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Canary in the Carpal Tunnel: A Pilot Study of the Value for Screening for Amyloidosis in Carpal Tunnel Syndrome
Logan Hansen, MD
1; Nevil M Khurana, M.S.
2; Trevor Ruesch, B.S.
2; Julia Malewicz, B.S.
2; Katiya Bharko, B.S.
2; Charles S Day, MD, MBA
31Henry Ford Hospital, Detroit, MI; 2Henry Ford Health, Detroit, MI; 3Henry Ford Health System, Detroit, MI
Introduction: Transthyretin (ATTR) amyloidosis, a form of systemic amyloidosis, can manifest as familial amyloid cardiomyopathy affecting the endomyocardial tissue of the heart. This can result in restrictive cardiomyopathy, and ultimately, heart failure with preserved ejection fraction. Patients presenting with this type of heart failure are often too late for treatment with current medication regimens, and early detection could increase patient lifespan. These misfolded amyloid proteins can also accumulate in the carpal tunnel, leading to carpal tunnel syndrome (CTS), which may be the earliest presentation of patients with amyloidosis. In this pilot study, we propose that early detection of amyloid via a biopsy of the transverse carpal ligament or tenosynovium in the carpal tunnel will allow early referral to cardiology clinic for consideration of disease-modifying treatment. Furthermore, a calculation using incremental cost effectiveness ratio (ICER) was used to determine the value of these biopsies in early detection of this disease process. Methods: Patients undergoing carpal tunnel release who meet criteria for biopsy, as described by Donnelly et al. in 2019, are included. Qualifying patients meet two tier 1 criteria - males aged 50 years or older, females aged 60 years or older, bilateral CTS or prior carpal tunnel release surgery; or one tier 1 criteria and one tier 2 criteria - spinal stenosis, biceps tendon rupture, atrial fibrillation or flutter (active or previous), presence of a pacemaker, congestive heart failure, or family history of ATTR amyloidosis. Tenosynovial biopsies are taken during open or endoscopic carpal tunnel release and are sent to pathology for congo red staining. If positive, patients are referred to high risk cardiology for evaluation, follow up for monitoring, and treatment of cardiac involvement. Additionally, ICER calculations including cost of hospitalizations, five-year mortality, cost of therapeutic intervention, and relative risk reduction of hospitalization with amyloidosis pharmacotherapy. Results: Of 38 patients matching eligibility criteria screened to date, 4 have had positive biopsy results and have been referred to high-risk cardiology. Of the 4 patients referred, two are currently undergoing advanced cardiac workup for consideration of initiating disease-modifying therapy. Discussion and Conclusion: This study demonstrates a potential service line link between orthopedic surgery and cardiology. Hand surgeons could potentially be a first point of contact for early diagnosis of transthyretin amyloidosis during carpal tunnel surgeries. This early diagnosis and interdisciplinary treatment pathway could allow for treatment to alter the disease's course and improve quality of life for patients with cardiac amyloidosis.
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