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Abstract Background Amyloid scintigraphy in combination with free light chain assessment results in an excellent diagnostic tool for cardiac transthyretin amyloidosis. In 57 patients, an abnormal Perugini Score was present in 57 patients, and the FLC test showed monoclonal protein in 60 patients. 29 patients with abnormal Perugini Score underwent the FLC test, which showed monoclonal protein. One more patient was diagnosed with concomitant AL and ATTR amyloidosis. 54 patients with Perugini Score II or III were diagnosed in 54 patients with Perugini Score II or III compared to 6 patients with Perugini II, providing a sensitivity of abnormal Perugini amyloidosis of 90 percent. ATTR was diagnosed in 55 patients and AL amyloidosis in 3 patients, with a positive predictive value of abnormal Perugini Score of 97%. Both AL patients had Pergini Score of II and one had a Perugini Score of III, while one had Perugini Score of III. The PPV of abnormal Perugini Score was 100% when excluding patients with monoclonal gammopathy. Conclusion When monoclonal gammopathy was excluded, our results support a PPV of abnormal amyloid scintigraphy of 100% for cardiac ATTR amyloidosis. Monoclonal gammopathy patients in mong patients with abnormal scintigraphy, one of ten patients with abnormal scintigraphy had AL amyloidosis as the cause. And in the case of Perugini Score II or III, tissue biopsy and histopathological testing should be performed in every patient with suspected amyloidosis and monoclonal gammopathy.
Source link: https://doi.org/10.1093/eurheartj/ehac544.903
Background and Background The prognostic role of bone tracer uptake in transthyretin cardiac amyloidosis is uncertain. Methods We reviewed data of ATTR-CA patients who underwent bone tracer scintigraphy with the acquisition of both planar and single photon emission computed tomography imaging. SpecT images were exhibited on 124 ATTR-CA patients. Patients with BiV uptake in NYHA 22653 were older and more often than those with isolated LV uptake in comparison to those with isolated LV uptake. During a median follow-up of 21 months, BiV uptake was associated with a higher rate of the primary outcome compared to isolated LV uptake, though the Perugini scale was not analyzed. Planar BiV uptake was independently linked to all-cause mortality in a prospective cohort of 463 ATTR-CA patients with a median follow-up of 30 months, with an increased risk of RV uptake in higher grades.
Source link: https://doi.org/10.1093/eurheartj/ehac544.1770
Methods Retrospective registry of 63 patients who underwent DPD due to suspicion of cardiac amyloidosis in our center between 2018 and 2021. We compared patients with ATTR, positive scintigraphy grade 2u20133, with patients with negative scintigraphy. Patients with a negative DPD were diagnosed with hypertensive heart disease. However, patients in echo3 had noticeably higher interventricular septum thickness and a worse biventricular function in comparison to the nATTR group. In the ATTR group, a significant rise in the IVS and decrease of left ventricular ejection fraction between echo1 and echo2 was noted. Conclusions The increase in the thickness of the IVS and decrease in the left ventricular ejection fraction during a follow-up of patients with ventricular hypertrophy of other etiologies could be a peculiar characteristic of ATTR.
Source link: https://doi.org/10.1093/eurheartj/ehac544.1691
A minimally invasive method that can distinguish between transthyretin amyloidosis and light-chain amyloidosis is pyrophosphate scintigraphy at Myocardial scintigraphy with technetium-99 million pyrophosphate. We discuss a case in which we helped identify the amyloidosis type in a 74-year-old man with cardiac amyloidosis and several previous admissions for acute decompensated heart failure in a 74-year-old man with cardiac amyloidosis and multiple prior admissions. Edema of bilateral lower extremity (adequate girth and abdominal girth were present in the patient. We were given a prescription-directed medical therapy for his acute decompensated heart disease with cardiorenal syndrome and decompensated cirrhosis. Patients' age, predominantly cardiac involvement, unremarkable serum protein electrophoresis result, and unusual free u03b/u03b light-chain ratio of 2. 24 had been established two years ago, a presumptive diagnosis of ATTR cardiomyopathy had been made. This case shows that technetium-99 million pyrophosphate scintigraphy can help diagnose ATTR cardiomyopathy and determining patients who may benefit from disease-modifying therapy.
Source link: https://doi.org/10.14503/thij-20-7379
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