The investigators will prospectively evaluate tissue samples obtained from patients undergoing carpal tunnel release surgery for amyloid in the soft tissue that is removed. Patients who have tissue that stains positive for amyloid will undergo cardiac testing to look for evidence of cardiac involvement.
Since the 1970s, the connection between amyloidosis and carpal tunnel syndrome has been described, with tenosynovial tissue staining positive for amyloid fibril deposition. Up to 30% of patients in prior studies with idiopathic carpal tunnel syndrome have biopsy tissue that stains positive for amyloid deposition. However, the prevalence of cardiac involvement at the time of carpal tunnel syndrome surgery has not been established. Furthermore, the role of TTR kinetic stability in the disease progression of amyloidosis is unclear. Diagnosing TTR amyloidosis at the time of carpal tunnel involvement could lead to an earlier identification of the disease at a stage where the cardiomyopathy may be occult or less advanced. In addition, abnormal TTR kinetics may even precede tissue deposition in the flexor tendon retinaculum. As several emerging pharmacological strategies are in development that may slow or even halt TTR amyloidosis, earlier diagnosis is advantageous. Identification and implementation of therapy for prevention or early disease treatment may alter the natural history of this progressive systemic disease.
Study Type
OBSERVATIONAL
Enrollment
96
Lutheran Hospital
Cleveland, Ohio, United States
Incidence of transthyretin (TTR) amyloidosis in patients undergoing carpal tunnel release surgery
Identify the incidence of TTR amyloid deposits in the soft tissue of patients undergoing carpal tunnel surgery for idiopathic carpal tunnel syndrome
Time frame: Biopsy completed on day of surgery
Cardiac progression of transthyretin (TTR) amyloidosis in carpal tunnel biopsy-positive patients
Assess the progression of cardiac disease in patients with TTR deposits found in soft tissue removed at the time of carpal tunnel surgery
Time frame: 4 years from baseline cardiac assessment.
Prevalence of abnormal transthyretin (TTR) kinetics in patients undergoing carpal tunnel surgery
Identify the prevalence of abnormal TTR kinetics in patients undergoing carpal tunnel surgery for idiopathic carpal tunnel syndrome (develop a new test that aids identification of misfolded TTR intermediates that is more specific and sensitive to diagnosis this disease in the blood or tissue)
Time frame: One-time kinetics test for biopsy-negative patients; baseline kinetics and annually for 4 years in biopsy-positive patients
Early transthyretin (TTR) amyloid cardiomyopathy identification in carpal tunnel biopsy-positive patients
Investigate whether early TTR cardiomyopathy can be identified in patients who have TTR amyloid deposits identified in the soft tissue removed at the time of carpal tunnel surgery using cardiac biomarkers, echocardiography with strain, and nuclear bone scintigraphy
Time frame: Baseline and annual testing for 4 years in biopsy-positive patients
This platform is for informational purposes only and does not constitute medical advice. Always consult a qualified healthcare professional.