Abstract

Emery-Dreifuss Muscular Dystrophy (EDMD) is characterised by a triad of progressive myopathic changes in skeletal muscles, early contractures of the neck, elbow, ankle and cardiac conduction defects. The incidence is 1 in 100,000. Pregnancy complications include respiratory problems, cardiomyopathy, preterm delivery, low birthweight and cephalopelvic disproportion. We present a case of known EDMD who was diagnosed as a child. She booked at a height of 145cm and weighed 35kgs. Her pregnancy was a result of ovulation induction by Letrozole. A dual chamber automated implantable cardiac defibrillator inserted last year because of the high risk of sudden cardiac death associated with her condition. She has regular follow up at the breathing support clinic. Being wheel chair bound, she had reduced mobility and was commenced on thromboprophylaxis. She developed pre-ecclampsia at 26 weeks and was delivered at 28 weeks by Caesarean section under regional anaesthetic.