Patient with severe left ventricular hypertrophy, bi-atrial enlargement, systolic dysfunction and BNP >8000. Cardiac MRI was negative for cardiac amyloidosis.
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Semi-quantitative Perugini grading scale is 3.0.
The ROI Ratio is 1.7 and positive for ATTR cardiac amyloidosis.
The patient is currently waiting for a cardiac biopsy for confirmation and corrective treatment.
Work up for myeloma and systemic amyloidosis is negative.
One can get a positive nuclear medicine scan despite normal cardiac MRI. So one needs to know the sensitivity and specificity for each of these modalities and how to use them and order them appropriately.
New treatments are available now for ATTR-cardiac amyloidosis.