Thailand ATTR-CM Registry

Mahidol University

Status: Enrolling

Conditions: Transthyretin Amyloid Cardiomyopathy (ATTR-CM)

Treatments: Other: No intervention

Study type: Observational

Funder types: Other; Industry

Identifiers

NCT06338696

COA-CREC070/2023

Details and patient eligibility

About

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.

Enrollment: 105 estimated patients

Sex: All

Ages: 18 to 100 years old

Volunteers: Accepts Healthy Volunteers

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

Trial design

105 participants in 1 patient group

ATTR Cardiomyopathy

Description:

Patients diagnosed with ATTR-CM (Transthyretin cardiomyopathy)

Treatment:

Other: No intervention