Impaired Autonomic and Repolarization Abnormalities are Observed in Patients with Facioscapulohumeral Dystrophy Despite Normal Myocardial Functions

Facioscapulohumeral Dystrophy (FSHD) is one of the most common adult muscular dystrophies. We aim to investigate structural, functional and arrhythmic abnormalities in patients with FSHD Type-1. Methods: Left ventricular mass, left ventricular tissue doppler analysis and myocardial performance index (MPI) were calculated in Type-1 FSHD and healthy controls. A 24-hour ambulatory electrocardiogram (ECG) analysis was performed. T wave peak to end (Tp-e) interval and Tp-e/QT ratio was calculated. Results: In the study average heart rate was significantly higher (93.6 ± 10.1 min-1 vs. 74.3 ± 5.6 min-1; p = 0.000) and standard deviation of N-N intervals was significantly lower (47.43 ± 15.49 vs. 69.85 ± 19.18; p = 0.001) in FSHD Group. Tp-e interval was longer (78.6 ± 14.6 msec vs. 64.3 ± 16.5 msec; p = 0.031) and Tp-e/QT ratio was larger (22.9 ± 3.2 vs. 17.8 ± 5.4; p = 0.018) in the FHSD Group. Conclusion: These findings suggest that autonomic and repolarization abnormalities might be observed in patients with FSHD in spite of normal structural and functional myocardial function.