Amyloid CardiomyopathyB-CARENCT04776824
Swiss Cardiac Amyloidosis REgistry (Swiss-CARE)
An observational study of Amyloid Cardiomyopathy, run by Insel Gruppe AG, University Hospital Bern at 7 sites in Switzerland.
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. Read more about this study
- Who is it for?
- Ages 18 years and older
- Conditions the study is for
- Amyloid Cardiomyopathy
- How many people
- 300 participants
- Where
- 7 sitesSwitzerland
- Study dates
- 22 Feb 2001to 1 May 2031
- How long will I take part?
- 10 Years
- Phase
- Observational
- Are there results?
- Not yet: the study is under way
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages 18 years and older
- All sexes
- No healthy volunteers
- Adult, Older adult
Study population: Confirmed diagnosis of amyloidosis w/wo cardiac involvement
Who may be able to join
- Confirmed diagnosis of amyloidosis w/wo cardiac involvement
- General Consent
Who cannot join
- Inability to give consent or existence of a written or documented oral refusal of the data subject.<18 years of age
Trial locations and contacts
7 sites in 1 country. Contact the study team or a site near you to ask about taking part.
7 sites in 1 country
Switzerland7 sites, 7 recruiting
- Recruiting
USB
Basel, 4031
Otmar Pfister, Prof. Dr., +41 61 328 74 82, otmar.pfister@unibas.ch
- Recruiting
Department of Cardiology, University Hospital Bern, Inselspital, Bern
Bern, 3010
Christoph Gräni, Prof, +41 31 632 45 08, christoph.graeni@insel.ch
- Recruiting
HUG
Geneva, 1211
Philippe Meyer, +41 22 372 13 13 ext. Dr., philippe.meyer@hug.ch
- Recruiting
CHUV
Lausanne, 1011
Sarah Hugelshofer, Dr. med., +41 21 314 11 11, sarah.hugelshofer@chuv.ch
- Recruiting
LUKS
Lucerne, 6000
Simon Stämpfli, Dr. med, +41 41 205 51 48, simon.staempfli@luks.ch
- Recruiting
KSSG
Sankt Gallen, 9007
Niklas Ehl, Dr. med, +41 71 494 10 51, niklas.ehl@h-och.ch
- Recruiting
Stadtspital Triemli
Zurich, 8063
Annina Studer Brüngger, Dr. med., +41 44 416 34 12, annina.studer@stadtspital.ch
About this study
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.
Read the 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.
- Conditions
- Amyloid Cardiomyopathy
- Keywords
- Cardiac Amyloidosis, Tafamidis, CMR feature tracking, Strain, T1 and T2 Mapping, Outcomes
- Medical subject headings
- Amyloid Neuropathies, Familial, Sprains and Strains
What participants receive
What participants receive. In a randomised study each person is assigned to one group by chance.
| Study group | Role | What they receive |
|---|---|---|
| Patients with confirmed amyloidosis | Confirmed diagnosis of amyloidosis w/wo cardiac involvement |
What is being measured
The primary outcome is the main question the study is designed to answer.
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.
Measured: 5 years
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.
Measured: 5 years
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.
Measured: 5 years
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.
Measured: 5 years
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted2 Mar 2021
- Study start22 Feb 2001
- Primary completion1 May 2031 (estimated)
- Study completion1 May 2031 (estimated)
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Publications (3)Papers the sponsor has linked to this study.
- Other trials for Amyloid CardiomyopathyStudies of the same condition, open and under way.
