Image Viability of Echocardiography in Adult Patients with Duchenne Muscular Dystrophy

Abstract

Increasingly, patients with Duchenne Muscular Dystrophy (DMD) are surviving to adulthood, requiring monitoring for DMD-related cardiomyopathy. Transthoracic echo (TTE) parameters, including LV ejection fraction, fractional shortening and LV end-diastolic diameter have been recommended to detect DMD-related cardiomyopathy. We examined the viability of obtaining these parameters, and the proportion of adult DMD patients on medications for cardiomyopathy. From 2000–2021 at a tertiary Australian hospital, 53 patients with DMD were identified. The number of patients who had a TTE, technical difficulty, echocardiographic parameters and medication data were obtained. Of the 53 patients, only 29 patients had TTE studies (54.7%). In 84% of these, the TTE was described as technically difficult due to the patient being wheelchair-bound, having severe scoliosis or respiratory failure. Other than LVEDD, no parameter was successfully obtained at a high frequency. The mean fractional shortening was 27.4+/-10.1% and the mean LVEDD was 4.4+/-1.9 cm. None of the 53 patients had a cardiac MRI scan performed as an alternate imaging modality. ACE inhibitors & ARBs (87%) were the most commonly prescribed medications at time of TTE, followed by beta blockers (31%). Although TTE is recommended for DMD patients, images are often difficult to reliably obtain. Failure to identify heart failure may reduce prescription of essential medications in this high-risk population.Tabled 1TTE parameterRecordedDifficult study61 (84%)Fractional shortening35 (48%)Ejection fraction (Simpson’s bi-plane)18 (25%)LV end-diastolic diameter60 (82%)LA diameter44 (60%)E/A ratio42 (58%)Global longitudinal strain0 (0%) Open table in a new tab