Concentrations of high-sensitivity cardiac troponin T (hs-cTnT) are frequently elevated in stable patients with confirmed muscle dystrophies. However, sparse information is available on the interpretation of serial concentration changes. Hs-cTnT was collected in 35 stable outpatients with confirmed skeletal muscle dystrophies at 0 and 1h and after 6-12months during scheduled outpatient visits. We simulated the effectiveness of the European Society of Cardiology (ESC) 0/1h algorithm and assessed biological variation at 6-12months using two established methods: reference change value (RCV) and minimal important difference (MID). Median baseline hs-cTnT concentrations were 34.4ng/L [inter-quartile range (IQR): 17.5-46.2], and values > 99th percentile upper limit of normal were present in 34 of 35 patients. All patients were stable without cardiovascular adverse events during a follow-up of 6.6months (IQR: 6-7). Median concentration change was 1.9ng/L (IQR: 0.7-3.2) and 0.8ng/L (IQR: 0-7.0) at 60min and 6-9months, respectively. Applying the criteria of the ESC 0/1h algorithm for triage of suspected acute coronary syndrome (ACS) showed poor overall effectiveness of baseline hs-cTnT values. No patient would qualify for rule-out based on hs-cTnT less than the limit of detection, whereas five cases would qualify for rule-in based on hs-cTnT≥52ng/L. Biological variabilities at 6-12months per MID and RCV were 1.2ng/L [95% confidence interval (CI): 0.7-2.1] and 28.6% (95% CI: 27.9-29.6), respectively. A total of 8 (22.9%) and 25 (71.4%) cases exceeded the biological variation range, suggesting some additional myocardial damage. The high prevalence of elevated hs-cTnT could negatively impact the effectiveness of rule-out and rule-in strategies based on a single hs-cTnT value. Knowledge of the physiological and biological variation of hs-cTnT after 6-12months is helpful to detect the progression of cardiac involvement or to search for cardiac complications including but not limited to arrhythmias that may trigger acute or chronic myocardial damage.