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Prevalence, risk factors and correlation with cardiac involvement of carpal tunnel syndrome in amyloidosis


Carpal tunnel syndrome (CTS) is one of the most common clinical manifestations of TTR-related amyloidosis, both hereditary (ATTR), and wild type (senile systemic amyloidosis, SSA) and often precedes cardiac symptoms. The exact prevalence of CTS in light-chain amyloidosis (AL), ATTR and SSA is not known. We therefore aimed to establish prevalence, risk factors and possible association with cardiac involvement in patients with TTR-related and AL amyloidosis.


We retrospectively analyzed clinical and instrumental (ECG and echocardographic) findings of 260 patients with TTR-related, and 175 with AL amyloidosis evaluated at our Centre between 1990 and September 2013.


Prevalence was 35% in TTR-related amyloidosis (35% in ATTR and 32% in SSA) and 8% in patients with AL (p< 0.001). Among TTR patients, CTS was more frequently associated with cardiac involvement (76% vs. 42%; p<0.0001) as reflected by the presence of pathological ECG and echocardiogram. Moreover, CTS manifested 9 years before the onset of cardiac symptoms. Among patients with cardiomyopathy with/without CTS there were no significant clinical/instrumental differences. At univariate analysis male gender and genotype were not associated with CTS.


CTS is specifically associated with TTR-related (but not AL) amyloidosis independently from patient gender. In TTR-related amyloidosis, CTS is more frequently associated with cardiac involvement, even though patients with cardiomyopathy with/without CTS have a comparable clinical/instrumental profile. CTS precedes cardiac symptoms onset by 9 years and this awareness is important for an early diagnosis of amyloidotic cardiomyopathy.

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This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver ( applies to the data made available in this article, unless otherwise stated.

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Milandri, A., Longhi, S., Gagliardi, C. et al. Prevalence, risk factors and correlation with cardiac involvement of carpal tunnel syndrome in amyloidosis. Orphanet J Rare Dis 10 (Suppl 1), P32 (2015).

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