- More trials run by Insel Gruppe AG, University Hospital BernThe sponsor's other registered studies.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Observational
- Observational model
- Case only
- Time perspective
- Prospective
- Enrolment
- 300 participants (estimated)
- Target duration
- 10 Years
Sponsor and investigators
- Lead sponsor
- Insel Gruppe AG, University Hospital Bern
- Sponsor type
- University, hospital or other
- Responsible party
- Sponsor
- Investigators
- Christoph Gräni, MD, PhD, Principal investigator, Department of Cardiology, University Hospital Bern, Inselspital, Bern
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT04776824
- Sponsor's protocol ID
- 2021-00135
- Acronym
- B-CARE
- Status last verified
- May 2025
- First submitted
- 25 Feb 2021
- Last updated
- 4 Nov 2026
- Results posted
- No
- Data monitoring committee
- No
- FDA-regulated drug
- No
- FDA-regulated device
- No
- Individual data sharing
- No
Publications
Dobner S, Tawo S, Noti F, Wieser F, Grani C, Nitsche C, Reichlin T, Hunziker L, Haeberlin A. Indication and electrical performance of conventional, resynchronization, and conduction system pacing in transthyretin amyloid cardiomyopathy. Heart Rhythm O2. 2026 Mar 27;7(7):1289-1301. doi: 10.1016/j.hroo.2026.03.026. eCollection 2026 Jul.
PubMed 42488169Caobelli F, Popescu CE, Gozlugol N, Rominger A, Zangeneh FA, Munsch LH, Ciocca N, Stortecky S, Dobner S, Hundertmark M, Grani C. Correlation of global and regional quantitative 99m Tc-3,3-diphosphono-1,2 propanodicarboxylicacid single-photon emission computed tomography with echocardiography in patients with suspected transthyretin-related cardiomyopathy. Nucl Med Commun. 2026 Jun 1;47(6):638-643. doi: 10.1097/MNM.0000000000002138. Epub 2026 Mar 10.
PubMed 41804669Caobelli F, Gozlugol N, Bakula A, Rominger A, Schepers R, Stortecky S, Hunziker Munsch L, Dobner S, Grani C. Prognostic Value of [99mTc]Tc-DPD Quantitative SPECT/CT in Patients with Suspected and Confirmed Amyloid Transthyretin-Related Cardiomyopathy and Preserved Left Ventricular Function. J Nucl Med. 2024 Jun 3;65(6):944-951. doi: 10.2967/jnumed.123.266926.
PubMed 38724281
Study at a glance
From the registry record, last updated by the sponsor on 4 Nov 2026. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- Swiss Cardiac Amyloidosis REgistry (Swiss-CARE)
- ClinicalTrials.gov ID
- NCT04776824
- Acronym / sponsor ID
- B-CARE / 2021-00135
- Conditions
- Amyloid Cardiomyopathy
- Keywords
- Cardiac Amyloidosis, Tafamidis, CMR feature tracking, Strain, T1 and T2 Mapping, Outcomes
- Study type
- Observational
- Recruitment status
- Recruiting
- Enrolment
- 300 participants (estimated)
- Lead sponsor
- Insel Gruppe AG, University Hospital BernUniversity, hospital or other
- Study start
- 22 Feb 2001 (actual)
- Primary completion
- 1 May 2031 (estimated)
- Study completion
- 1 May 2031 (estimated)
- First posted
- 2 Mar 2021 (actual)
- Results
- Not posted in the source record
Clinical summary
The research question as the sponsor registered it. This describes what the study plans to test, not what it found.
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 design
- Observational model
- Case only
- Time perspective
- Prospective
- Target follow-up
- 10 Years
- Number of arms
- 1
| Arm | Type | Receives |
|---|---|---|
| Patients with confirmed amyloidosis | – | –Confirmed diagnosis of amyloidosis w/wo cardiac involvement |
Who may qualify
- Ages
- 18 years and older
- Sex
- All
- Healthy volunteers
- Not accepted
- Age groups
- Adult, Older adult
- Study population
- Confirmed diagnosis of amyloidosis w/wo cardiac involvement
Key inclusion criteria (2 listed)
- Confirmed diagnosis of amyloidosis w/wo cardiac involvement
- General Consent
Key exclusion criteria (1 listed)
- Inability to give consent or existence of a written or documented oral refusal of the data subject.<18 years of age
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Primary outcome
LV (left ventricle) and RV (right ventricle) function as assessed by CMR feature tracking as predictor for MACE (major adverse cardiac event)
Time frame: 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.
