The effect of enalapril and carvedilol on left ventricular dysfunction in middle childhood and adolescent patients with muscular dystrophy

Abstract

Background and objectives: In Duchenne and Becker muscular dystrophies, cardiac function deteriorates with time resulting in heart failure which is often fatal. We prospectively evaluated the effect of enalapril and carvedilol on left ventricular (LV) dysfunction in middle childhood and adolescent patients with muscular dystrophy.

Subjects and methods: Twenty-three patients with LV dysfunction (22 with Duchenne muscular dystrophy, 1 with Becker muscular dystrophy) were enrolled. We prescribed enalapril (13 patients) or carvedilol (10 patients) randomly from July 2008 to August 2010 and followed up the patients until September 2011. The changes in LV function parameters before and after the treatment were evaluated by echocardiography.

Results: The mean age at the start of treatment with enalapril or carvedilol was 12.6±3.7 years (median 13 years), and mean follow-up duration was 20.1±8.9 months. In the enalapril group, LV fractional shortening (FS) increased from 25.8±2.1 to 26.6±3.0 (p=0.241). In the carvedilol group, LV FS increased from 26.4±1.1 to 28.6±4.2 (p=0.110). In all 23 patients, LV FS significantly increased from 26.1±1.7 (before) to 27.6±3.7 (after treatment) (p<0.046). Indexed LV dimension at end diastole and LV end-diastolic volume decreased slightly, but without statistical significance by tri-plane volumetry. LV diastolic functional parameters were maintained during follow-up period.

Conclusion: Enalapril or carvedilol could improve LV systolic function in middle childhood and adolescent patients with muscular dystrophy without significant adverse effects.

Keywords: Cardiomyopathies; Carvedilol; Echocardiography; Enalapril; Muscular dystrophies.

Fig. 1

Left ventricular (LV) peak longitudinal strain and 3-dimensional LV volume measurement. A: LV peak global longitudinal strain at the apical 4-chamber view, which was -17% in 1 patient. B: 3-Dimensional LV volume measurement and acquisition of ejection fraction by tri-plane volumetry, which was 36.8% in 1 patient.

Fig. 2

Left ventricular (LV) changes in dimension and systolic function before and after treatment in the enalapril-treated (A) and carvedilol-treated (B) groups. Patients of both groups showed decreased LV end-diastolic and end-systolic dimension, but the effect was not statistically significant. LV fractional shortening (FS) showed slight improvement in both groups after treatment, but without statistical significance. LVEDd: LV end-diastolic diameter, LVESd: LV end-systolic diameter, EF: ejection fraction.

Fig. 3

Left ventricular (LV) changes in dimension and systolic function before and after treatment with enalapril or carvedilol. Overall end-diastolic dimension (LVEDd) and end-systolic LV dimension (LVESd) decreased after treatment with enalapril or carvedilol for 20.1±8.9 months (A and B). Overall LV fractional shortening (FS) increased significantly (C). Ejection fraction (EF) showed slight improvement after treatment, but without statistical significance (C and D).