved ejection fraction - all features, which lead to a reduction in stroke volume, the underlying mechanism of the low flow condition as observed in PLFLG AS patients.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Patients with symptomatic severe aortic stenosis | Elderly patients referred for TAVR evaluation |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Tc-99m-DPD scintigraphy | Diagnostic Test | Myocardial scintigraphy |
|
| Measure | Description | Time Frame |
|---|---|---|
| Proportion of patients with grade 2 or 3 cardiac uptake in scintigraphy and without pathological light chains in immunological assays | Evaluation of incidence rate | In-Hospital [7 days] |
| Measure | Description | Time Frame |
|---|---|---|
| All-cause and cardiovascular mortality | Clinical Endpoints | 30 days, 12months |
| CPET - maximal exercise capacity | Functional Endpoints |
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Inclusion Criteria:
Exclusion Criteria:
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Patients with symptomatic, severe aortic stenosis undergoing pre-evaluation for (transcatheter) aortic valve replacement
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| Name | Affiliation | Role |
|---|---|---|
| Stefan Stortecky, MD | Swiss Cardiovascular Center Bern | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| University Hospital Bern - Inselspital | Bern | Canton of Bern | 3010 | Switzerland |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 38085082 | Derived | Bernhard B, Leib Z, Dobner S, Demirel C, Caobelli F, Rominger A, Schutze J, Grogg H, Alwan L, Spano G, Boscolo Berto M, Lanz J, Pilgrim T, Windecker S, Stortecky S, Grani C. Routine 4D Cardiac CT to Identify Concomitant Transthyretin Amyloid Cardiomyopathy in Older Adults with Severe Aortic Stenosis. Radiology. 2023 Dec;309(3):e230425. doi: 10.1148/radiol.230425. | |
| 37581394 |
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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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| In-Hospital [7 days] |
| Dobner S, Pilgrim T, Hagemeyer D, Heg D, Lanz J, Reusser N, Grani C, Afshar-Oromieh A, Rominger A, Langhammer B, Reineke D, Windecker S, Stortecky S. Amyloid Transthyretin Cardiomyopathy in Elderly Patients With Aortic Stenosis Undergoing Transcatheter Aortic Valve Implantation. J Am Heart Assoc. 2023 Aug 15;12(16):e030271. doi: 10.1161/JAHA.123.030271. Epub 2023 Aug 10. |
| 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 |