Short summary

AL amyloidosis is a plasma cell disease, in which there is an increased production of abnormal proteins (light chains). These proteins can be deposited in different organs, thereby causing organ dysfunction. The involvement of the heart causes heart failure. The main treatment is directed against the production of the light chains in the bone marrow. Treatment of heart failure is supportive. The present study will test the hypothesis that cardiac MRI is better than echocardiography to detect positive changes in the heart after treatment of the light chains.

Rationale

Treatment of heart failure in patients with cardiac AL amyloidosis is supportive. To date there is no treatment that can clear the deposition of the light chains in the heart. Further, the well-established therapies for other types of heart failure are ineffective. Similarly, typical features of cardiac amyloidosis such as the hypertrophy of the myocardium remain unchanged despite successful treatment of the light chains. Nevertheless, once the light chains are suppressed with chemotherapy, many patients experience increased functional capacity. These observations lead to the assumption that there could be small changes in the heart that are undetectable with the traditional methods of imaging. 

Both echocardiography and cardiac magnetic resonance (CMR) can accurately detect hypertrophy of the cardiac muscle in the case of cardiac amyloidosis. Furthermore, both have additional modalities (measurement of strain rate in echocardiography and measurement of diffuse fibrosis in CMR), which are more sensitive to changes in the heart and also become abnormal in a typical manner in patients with cardiac amyloidosis. Whether the latter changes also are somewhat reversible after successful treatment of the light chains is unknown.

We will examine a group of 15 patients with cardiac amyloidosis with echocardiography and CMR at the beginning of the treatment and after their light chains have been suppressed by the treatment. We will compare the results of these examinations before and after treatment to see whether they can detect any improvements in the heart. We hypothesize that the CMR method to detect diffuse fibrosis will be able to detect changes and it is more sensitive than strain rate on echocardiography for the detection of these changes. As a control group we will examine 15 patients with ATTR amyloidosis (another type of cardiac amyloidosis, for which no specific treatment exists and as such no improvement is expected) twice with a time interval of about 12 months. Lastly, we will also examine a group of about 35 normal subjects in order to have some baseline T1 values for CMR. T1 values those CMR measurements, which become abnormal due to diffuse fibrosis.

Description of the cohort

Group 1. 15 adults, men and women, diagnosed with AL amyloidosis and with involvement of the heart, as defined on either echocardiography or CMR.

Group 2. 15 adults, men and women, diagnosed with ATTR amyloidosis and with involvement of the heart, as defined on either echocardiography or CMR.

Gropu 3. 35 healthy adults, men and women between 25 and 90 years old.

Data and biological material

Patient charts for functional status, cardiac markers, level of light chains. Echocardiographic and CMR data.

Collaborating researchers and departments

Department of cardiology, Odense University Hospital

• Professor Jens Mogensen, MD PhD
• Consultant Eva Søndergaard MD PhD

Department of hematology, Odense University Hospital

• Professor Niels Abilgaard MD DmSC
• Consultant Charlotte Toftmann Hansen MD PhD

Department of Radiology and Nuclear Medicine, Esbjerg Hospital

• Associate professor Søren Hess MD PhD

Department of Clinical Pathology, Odense University Hospital

• Professor Niels Marcussen, MD DmSC
• Consultant Hanne Møller, MD

Department of Clinical Biochemistry

• Senior Researcher Hans Christian Beck