TY - JOUR T1 - Cardiac involvement in Emery Dreifuss muscular dystrophy: a case series JF - Heart JO - Heart SP - 105 LP - 108 DO - 10.1136/hrt.82.1.105 VL - 82 IS - 1 AU - A E Buckley AU - J Dean AU - I R Mahy Y1 - 1999/07/01 UR - http://heart.bmj.com/content/82/1/105.abstract N2 - 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. ER -