Key result
Endomyocardial biopsy confirms AL cardiac amyloidosis despite false-positive grade 3 bone scintigraphy uptake.
Why the study?
Bone scintigraphy interpretation for cardiac amyloidosis diagnosis remains challenging when monoclonal gammopathy is present, requiring clarification of diagnostic accuracy.
Case Report (n=1)
No
Even Perugini grade 3 myocardial uptake on bone scintigraphy does not definitively establish ATTR-CA in the presence of monoclonal gammopathy, making tissue biopsy essential.
Tissue biopsy remains essential with monoclonal gammopathy despite grade 3 uptake; this case leaves open scintigraphy reliability for ATTR-CA.
BACKGROUND: Bone scintigraphy has improved noninvasive diagnosis of cardiac amyloidosis, but interpretation remains challenging when monoclonal gammopathy is present. CASE SUMMARY: A 76-year-old man with heart failure with preserved ejection fraction, chronic kidney disease, permanent atrial fibrillation, and persistent troponin elevation underwent coronary angiography showing significant left anterior descending artery stenosis treated with percutaneous coronary intervention. Persistent symptoms, recurrent heart failure decompensations, disproportionate natriuretic peptide elevation, increased wall thickness, and apical sparing raised suspicion for infiltrative cardiomyopathy. Laboratory testing revealed monoclonal gammopathy. Tc-99m-pyrophosphate scintigraphy showed Perugini grade 3 uptake, strongly suggestive of transthyretin cardiac amyloidosis (ATTR-CA), but endomyocardial biopsy established light-chain (AL) cardiac amyloidosis. AL-directed therapy was initiated, but the patient died of severe pneumonia with respiratory failure. DISCUSSION: In the presence of monoclonal gammopathy, even Perugini grade 3 uptake does not establish ATTR-CA. Recent ischemic injury may further confound scintigraphic interpretation. TAKE-HOME MESSAGE: Tissue confirmation remains essential for accurate diagnosis and timely AL-directed therapy.
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Cacciapuoti et al. (2026) conducted a case report in Cardiac amyloidosis (n=1). Tc-99m-pyrophosphate scintigraphy was evaluated on Amyloid subtype confirmation by endomyocardial biopsy. Tissue confirmation established light-chain (AL) cardiac amyloidosis in a patient with monoclonal gammopathy, despite Perugini grade 3 uptake on bone scintigraphy mimicking transthyretin cardiac amyloidosis.
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