Swiss Cardiac Amyloidosis REgistry (Swiss-CARE)

Recruiting

• Conditions: Amyloid Cardiomyopathy

• Registration Number: NCT04776824
• Lead Sponsor: Insel Gruppe AG, University Hospital Bern

Brief 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.

Detailed 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.

Study Timeline

• Study Start: 2001-02-22
• Study First Submitted: 2021-02-25
• Study First Submitted QC: 2021-02-25
• Study First Posted: 2021-03-02
• Status Verified: 2025-05
• Last Update Submitted: 2025-05-01
• Last Update Posted: 2025-05-06
• Primary Completion: 2031-05-01
• Study Completion: 2031-05-01

Recruitment & Eligibility

• Status: RECRUITING
• Sex: All
• Target Recruitment: 300

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

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
LV (left ventricle) and RV (right ventricle) function as assessed by CMR feature tracking as predictor for MACE (major adverse cardiac event)	5 years	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.
Late gadolinium enhancement as predictor for MACE	5 years	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.
LV and RV tissue characterization as assessed by T1 and T2 mapping as predictor for MACE	5 years	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.
Extracellular volume (ECV) as predictor for MACE	5 years	ECV (%) as a marker of myocardial tissue remodelling is calculated from native and post-contrast T1 mapping and haematocrit before and during therapy.

Trial Locations

Locations (7)

USB; 🇨🇭 Basel, Switzerland

HUG; 🇨🇭 Genf, Switzerland

CHUV; 🇨🇭 Lausanne, Switzerland

LUKS; 🇨🇭 Luzern, Switzerland

KSSG; 🇨🇭 St. Gallen, Switzerland

Stadtspital Triemli; 🇨🇭 Zürich, Switzerland

Department of Cardiology, University Hospital Bern, Inselspital, Bern; 🇨🇭 Bern, Switzerland