Transthyretin cardiac amyloidosis
Case submitted by Nazim Coskun
74-year-old female
Echocardiography → IVS 19 mm, EF 55%
Serum and urine immunofixation negative for a monoclonal protein
- Planar and SPECT/CT mages acquired 3 hours after the injection of Tc-99m PYP (555 MBq, 15 mCi) showed increased myocardial uptake (Perugini Grade 3, H/CL: 2.6).
- SPECT/CT images also show effusion in the left pleural cavity, free abdominal fluid and bilateral 7th rib fractures.
Case Notes
Cardiac amyloidosis is a restrictive cardiomyopathy caused by extracellular deposition of misfolded amyloid fibrils. Although it was considered a rare disease until recently, emerging data suggests that a significant portion of patients with heart failure with preserved ejection fraction and severe aortic stenosis have underlying cardiac amyloidosis. Radionuclide imaging with bone seeking agents is a non-invasive method that plays an important for the diagnosis of ATTR cardiac amyloidosis. Increased myocardial uptake of bone seeking agents is a highly accurate diagnostic finding for ATTR cardiac amyloidosis, provided that AL amyloidosis is excluded.