Carpal Tunnel Syndrome and Amyloid Cardiomyopathy

Abstract

There is no financial information to disclose. Carpal tunnel syndrome (CTS) can be the initial manifestation in patients with amyloidosis, often presenting years before cardiac involvement becomes apparent. We hypothesized that a significant percentage of older patients undergoing surgery for idiopathic CTS would be diagnosed with amyloidosis through tenosynovial biopsy, and that early detection of amyloid cardiomyopathy using advanced cardiac imaging techniques may be possible. This is an ongoing prospective, longitudinal study that includes males = 50 and females = 60 years of age undergoing carpal tunnel decompression. Patients with a known diagnosis of amyloidosis or rheumatoid arthritis are excluded. Tenosynovial biopsy is taken at the time of surgery, and Congo-red staining is performed to detect amyloid deposits. Tissue positive for amyloid is further analyzed by mass spectrometry and immunohistochemistry for subtyping. Patients with amyloid deposits are referred to a cardiologist, where physical examination, laboratory studies, EKG, echocardiography with longitudinal strain, and 99m-technetium pyrophosphate nuclear scintigraphy are performed to evaluate for evidence of cardiac amyloid involvement. Patients will be followed for 4 years with yearly cardiac testing. Preliminarily, 205 patients undergoing carpal tunnel decompression were screened for eligibility (Fig. 47-1). Of the 120 patients who met inclusion criteria, 58 consented to participate and underwent biopsy. Seven patients (7/58, 12.1%) were found to have amyloidosis. Tissue subtyping revealed that five patients had transthyretin amyloidosis (ATTR) and two patients had light chain amyloidosis (AL). Upon further cardiac workup, two patients were found to have associated amyloid cardiomyopathy (one patient with ATTR and one patient with AL). •Seven patients (12.1%) undergoing carpal tunnel decompression for ‘idiopathic’ carpal tunnel syndrome were diagnosed with amyloidosis through tenosynovial biopsy.•Two out of seven of these patients were found to have evidence of amyloid cardiomyopathy upon further work up with advanced cardiac imaging.•Two patients were found to have the AL subtype caused by a plasma cell disorder. This subtype is rapidly progressive and early diagnosis and intervention is of paramount importance for survival.•Hand surgeons should be aware of the association between carpal tunnel syndrome and amyloidosis. Performing a tenosynovial biopsy and staining for amyloid with Congo red in an older cohort with idiopathic CTS may lead to early diagnosis of amyloidosis, thereby allowing for timely intervention of this life-threatening disease.