Cardiovascular magnetic resonance for therapy monitoring in cardiomyopathies

Figures

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  Figure 1. Cardiac magnetic resonance (CMR) images in a patient with hypertrophic cardiomyopathy (HCM) at baseline (pre-treatment) and after 1 year of treatment with aficamten (post-treatment). Follow-up CMR demonstrates a reduction in left ventricular (LV) mass (107 to 94 g) and maximal wall thickness (19 to 17 mm), consistent with LV reverse remodelling during myosin inhibitor therapy. Native T1 relaxation time decreased from an elevated baseline of 1103 to 980 ms following aficamten therapy (institutional normal reference range: 996–1092 ms).

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  Figure 2. Cardiovascular magnetic resonance (CMR) for therapy monitoring in light chain cardiac amyloidosis (AL-CA). (Panel A): CMR images from a patient with AL-CA acquired at baseline and after treatment with daratumumab–cyclophosphamide–bortezomib–dexamethasone (Dara-CyBorD). At baseline (top panels), late gadolinium enhancement (LGE) images showed diffuse myocardial hyperintensity in both ventricles and both atria (hardly distinguishable from bloodpool hyperintensity), consistent with high myocardial gadolinium uptake due to diffuse amyloid infiltration. Native T1 mapping (global native T1 1097 ms) and extracellular (ECV) mapping (global ECV 62%) were very high, confirming myocardial amyloid infiltration. After two years of treatment (bottom panels), LGE images still showed diffuse amyloid infiltration, while native T1 mapping (global native T1 1079 ms) and ECV mapping (globale ECV 45%) were significantly improved compared to baseline, consistent with a lower myocardial amyloid burden after therapy. (Panel B): CMR images from a patient with multiple myeloma showing the natural progression of AL-CA. Baseline images (top panels) demonstrated limited subendocardial LGE in the…

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  Figure 3. Cardiovascular magnetic resonance (CMR) for therapy monitoring in Fabry disease. A middle-aged lady with Fabry disease underwent CMR at baseline and after 2 years of treatment with enzyme replacement therapy (ERT). Baseline CMR (top panels) demonstrated mild intramyocardial late gadolinium enhancement (LGE) in the basal inferolateral left ventricular wall (arrow), as well as a typical diffuse reduction of native T1 mapping values (global native T1 879 ms). After therapy (bottom panels), the inferolateral LGE area remained unchanged (arrow), but native T1 mapping values significantly increased to nearly normal values (global native T1 961 ms).

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  Figure 4. Cardiovascular magnetic resonance (CMR) for therapy monitoring in dilated cardiomyopathy (DCM). Evolution of dilated cardiomyopathy in a patient with pathogenic titin gene mutation. Before therapy (top row), he had 38% left ventricular ejection fraction, midwall late gadolinium enhancement (LGE) in the basal segments of the LV, with a ring-like appearance (white arrows), reduced global strain values: GLS (global longitudinal strain) −12%, GCS (global circumferential strain) −8%, GRS (global radial strain) 10%. After 3 years of optimized medical therapy (bottom row), he presented improved left ventricular ejection fraction (50%), same extent and distribution of LGE (white arrows), improved global strain values: GLS −15%, GCS −13%, GRS 18%.