Subclinical cardiomyopathy in Becker muscular dystrophy

Abstract

Objective-To investigate the prevalence, age distribution, and spectrum of cardiac involvement in a cohort of patients with Becker muscular dystrophy.Design (Br Heart J 1992;68:304-8)

Evolution of cardiac abnormalities in Becker muscular dystrophy over a 13-year period

“…We found a high proportion of ECG abnormalities, which is in accordance with others [23,45]. The most frequently observed abnormalities consisted of high Rwaves and pathological Q-waves.…”

“…Our findings have been confirmed by others [11,23,32,33,45]. In the present study with a mean follow-up of 12.5 years, cardiac abnormalities had progressed in 63% of patients.…”

“…In BMD estimates of the prevalence of abnormal cardiac findings vary from 17% to 74% [1,11,12,21,23,32,45]. Dilated cardiomyopathy (DCM) in BMD is independent of age and severity of muscle weakness [1,11,12,23,41,45]. It can even be the only clinical manifestaAbstract We evaluated the course of cardiac involvement in 27 previously reported patients with Becker muscular dystrophy (BMD) originating from nine kindreds.…”

“…Electrocardiographic (ECG) changes similar to those seen in DMD can be found in 41%-74% of BMD patients [7,11,12,23,45]. Echocardiographic abnormalities, including global hypokinesia [11,23,45], left ventricular dilatation or DCM [11,12,23,45], right ventricular dilatation [23], hypertrophic cardiomyopathy [20,32,33] and other wall motion abnormalities [11,23,45] are seen in 17%-67% of patients [11,12,23,32,45]. Despite the high proportion of DCM in BMD, symptomatic patients are not common.…”

“…In recent years the correlation between cardiac involvement and the defect at the DNA level has been studied [11,23,25,32,40,45]. To date, several mutations have been described which may give rise to DCM.…”