Duchenne muscular dystrophy (DMD) is due to mutations in the dystrophin gene on chromosomeXp21.1. Dystrophin is a sarcolemmal protein which binds actin to extra cellular matrix. A deficiency in dystrophin leads to breakdown of muscle membrane and muscular alteration [1]. The disease is clinically characterized by proximal weakness and wasting, leading to loss of ambulation by 12 years of age. The heart is affected to various degrees, depending on the stage of the disease. The most common cardiac abnormality in DMD is dilated cardiomyopathy. Conduction system disease and tachyarrhythmias may occur in some patients. Death occurs in early adulthood secondary to respiratory or cardiac failure [2], even if recent advances in the use of inspiratory and expiratory muscle aids have greatly reduced the risk of pulmonary morbidity and mortality rates [3].The typical initial manifestation of cardiac involvement in DMD is sinus tachycardia [1]. Cardiac involvement is due to progressive replacement of the cardiomyocytes and the Purkinje system by connective tissue or fat [1]. Impairment of cardiac autonomic function in patients with DMD is well known. Kirchmann found sinus tachycardia in 26% and reduced heart rate variability in 51% [4]. In human cardiac purkinje fibers, immunocytochemical staining showed that dystrophin was localised to the membrane surface of the purkinjer fiber [5]. The cardiac rhythm abnormalities play a significant role in morbidity and mortality in this disease [6]. Supraventricular ectopy or couplets are reported to indicate increasing severity of arrhythmia with the progression of disease [7]. 33% of DMD patients had ventricular premature beats in a study including 45 Duchenne muscular dystrophy patients without congestive heart failure and followed up for 3 years [8].