Cardiac Evaluation in Duchenne Muscular Dystrophy Patients Presenting with Acute Severe Chest Pain

Cardiovascular complications are a leading cause of morbidity and mortality in patients with Duchenne muscular dystrophy (DMD). Chest pain in the DMD patient is often overlooked as it is frequently attributed to the musculoskeletal system. Rarely does it prompt a complete cardiac evaluation. We report a case series of 7 DMD patients (2010–2016) who presented with acute severe chest pain, ECG abnormalities and acute marked troponin elevation. All ECGs demonstrated sinus tachycardia and ST and T wave abnormalities that resolved within several days. No patient had ECG findings consistent with myocardial infarction (STEMI). Troponin-I elevation ranged from 25 to 80 ng/ml (normal < 0.029 ng/ml). All but one patient underwent cardiac magnetic resonance imaging (CMR). In all cases CMR demonstrated worsening of left ventricular function as well as the presence of new or progressive myocardial fibrosis. Viral titers were negative in all 7 patients. All patients survived however on follow-up demonstrated no improvement in the degree of fibrosis seen on CMR. The DMD patient presenting with significant chest pain should undergo cardiac evaluation. Chest pain may be a symptom of acute myocardial injury. Evaluation should include ECG, serial troponin-I measurements and myocardial imaging to include CMR. The mechanism of acute injury is unclear though does not appear to be viral in origin. Further evaluation may provide insight into the mechanism of cardiac disease progression. This has important implications to patient evaluation and treatment.