P315 Electrocardiographic changes in duchenne muscular dystrophy

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Abstract

Background

Duchenne Muscular Dystrophy (DMD) is an X-linked disorder, the most common muscular dystrophy in children. It presents in early childhood and become fatal in the 2nd decade, mostly because of respiratory involvement and approximate 10% of cases because of cardiac failure.

Background

Cardiac changes become present after first decade. It leads to dilated cardiomiopathy, congestive cardiac failure, arrhythmias and finally cardiac death. Main ECG changes are sinus tachycardia, short PR interval, right ventricular hypertrophy, prolonged QTc, ST depression and rarely may be associated with WPW syndrome.

Aim

Study the ECG findings in DMD patients.

Methods

We analysed a total of 22 male patients admitted in‘Dr. Victor Gomoiu’ Children’s Clinical Hospital in paediatric cardiology department. The diagnostic of DMD was established using clinical and biochemical criteria and confirmed by genetic test. All patients were under corticotherapy. Patients underwent 12 lead ECG, 24 hour ECG holter, in order to detect ECG changes. It was noted the HR, R waves, R/S ratio, PR interval. Q waves, QT interval, ST segment, T waves. The parameters were measured manually and compared with standard age matched normal values.

Results

The characteristic of the study were: the mean age was 11 years; the mean weight of patients was 38.3 kg, 32% of them were wheelchair bounded.

Results

Tall R waves with R/S ratio more than 1 were present in 54% of all patients, RR` patter was found in 32%, QTc at higher limit was found of 40%.

Conclusions

Electrocardiographical changes are common in DMD patients, revealing early cardiac involvement. In order to delay cardiac changes, further studies (such as strain and cardiac RM) need to be done in order to sustain cardiac function in normal limits as long as possible.

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