TY - JOUR

T1 - Screening for Cardiac Amyloidosis 5 to 15 Years After Surgery for Bilateral Carpal Tunnel Syndrome

AU - Westin, Oscar

AU - Fosbøl, Emil L.

AU - Maurer, Mathew S.

AU - Leicht, Birgitte P.

AU - Hasbak, Philip

AU - Mylin, Anne Kærsgaard

AU - Rørvig, Sara

AU - Lindkær, Thomas Hartvig

AU - Johannesen, Helle Hjorth

AU - Gustafsson, Finn

N1 - Funding Information: The study was funded by independent research grants from Erik og Susanna Olesens Almenvelgørende Fond, Pfizer, Arvid Nilssons Fond, Højmosegård-legatet, Frimodt-Heineke Fonden, and Hjertecentrets forskningsudvalg, Rigshospitalet (Dr Westin). Dr Fosbøl has received an independent research grant from Novo Nordisk Foundation, outside the submitted work. Dr Maurer has received grant support from NIH grants R01HL139671, R21AG058348, and K24AG036778; has been a consultant for Intellia, Novo-Nordisk, Pfizer, Eidos, Prothena, Akcea, and Alnylam; and his institution has received clinical trial funding from Pfizer, Prothena, Eidos, and Alnylam. Dr Gustafsson has received personal fees from Abbott, AstraZeneca, Pfizer, Boehringer Ingelheim, Novartis, and Orion Pharma outside the submitted work. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose. Funding Information: The authors thank Kirsten Arnaa, medical secretary, for management of grants; the cardiovascular research nurses for day-to-day assistance; Lesli Hingstrup Larsen for assistance with set-up of project biobank; and Lærke Marie Sidenius Nelson for assistance with planning the study and acquiring necessary approvals.

PY - 2022

Y1 - 2022

N2 - Background: Bilateral carpal tunnel syndrome (CTS) is a common extracardiac manifestation of amyloidosis and usually predates overt cardiac amyloidosis (CA) by several years. Screening studies on patients undergoing CTS surgery have shown a low yield of CA (2.0%), but high prevalence of amyloid in the carpal ligament. The proportion of patients with amyloid in the carpal ligament who later develop CA is unknown. Objectives: The authors sought to investigate the prevalence of undiagnosed CA 5 to 15 years after surgery for bilateral CTS. Methods: Using national registries, the authors identified subjects aged 60 to 85 years with prior CTS surgery, where the first procedure on the second wrist was performed 5 to 15 years earlier. Invitations to participate in the study were sent by mail. Per international recommendations, the initial cardiac evaluation included echocardiography, 99mtechnetium-pyrophosphate scintigraphy, and assessment of monoclonal proteins in serum and urine. Results: A total of 250 subjects (35.7% of those invited) participated in the study. The median age was 70.4 years, and 50% were female. CA was diagnosed in 12 patients (4.8%; 95% CI: 2.5%-8.2%), and all cases were wild-type transthyretin amyloidosis (ATTRwt). The prevalence of ATTRwt in men was 8.8% (95% CI: 4.5%-15.2%; n = 11), and 21.2% (95% CI: 11.1%-34.7%) in male subjects ≥70 years with a BMI <30 kg/m2. All but 2 patients diagnosed with ATTRwt were in the lowest disease severity score (Mayo score). Conclusions: Screening for CA in patients with prior surgery for bilateral CTS finds approximately 5% with early-stage transthyretin CA. The clinical yield was higher (>1 in 5) when focusing on nonobese men ≥70 years, showing potential for systematic screening.

AB - Background: Bilateral carpal tunnel syndrome (CTS) is a common extracardiac manifestation of amyloidosis and usually predates overt cardiac amyloidosis (CA) by several years. Screening studies on patients undergoing CTS surgery have shown a low yield of CA (2.0%), but high prevalence of amyloid in the carpal ligament. The proportion of patients with amyloid in the carpal ligament who later develop CA is unknown. Objectives: The authors sought to investigate the prevalence of undiagnosed CA 5 to 15 years after surgery for bilateral CTS. Methods: Using national registries, the authors identified subjects aged 60 to 85 years with prior CTS surgery, where the first procedure on the second wrist was performed 5 to 15 years earlier. Invitations to participate in the study were sent by mail. Per international recommendations, the initial cardiac evaluation included echocardiography, 99mtechnetium-pyrophosphate scintigraphy, and assessment of monoclonal proteins in serum and urine. Results: A total of 250 subjects (35.7% of those invited) participated in the study. The median age was 70.4 years, and 50% were female. CA was diagnosed in 12 patients (4.8%; 95% CI: 2.5%-8.2%), and all cases were wild-type transthyretin amyloidosis (ATTRwt). The prevalence of ATTRwt in men was 8.8% (95% CI: 4.5%-15.2%; n = 11), and 21.2% (95% CI: 11.1%-34.7%) in male subjects ≥70 years with a BMI <30 kg/m2. All but 2 patients diagnosed with ATTRwt were in the lowest disease severity score (Mayo score). Conclusions: Screening for CA in patients with prior surgery for bilateral CTS finds approximately 5% with early-stage transthyretin CA. The clinical yield was higher (>1 in 5) when focusing on nonobese men ≥70 years, showing potential for systematic screening.

KW - cardiac amyloidosis

KW - carpal tunnel syndrome

KW - early detection

KW - screening

U2 - 10.1016/j.jacc.2022.06.026

DO - 10.1016/j.jacc.2022.06.026

M3 - Journal article

C2 - 36049804

AN - SCOPUS:85136314270

VL - 80

SP - 967

EP - 977

JO - Journal of the American College of Cardiology

JF - Journal of the American College of Cardiology

SN - 0735-1097

IS - 10

ER -