Key result
Cardiac medications including ACE inhibitors, beta-blockers, and inotropics significantly changed echocardiographic parameters and better preserved LV EF in DMD children with baseline EF < 45%.
Why the study?
Do cardiac medications and noninvasive ventilator support preserve cardiac function in children with Duchenne muscular dystrophy cardiomyopathy?
Observational (n=48)
Do cardiac medications and noninvasive ventilator support preserve cardiac function in children with Duchenne muscular dystrophy cardiomyopathy?
p-value: p=< 0.05
Individualized combination medical therapy, including inotropes, may help preserve left ventricular ejection fraction in children with Duchenne muscular dystrophy and baseline LVEF < 45%.
May support individualized therapy in DMD cardiomyopathy with reduced EF; hypothesis-generating and requires randomized confirmation.
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%.
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Kim et al. (2020) conducted an observational in Duchenne muscular dystrophy cardiomyopathy (n=48). Cardiac medications (ACEIs, beta-blockers, inotropics) and noninvasive ventilator support vs. Baseline LV EF ≥ 45% vs LV EF < 45% was evaluated on Change in echocardiographic measurements (IVSd, IVSs, LVIDs, LVPWd, EF, FS, DT, DT slope, Lat A’ and Lat E/E’) (p=< 0.05). Cardiac medications including ACE inhibitors, beta-blockers, and inotropics significantly changed echocardiographic parameters and better preserved LV EF in DMD children with baseline EF < 45%.
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