Transthyretin Amyloid Cardiomyopathy (ATTR-CM)
Conditions
Keywords
Transthyretin amyloid cardiomyopathy (ATTR-CM), Heart Failure, Cardiomyopathy
Brief summary
The investigators of this registry aim to study the natural history, clinical presentation, characteristics, and imaging findings of patients diagnosed with ATTR amyloidosis in Thailand longitudinally. This will enable the investigators to study this disease in depth. With a better understanding, the investigators can aim to develop early screening programs for at-risk patients, raising awareness among non-cardiologists. As amyloid-specific therapies have now become available in Thailand, the findings of this registry can be helpful for epidemiological studies in Thailand.
Interventions
OTHERNo intervention
No intervention
Sponsors
Pfizer
Mahidol University
Study design
Observational model
OTHER
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to 100 Years
Healthy volunteers
Yes
Inclusion criteria
* Age \> 18 years. * All patients diagnosed with ATTR-CM (Transthyretin cardiomyopathy) who have either symptomatic or asymptomatic must have evidence of cardiac involvement by echocardiogram as defined by left ventricle wall thickness of ≥ 12 mm and with at least one of the following criteria: * Positive scintigraphy by 99mTC-DPD \[99mTC-3,3-diphosphono-1,2-propano-dicarboxylic acid\] or 99mTC-PYP \[Pyrophosphate\] or 99mTC-HMDP \[hydroxymethylene diphosphate\] with Perugini grade ≥ 2 and/or H/CL (heart to contralateral lung) ratio \> 1.5 * Positive scintigraphy by 99mTC-DPD \[99mTC-3,3-diphosphono-1,2-propano-dicarboxylic acid\] or 99mTC-PYP \[Pyrophosphate\] or 99mTC-HMDP \[hydroxymethylene diphosphate\] with Perugini grade 1 and positive cardiac biopsy tissue confirmed which Congo red stain ≥ 4 sites * Positive scintigraphy by 99mTC-DPD \[99mTC-3,3-diphosphono-1,2-propano-dicarboxylic acid\] or 99mTC-PYP \[Pyrophosphate\] or 99mTC-HMDP \[hydroxymethylene diphosphate\] with Perugini grade 1 with positive non-cardiac biopsy tissue confirmed by Congo red stain * Abnormalities on CMR (Cardiac MRI) \[Subendocardium LGE (late gadolinium enhancement) and/or elevated native T1 value and/or increased extracellular volume (ECV \> 0.4)\] with positive non-cardiac and/or cardiac tissue biopsy confirmed by Congo red stain
Exclusion criteria
* Diagnosed with amyloidosis caused by proteins other than transthyretin (light chain Amyloidosis, etc.) * Diagnosed with secondary amyloidosis
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Rate of death | 3 years |
| Rate of heart failure hospitalization | 3 years |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Transthyretin (ATTR) amyloidosis medication | 3 years | Rate of use and rate of adverse effect of Transthyretin (ATTR) amyloidosis medication |
Countries
Thailand
Contacts
Primary ContactKanchalaporn Jirataiporn, B.N.S.
Outcome results
None listed