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Cardiac Involvement and Treatment in AL Amyloidosis
Quick Takes
- In a cohort of newly diagnosed AL amyloidosis patients, cMRI with extracellular volume was the only significant predictor of survival with serum cardiac biomarkers and echo changes lacking predictive power.
- When combined with hematological response to treatment, cMRI improved prognostication and may serve as a tool for guiding therapy.
Study Questions:
What is the role of cardiac magnetic resonance imaging (cMRI) with extracellular volume (ECV) mapping in systemic light chain (AL) amyloidosis compared with serum biomarkers and echocardiography?
Methods:
This study analyzed all patients with AL amyloidosis from a prospective registry of newly diagnosed patients at the National Amyloidosis Center in the United Kingdom. All patients with an echo, serum biomarkers, and cMRI with ECV mapping were included. The primary outcome was all-cause mortality and hematological response defined as no response, partial response, very good partial response, and complete response.
Results:
A total of 560 patients with newly diagnosed AL amyloidosis were included. All patients received bortezomib. The median age was 68 years, 62% were male, and median NT-proBNP was 2148 ng/mL. During median follow-up of 40.5 months, 38% of patients died and in multivariable models, only ECV was an independent predictor of survival with patients. In the landmark 1-month analysis, patients with ECV <0.30% (no cardiac involvement) and ECV 0.31-0.40% (early cardiac AL amyloidosis) had no difference in survival regardless of hematological response. However, survival in patients with ECV >0.40% (moderate and severe cardiac infiltration) depended on hematological response. In the landmark analysis at 6 months, survival in patients with ECV <0.30% did not depend on hematological response but for patients with ECV ≥0.30%, survival depended on hematological response. In patients with ECV <0.40%, survival did not depend on speed of achieving deep hematological response. In patients with ECV >0.40%, survival was better in patients achieving earlier deep hematological response.
Conclusions:
In a prospective cohort of newly diagnosed AL amyloidosis patients, ECV mapping on cMRI was an independent predictor of mortality and extent of ECV was predictive of prognosis when combined with extent and speed of hematological response.
Perspective:
Systemic AL amyloidosis commonly involves the heart, which is the primary driver for mortality. At present, diagnosis of cardiac involvement largely relies on serum biomarkers, characteristic echo abnormalities, or an endomyocardial biopsy. Serum biomarkers, however, vary based on body mass index, arrhythmia, etc., and may not always represent cardiac AL. Echo changes of left ventricular hypertrophy can also be seen in hypertension and may not be picked up in early cardiac AL. This study suggests that cMRI with ECV measurement can characterize cardiac involvement. In addition, when compared to biomarkers and echo in a multivariable model, ECV was the only independent predictor of prognosis. In combination with hematological response, it improved prognostication, suggesting it can guide therapy changes in case of nonresponders.
Clinical Topics: Cardiovascular Care Team, Heart Failure and Cardiomyopathies, Noninvasive Imaging, Acute Heart Failure, Magnetic Resonance Imaging
Keywords: Heart Failure, Immunoglobulin Light-chain Amyloidosis, Magnetic Resonance Imaging
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