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Selected Publication:
Ashjaei, M.
Diagnostic and prognostic value of amyloid scintigraphy in patients with suspected cardiac amyloidosis
a retrospective study
Humanmedizin; [ Diplomarbeit ] Medizinische Universität Graz; 2024. pp. 63
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- Authors Med Uni Graz:
- Advisor:
- Aigner Reingard
- Stanzel Susanne
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- Abstract:
- Introduction: Systemic amyloidosis encompasses a group of disorders characterized by the extracellular buildup of insoluble fibrillar proteins, leading to structural disturbances in affected tissues and impaired organ function. Of particular interest is cardiac form of amyloidosis. The clinical presentation of amyloidosis patients varies depending on the specific type of cardiac involvement. While echocardiography is regarded as the gold standard for non-invasive diagnosis of amyloidotic cardiomyopathy, a definitive diagnosis of amyloidosis typically necessitates a biopsy of the affected organ. In recent times, amyloid-scintigraphy using 99mTc- pyrophosphate (PYP) has emerged as a valuable clinical tool for cardiac imaging in amyloidosis. Aim: The aim of this study is to assess both the diagnostic and prognostic value of amyloid scintigraphy utilizing 99mTc-pyrophosphate in suspected cardiac amyloidosis. Methods and Materials: A retrospective analysis was conducted, involving 150 individuals who were suspected of having cardiac amyloidosis. Between July 2018 and June 2022 amyloid scintigraphy with 99mTc-pyrophosphate was performed. The results obtained from the amyloid scintigraphy (the Perugini score ranging from 0 to 3, heart-to-lung ratio (H/CL ratio)) were compared with the outcomes of myocardial biopsy and/or cardiac MRI. The patients were categorized into different groups based on the Perugini Score (0, 1, 2, 3) and H/CL ratio (greater or less than 1.5). The median follow-up time was 9 months. Additionally, the association between the Perugini score and life expectancy was examined by the long-rank test. Results: Myocardial biopsies were performed on 16 patients, and 77 patients underwent cardiac MRI. Amyloid scintigraphy with 99mTc-PYP was considered positive in 133 patients (88.7%) based on visual assessment on planar images (Perugini score 1-3). By contrast, amyloid scintigraphy was considered negative in 17 patients (11.3%) (Perugini score 0). In the semi-quantitative assessment, 80 patients (53.3%) had a H/CL ratio > 1.5, and 70 patients (46.7%) a H/CL ratio ≤ 1.5. When using myocardial biopsy as the gold standard, the Perugini score showed a sensitivity of 92% and a specificity of 33%, and when using cardiac MRI as the gold standard, 91% and 15%, respectively. In contrast, the quantitative H/CL ratio demonstrated a specificity of 67% and a sensitivity of 54% using myocardial biopsy as gold standard and 64% and 64%, respectively using cardiac MRI as gold standard. Regarding the prognostic value of amyloid scintigraphy with 99mTc-PYP, no significant differences in survival between patients with Perugini scores 1-3 and patients with Perugini score 0, as well as between patients with H/CL ratio >1.5 and <1.5 was found. Conclusion: Our study demonstrates the substantial diagnostic sensitivity of 99mTc- PYP scintigraphy, offering a non-invasive diagnostic alternative that complements conventional diagnostic methods like myocardial biopsy and cardiac MRI in the evaluation of patients with cardiac amyloidosis. Patients with Perugini score 0 and a H/CL ratio ≤ 1.5, respectively had no survival benefit compared to patients with Perugini score 1-3 and a H/CL ratio > 1.5, respectively. This shows that stratification according to Perugini score and H/CL ratio has no prognostic value in patients with suspected cardiac amyloidosis.