Duchenne muscular dystrophy: an overview to the cardiologist.

INTRODUCTION

Duchenne muscular dystrophy (DMD) is the most common form of muscular dystrophy in children, affecting approximately one in 3,500-5,000 liveborn boys. The main signs and symptoms include gait disturbances beginning in early childhood, with later onset of respiratory and cardiac function disorders, both directly affecting the prognosis.

AREAS COVERED: The recent improvement of mechanical ventilation increased the mean DMD survival age; however, there has been little progress in the treatment and prevention of cardiac complications, which currently predominantly impact survival. Cardiological evaluation with imaging methods, such as echocardiography and magnetic resonance imaging, can improve the understanding and detect changes in cardiac function early.

EXPERT OPINION: Close monitoring by the cardiologists and early treatment, with adequate heart disease stratification, may be the key to prolong the lives of these patients until more promising therapies are available and can predict DMD prognosis and progression more accurately. The objective of this brief review is to update the cardiologists by highlighting the most relevant aspects of treatment and follow-up, in a practical and concise way.