Light-chain cardiac amyloidosis (stiff heart syndrome) is a life-limiting condition that occurs when plaques of protein called amyloid build up in heart muscle, affecting its ability to pump blood, and without treatment can rapidly lead to heart failure and death. However, assessing the condition has been difficult, as while clinicians can detect the presence of amyloid in the heart, there has been no safe test to measure the amount. This has also meant there has been no way of measuring the therapeutic effect of chemotherapy – the normal first line treatment. A patient’s response is currently assessed with indirect biological markers, but these do not measure the amount (or reduction) of cardiac amyloid – the drug’s ultimate target – and doctors find the markers less useful when trying to assess second-line chemotherapy treatments. Now, an advanced form of cardiac MRI has for the first-time enabled clinicians to measure the effectiveness of chemotherapy in patients with ‘stiff heart syndrome’.

Researchers at University College London (London, UK) have, for 10 years, been developing and refining Cardiovascular Magnetic Resonance (CMR) Extracellular Volume Mapping (ECV) for amyloid. This non-invasive technique enables clinicians to measure both the presence and amount of amyloid protein using MRI. Now for the first time they have used the technology to evaluate the success of chemotherapy treatment, by assessing cardiac amyloid regression or progression.

For the study 176 patients with light-chain cardiac amyloidosis had Cardiovascular Magnetic Resonance with Extracellular Volume Mapping. CMR scans with ECV mapping were done at diagnosis, and then at six, 12 and 24 months after starting chemotherapy. The advanced MRI technique allowed researchers to accurately measure the amount of amyloid protein in hearts, and, for the first-time ever, to measure the changes in response to chemotherapy on repeat scans. By measuring the changes they could detect which patients would have a better or worse prognosis. Further, combining the results with blood tests for the disease, it was found almost 40% of patients had a substantial improvement (reduction) in amyloid deposition, something that was not thought to be possible before – showing how effective chemotherapy can be. The researchers believe that the MRI technique should now be used immediately to diagnose and assess all cases of light-chain cardiac amyloidosis.

“Since MRI scans are widely available, by developing the use of ECV mapping in a machine that already is used for these patients, we hope that its use can be made available to more patients to help improve their care,” said Professor Marianna Fontana (UCL Division of Medicine), senior author and a British Heart Foundation (BHF) Clinical Fellow. “The aim would be to use these scans routinely for all patients with the disease to help doctors monitor the response to chemotherapy to help improve patient survival, which is very poor in patients who do not respond to treatment.” 

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