LV and RV tissue characterization as assessed by T1 and T2 mapping as predictor for MACE
Time frame: 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.
Late gadolinium enhancement as predictor for MACE
Time frame: 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.
Extracellular volume (ECV) as predictor for MACE
Time frame: 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.
Results and safety
This study is still under way. Results are not available in the source record yet.
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 4 Nov 2026.
- Derived from this study: PubMed 42488169Dobner S, Tawo S, Noti F, Wieser F, Grani C, Nitsche C, Reichlin T, Hunziker L, Haeberlin A. Indication and electrical performance of conventional, resynchronization, and conduction system pacing in transthyretin amyloid cardiomyopathy. Heart Rhythm O2. 2026 Mar 27;7(7):1289-1301. doi: 10.1016/j.hroo.2026.03.026. eCollection 2026 Jul.
- Derived from this study: PubMed 41804669Caobelli F, Popescu CE, Gozlugol N, Rominger A, Zangeneh FA, Munsch LH, Ciocca N, Stortecky S, Dobner S, Hundertmark M, Grani C. Correlation of global and regional quantitative 99m Tc-3,3-diphosphono-1,2 propanodicarboxylicacid single-photon emission computed tomography with echocardiography in patients with suspected transthyretin-related cardiomyopathy. Nucl Med Commun. 2026 Jun 1;47(6):638-643. doi: 10.1097/MNM.0000000000002138. Epub 2026 Mar 10.
- Derived from this study: PubMed 38724281Caobelli F, Gozlugol N, Bakula A, Rominger A, Schepers R, Stortecky S, Hunziker Munsch L, Dobner S, Grani C. Prognostic Value of [99mTc]Tc-DPD Quantitative SPECT/CT in Patients with Suspected and Confirmed Amyloid Transthyretin-Related Cardiomyopathy and Preserved Left Ventricular Function. J Nucl Med. 2024 Jun 3;65(6):944-951. doi: 10.2967/jnumed.123.266926.
- Individual participant dataSharing planned: No
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Referral and contact
AzTrials does not enrol participants or take referrals. Contact the study team or a site listed below; they decide who can take part.
Christoph Gräni, MD, PhD principal investigator
Department of Cardiology, University Hospital Bern, Inselspital, Bern
7 sites in 1 country: see every site and its status.
Questions about this trial
What is this clinical trial studying?
An observational study of Amyloid Cardiomyopathy, run by Insel Gruppe AG, University Hospital Bern at 7 sites in Switzerland.
The full registered description is in the About this study section and the official record.
Who may be eligible to take part?
The sponsor lists 2 inclusion and 1 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.
Is this clinical trial currently recruiting?
The record says: looking for participants now, as of the sponsor's last update on 4 Nov 2026. Status can change between updates, so confirm with the study contact before making plans.
Where is the study taking place?
The record lists 7 sites in Switzerland. Whether a site is taking part right now should be confirmed with that site. See every site.
What are the possible benefits and risks?
They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.
Are results available for this study?
This study is still under way. Results are not available in the source record yet.
How can a doctor refer a patient or contact the research team?
Contact Christoph Gräni, MD, PhD (+41 31 632 4508, christoph.graeni@insel.ch), or a site near the patient. AzTrials does not enrol participants or take referrals.
What does the primary outcome measure?
LV (left ventricle) and RV (right ventricle) function as assessed by CMR feature tracking as predictor for MACE (major adverse cardiac event) (time frame: 5 years). The study lists 4 primary outcomes in all. It is what the study was designed to measure, not a finding.
Where can I read the original study information?
On the ClinicalTrials.gov record for NCT04776824, which also lists 3 linked publications.
Does a listing mean the treatment is approved or proven to work?
No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.
How often is this information updated?
AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 4 Nov 2026.