Swiss Cardiac Amyloidosis REgistry (Swiss-CARE)

Amyloid Cardiomyopathy Clinical Trial

— B-CARE  

Official title:

Swiss Cardiac Amyloidosis REgistry (Swiss-CARE)

Clinical Trial Details — Status: Recruiting

Administrative data

• NCT number: NCT04776824
• Other study ID #: 2021-00135
• Status: Recruiting
• Start date: February 22, 2001
• Est. completion date: May 1, 2031

Study information

• Verified date: October 2023
• Source: Insel Gruppe AG, University Hospital Bern
• Contact: Christoph Gräni, MD, PhD
• Phone: +41 31 632 4508
• Email: christoph.graeni[@]insel.ch
• Is FDA regulated: No
• Study type: Observational [Patient Registry]

Clinical Trial Summary

Cardiac transthyretin amyloidosis (ATTR), caused by ventricular depositions of misfolded transthyretin, results in an infiltrative cardiomyopathy, progressing from pronounced myocardial wall thickening, diastolic and systolic dysfunction to the development of terminal heart failure. Recently, treatment options for TTR amyloidosis have become available. However costs for therapy are enormous and previous trials were not able to differentiate between patients that might benefit from treatment and those without a need for treatment.

the investigators study aims to determine markers, as assessed by cardiac magnet resonance imaging (CMR) feature tracking (FT) and T1- and T2- mapping, that might reliably indicate disease severity and could help to identify patients that might benefit from (ongoing) TTR stabilization treatment.

Description:

Cardiac transthyretin amyloidosis (ATTR), the most common amyloidosis form with cardiac involvement, is caused by tissue deposition of misfolded TTR, a transport Protein for thyroxine and retinol. Ventricular depositions of amyloid fibrils results in an infiltrative cardiomyopathy, progressing from pronounced myocardial wall thickening, to diastolic and systolic dysfunction and finally chronic heart failure.

While treatment options are now available, it remains unclear how to monitor therapy response and disease progression. No makers have been identified that predict outcome prior to initiation of therapy, thus patient selection for therapy remains challenging.

The investigators study will address these issues and will provide systematically assessed CMR data before and over the course of 18 months after therapy initiation. Clinical and laboratory follow-up will be performed every 3-6 months. The investigators study is based on an open, uncontrolled, structured collection of retrospective and prospective data from all patients diagnosed with amyloidosis at the Inselspital Bern with the aim to follow patients undergoing therapy.

The investigators hypothesize that CMR feature tracking (FT) and measures of T1- and T2- mapping, such as extracellular volume (ECV) may better correlate with disease severity and help to identify patients likely to benefit from (ongoing) TTR stabilizing therapy. Beside standard CMR assessments, the investigators will use CMR feature tracking to quantify global and regional myocardial function. FT has proven to be an excellent predictor in various cardiomyopathies.

The proposed study will evaluate the potential of CMR to identify patients likely to benefit from therapy, monitor treatment response and balance individual patient benefit and health care cost.

Recruitment information / eligibility

• Status: Recruiting
• Enrollment: 300
• Est. completion date: May 1, 2031
• Est. primary completion date: May 1, 2031
• Accepts healthy volunteers: No
• Gender: All
• Age group: 18 Years and older

Eligibility

Inclusion Criteria:

• Confirmed diagnosis of amyloidosis w/wo cardiac involvement

• General Consent

Exclusion Criteria:

• Inability to give consent or existence of a written or documented oral refusal of the data subject.<18 years of age

Related Conditions & MeSH terms

• Amyloid Cardiomyopathy
• Amyloidosis
• Cardiomyopathies

Locations

Country	Name	City	State
Switzerland	Department of Cardiology, University Hospital Bern, Inselspital, Bern	Bern

Sponsors (4)

Lead Sponsor	Collaborator
Insel Gruppe AG, University Hospital Bern	Cantonal Hospital of St. Gallen, Luzerner Kantonsspital, Triemli Hospital

Country where clinical trial is conducted

Switzerland, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	LV (left ventricle) and RV (right ventricle) function as assessed by CMR feature tracking as predictor for MACE (major adverse cardiac event)	Global and regional longitudinal (%), circumferential (%) and radial (%) strain measurements are used to quantify LV and RV function before and after therapy initiation. MACE is defined as a composite of sustained ventricular tachycardia, hospitalization for heart failure and all-cause death.	5 years
Primary	LV and RV tissue characterization as assessed by T1 and T2 mapping as predictor for MACE	Global and regional tissue characteristics are assessed by repetitive T1 and T2 mapping (global and regional T1 and T2 time (ms)) before and during therapy. MACE is defined as a composite of major cardiovascular endpoints listed above.	5 years
Primary	Late gadolinium enhancement as predictor for MACE	Global and regional myocardial tissue is characterized by gadolinium contrast agent application. The presence and extent (% and total mass (g)) of late gadolinium enhancement is evaluated as a predictor for MACE.	5 years
Primary	Extracellular volume (ECV) as predictor for MACE	ECV (%) as a marker of myocardial tissue remodelling is calculated from native and post-contrast T1 mapping and haematocrit before and during therapy.	5 years