*Clinical Course*

Typically, this devasting disease is characterized by progressive skeletal muscle waste, with loss of ambulatory capacity and decline of respiratory and cardiac functions. The onset of muscle weakness is at 7–12 years old and the patient become wheel chair bound at 13 years [15]. The standard use of non-invasive home ventilators and the advance in respiratory care has changed the prognosis and prolonged survival [16]. DCM can occur at any age but often presents around 14–15 years and is very common in patients over 18 years of age [17]. It remains asymptomatic for many years in spite of the progression of cardiac dysfunction, because energy expenditure and oxygen consumption are severely diminished by muscle weakness. The degree of skeletal muscle weakness does not correlate with the severity of cardiomyopathy in patients, individual evaluation is important in order to tailor therapy and clinical assessment. Connuck et al. [5] demonstrated that the mortality rate for DMD patients with cardiomyopathy is significantly worse than that of BMD patients (who often undergo transplant) and similarly aged myocarditis and idiopathic DCM patients. The echocardiographic analysis on clinical course showed that the progression of the cardiomyopathy is slower in DMD when compared to Becker or other forms of cardiomyopathy.

#### **3. Cardiovascular Management**

Current 2018 DMD Care Considerations assessed that regular cardiac assessment is essential for DMD care [4]. From the time of DMD diagnosis, every effort should be focused to detect the early onset and the progression of the DCM. Early recognition is also crucial for therapy, conditioning the life expectancy. In non-ambulatory, asymptomatic patient serial evaluation is necessary to assess the progression of the disease. Clinical evaluation remains challenging because most of these patients have often low blood pressure values, cool extremities because of reduced skeletal muscular mass. Therefore, these clinical features require multiparametric evaluation in order to differentiate whether the cardiac process is ongoing.
