Background:We evaluated the effect of enalapril and carvedilol on LV dysfunction and compared the efficacy of those drugs in patients with muscular dystrophy. Methods:Among 284 patients with muscular dystrophy on active follow-up, 52 patients (48 with Duchenne, 4 with Becker muscular dystrophy) were enrolled in this study from October 1994 to June 2010 in the Seoul National University Children’s Hospital. We reviewed medical records of all the patients with enalapril and/or carvedilol retrospectively and newly prescribed enalapril or carvedilol at random from July 2008 in 22 patients (enalapril; 12 patients, carvedilol; 10 patients). The changes of LV dimension, volume, various systolic (fractional shortening, ejection fraction, peak global longitudinal strain, etc) and diastolic function (transmitral inflow pattern, etc) before and after administration of enalapril and/or carvedilol were evaluated. Results:The mean starting age of medication was 12.8±3.6 years and mean follow-up duration was 1.8±0.9 years. The prescribed medication was enalapril only in 34 patients, carvedilol only in 10 patients and combination of enalapril and carvedilol in 8 patients. Indexed LV dimension at end-diastole decreased from 37.9±7.8 to 36.1±6.8 mm/m2 (p=0.73). LV fractional shortening (from 24.1±6.0 to 24.1±7.2%, p=0.778) and ejection fraction (EF, from 46.4±9.1 to 46.3±8.8%, p=0.258) did not change significantly. LV diastolic functional parameters including transmitral inflow pattern maintained during follow-up periods. Overall, 4 patients died during follow-up; one patient from respiratory failure, 2 patients from heart failure, 1 patient after open heart surgery for associated atrial septal defect. After randomization of enalapril and carvedilol in 22 patients, mean treatment duration was 1.11±0.33 years. Indexed LV volume at end-diastole by 3-dimensional volume acquisition decreased from 46.5±12.4 to 41.3±6.3 mL/m2 (p=1.0) in enalapril group and increased from 50.9±13.1 to 55.6±33.4 mL/m2 (p=0.069) in carvedilol group. EF from 3-dimensional volume acquisition slightly decreased from 51.2±6.6 to 48.2±5.2% (p=0.116) in enalapril group and slightly increased from 48.2±4.1 to 48.4±6.3% (p=0.672) in carvedilol group. LV diastolic functional parameters showed no significant change during follow-up in both groups. There was no adverse effect from medication. Conclusion:Enalapril and/or carvedilol administration could prevent the progression of LV systolic dysfunction in patients with muscular dystrophy. Three-dimensional volume acquisition could give additional information about LV dysfunction.
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