Abstract
In patients with Duchenne muscular dystrophy (DMD), death secondary to cardiac or respiratory failure typically occurs in the second or third decade without treatment. Although cardiac dysfunction is treated with standard heart-failure strategies, it remains insufficient in DMD children. The purpose of this study was to evaluate the efficiency of cardiac medication and noninvasive ventilator support in DMD cardiomyopathy children with analyzing echocardiographic data. Forty-eight DMD children patients were included and divided into 2 groups by left ventricular (LV) ejection fraction (EF) at the time of initial treatment. Group 1: LV EF ≥ 45% and Group 2: LV EF < 45%. p-values were calculated using a Linear mixed model to estimate the association between cardiac medications and echocardiographic measurements. Before and after cardiac medications, the change values were significantly different in interventricular septal thickness at end diastole (IVSd), interventricular septal thickness at end systole (IVSs), left ventricular internal diameter end systole (LVIDs), left ventricular posterior wall thickness end diastole (LVPWd), ejection fraction (EF), fractional shortening (FS), deceleration time (DT), DT slope, Lat A’ and Lat E/E’ (p < 0.05). Group 2 patients revealed to take more kinds of cardiac medications than Group 1 (p < 0.05) including ACEIs, beta-blocker, and inotropics, then LV EF was better preserved in Group 2 than Group 1. It is certainly helpful to take individualized medical combination therapy including inotropic agents for cardiomyopathy in DMD children patients with EF < 45%.
Highlights
Duchenne muscular dystrophy (DMD), an X-linked disorder due to lack of dystrophin, is characterized by progressive muscle weakness and myocardial dysfunction [1]
We retrospectively reviewed the medical record of their cardiac medications (Beta-blocker, ACE inhibitor, Diuretics, Inotropics, Aspirin etc.), echocardiographic data, demographic data and non-invasive ventilator apply time per day
To analyze the treatment outcome between the two groups which is divided based upon left ventricular (LV) ejection fraction (EF), we evaluated relationship between the change values of echocardiographic parameters and cardiac medication and respiratory care with linear mixed interaction test
Summary
Duchenne muscular dystrophy (DMD), an X-linked disorder due to lack of dystrophin, is characterized by progressive muscle weakness and myocardial dysfunction [1]. DMD is typically diagnosed between the ages of 3 and 7 years and is characterized by progressive skeletal muscle weakness with loss of ambulation between the ages of 7 and 13 years [2]. Respiratory care of DMD patients has improved as a result of the development of supportive equipment and techniques [3]. Advances in the respiratory care of patients with DMD have improved their prognosis. Nocturnal home ventilators and mechanically assisted coughing lead to improved survival of patients with DMD [4]. The American Thoracic Society has published a statement regarding the respiratory care of patients with DMD, including evaluation and management
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