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A 13-year-old boy presented with progressive weakness of both lower limbs from the age of 5 years. He had a history of delayed milestones from birth and difficulty in getting up from sitting posture and climbing uphill, which progressively worsened necessitating wheelchair support over the past 1 year. Clinically, there was flaccid weakness of proximal lower limb muscles, absent reflexes, characteristic pseudohypertrophy of calf muscles, waddling gait and Gowers sign. There was marked scoliosis and lumbar lordosis. His cardiovascular examination was normal. Genetic analysis revealed deletion of exon involving DMD gene.

Electrocardiogram displayed classical features of Duchenne muscular dystrophy in the form of tall R wave in lead V1 with R/S amplitude >1. Deep negative q waves were seen in lateral leads involving V6. There was short PR interval (90 ms) and sinus arrhythmia with longest cycle length exceeding minimum cycle length by >120 ms (Figure 1).

Echocardiogram (ECG) could be the earliest marker of cardiac involvement. ECG including the global longitudinal strain was normal in our patient. The characteristic ECG pattern includes tall R in V1 with R/S > 1, deep q waves in lateral leads, short PR interval, notching of QRS and sinus tachycardia due to autonomic dysregulation.1

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