Caesarean delivery in a parturient with Holt-Oram syndrome and implantable cardioverter defibrillator: anaesthetic considerations

Holt-Oram syndrome is a rare autosomal dominant disorder characterized by skeletal upper-limb dysplasias and congenital cardiac defects. Clinical manifestations of this syndrome vary, and range from sub clinical radiologic findings to overt, life-threatening disease. The author of this article reports safe and effective use of low dose sequential combined spinal and epidural anaesthesia for caesarean section in a parturient with Holt-Oram syndrome and automatic implantable cardioverter defibrillator (AICD). Concerns with regards to anaesthetic management include problems with vascular access, possible arrhythmias, the presence of an AICD, and the possibility of cardiovascular instability. The characteristics of this syndrome and anaesthetic implications are discussed.