Objective: 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=0.0001) 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. 

You may cite this article as: Çağlıyan ÇE, Gelincik H, Çelik Aİ, Koç F. Impaired autonomic and repolarization abnormalities in patients with facioscapulohumeral dystrophy. Neurol Sci Neurophysiol 2018; 35(3): 127-131.