Abstract
Cardiac complications are frequently diagnosed in Duchenne muscular dystrophy (DMD), clinical manifestations generally appear after age 10. ECG recordings objectified various changes in these patients, the most common being represented by extensive R waves in V1, left deep Q waves, conduction abnormalities and arrhythmias. Echocardiographic examination may objective the presence of subclinical cardiac dysfunction in children under 12 years old diagnosed with DMD. In patients where standard echocardiographic examination is normal, myocardial performance index is a parameter useful in early detection of asymptomatic cardiac abnormalities. Cardiovascular magnetic resonance provides information both on left ventricular systolic function and myocardial tissue changes and occurrence of fibrosis, lesions rarely objectified before the age of 10 years. All these explorations should be considered in children with DMD at the end of a complete neurological exam.
Highlights
Progressive muscular dystrophies include a heterogeneous group of disorders characterized by primitive, progressive muscle degeneration genetically determined
(2007) believes that most patients with Duchenne muscular dystrophy (DMD) who reach the third decade of life, present cardiomyopathy, but frequently diagnosis are delayed because physical inactivity of the patients masks the symptoms of heart failure
According to studies by nuclear magnetic resonance myocardial fibrosis is initially located at the basal-posterior and lateral wall of the left ventricle (Silva, 2007; Puchalski, 2009), the consequence being the reduction of contractile force and the disturbance of the electrical activity of myocardial cells
Summary
Electrocardiograms (ECG) have objectified in children with DMD various changes (Fig. 1, Fig. 2), the most frequently changes being represented by:. Takani (2008) performed 136 ECG recordings from a group of 69 patients aged under 18 years, diagnosed with DMD, following potential cardiac electrical changes. The author observed that 91.3% of the studied children had one or more ECG abnormalities. He noted that 84.8% of patients younger than 10 years had ECG changes, the most common being represented by the presence of a deep Q waves, and the typical appearance described in DMD represented by ample R wave in V1-V5 and deep Q wave was more common in adolescents
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