PMC6936921_01
Total body scintigraphic scans at 3 hours (late) after the intravenous injection of 99mTc-DPD: (a) whole-body scan: anterior view, (b) zoom of thoracic view, (c) no uptake of the tracer was evident at the myocardial level.
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Total body scintigraphy showed no cardiac 99mTc-DPD uptake (Fig. 1).
Myocardial biopsy. Congo red staining showed reddish amyloid material in the myocardium (left) with apple-green birefringence under polarised light (right), x200.
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Myocardial biopsy showed amyloid deposition at muscle fibre level (Fig. 2).
Renal biopsy. Congo red staining showed reddish amyloid material in the renal medulla (left) with apple-green birefringence under polarised light (right), x200.
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The patient was submitted to a renal biopsy that showed amyloid deposition restricted to the medulla with no involvement of glomeruli (Fig. 3), which raised the suspicion of systemic amyloidosis related to apolipoprotein deposition.
The table shows the list of the most abundant proteins identified in the tested sample (myocardial biopsy). The ApoA4 protein is highlighted with 90% sequence coverage. The graphic indicates the relative abundance of peptide (y-axis) in relation to peptide retention time (x-axis).
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MS-based proteomic analysis, performed to determine the proteome profile of the cardiac amyloid, identified and quantified more than 800 proteins and disclosed large amounts of AApoAIV amyloid deposits, one of the most abundant proteins detected in the biopsy (Fig. 4).
Transthoracic echocardiogram. (a) Longitudinal axis and short axis showing increased thickness of LV wall, (b) restrictive transmitral filling pattern, (c) longitudinal strain imaging showing a basal to apical gradient.
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Cardiac ultrasound revealed severe LV dysfunction with an ejection fraction of 28%, a restrictive mitral inflow pattern and an E/e' ratio of 19.5 compatible with high LV filling pressures (Fig. 5).