Abstract

BackgroundWe aimed to characterize patients with systemic amyloidosis stratified by a prior diagnosis of carpal tunnel syndrome (CTS) and to describe early echocardiographic parameters concomitant with CTS. Methods and ResultsPatients with suspected amyloidosis during CTS diagnosis were excluded. Our cohort included 108 patients with systemic amyloidosis of which 36% had a prior CTS at a median of 4 years (interquartile range [IQR] 2.8–6.7 years) before disease diagnosis. Patients with prior CTS were more likely to present subsequently with cardiac amyloidosis (78% vs 53%, P = .013), yet overall survival was comparable between groups (53% vs 61%, P = .825). Prior CTS was more commonly diagnosed in subsequent patients with transthyretin (62%) than in patients with immunoglobulin light chain (24%, P < .001). Furthermore, in a subanalysis of patients subsequently diagnosed with cardiac amyloidosis, findings at CTS diagnosis (n = 17) demonstrated a mild increase in septal thickness 1.3 cm (IQR 1.2–1.5 cm), increased relative wall thickness 0.46 cm (IQR 0.45–0.58 cm), and increased left ventricular mass index 155 g/m2(IQR 92–177 g/m2) compared with age-adjusted normal range echocardiographic values. Doppler mitral flow data was supportive of left ventricular diastolic dysfunction. ConclusionsEarly echocardiographic findings at CTS diagnosis, preceding the diagnosis of cardiac amyloidosis by several years, are suggestive of increased wall thickness and diastolic dysfunction.

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