Carpal tunnel syndrome and spinal canal stenosis: harbingers of transthyretin amyloid cardiomyopathy?

  • Fabian aus dem SiepenEmail author
  • Selina Hein
  • Sofie Prestel
  • Christian Baumgärtner
  • Stefan Schönland
  • Ute Hegenbart
  • Christoph Röcken
  • Hugo A. Katus
  • Arnt V. Kristen
Original Paper



Carpal tunnel syndrome (CTS) and spinal canal stenosis can be frequently observed in the medical history of patients with transthyretin amyloidosis (ATTR), both in the hereditary (mt-ATTR) and wild-type (wt-ATTR) form. The aim of this retrospective single-center analysis was to determine the prevalence of these findings, delay to diagnosis of systemic amyloidosis and the prognostic value in a large cohort of patients with wt-ATTR and mt-ATTR amyloidosis.


Medical records of 253 patients diagnosed with wt-ATTR, 136 patients with mt-ATTR and 77 asymptomatic gene carriers were screened for history of CTS and spinal canal stenosis and laboratory analysis, electrocardiography and echocardiographic results, respectively. Clinical follow-up was performed by phone assessment.


History of CTS was present in 77 patients (56%) with mt-ATTR, in 152 patients (60%) with wt-ATTR and even in 10 of the asymptomatic gene carriers (13%). Latency between carpal tunnel surgery and first diagnosis of systemic amyloidosis was significantly longer in wt-ATTR compared to mt-ATTR (117 ± 179 months vs. 66 ± 73 months; p = 0.02). In total, 36 patients (14%) with wt-ATTR and 7 patients (5%) with mt-ATTR had a history of clinically significant spinal canal stenosis. In the subgroup of mt-ATTR, patients with CTS had thicker IVS (19 ± 5 mm vs. 16 ± 5 mm, p < 0.05), higher LV mass index (225 ± 78 g vs. 193 ± 98 g, p < 0.05), lower Karnofsky index (78 ± 15% vs. 83 ± 17%, p < 0.05), and lower mitral annular plane systolic excursion (MAPSE; 9 ± 4 mm vs. 11 ± 5 mm, p < 0.05) compared to patients without CTS, whereas in wt-ATTR no significant differences could be observed. No significant difference in survival was observed between patients with and without CTS (wt-ATTR: 67 vs. 63 months, p = 0.45; mt-ATTR: 74 vs. 63 months, p = 0.60). A combination of CTS and spinal stenosis was present in 32 wt-ATTR patients (12%) and 3 mt-ATTR patients (2.2%).


The prevalence of CTS is high and the latency between CTS surgery and diagnosis of amyloidosis is long among patients with wt-ATTR and mt-ATTR. CTS might be predictive for future occurrence of systemic (predominantly cardiac) ATTR amyloidosis.


Amyloidosis Carpal tunnel syndrome Spinal canal stenosis Heart failure 


Compliance with ethical standards

Conflict of interest

On behalf of all authors, the corresponding author states that there is no conflict of interest.


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  • Fabian aus dem Siepen
    • 1
    Email author
  • Selina Hein
    • 1
  • Sofie Prestel
    • 1
  • Christian Baumgärtner
    • 1
  • Stefan Schönland
    • 2
  • Ute Hegenbart
    • 2
  • Christoph Röcken
    • 3
  • Hugo A. Katus
    • 1
    • 4
  • Arnt V. Kristen
    • 1
  1. 1.Department of Cardiology, Angiology and Respiratory Medicine, Amyloidosis CenterUniversity Hospital HeidelbergHeidelbergGermany
  2. 2.Department of Hematology and Oncology, Amyloidosis CenterUniversity Hospital HeidelbergHeidelbergGermany
  3. 3.Institute of PathologyChristian-Albrechts-University KielKielGermany
  4. 4.Partner Site Heidelberg/Mannheim, DZHK (German Center for Cardiovascular Research)University of HeidelbergHeidelbergGermany

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