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Abstract Introduction Heart disease remains a leading cause of mortality in patients with muscular dystrophy (MD), and cardiac assessment by standard imaging modalities is challenging due to the prominence of physical limitations. Methods In this prospective cohort study of 169 MD patients and 34 negative control patients, we demonstrate the clinical utility of a 12‐lead electrocardiogram (ECG) as an effective modality for the assessment of cardiac status in patients with MD. We assessed the utility of conventional criteria for electrocardiogram‐indicated left ventricular hypertrophy (ECG‐LVH) as well as ECG morphologies. Results Cornell voltage, Cornell voltage‐duration, Sokolow–Lyon voltage, and Romhilt‐Estes point score criteria demonstrated low sensitivity and minimal positive predictive value for ECG‐LVH when compared with cardiac imaging. Patients with LBBB had a high probability of a cardiomyopathy (relative risk [RR], 2.75; 95% confidence interval [CI], 2.14–3.53; p < .001), and patients with QRS fragmentation (fQRS) had a high probability of a cardiomyopathy (RR, 1.76; 95% CI, 1.20–2.59; p = .004), requiring cardiac medication and device intervention. We found that an R/S ratio >1 in V1 and V2 is highly specific (specificity, 0.89; negative predictive value [NPV], 0.89 and specificity, 0.82; NPV, 0.89, respectively) for patients with dystrophinopathies compared with other types of MD. Conclusion The identification of LBBB and fQRS was linked to cardiomyopathy in patients with MD, while ECG‐LVH was of limited utility. Importantly, these findings can be applied to effectively screen a broad cohort of MD patients for structural heart disease and prompt further evaluation and therapeutic intervention.