Purpose
Systemic amyloidosis is a rare multisystem disease caused by the deposition of misfolded protein in various organs [1]. Cardiac involvement represents the most important prognostic factor and thus early diagnosis is of utmost importance, influencing further management of the patients [2].
There are several types of systemic amyloidosis but the commonest types affecting the heart are light-chain (AL) amyloidosis and transthyretin (ATTR) amyloidosis, the latter being further subdivided into senile amyloidosis and variant ATTR amyloidosis, caused by mutations in the TTR gene. Even though cardiac...
Methods and materials
Patient population. Fifty healthy subjects (32 subjects aged 18-65 and 18 subjects aged > 65) and ten patients with amyloidosis were prospectively recruited between February 2019 and October 2019. Four patients presented with the diagnosis of ATTR-amyloidosis proven by 99mTc-scintigraphy and six patients with suspected amyloidosis (two were first-degree relatives of a patient diagnosed with ATTRm amyloidosis, two were affected by hypertrophic cardiomyopathy, two presented with carpal tunnel syndrome). None of them had endomyocardial biopsy.
Data acquisition.CMR studies were performed using a 3T MR system...
Results
A Gauss curve distribution of T1 values obtained in healthy subjects was shown at statistical analysis, with similar distribution trend also considering two subgroups of age 18–65 years and >65 years. The average T1 value in healthy subjects was 1226±64 ms, which was adopted as local reference standard.
Secondly a comparison was made between values of native T1 and ECV obtained in healthy subjects and in patients with amyloidosis: the average native T1 value in this last group was 1386±72 ms and the average ECV...
Conclusion
There are few studies in literature about the diagnostic role of T1-mapping in cardiac amyloidosis especially on 3T MR scanners [10]. Our results, although obtained among a small number of patients compared to healthy subjects, demonstrate that T1-mapping can be a helpful non-invasive technique to diagnose cardiac amyloidosis, with diagnostic T1 cut-off at about 1300 ms. In accordance with literature [3, 11] our native T1 and ECV values are higher in ATTR patients than in other forms of amyloidosis.
Native T1 better accuracy respect to...
Personal information and conflict of interest
A. Cassar Scalia; Modena/IT - nothing to disclose L. Ruoli; Modena/IT - nothing to disclose F. Fiocchi; Modena/IT - nothing to disclose G. Ligabue; Modena/IT - nothing to disclose P. Torricelli; Modena/IT - nothing to disclose
References
1.Banypersad SM, Moon JC, Whelan C, Hawkins PN, Wechalekar AD (2012) Updates in cardiac amyloidosis: a review. J Am Heart Assoc; 1:e000364.
2.Fikrle M, Palečekab, T., Kuchynka, P., Němečeka, E., Bauerovác, L., Straub, J., Ryšaváe, R (2013) Cardiac amyloidosis: a comprehensive review. Cor et vasa; 55:e60-e75.
3.Banypersad SM. The Evolving Role of Cardiovascular Magnetic Resonance Imaging in the Evaluation of Systemic Amyloidosis (2019) Magn Reson Insights; 12:1178623X19843519.
4.Tang CX, Petersen SE, Sanghvi MM, Lu GM, Zhang LJ (2018) Cardiovascular magnetic resonance imaging for amyloidosis: The...