Cardiac Transthyretin Amyloidosis: A Nuclear Medicine Leading Role. Situation in a Spanish Center and "State of the Art" in Nuclear Medicine.

ABSTRACT

PURPOSE: Amyloidosis is a heterogeneous group of diseases caused by abnormal extracellular deposition of insoluble proteins and can involve myocardium. One of the causes of myocardial involvement is TTR amyloidosis. Our objective has been to evaluate the situation of cardiac amyloidosis (CA) in our center and the role of nuclear medicine, and to review the state of the art of nuclear medicine in this entity. PATIENTS AND METHODS: We have evaluated retrospectively 186 patients with clinical suspicion of CA and analyzed the clinical characteristics, free light chains and immunofixation in serum and/or urine, and the most relevant biomarkers associated with transthyretin CA (C-ATTR) of these patients and compared them with the results of the 99mTc-DPD scintigraphy. RESULTS: We have verified the growing bibliographic evidence concerning C-ATTR. A total of 51 scintigraphies (27.4%) were positive, 2 (1.1%) indeterminate and 133 (71.5%) negative according to the Perugini score. ATTR was diagnosed in 22 (11.8%; 77.3% males; mean age, 79.4 years). Of these, 12 (75% men; 82.3 years) were ATTRwt (wild-type or age-associated) patients, 2 (50% men; 52 years) experienced ATTRv (variant or hereditary), and 8 (87.5% men; 82.3 years) were not classified because of the absence genetic test. The origin of amyloidosis could not be determined in 31 (16.7%; 80.7% males; 84.5 years). In 29 of them (93.6%), it was because there was no study of free light chains or immunofixation. CONCLUSIONS: Nuclear medicine is playing an increasing role in the diagnosis and classification of CA. However, the monitoring of these is still patchy.