PT - JOURNAL ARTICLE AU - A E Buckley AU - J Dean AU - I R Mahy TI - Cardiac involvement in Emery Dreifuss muscular dystrophy: a case series AID - 10.1136/hrt.82.1.105 DP - 1999 Jul 01 TA - Heart PG - 105--108 VI - 82 IP - 1 4099 - http://heart.bmj.com/content/82/1/105.short 4100 - http://heart.bmj.com/content/82/1/105.full SO - Heart1999 Jul 01; 82 AB - Three patients with Emery Dreifuss muscular dystrophy are reported. Emery Dreifuss muscular dystrophy is an X linked muscular dystrophy, in which locomotor involvement is characteristically mild and slowly progressive. The effect on the heart becomes apparent in the teenage years and is characterised by cardiac conduction defects and infiltration of the myocardium by fibrous and adipose tissue. It first affects the atria, which results in atrial paralysis; treatment with ventricular pacing is usually needed. Female carriers can develop heart problems and are at risk of sudden death. Relatives of affected patients should be offered screening with electrocardiography and echocardiography.