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Updated: May 25, 2025
Genetic Analysis of Hereditary Transthyretin Ala97Ser Related Amyloidosis
Published on: June 9, 2018
Future Directions in Quantitative SPECT-CT Evaluation of Cardiac Transthyretin Amyloidosis: Correlation with Clinical
Mirela Gherghe1,2, Mario-Demian Mutuleanu1,2, Tatiana Lucia Suta2
1Nuclear Medicine Department, University of Medicine and Pharmacy "Carol Davila", 050474 Bucharest, Romania.
Insights
Quantitative SPECT-CT shows promise for early diagnosis of cardiac transthyretin amyloidosis (ATTR). Advanced metrics correlate with clinical and imaging findings, aiding in identifying this underrecognized heart failure cause.
Area of Science:
- Nuclear medicine
- Cardiology
- Radiology
Background:
- Cardiac amyloidosis (ATTRv and ATTRwt) is an underdiagnosed cause of heart failure with preserved ejection fraction.
- Diagnosis is challenging due to low suspicion and overlap with other conditions.
- Current diagnostic methods have limitations.
Purpose of the Study:
- To evaluate the utility of quantitative [99mTc]-PYP SPECT-CT for volumetric assessment in cardiac amyloidosis.
- To explore correlations between quantitative SPECT-CT metrics and clinical/imaging parameters.
- To establish SPECT-CT as a tool for early diagnosis of cardiac ATTR.
Main Methods:
- A prospective monocentric study of 22 patients with hereditary cardiac transthyretin amyloidosis (ATTR).
- Quantitative analysis of [99mTc]-PYP SPECT-CT scans, including volumetric evaluation.
- Correlation analysis with electrocardiography (ECG), echocardiography (EchoGLS), cardiac MRI (T1 mapping), and clinical data (neuropathy).
Main Results:
- SUVmaxMyocardium/SUVmaxBone correlated with ECG low voltage and EchoGLS.
- SUVmaxMyocardium/SUVmaxLiver correlated with myocardial gadolinium kinetics, T1 mapping, diastolic dysfunction, neuropathy, and EchoGLS.
- SUVmaxMyocardium/SUVmeanBone and SUVmaxMyocardium/SUVmaxSoft tissue showed correlations with diastolic dysfunction, neuropathy, and S II.
Conclusions:
- Advanced quantitative SPECT-CT metrics demonstrate moderate to strong correlations with clinical and paraclinical data.
- These findings support the use of quantitative SPECT-CT for early diagnosis of cardiac ATTR.
- Further multicenter studies are required to validate these novel quantitative SPECT-CT parameters.
Abstract:
Background: ATTRv and ATTRwt cardiac amyloidosis (CA) are underrecognized causes of heart failure with preserved left ventricular ejection fraction. The diagnosis of CA remains challenging due to low diagnostic suspicion and clinical overlap with more common diseases. The aim of this study was to use [99mTc]-PYP SPECT-CT to perform a volumetric evaluation of bone scintigraphy to overcome the limitations of current practices. Methods: A monocentric prospective study was conducted to evaluate a lot of 22 patients with a mean age of 52.86 ± 13.80 years, diagnosed with hereditary cardiac transthyretin amyloidosis (ATTR). Results: Correlations between the quantitative SPECT-CT, clinical data, and morphological parameters were performed, demonstrating moderate to strong correlation of SUVmaxMyocardium/SUVmaxBone to both ECG low voltage and EchoGLS, SUVmaxMyocardium/SUVmaxLiver to myocardial gadolinium kinetics with T1 mapping MRI, diastolic disfunction, sensory-motor polyneuropathy, and EchoGLS, SUVmaxMyocardium/SUVmeanBone with diastolic disfunction and sensory-motor polyneuropathy, as well as SUVmaxMyocardium/SUVmaxSoft tissue to S II, respectively. Conclusions: The moderate to strong correlations among advanced quantitative SPECT-CT metrics and clinical and paraclinical data create the premises to use these parameters for early diagnosis of cardiac ATTR. Further multicentric studies in a larger patient population are needed to validate the newly identified quantitative SPECT-CT parameters.
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