TY - JOUR T1 - Dystrophin analysis in idiopathic dilated cardiomyopathy. JF - Journal of Medical Genetics JO - J Med Genet SP - 955 LP - 957 DO - 10.1136/jmg.30.11.955 VL - 30 IS - 11 AU - V V Michels AU - G M Pastores AU - P P Moll AU - D J Driscoll AU - F A Miller AU - J C Burnett AU - R J Rodeheffer AU - J A Tajik AU - A H Beggs AU - L M Kunkel Y1 - 1993/11/01 UR - http://jmg.bmj.com/content/30/11/955.abstract N2 - Idiopathic dilated cardiomyopathy (DCM) is characterised by ventricular dilatation and impaired systolic function resulting in congestive heart failure and frequently death. A dilated cardiomyopathy is common in patients with symptomatic Duchenne/Becker muscular dystrophy, a disease caused by dystrophin gene defects. However, cardiomyopathy is rarely the predominant clinical feature of this form of muscular dystrophy. To determine whether dystrophin gene defects might account for a significant number of patients with apparently isolated idiopathic DCM, we performed dystrophin gene analysis in 27 DCM patients, who were ascertained as part of a prospective study on idiopathic DCM. No dystrophin gene defects were found in our patients, whose average age was 50 years. These data suggest that dystrophin defects are not a common cause of idiopathic DCM in this age group in the absence of skeletal muscle cramps or weakness. ER -