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There is little clinical data in the literature on the anaesthetic management of paediatric patients with Eisenmenger's syndrome undergoing non-cardiac surgery. This paper reviews our experiences with either such patients who underwent a total of 11 surgical procedures. Of the eight children, six had Down's syndrome and an atrio-ventricular septal defect, one had a ventricular septal defect and one an atrial septal defect. Nine of the eleven operations consisted of minor dental, plastic or ENT procedures, while one patient underwent two laparotomies. Premedication (trimeprazine/ meperidine combination or midazolam) was administered on three occasions. Induction of anaesthesia was achieved by either inhalation of halothane (2), or intravenously with thiopentone (6), ketamine (2) or propofol (1). Muscle relaxation and mechanical ventilation were employed only for both intra-abdominal procedures, otherwise patients were allowed to breathe spontaneously with, or without, manual assistance. Halothane (8), isoflurane (2) and enflurane (1) were all used for maintenance of anaesthesia. Non-invasive monitoring was applied intraoperatively for minor procedures, and arterial and central venous catheters inserted for the laparotomies. Postoperative analgesia for both these cases was provided by an epidural infusion of bupivacaine 0.125% and fentanyl 5 micrograms x ml(-1). A single im bolus of morphine was required following a dental clearance, otherwise pain relief for the rest of the cases was achieved by local anaesthetic infiltration and NSAIDS. With the exception of a single episode of bradycardia, induction, maintenance and recovery from anaesthesia were well tolerated in all cases. In conclusion, our experience suggests that despite theoretical risks, children with Eisenmenger's syndrome appear to tolerate a variety of anaesthetic techniques.

The anaesthetic management of the child with Eisenmenger’s syndrome