Donut Morphology of Cardiac Amyloidosis

Figures

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  Figure 1. Electrocardiogram Electrocardiogram reveals atrial fibrillation, normal voltage, poor R wave progression (pseudoinfarct pattern), and nonspecific ST-segment changes.

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  Figure 2. T1-Weighted Cine CMR Images T1-weighted cine (steady-state free precession) images from cardiac magnetic resonance (CMR) demonstrate isolated basal left ventricular wall thickening (19 mm basal septum, 17 mm basal inferolateral wall) with otherwise normal wall thickness in the mid–left ventricular to apical left ventricular walls, right ventricular free walls, and atrial walls.

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  Figure 3. Delayed Post-Gadolinium Contrast CMR Images Delayed post-gadolinium contrast images from cardiac magnetic resonance (CMR) show prominent near-circumferential enhancement of the basal left ventricular segments (A to C) and basal right ventricular free wall (A and C). There is additional mild subepicardial enhancement at the mid-inferior right ventricular insertion site (D) with otherwise normal-appearing myocardium in mid-segments to apical segments. A trace simple pericardial effusion is visualized along the basal lateral heart (A to C).

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  Figure 4. T1 Parametric Mapping From CMR T1 parametric mapping from cardiac magnetic resonance (CMR) shows prominent elevation of native T1 values in the basal segments (up to 1,198 ms) with a base-to-apex gradient of more normal values throughout the mid- and apical myocardium. ROI = region of interest.

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  Figure 5. Technetium-99m Pyrophosphate Imaging in Cardiac Amyloidosis Cardiac amyloid radionuclide imaging performed at 1 hour with technetium-99m pyrophosphate shows a subtle abnormality on planar imaging (A) with activity adjacent to the sternum. On single-photon emission computed tomography images (B and C), nondiffuse (grade 2) myocardial uptake is clearly visualized in the basal left ventricle with more muted (grade 1) uptake in the mid-septum, closely matching the distribution of late enhancement on fused Cardiac amyloid radionuclide imaging/cardiac magnetic resonance late gadolinium enhancement images (D).

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  Visual Summary. Timeline of Clinical Events Timeline of clinical events from initial presentation and outside evaluation, through CMR-based identification of donut morphology, repeat scintigraphy confirming the diagnosis, initiation of tafamidis, and clinical improvement at 3-month follow-up. AF = atrial fibrillation; ATTR-CM = transthyretin cardiac amyloidosis; CMR = cardiac magnetic resonance; CTS = carpal tunnel syndrome; ECG = electrocardiogram; ECV = extracellular volume; ED =emergency department; GLS = global longitudinal strain; HF = heart failure; HFpEF = heart failure with preserved ejection fraction; hsTnT = high-sensitivity cardiac troponin T; IVS = interventricular septum; JVP = jugular venous pressure; LGE = late gadolinium enhancement; LV = left ventricle/ventricular; NT-proBNP = N-terminal pro–B-type natriuretic peptide; PYP = pyrophosphate; SPIFE = serum protein immunofixation electrophoresis; subendo-midmyo = subendocardial to mid-myocardial.