Author + information
- Received May 10, 2018
- Revision received July 23, 2018
- Accepted July 26, 2018
- Published online October 15, 2018.
- Brett W. Sperry, MDa,b@BrettSperryMD,
- Bryan A. Reyes, MDc,
- Asad Ikram, MBBSa,
- Joseph P. Donnelly, MDa,
- Dermot Phelan, MD, PhDa,
- Wael A. Jaber, MDa,
- David Shapiro, MDc,
- Peter J. Evans, MD, PhDc,
- Steven Maschke, MDc,
- Scott E. Kilpatrick, MDd,
- Carmela D. Tan, MDd,
- E. Rene Rodriguez, MDd,
- Cecilia Monteiro, MDe,
- W.H. Wilson Tang, MDa,
- Jeffery W. Kelly, PhDe,
- William H. Seitz Jr., MDc and
- Mazen Hanna, MDa,∗ (, )@maz_hanna@ClevelandClinic
- aDepartment of Cardiovascular Medicine, Cleveland Clinic Foundation, Cleveland, Ohio
- bMid America Heart Institute, Saint Luke’s Hospital, Kansas City, Missouri
- cDepartment of Orthopaedic Surgery, Cleveland Clinic Foundation, Cleveland, Ohio
- dDepartment of Pathology, Cleveland Clinic Foundation, Cleveland, Ohio
- eDepartments of Chemistry and Molecular Medicine, The Scripps Research Institute, La Jolla, California
- ↵∗Address for correspondence:
Dr. Mazen Hanna, Cleveland Clinic, 9500 Euclid Avenue, J3-4, Cleveland, Ohio 44195.
Background Patients with cardiac amyloidosis often have carpal tunnel syndrome that precedes cardiac manifestations by several years. However, the prevalence of cardiac involvement at the time of carpal tunnel surgery has not been established.
Objectives The authors sought to identify the prevalence and type of amyloid deposits in patients undergoing carpal tunnel surgery and evaluate for cardiac involvement. The authors also sought to determine if patients with soft tissue transthyretin (TTR) amyloid had abnormal TTR tetramer kinetic stability.
Methods This was a prospective, cross-sectional, multidisciplinary study of consecutive men age ≥50 years and women ≥60 years undergoing carpal tunnel release surgery. Biopsy specimens of tenosynovial tissue were obtained and stained with Congo red; those with confirmed amyloid deposits were typed with mass spectrometry and further evaluated for cardiac involvement with biomarkers, electrocardiography, echocardiography with longitudinal strain, and technetium pyrophosphate scintigraphy. Additionally, serum TTR concentration and tetramer kinetic stability were examined.
Results Of 98 patients enrolled (median age 68 years, 51% male), 10 (10.2%) had a positive biopsy for amyloid (7 ATTR, 2 light chain [AL], 1 untyped). Two patients were diagnosed with hereditary ATTR (Leu58His and Ala81Thr), 2 were found to have cardiac involvement (1 AL, 1 ATTR wild-type), and 3 were initiated on therapy. In those patients who had biopsy-diagnosed ATTR, there was no difference in plasma TTR concentration or tetramer kinetic stability.
Conclusions In a cohort of patients undergoing carpal tunnel release surgery, Congo red staining of tenosynovial tissue detected amyloid deposits in 10.2% of patients. Concomitant cardiac evaluation identified patients with involvement of the myocardium, allowing for implementation of disease-modifying therapy. (Carpal Tunnel Syndrome and Amyloid Cardiomyopathy; NCT02792790)
This study was funded by Dr. Hanna’s Term Chair in Amyloid Heart Disease: Case Western Reserve University/Cleveland Clinic CTSA Grant Number UL1TR000439 from the National Center for Advancing Translational Sciences (NCATS), a component of the National Institutes of Health (NIH). Its contents are solely the responsibility of the authors and do not necessarily represent the official view of NCATS or NIH. Dr. Sperry has received consulting fees from GlaxoSmithKline. Dr. Monteiro has been supported by an American Heart Association Predoctoral Grant (16PRE31130009). Dr. Tang has been supported by grants from the NIH (R01HL103931) and Collins Family Fund; and has served as a consultant for the Advisory Board Company. Dr. Kelly has been supported by NIH NIDDK 046335; is a shareholder of FoldRx/Pfizer equity; and receives tafamidis royalties from Pfizer. Dr. Seitz Jr. has served as a consultant for Stryker, Zimmer/Biomet, and Kapp Surgical; and has served as an orthopedic surgery consultant. Dr. Hanna has received funding from the NCATS/NIH (UL1TR000439); and has received consulting fees from Pfizer, Ionis, and Eidos pharmaceuticals. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.
- Received May 10, 2018.
- Revision received July 23, 2018.
- Accepted July 26, 2018.
- 2018 American College of Cardiology Foundation
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