Brett W Sperry1, Bryan A Reyes2, Asad Ikram3, Joseph P Donnelly3, Dermot Phelan3, Wael A Jaber3, David Shapiro2, Peter J Evans2, Steven Maschke2, Scott E Kilpatrick4, Carmela D Tan4, E Rene Rodriguez4, Cecilia Monteiro5, W H Wilson Tang3, Jeffery W Kelly5, William H Seitz2, Mazen Hanna6. 1. Department of Cardiovascular Medicine, Cleveland Clinic Foundation, Cleveland, Ohio; Mid America Heart Institute, Saint Luke's Hospital, Kansas City, Missouri. Electronic address: https://twitter.com/BrettSperryMD. 2. Department of Orthopaedic Surgery, Cleveland Clinic Foundation, Cleveland, Ohio. 3. Department of Cardiovascular Medicine, Cleveland Clinic Foundation, Cleveland, Ohio. 4. Department of Pathology, Cleveland Clinic Foundation, Cleveland, Ohio. 5. Departments of Chemistry and Molecular Medicine, The Scripps Research Institute, La Jolla, California. 6. Department of Cardiovascular Medicine, Cleveland Clinic Foundation, Cleveland, Ohio. Electronic address: hannam@ccf.org.
Abstract
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).
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).
Authors: Sharmila Dorbala; Yukio Ando; Sabahat Bokhari; Angela Dispenzieri; Rodney H Falk; Victor A Ferrari; Marianna Fontana; Olivier Gheysens; Julian D Gillmore; Andor W J M Glaudemans; Mazen A Hanna; Bouke P C Hazenberg; Arnt V Kristen; Raymond Y Kwong; Mathew S Maurer; Giampaolo Merlini; Edward J Miller; James C Moon; Venkatesh L Murthy; C Cristina Quarta; Claudio Rapezzi; Frederick L Ruberg; Sanjiv J Shah; Riemer H J A Slart; Hein J Verberne; Jamieson M Bourque Journal: J Nucl Cardiol Date: 2020-04 Impact factor: 5.952