Key result
Cardiac 123I-MIBG uptake is ~70% lower in α-synucleinopathies compared to ARVC.
Why the study?
Although 123I-MIBG SPECT/CT assesses cardiac sympathetic denervation in both ARVC and α-synucleinopathies, quantitative and semi-quantitative cardiac innervation had not been directly compared between these diseases.
Does quantitative and semi-quantitative 123I-MIBG SPECT/CT differentiate cardiac sympathetic innervation between patients with α-synucleinopathies and ARVC?
Observational (n=28)
No
Does quantitative and semi-quantitative 123I-MIBG SPECT/CT differentiate cardiac sympathetic innervation between patients with α-synucleinopathies and ARVC?
Quantitative and semi-quantitative 123I-MIBG SPECT/CT demonstrates significantly lower cardiac sympathetic innervation in patients with α-synucleinopathies compared to those with ARVC, suggesting the need for disease-specific uptake ranges.
Answers are grounded in this paper and cite their sources.
Suggests 123I-MIBG SPECT/CT may differentiate sympathetic denervation in α-synucleinopathies from ARVC; leaves open prospective validation of disease-specific thresholds.
Background/Objectives: 123Iodo-metaiodobenzylguanidine single photon emission computed tomography/computed tomography (123I-MIBG SPECT/CT) is used to evaluate the cardiac sympathetic nervous system in cardiac diseases such as arrhythmogenic right ventricular cardiomyopathy (ARVC) and α-synucleinopathies such as Parkinson’s diseases. A common feature of these diseases is denervation. We aimed to compare quantitative and semi-quantitative cardiac sympathetic innervation using 123I-MIBG imaging of ARVC and α-synucleinopathies. Methods: Cardiac innervation was assessed using 123I-MIBG SPECT/CT in 20 patients diagnosed with definite ARVC and 8 patients with clinically diagnosed α-synucleinopathies. Heart-to-mediastinum-ratio (H/M-ratio), as semi-quantitative, was evaluated. Additionally, standardized uptake value (SUV), as quantitative, was measured as SUVmedian, SUVmax, and SUVpeak in the left ventricle (LV), the right ventricle (RV), and in the global heart, based on a CT scan following quantitative image reconstruction. Results: The quantification of 123I-MIBG uptake in the LV, the RV, and the global heart was feasible in patients suffering from α-synucleinopathies. SUVmedian, and SUVpeak demonstrated a significant difference between ARVC and α-synucleinopathies across all regions, with the α-synucleinopathy group showing a lower uptake. In addition, the H/M ratio showed significantly lower uptake in patients with α-synucleinopathies than in patients with ARVC. Conclusions: Patients with α-synucleinopathies demonstrate significantly lower cardiac innervation in semi-quantitative and quantitative examinations than ARVC patients. The comparison of semi-quantitative and quantitative examinations suggests that quantitative examination offers an advantage. Quantitative analysis can be performed separately for the LV, RV, and global heart. However, analyzing the LV or RV does not provide additional benefit over analyzing the global heart in distinguishing between α-synucleinopathies and ARVC. Considering the different clinical manifestations of these two diseases, the absolute SUV values should not be generalized across different pathologies, and disease-specific ranges should be used instead.
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Hagen et al. (2024) conducted an observational in Arrhythmogenic Right Ventricular Cardiomyopathy and α-synucleinopathies (n=28). α-synucleinopathies vs. Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) was evaluated on Global heart standardized uptake value median (heart-SUVmedian) of 123I-MIBG (Cohen's d 2.88, 95% CI 1.71-4.06, p=<0.001). Patients with α-synucleinopathies demonstrated lower global heart 123I-MIBG uptake compared to patients with ARVC.
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