Research: Advanced MRI benefits patients with heart stiffening disease

Researchers at UCL and the Royal Free Hospital have created an advanced kind of cardiac MRI that allows doctors to evaluate the efficacy of chemotherapy in patients with the terminal ailment of stiff heart syndrome.

The finding, which was reported in the European Heart Journal, allows clinicians to better direct treatment plans, which in turn improves patient prognosis, according to researchers.

When amyloid protein plaques form in the heart muscle, they limit the heart’s ability to pump blood. This condition is known as stiff heart syndrome and, if left untreated, can quickly result in heart failure and death.

However, diagnosing the condition has proven challenging because there is no reliable test to determine the amount of amyloid in the heart, even if clinicians can feel its presence.

This also meant that chemotherapy—the standard first-line treatment—could not be evaluated clinically.

Currently, clinicians employ indirect biological markers to evaluate a patient’s response, but these don’t measure the quantity (or reduction) of cardiac amyloid, the drug’s primary target, and specialists find the indicators less helpful when evaluating second-line chemotherapy therapies.

Cardiovascular Magnetic Resonance (CMR) Extracellular Volume Mapping (ECV) for amyloid has been developed and improved upon for the past ten years by UCL researchers at the National Amyloidosis Centre. Clinicians can use MRI to assess the quantity and presence of amyloid protein utilizing this non-invasive approach.

They are now using the technology for the first time to determine if cardiac amyloid is advancing or going backward in order to assess the efficacy of chemotherapy treatment.

For the study, Cardiovascular Magnetic Resonance with Extracellular Volume Mapping was performed on 176 individuals with light-chain cardiac amyloidosis. At diagnosis and then six, 12, and twenty-four months following the commencement of chemotherapy, CMR scans with ECV mapping were conducted.

Researchers were able to precisely detect the amount of amyloid protein in hearts using the cutting-edge MRI technique, and they were also able to measure for the first time ever alterations brought on by chemotherapy on subsequent scans. They may identify which patients would have a better or worse prognosis by measuring the changes.

First author, Dr. Ana Martinez-Naharro (UCL Division of Medicine) said: “In this study, we were assessing the ability and value of CMR to carry out ECV mapping to measure directly the changes in amyloid proteins in the heart in response to chemotherapy and how they correlate with the indirect markers that currently exist.

“The scans and data made available using this technique, gave us the information to both see the amount of amyloid protein and also the regression in amyloid during the course of chemotherapy treatment.

“This is incredibly valuable for clinicians; knowing the amount, rather than just the presence of amyloid, means they can better guide treatment option, by more accurately deciding timing and protocol of second-line chemotherapy treatments.”

Senior author, Professor Marianna Fontana (UCL Division of Medicine), a British Heart Foundation (BHF) Clinical Fellow, said this UCL-developed 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.

“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.”

Professor Fontana added: “Twenty years ago, another MRI technique called T2*, was proven to be able to do the same thing for measuring the amount of iron in the heart in a condition called thalassemia.

“For the last two decades, it has been used to help monitor patients’ response to treatment and allow doctors to make changes along the way. As a result, survival has improved for thalassemia.

“As T2* has changed the landscape for this condition, tracking changes using ECV mapping of the heart may completely change the landscape for patients with amyloidosis, with the potential of guide treatment and, in the process, lead to improvements in patient outcomes.”

This article originally appeared in Miragenews and has been updated.